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1. Children with special health care needs attending emergency department in Italy: analysis of 3479 cases

Author: Cianci, P., D'Apolito, V., Moretti, A., Barbagallo, M., Paci, S., Carbone, M. T., Lubrano, R., Urbino, A., Dionisi Vici, C., Memo, L., Zampino, G., La Marca, G., Villani, A., Corsello, G., Selicorni, A., Campania, A., Geremia, C., Castagno, E., Masi, S., Poggi, G., Vestri, M., Fossali, E., Rocchi, A., Dadalt, L., Arrighini, A., Chiappa, S., Renna, S., Piccotti, E., Borgna, C., Govoni, M. R., Biondi, A., Fossati, C., Iughetti, L., Bertolani, P., Salvatoni, A., Agosti, M., Fuca, F., Ilardi, A., Giuffrida, S., Diguardo, V., Boni, S., D'Antiga, L., Ruggeri, M., Chiaretti, A., Amarri, S., Peduto, A., Bernardi, F., Corsini, I., Deangelis, G. L., Ruberto, C., Zuccotti, G. V., Stringhi, C., Lombardi, G., Salladini, C., Dimichele, S., Parola, L., Porta, A., Biasucci, G., Bellini, M., Ortisi, M. T., Apuril, E., Midulla, F., Tarani, L., Parlapiano, G., Lietti, D., Sforzini, C., Marseglia, G. L., Savasta, S., Falsaperla, R., Vitaliti, M. C., Chiarelli, F., Rossi, N., Banderali, G., Giacchero, R., Bernardo, L., Pinto, F., Fabiani, E., Ficcadenti, A., Pellegrini, G., Giacoma, S., Biban, P., Spada, S., Tipo, V., Ghitti, C., Bolognini, S., Mariani, G., Russo, A., Colella, M. G., Verrico, A., Bruni, P., Poddighe, D., Cagnoli, G., Morandi, F., Gadaleta, A., Barbi, E., Bruno, I. I., Graziano, R., Sgaramella, P., Catalani, M. P., Baldoni, I., Colarusso, G., Galvagno, G., Barone, A. P., Longo, A., Nardella, G., Portale, G., Garigali, G., Bona, G., Erbela, M., Agostiniani, R., Nanni, L., Schieven, E., Dona, M., Varisco, T., Russo, F., Distefano, V. A., Dipietro, F., Tarallo, L., Imperato, L., Parisi, G., Salzano, R., Raiola, G., Talarico, V., Bellu, R., Cannone, A., Ferrante, P., Cianci, P, D'Apolito, V, Moretti, A, Barbagallo, M, Paci, S, Carbone, M, Lubrano, R, Urbino, A, Dionisi Vici, C, Memo, L, Zampino, G, La Marca, G, Villani, A, Corsello, G, Selicorni, A, Campania, A, Geremia, C, Castagno, E, Masi, S, Poggi, G, Vestri, M, Fossali, E, Rocchi, A, Dadalt, L, Arrighini, A, Chiappa, S, Renna, S, Piccotti, E, Borgna, C, Govoni, M, Biondi, A, Fossati, C, Iughetti, L, Bertolani, P, Salvatoni, A, Agosti, M, Fuca, F, Ilardi, A, Giuffrida, S, Diguardo, V, Boni, S, D'Antiga, L, Ruggeri, M, Chiaretti, A, Amarri, S, Peduto, A, Bernardi, F, Corsini, I, Deangelis, G, Ruberto, C, Zuccotti, G, Stringhi, C, Lombardi, G, Salladini, C, Dimichele, S, Parola, L, Porta, A, Biasucci, G, Bellini, M, Ortisi, M, Apuril, E, Midulla, F, Tarani, L, Parlapiano, G, Lietti, D, Sforzini, C, Marseglia, G, Savasta, S, Falsaperla, R, Vitaliti, M, Chiarelli, F, Rossi, N, Banderali, G, Giacchero, R, Bernardo, L, Pinto, F, Fabiani, E, Ficcadenti, A, Pellegrini, G, Giacoma, S, Biban, P, Spada, S, Tipo, V, Ghitti, C, Bolognini, S, Mariani, G, Russo, A, Colella, M, Verrico, A, Bruni, P, Poddighe, D, Cagnoli, G, Morandi, F, Gadaleta, A, Barbi, E, Bruno, I, Graziano, R, Sgaramella, P, Catalani, M, Baldoni, I, Colarusso, G, Galvagno, G, Barone, A, Longo, A, Nardella, G, Portale, G, Garigali, G, Bona, G, Erbela, M, Agostiniani, R, Nanni, L, Schieven, E, Dona, M, Varisco, T, Russo, F, Distefano, V, Dipietro, F, Tarallo, L, Imperato, L, Parisi, G, Salzano, R, Raiola, G, Talarico, V, Bellu, R, Cannone, A, Ferrante, P, Paola Cianci, Valeria D'Apolito, Alex Moretti, Massimo Barbagallo, Sabrina Paci, Maria Teresa Carbone, Riccardo Lubrano, Antonio Urbino, Carlo Dionisi Vici, Luigi Memo, Giuseppe Zampino, Giancarlo La Marca, Alberto Villani, Giovanni Corsello, Angelo Selicorni, Cianci, P., D'Apolito, V., Moretti, A., Barbagallo, M., Paci, S., Carbone, M. T., Lubrano, R., Urbino, A., Dionisi Vici, C., Memo, L., Zampino, G., La Marca, G., Villani, A., Corsello, G., Selicorni, A., Campania, A., Geremia, C., Castagno, E., Masi, S., Poggi, G., Vestri, M., Fossali, E., Rocchi, A., Dadalt, L., Arrighini, A., Chiappa, S., Renna, S., Piccotti, E., Borgna, C., Govoni, M. R., Biondi, A., Fossati, C., Iughetti, L., Bertolani, P., Salvatoni, A., Agosti, M., Fuca, F., Ilardi, A., Giuffrida, S., Diguardo, V., Boni, S., D'Antiga, L., Ruggeri, M., Chiaretti, A., Amarri, S., Peduto, A., Bernardi, F., Corsini, I., Deangelis, G. L., Ruberto, C., Zuccotti, G. V., Stringhi, C., Lombardi, G., Salladini, C., Dimichele, S., Parola, L., Porta, A., Biasucci, G., Bellini, M., Ortisi, M. T., Apuril, E., Midulla, F., Tarani, L., Parlapiano, G., Lietti, D., Sforzini, C., Marseglia, G. L., Savasta, S., Falsaperla, R., Vitaliti, M. C., Chiarelli, F., Rossi, N., Banderali, G., Giacchero, R., Bernardo, L., Pinto, F., Fabiani, E., Ficcadenti, A., Pellegrini, G., Giacoma, S., Biban, P., Spada, S., Tipo, V., Ghitti, C., Bolognini, S., Mariani, G., Russo, A., Colella, M. G., Verrico, A., Bruni, P., Poddighe, D., Cagnoli, G., Morandi, F., Gadaleta, A., Barbi, E., Bruno, I. I., Graziano, R., Sgaramella, P., Catalani, M. P., Baldoni, I., Colarusso, G., Galvagno, G., Barone, A. P., Longo, A., Nardella, G., Portale, G., Garigali, G., Bona, G., Erbela, M., Agostiniani, R., Nanni, L., Schieven, E., Dona, M., Varisco, T., Russo, F., Distefano, V. A., Dipietro, F., Tarallo, L., Imperato, L., Parisi, G., Salzano, R., Raiola, G., Talarico, V., Bellu, R., Cannone, A., and Ferrante, P.
Subjects: Male, Metabolic disease, Hospitalization rate, Congenital skeletal condition, Children with special health care needs, Emergency department, Isolated CNS malformation, Metabolic diseases, Multiple AED therapy, Neuromuscular diseases, Syndromic disorders, True isolated microcephaly, 0302 clinical medicine, Clinical history, Medicine, Child, education.field_of_study, Neuromuscular disease, Settore MED/38, Disabled Children, Hospitalization, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Italy, Child, Preschool, Female, Children with special health care need, Emergency Service, Hospital, medicine.medical_specialty, Adolescent, Population, Triage Code, 03 medical and health sciences, Pharmacotherapy, 030225 pediatrics, Humans, Medical prescription, education, Retrospective Studies, Health Services Needs and Demand, Syndromic disorder, business.industry, Research, Infant, Newborn, Infant, 030208 emergency & critical care medicine, Children with special health care needs, Congenital skeletal conditions,Emergency department, Hospitalization rate, Isolated CNS malformation, Metabolic diseases, Multiple AED therapy, Neuromuscular diseases, Syndromic disorders, True isolated microcephaly, Family medicine, Chronic Disease, business, Facilities and Services Utilization
Abstract: Background Although children with special health care needs (CSHCN) represent a minority of the population, they go through more hospitalizations, more admissions to the Emergency Department (ED), and receive a major number of medical prescriptions, in comparison to general pediatric population. Objectives of the study were to determine the reasons for admission to the ED in Italian CSHCN, and to describe the association between patient’s demographic data, clinical history, and health services requirements. Methods Ad hoc web site was created to collect retrospective data of 3479 visits of CSHCN to the ED in 58 Italian Hospitals. Results Seventy-two percent of patients admitted to ED were affected by a previously defined medical condition. Most of the ED admissions were children with syndromic conditions (54%). 44.2% of the ED admissions were registered during the night-time and/or at the weekends. The hospitalization rate was of 45.6% among patients admitted to the ED. The most common reason for admission to the ED was the presence of respiratory symptoms (26.6%), followed by gastrointestinal problems (21.3%) and neurological disorders (18.2%). 51.4% of the access were classified as ‘urgent’, with a red/yellow triage code. Considering the type of ED, 61.9% of the visits were conducted at the Pediatric EDs (PedEDs), 33.5% at the Functional EDs (FunEDs) and 4.6% at the Dedicated EDs (DedEDs). Patients with more complex clinical presentation were more likely to be evaluated at the PedEDs. CSHCN underwent to a higher number of medical procedures at the PedEDs, more in comparison to other EDs. Children with medical devices were directed to a PedED quite exclusively when in need for medical attention. Subjects under multiple anti-epileptic drug therapy attended to PedEDs or FunEDs generally. Patients affected by metabolic diseases were more likely to look for medical attention at FunEDs. Syndromic patients mostly required medical attention at the DedEDs. Conclusions Access of CSHCN to an ED is not infrequent. For this reason, it is fundamental for pediatricians working in any kind of ED to increase their general knowledge about CHSCN and to gain expertise in the management of such patients and their related medical complexity.
Published: 2020
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2. Pathologic complete response (pCR) to neoadjuvant treatment with or without atezolizumab in triple-negative, early high-risk and locally advanced breast cancer: NeoTRIP Michelangelo randomized study

Author: Gianni L, Huang CS, Egle D, Bermejo B, Zamagni C, Thill M, Anton A, Zambelli S, Bianchini G, Russo S, Ciruelos EM, Greil R, Semiglazov V, Colleoni M, Kelly C, Mariani G, Del Mastro L, Maffeis I, Valagussa P, and Viale G
Subjects: Paclitaxel, neoadjuvant therapy, Triple Negative Breast Neoplasms, Hematology, Antibodies, Monoclonal, Humanized, B7-H1 Antigen, Neoadjuvant Therapy, Carboplatin, atezolizumab, Oncology, Antineoplastic Combined Chemotherapy Protocols, Humans, Female, triple-negative breast cancer
Abstract: BACKGROUND: High-risk triple-negative breast cancers (TNBCs) are characterized by poor prognosis, rapid progression to metastatic stage and onset of resistance to chemotherapy, thus representing an area in need of new therapeutic approaches. Programmed death-ligand 1 (PD-L1) expression is an adaptive mechanism of tumour resistance to tumour-infiltrating lymphocytes, which in turn are needed for response to chemotherapy. Overall, available data support the concept that blockade of PD-L1/programmed cell death protein 1 checkpoint may improve efficacy of classical chemotherapy. PATIENTS AND METHODS: Two hundred and eighty patients with TNBC were enrolled in this multicentre study (NCT002620280) and randomized to neoadjuvant carboplatin area under the curve 2 and nab-paclitaxel 125 mg/m(2) intravenously (i.v.) on days 1 and 8, without (n = 142) or with (n = 138) atezolizumab 1200 mg i.v. on day 1. Both regimens were given q3 weeks for eight cycles before surgery followed by four cycles of an adjuvant anthracycline regimen. The primary aim of the study was to compare event-free survival (EFS), and an important secondary aim was the rate of pathological complete response (pCR defined as the absence of invasive cells in breast and lymph nodes). The primary population for all efficacy endpoints is the intention-to-treat (ITT) population. RESULTS: The ITT analysis revealed that pCR rate after treatment with atezolizumab (48.6%) did not reach statistical significance compared to no atezolizumab [44.4%: odds ratio (OR) 1.18; 95% confidence interval 0.74-1.89; P = 0.48]. Treatment-related adverse events were similar with either regimen except for a significantly higher overall incidence of serious adverse events and liver transaminase abnormalities with atezolizumab. CONCLUSIONS: The addition of atezolizumab to nab-paclitaxel and carboplatin did not significantly increase the rate of pCR in women with TNBC. In multivariate analysis, the presence of PD-L1 expression was the most significant factor influencing the rate of pCR (OR 2.08). Continuing follow-up for the EFS is ongoing, and molecular studies are under way.
Published: 2022

3. Trends in Perinatal Practices and Neonatal Outcomes of Very Low Birth Weight Infants during a 16-year Period at NEOCOSUR Centers

Author: D'Apremont, I., Marshall, G., Musalem, C., Mariani, G., Musante, G., Bancalari, A., Fabres, J., Mena, P., Zegarra, J., Tavosnanska, J., Lacarrubba, J., Solana, C., Vaz Ferreira, C., Herrera, T., Villarroel, L., Tapia, J.L., and NEOCOSUR Neonatal Network
Subjects: Adult, medicine.medical_specialty, Birth weight, Perinatal care, Gestational Age, morbidity, Infant, Premature, Diseases, survival, 03 medical and health sciences, 0302 clinical medicine, Early onset sepsis, Adrenal Cortex Hormones, Enterocolitis, Necrotizing, 030225 pediatrics, Sepsis, medicine, Retrospective analysis, Humans, Infant, Very Low Birth Weight, Retinopathy of Prematurity, obstetrical care, 030212 general & internal medicine, preterm infants, neonatal care, database, Bronchopulmonary Dysplasia, Cerebral Hemorrhage, Retrospective Studies, purl.org/pe-repo/ocde/ford#3.02.03 [https], Obstetrics, business.industry, Cesarean Section, Infant, Newborn, Gestational age, Low birth weight, Perinatal Care, Treatment Outcome, Neonatal outcomes, Pediatrics, Perinatology and Child Health, Gestation, Female, medicine.symptom, business, Infant, Premature, Hydrocephalus, Maternal Age
Abstract: Objective: To describe trends in mortality, major morbidity, and perinatal care practices of very low birth weight infants born at NEOCOSUR Neonatal Network centers from January 1, 2001, through December 31, 2016. Study design: A retrospective analysis of prospectively collected data from all inborn infants with a birthweight of 500-1500 g and 23-35 weeks of gestation. Results: We examined data for 13 987 very low birth weight infants with a mean birth weight of 1081 ± 281 g and a gestational age of 28.8 ± 2.9 weeks. Overall mortality was 26.8% without significant changes throughout the study period. Decreases in early onset sepsis from 6.3% to 2.8% (P
Published: 2020

4. Phase 3 Trial of 177Lu-Dotatate for Midgut Neuroendocrine Tumors

Author: Strosberg, J, El Haddad, G, Wolin, E, Hendifar, A, Yao, J, Chasen, B, Mittra, E, Kunz, Pl, Kulke, Mh, Jacene, H, Bushnell, D, O'Dorisio, Tm, Baum, Rp, Kulkarni, Hr, Caplin, M, Lebtahi, R, Hobday, T, Delpassand, E, Van Cutsem, E, Benson, A, Srirajaskanthan, R, Pavel, M, Mora, J, Berlin, J, Grande, E, Reed, N, Seregni, E, Öberg, K, Lopera Sierra, M, Santoro, P, Thevenet, T, Erion, Jl, Ruszniewski, P, Kwekkeboom, D, Krenning, E, Ansquer, C, Baudin, E, Courbon, F, Giammarile, F, Taieb, D, Scheidhauer, K, Weber, M, Bodei, L, Brianzoni, E, DELLE FAVE, Gianfranco, Chiara Grana, M, Mariani, G, Rindi, G, Severi, S, Azevedo, I, Sundin, A, Al‐nahhas, A, Freemantle, N, Grossman, A, Manoharan, P, Anthony, L, Benson, Ab, Garbus, S, Kulke, M, Kvols, L, Metz, D, Morse, M, Schipper, M, Yao, J., and Radiology & Nuclear Medicine
Subjects: Male, medicine.medical_specialty, Octreotide, Antineoplastic Agents, Kaplan-Meier Estimate, Neuroendocrine tumors, Gastroenterology, Disease-Free Survival, Drug Administration Schedule, 030218 nuclear medicine & medical imaging, law.invention, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Randomized controlled trial, law, Internal medicine, Organometallic Compounds, medicine, Clinical endpoint, Humans, Infusions, Intravenous, Telotristat ethyl, Aged, Gastrointestinal Neoplasms, Tumors, DOTA-TATE, Intestins, business.industry, Nausea, General Medicine, receptor radionuclide therapy, radiolabeled somatostatin analog, carcinoid-syndrome, prognostic-factors, survival, prrt, lu-177-dota-octreotate, lu-177-octreotate, scintigraphy, octreotide, Middle Aged, medicine.disease, Surgery, Intestines, Clinical trial, Neuroendocrine Tumors, chemistry, Delayed-Action Preparations, 030220 oncology & carcinogenesis, Radionuclide therapy, Female, business, medicine.drug
Abstract: Background: Patients with advanced midgut neuroendocrine tumors who have had disease progression during first-line somatostatin analogue therapy have limited therapeutic options. This randomized, controlled trial evaluated the efficacy and safety of lutetium-177 ((177)Lu)-Dotatate in patients with advanced, progressive, somatostatin-receptor-positive midgut neuroendocrine tumors. Methods: We randomly assigned 229 patients who had well-differentiated, metastatic midgut neuroendocrine tumors to receive either (177)Lu-Dotatate (116 patients) at a dose of 7.4 GBq every 8 weeks (four intravenous infusions, plus best supportive care including octreotide long-acting repeatable [LAR] administered intramuscularly at a dose of 30 mg) ((177)Lu-Dotatate group) or octreotide LAR alone (113 patients) administered intramuscularly at a dose of 60 mg every 4 weeks (control group). The primary end point was progression-free survival. Secondary end points included the objective response rate, overall survival, safety, and the side-effect profile. The final analysis of overall survival will be conducted in the future as specified in the protocol; a prespecified interim analysis of overall survival was conducted and is reported here. Results: At the data-cutoff date for the primary analysis, the estimated rate of progression-free survival at month 20 was 65.2% (95% confidence interval [CI], 50.0 to 76.8) in the (177)Lu-Dotatate group and 10.8% (95% CI, 3.5 to 23.0) in the control group. The response rate was 18% in the (177)Lu-Dotatate group versus 3% in the control group (PConclusions: Treatment with (177)Lu-Dotatate resulted in markedly longer progression-free survival and a significantly higher response rate than high-dose octreotide LAR among patients with advanced midgut neuroendocrine tumors. Preliminary evidence of an overall survival benefit was seen in an interim analysis; confirmation will be required in the planned final analysis. Clinically significant myelosuppression occurred in less than 10% of patients in the (177)Lu-Dotatate group. (Funded by Advanced Accelerator Applications; NETTER-1 ClinicalTrials.gov number, NCT01578239 ; EudraCT number 2011-005049-11 .).
Published: 2017
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5. A multicenter observational study on the management of hyperglycemia in patients with acute coronary syndrome

Author: Avanzini, F, Mafrici, A, Riva, E, Franzosi, MG, Milani, V, Giudici, V, Marelli, G, Mariani, G, Piatti, PM, Roncaglioni, MC, on behalf of GLICINE-SPIDER Collaborative Group, Margonato, A, Cianflone, D, Avanzini, F, Mafrici, A, Riva, E, Franzosi, Mg, Milani, V, Giudici, V, Marelli, G, Mariani, G, Piatti, Pm, Roncaglioni, Mc, on behalf of GLICINE-SPIDER Collaborative, Group, Margonato, A, and Cianflone, D
Subjects: Blood Glucose, Male, Diabetes mellitu, Poor prognosis, Acute coronary syndrome, medicine.medical_specialty, Prognosi, Endocrinology, Diabetes and Metabolism, medicine.medical_treatment, Medicine (miscellaneous), Diabetes Complications, Diabetes Complication, Diabetes mellitus, Internal medicine, Diabetes Mellitus, Nutrition and Dietetic, Humans, Insulin, Medicine, In patient, Prospective Studies, Acute Coronary Syndrome, Prospective cohort study, Intensive care medicine, Coronary Care Unit, Aged, Nutrition and Dietetics, business.industry, Incidence (epidemiology), Coronary Care Units, Intensive cardiac care unit, Middle Aged, Prognosis, medicine.disease, Prospective Studie, Treatment Outcome, Italy, Hyperglycemia, Female, Observational study, Cardiology and Cardiovascular Medicine, business, Human
Abstract: Aim To assess the prevalence, risk and management of hyperglycemia in patients with acute coronary syndrome (ACS). Methods and Results Design: a multicenter prospective observational study of a representative sample of patients with ACS consecutively admitted to intensive cardiac care units (ICCU). Setting: 31 out of 61 ICCUs in Lombardy, the most heavily populated Italian region. From May 2009 to April 2010 1260 patients (69.4% male; mean age 68 ± 13 years) were included in the study: 301 (23.9%) were known diabetic patients (D) and 265 (21.0%) had hyperglycemia (H) (blood glucose >180 mg/dL) at hospital admission, 174 with a history of diabetes (D+H+) and 91 without (D−H+). On the first day after admission intravenous insulin infusion was prescribed to 72 D+H+ (41.4%) and 10 D−H+ (11.0%), according to different protocols. Approximately one third of D+H+ patients (59) and one fifth (17) of D−H+ maintained mean blood glucose higher than 180 mg/dL during the first day in the ICCU. Patients with diabetes or hyperglycemia had a higher incidence of major adverse cardiovascular events or death in hospital. However, at multivariable analysis neither diabetes nor blood glucose at admission was associated with a poor prognosis whereas mean blood glucose on the first day was an independent negative prognostic predictor (OR 1.010, 95% CI 1.002–1.018, p = 0.016). Conclusion Hyperglycemia is frequent in patients with ACS and is independently associated with a poor in-hospital prognosis if it persists in first day. Unfortunately, however, this condition is still poorly treated, with far from optimal blood glucose control.
Published: 2015
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6. Everolimus Plus Exemestane in Advanced Breast Cancer: Safety Results of the BALLET Study on Patients Previously Treated Without and with Chemotherapy in the Metastatic Setting

Author: Generali D, Montemurro F, Bordonaro R, Mafodda A, Romito S, Michelotti A, Piovano P, Ionta MT, Bighin C, Sartori D, Frassoldati A, Cazzaniga ME, Riccardi F, Testore F, Vici P, Barone CA, Schirone A, Piacentini F, Nolè F, Molino A, Latini L, Simoncini EL, Roila F, Cognetti F, Nuzzo F, Foglietta J, Minisini AM, Goffredo F, Portera G, Ascione G, Mariani G, Cazzaniga M, contributor, Generali, D, Montemurro, F, Bordonaro, R, Mafodda, A, Romito, S, Michelotti, A, Piovano, P, Ionta, M, Bighin, C, Sartori, D, Frassoldati, A, Cazzaniga, M, Riccardi, F, Testore, F, Vici, P, Barone, C, Schirone, A, Piacentini, F, Nolè, F, Molino, A, Latini, L, Simoncini, E, Roila, F, Cognetti, F, Nuzzo, F, Foglietta, J, Minisini, A, Goffredo, F, Portera, G, Ascione, G, Mariani, G, Contributor, Generali, Daniele, Montemurro, F., Bordonaro, R., Mafodda, A., Romito, S., Michelotti, A., Piovano, P., Ionta, M. T., Bighin, C., Sartori, D., Frassoldati, A., Cazzaniga, M. E., Riccardi, F., Testore, F., Vici, P., Barone, C. A. ., Schirone, Alice, Piacentini, F., Nolè, F., Molino, A., Latini, L., Simoncini, E. L., Roila, F., Cognetti, Francesca, Nuzzo, Federica, Foglietta, J., Minisini, A. M., Goffredo, F., Portera, G., Ascione, G., and Mariani, G.
Subjects: 0301 basic medicine, Oncology, Cancer Research, Advanced breast cancer, Everolimus, Hormone-receptor positive, Real life, Safety, medicine.medical_treatment, chemistry.chemical_compound, 0302 clinical medicine, Exemestane, Antineoplastic Combined Chemotherapy Protocols, Medicine, Neoplasm Metastasis, Aged, 80 and over, advanced breast cancer, everolimus, hormone-receptor positive, real life, safety, Middle Aged, Metastatic breast cancer, Everolimu, Italy, 030220 oncology & carcinogenesis, Female, medicine.drug, Hormone‐receptor positive, Adult, medicine.medical_specialty, Drug-Related Side Effects and Adverse Reactions, Socio-culturale, Breast Neoplasms, Everolimus Plus Exemestane, Advanced Breast Cancer, BALLET Study, 03 medical and health sciences, Breast cancer, Internal medicine, Post-hoc analysis, Breast Cancer, Humans, Adverse effect, Aged, Chemotherapy, business.industry, medicine.disease, Androstadienes, Regimen, 030104 developmental biology, chemistry, business
Abstract: BACKGROUND: The BALLET study was an open-label, multicenter, expanded access study designed to allow treatment with everolimus plus exemestane in postmenopausal women with hormone receptor-positive metastatic breast cancer progressed following prior endocrine therapy. A post hoc analysis to evaluate if previous chemotherapy in the metastatic setting affects the safety profile of the combination regimen of everolimus and exemestane was conducted on the Italian subset, as it represented the major part of the patients enrolled (54%). PATIENTS AND METHODS: One thousand one hundred and fifty-one Italian patients were included in the present post hoc analysis, which focused on two sets of patients: patients who never received chemotherapy in the metastatic setting (36.1%) and patients who received at least one chemotherapy treatment in the metastatic setting (63.9%). RESULTS: One thousand one hundred and sixteen patients (97.0%) prematurely discontinued the study drug, and the main reasons reported were disease progression (39.1%), local reimbursement of everolimus (31.1%), and adverse events (AEs) (16.1%). The median duration of study treatment exposure was 139.5 days for exemestane and 135.0 days for everolimus. At least one AE was experienced by 92.5% of patients. The incidence of everolimus-related AEs was higher (83.9%) when compared with those that occurred with exemestane (29.1%), and the most commonly reported everolimus-related AE was stomatitis (51.3%). However, no significant difference in terms of safety related to the combination occurred between patients without and with chemotherapy in the metastatic setting. CONCLUSION: Real-life data of the Italian patients BALLET-related cohort were an adequate setting to state that previous chemotherapy did not affect the safety profile of the combination regimen of everolimus and exemestane. The Oncologist 2017;22:1-8Implications for Practice: With the advent of new targeted agents for advanced or metastatic breast cancer, multiple lines of therapy may be possible, and components of the combined regimens can overlap from one line to another. Thus, it is important to assess even the potential of cumulative and additive toxic effects among the drugs. Previous chemotherapy did not affect the safety profile of the combination regimen of everolimus and exemestane. The continuous monitoring of the safety signals of this drug combination from general clinical practice is important, in particular for stomatitis.
Published: 2016

7. Association of kidney disease measures with risk of renal function worsening in patients with type 1 diabetes

Author: Mirijello, Antonio, Viazzi, Francesca, Fioretto, Paola, Giorda, Carlo, Ceriello, Antonio, Russo, Giuspina T., Guida, Pietro, Pontremoli, Roberto, De Cosmo, Salvatore, Cimino, Antonino, Fava, Danila, Giorda, Carlo Bruno, Meloncelli, Illidio, Nicolucci, Antonio, Pellegrini, Fabio, Rossi, Maria Chiara, Turco, Salvatore, Vespasiani, Giacomo, Pellegrini, F., Graziano, G., Lucisano, G., Memmo, R., Pellicciotta, E., Paciotti, V., Pupillo, M., Armentano, G., Giovannini, C., Armentano, V., Laudato, M., Turco, S., Acquati, S., Ciardullo, A. V., Laffi, G., Felace, G., Taboga, C., Tortul, C., Santantonio, G., Suraci, C., Ghisoni, G., Raffa, M., Genovese, S., Lovagnini-Scher, C. A., Rampini, P., Rocca, A., Ruggeri, P., Tortato, E., Cotti, L., Cristofaro, M. R., Tagliaferri, M., Comoglio, M., Fornengo, R., De Cosmo, S., Gentile, F. M., Gigante, A., Mastinu, F., Di Benedetto, A., Pata, P., Arcangeli, A., Orsini, P., Acler, P., De Blasi, G., Cicioni, G., Pocciati, S., Marangoni, A., Nogara, A., Lanero, M., Bertero, M. G., Damassino, R., Bergonzini, C., Schumtz, L., Seksich, L., Pipitone, A., Boaretto, M., Manfroi, I., Parmesan, L., Conte, B., Soccol, F., Pagano, A., Papini, E., Rinaldi, R., Petrucci, L., Graziano, F., Chianelli, M., Silvagni, S., Rosco, M., Ansaldi, E., Malvicino, F., Battezzati, M., Maresca, P., Palenzona, C., Boemi, M., Rabini, R. A., Brandoni, G., Lanari, L., Gatti, C., Testa, I., Cherubini, V., Doveri, G., Pecorelli, L., Ciccarelli, A., Gallardini, M. B., Courthoud, R., Sara Bredy, S., Ricciardi, G. P., Vitalone, G., Setti, D., Contrini, P., Corsi, A., Ghigliotti, V., Oddone, G., Ponzani, P., Valbonesi, G., Mazzini, V., Di Berardino, P., Colleluori, P., Montani, V., Trosini, V., Velussi, M., Alfidi, P., Verdecchia, B., Baliva, L., Di Pietro, A., Franchi, G., Luce, R. P., Pianta, A., Ferrari, M., Balzano, S., Beltranello, G., Dal Fabbro, S., Aricò, C. N., Cervo, L., Zanon, R., Rossa, S., Di Pace, M. C., Ciavarella, A., Giangiulio, S., Grimaldi, M., Mustacchio, A., Santacroce, G., Fattor, B., Monauni, T., Cristini, M., Orion, G., Crazzolara, D., Amor, F., Eisath, J. E., Lintner, S., Garavelli, S., Calari, T., Marini, P., Sandri, O., Scala, M., Stroppa, C., Trentin, A., Carlin, R., Carli, B., Sandonà, M., Zortea, C., Bonet, L., Pradel, L., Reato, S., Buschini, M., Bonfiglioli, D., Mones, D., Beldì, F., Morea, A., Bondesan, L., Perbellini, S., Cimino, A., Valentini, U., Agosti, B., Corsini, R., Girelli, A., Zarra, E., Rocca, L., Bergmann, M., Pradi, I., Unterkircher, S., Piok, M., Pichler, M., Trinchera, A., Palamà, G., Palma, P., Carboni, L., Murtas, M. G., Mudadu, T., Turco, M. P., Floris, M., Delogu, A., Farris, L., Songini, M., Piras, G., Seguro, R., Floris, R., Corona, G., Lai, M., Piras, E., Contini, P. P., Cocco, S., Pilosu, R. M., Sannia, M. C., Spanu, F., Busciantella Ricci, N., Cartechini, M. G., Agostinelli, G., Fiorelli, C., Nuzzi, A., Ballauri, C., Giorda, C. B., Lesina, A., Romeo, F., Giudici, G., Maciejewska, E. G., Deroma, A., Paduano, M., Rossi, L., Vagnini, C., Dolci, M., Mori, M., Baccetti, F., Gregori, G., Straface, E., Pozzuoli, G., Barone, M., Stasio, G. B., Tondini, S., Borgoni, F., Grosso, J., Scarsellato, C., Sciulli, A., De Marco, F., Confortin, L., Marin, N., Lamonica, M., Gialdino, S., Borzì, V., Gatta, C., Rapisard, R., Strano, S., Calabrò, M., Puccio, L., Zolli, M., Coracina, A., Starnone, V., Del Buono, A., Terracciano, A. M., Monda, M. V., Castro, F., Guaglianone, A., Maccari, V., Corsi, L., Versari, G., Falivene, M. R., Boletto, N., Corsi, S., Marafetti, L., Vitacolonna, E., Capani, F., Caputo, L., Di Nisio, L., Simonetti, F., Boscolo Bariga, A., Ballarin, G., De Boni, S., Di Benedetto, S., Chiambretti, A. M., Di Vito, L., Pascuzzo, M. D., Urli, P., Rumi, P., Balzarini, B., Galli, P., Castellan, M., Giannetti, A., Russotti, C., De Blasi, A., Perna, A., Campanelli, C., Ranchelli, A., Biccheri, D., Dadi, G., Massa, L., Baldi, G. P., Sciacca, F., Costanzo, E., Spada, M., Paolini, G., Ziller, P., Portolan, F., Pasolini, G., Ghilardi, G., Fiorina, P., Grata, M. L., Capretti, L., Speroni, G., Fugazza, L., Massafra, C., Lovagnini Scher, A., Cimicchi, M. C., Percudani, C., Risolo, T., Saccò, P., Gidoni Guarnieri, G. L., Piccolo, D., Bravin, C., De Noni, E., Scarpel, M., Marcon, M., Giacon, F., Panebianco, G., Tadiotto, F., Da Tos, V., D'Ambrosio, M., Pellizzola, D., Zampini, M. A., Frezzati, E., Mari, E., Raminelli, E., Gaiti, D., Bosi, E. A., Chierici, G., Pilla, S., Copelli, M., Zanichelli, P., Bertelli, L., Caretta, P., Vezzani, V., Bodecchi, S., Longobucco, A., Di Lembo, S., Spotti, E., Carrai, E., Degli Innocenti, A., Manini, L., Persico, R., Rossi, C., Magro, G., Marelli, G., Vilei, V., Andrioli, M., Bellato, L., Fedeli, M., Merlini, A., Pinelli, G., Marin, G., Contin, M. L., Gallo, A., Parlato, P., Pecchielan, W., Jacovacci, J., Placentino, G., Richini, D., Molinari, S., Strazzeri, R., Fabbri, T., Di Bartolo, P., Garrapa, G., D'Incau, F., Lagomanzini, P., Conte, P., Todesco, F., Foglini, P., Pantanetti, P., Bedetta, C., Maricotti, R., Tomasi, F., Monesi, M., Graziani, R., Beretta, F., Penna, L., Guberti, A., Dazzi, D., Forte, E., Gasbarrone, A., Marrocco, T., Moschetta, R., Tuccinardi, F., De Meo, F., Coppola, A., Pirolozzi, P., Placitelli, E., Vallefuoco, R., Catone, B., Ceschia, S., Urban, M., Fabbri, F., Torresani, M., Crovetto, R., Battistini, M., Carosia, P., Viviani, G. L., Durante, A., Pais, F., Lilliu, V., Quieto, C., D'Ugo, E., Squadrone, M., Amenduni, T., Iovannisci, M. M., Della Penna, L., Potente, F., Delle Donne, T., Massa, C., Ulisse, M. A., De Berardinis, S., Guarnieri, I., Pace, S., Splendiani, M., Di Giuseppe, R., Brunato, B., Assaloni, R., Muraro, R., Loro, R., Bucciol, S., Lavacca, C., Rossi, M., Sabbatini, G., Quadri, F., Sambuco, L., Santacroce, C., Paola Caretta, D., Marino, C., Micheletti, A., Petrelli, A., Corda, A., Pisano, L., Guaita, G., Deias, C., Trevisan, G., Coletti, I., Iannarelli, R., De Luca, A., Minnucci, A., Antenucci, D., Di Florio, C., Angelicola, G., Bosco, A., Fresco, R., Di Marco, G., Ugolotti, D., Cadossi, T., Di Caro, P., Mazzocchetti, M., Buzzetti, R., Leto, G., Gnessi, C., Cipolloni, L., Foffi, C., Moretti, C., Venditti, C., Meniconi, R., Bertoli, S., Cosimi, S., Di Cianni, G., Turco, A., Richini, A., Marconi, S., Sannino, C., Lemmi, P., Giuntoli, S., Manfrè, N., Giannini, F., Di Carlo, A., Casadidio, I., Melandri, P., Maolo, G., Polenta, B., Piccinini, N., Vincenti, C., Pastore, N., Mega, P., Magurano, E., Cananiello, A., Francescutto, C. A., Brussa Toi, E., Gaspardo, G., Angeli, L., Ronchese, L., Sciangula, L., Ciucci, A., Contartese, A., Banfi, E., Castelli, E., Tatti, P., Bloise, D., Di Mauro, P., Masselli, L., Lo Presti, A., Scarpitta, A. M., Gambina, F., Venezia, A., Morea, R., Lagonigro, G., Copeta, G., Iannucci, V., Milano, V., Trupo, M., Lochmann, A., Marchetto, P. E., Incelli, G., De Paola, G., Steiger, M. M., Gamper, M. A., Breitenberger, S., Holzner, M., Frischmann, J., Lambiase, C., Di Vece, T., D'Aniello, M., Fezza, M., Giordano, C., Leo, F., Saitta, G., Cucinotta, D., Di Vieste, G., Pintaudi, B., Mancuso, T., Musacchio, N., Giancaterini, A., Pessina, L., Salis, G., Schivalocchi, F., Testori, G., Rampini, P. A., Cerutti, N., Morpugo, P. S., Cavaletto, M. L., Bonino, G., Morreale, F., Mariani, G., Ragonesi, P. D., Bollati, P., Colapinto, P., Bosi, E., Falqui, L., Bortolato, L., Cosma, A., Pistolato, P., Centenaro, B., Ceccato, A., Campobasso, G., Zaurino, F., Mazzotta, G., Manti, R., Da Ros, R., Carlucci, S., Narduzzi, L., Bortolotto, D., D'Acunto, L., Stanic, L., Volpi, A., Cospite, A. M., Manicardi, V., Michelini, M., Finardi, L., Borghi, F., Manicardi, E., Lombardi, S., Tommasi, C., Iaccarino, M., Cozza, S., Binotto, M., Marini, F., Mecenero, I., Massignani, S., Stecco, P., Urbani, E., Massariol, W., Parolin, R., Gatti, A., Bonavita, M., Creso, E., Giannettino, R., Gobbo, M., Iovine, C., Turco, A. A., Riccardi, G., Iazzetta, N., Giannattasio, C., Egione, O., Galdieri, S., Velotti, A., Azzolina, A., Annicelli, G., Sorrentino, T., Gaeta, I., Zenari, L., Bertolini, L., Sorgato, C., Grippaldi, F., Stroppiana, M., Popolizio, R., Carbone, N., Grasso, S., Abate, S., Gaggero, G. C., Strazzabosco, M., Brun, E., Carlesi, G. P., Garrone, S., Cicalò, A. M., Clausi, C., Cau, R., Manconi, A., Carboni, A., Angius, M. F., Pinna, A. A., Caria, S., Filigheddu, G. D., Tonolo, G., Carta, I., Calebich, S., Burlotti, C., Saglietti, G., Schellino, A., Madau, G., Cossu, M., Mulas, F., Zoccheddu, S., Balsanelli, M., Fetonti, M., Rotolo, A., Sambo, P., Secchi, E., Angotzi, M. A., Loddoni, S., Brundu, I., Careddu, F., Becciu, A., Gabriella Piras, G., Novara, F., Cipro, F., Torchio, G., Palumbo, P., Bianchi, A., Colucci, G., La Motta, G., Tiengo, A., Avogaro, A., Bruttomesso, D., Crepaldi, C., Fadini, G., Guarnieri, G., Lavagnini, M. T., Maran, A., Vedovato, M., De Kreutzenberg, V., Fedele, D., Lapolla, A., Sartore, G., Bax, G., Cardone, C., Dalfrà, M. G., Masin, M., Toniato, R., Piarulli, Francesco, Mattina, G., Fulantelli, M. A., Gioia, D., Conti, M., Ridola, G., D'Agati, F., Grossi, G., De Berardinis, F., Zavaroni, I., Dei Cas, A., Franzini, L., Usberti, E., Antonimi, M., Anelli, N., Poli, R., Ridolfi, V., Michela, M., Haddoub, S., Prampolini, G., Muoio, A., Filippi, D., Bucci, F., Tardio, S. M., Calderini, M. C., Magotti, M. G., Quarantelli, C., Vernazza, M. A., Carolfi, A., Saracca, R., Picchio, E., Del Sindaco, P., Spalluto, A., Maggiulli, L., Torreggiani, V., Rastelletti, S., Ugolini, C., Pucci, N., Magi, S., Muratori, S., La Penna, G., Consoli, A., Galeone, F., Magiar, A. V., Gherardini, V., Moretti, L., Bientinesi, M., Landi, L., Bernardi, A., Del Prato, S., Miccoli, R., Bianchi, C., Penno, G., Venditti, F., Anichini, R., De Bellis, A., Bruschi, T., Butelli, L., Gioffredi, M., Gori, R., Picciafuochi, R., Malagoli, R., Bernini, A., Gelisio, R., Zanon, M., Del Bianco, A., Bamiston, A., Signorato, M., Citro, G., Calabrese, M., Ianni, L., Lorenzetti, M., Marsocci, A., Guizzotti, S., Memoli, G., Cabasino, F., Farci, F., Atzori, A., Sanna, A., Ghiani, M., Siotto, I., Sedda, M., Manis, A., Loddo, C., Loddo, I., Seguro, P., Cuomo, A., Orlando, L., Olanda, G. B., Pucci, A., Massenzo, M., Sardu, C., Perrone, G., Corazziere, F., La Puzza, I., Tripodi, P. F., Riggio, S., Giampaolo, A., Mannino, D., Aleandri, A. R., Guidi, M. V., Battisti, B., Faraglia, M. R., Lilli, V., Leotta, S., Visalli, N., Gagliardi, A., Fontana, L., Altomare, M., Carletti, S., Abbruzzese, S., Chiaramonte, F., Giordano, R., Rossini, M., Migneco, G., Cappelloni, D., Urbani, A., Piergiovanni, F., Fava, D., Simonetta, A., Massimiani, F., Bulzomì, R., Giuliano, M., Pennafina, M. G., Di Perna, P., D'Accinni, M. P., Paolucci, D., D'Ubaldi, A., D'Angelo, M. T., Masaro, G., Pietrantoni, M., Fratini, M., La Rosa, R., Poggi, M., Piccirilli, F., Pisano, R., Saponara, C., Conforti, I., Penza, A., Scalpone, R., Lo Pinto, S., Iacovella, L., Caccamo, C., Sposito, S., Teodonio, C., Restuccia, M. G., Mirto, G., Girardello, R., Gennaro, R., De Moliner, L., Bettini, E., Mattuzzi, A., Speese, K., Frisinghelli, F., Locatelli, F., Nicoletti, M., Trojan, N., Centis, R., L Volsi, P., Levis, E., Zanette, G., Comba, G., Ballatore, L., Cattaneo, A., Aglialoro, A., Guido, R., Patrone, M., Zecchini, M., Vespasiani, G., Meloncelli, I., Clementi, L., Galetta, M., Marconi, V., Bordin, P., Perale, L., Vinci, C., Sira Zanon, M., Geretto, L., Toffolo, C., Furlan, M. G., Mazzanti, G., Vinci, M., Sica, V., Armeni, M., Derai, R., Ennas, O., Mamusa, S., Pisano, M. A., Carreras, L., Rauseo, A., Cervone, S., Leggieri, A., Pontonio, M., Sturaro, R., Quattrocchi, F., Molinaro, M., Trasatti, M., Ferretti, B., Labarile, G., Baule, G. M., Gentilini, A., Spanu, M. A., Fancellu, A., Bianco, P., Lione, L., Massazza, G., Bocchio, G., Bosco, E., Monachesi, M., Carta, G., Boschetti, M., Ceresola, E., Venier, E., Calcaterra, F., Cataldi, F., Miola, M., Manfrini, S., Lai, A., Locci, B., Putzu, D., Tanganelli, I., Leonini, M., Egger, K., Marchiotto, W., Vincis, L., Orlandini, V., Pilloni, C., Farci, R., Pelligra, I., Renier, G., Mameli, M., Pala, A., Devigus, E., Fumagalli, I., Lalli, C., Leandri, M., Agliani, M., De Pascalis, L., Malci, F., De Ciocchis, A., Diodati, M. B., Macerola, B., Davì, S., Caccavale, A., Brocato, L., Pognant Gros, M., Borla, S., Lattanzi, E., Piersanti, C., Piersanti, A., Spinelli, I., Tuzzoli, L., Tulini, V., Quaranta, G., Iorio, V., Tirabovi, M., De Terlizi, Candia, Massarelli, M. G., Venturi, S., Travaglini, A., Draghi, P., Pomante, P., Richiardi, L., Clerico, A., Bruno, A., Cavallo Perin, P., Ghigo, E., Porta, M., Scuntero, P., Arcari, R., Bertaina, S., Bo, S., Broglio, F., Bruno, G., Degiovanni, M., Fornengo, P., Grassi, G., Inglese, V., Maccario, M., Maghenzani, G., Marena, S., Martina, V., Passera, P., Ruiu, G., Tagliabue, M., Zanone, M., Monge, M., Boffano, G. M., Macrì, K., Maio, P., Ozzello, A., Pergolizzi, E., Gaia, D., Gennari, P., Micali, G., Rossetto, E., Dalmazzo, C., Oreglia, M., Stefani, T., Dossena, C., Paglia, P., Bosoni, S., Romanelli, T., Inchiostro, S., Dauriz, M., Bossi, C. A., Meregalli, G., Balini, A., Berzi, D., Filippini, B., Crotto, G., Paccagnella, A., Orrasch, M., Sambataro, M., Citro, T., Kiwanuka, E., Bagolin, E., Almoto, B., Macchia, A., Branca, M. T., Filesi, M., Candido, R., Caroli, E., Manca, E., Petrucco, A., Tommasi, E., Jagodnik, G., Baskar, B., Daris, N., Dal Col, P., Pellegrini, M. A., Tonutti, L., Venturini, G., Andreani, M., Turchi, F., Fedrighelli, F., Martinelli, G., Rongioletti, R., Candidi, M., Pais, M., Moro, E., Cervellino, F., Sinisi, R., Zampino, A., Mingardi, R., Lora, L., Reitano, R., Stocchiero, C., Simoncini, M., Mesturino, C. A., Zen, F., Di Pietro, S., Scoponi, C., Tilaro, L., Pelliccioni, S., Slongo, R., Vita, E., Garofalo, A., Vitale, F., Campanella, B., Mastrilli, V., Borrelli, T., D'Avino, A., Perbellini, A., Mirijello, Antonio, Viazzi, Francesca, Fioretto, Paola, Giorda, Carlo, Ceriello, Antonio, Russo, Giuspina T, Guida, Pietro, Pontremoli, Roberto, and De Cosmo, Salvatore, Giordano, Carla
Subjects: Adult, Male, Nephrology, medicine.medical_specialty, endocrine system diseases, Renal function, 030209 endocrinology & metabolism, 030204 cardiovascular system & hematology, lcsh:RC870-923, Kidney, urologic and male genital diseases, GFR, 03 medical and health sciences, 0302 clinical medicine, Risk Factors, Internal medicine, Diabetes mellitus, medicine, Humans, Albuminuria, Diabetic kidney disease, Type 1 diabetes, urogenital system, business.industry, Incidence (epidemiology), Middle Aged, lcsh:Diseases of the genitourinary system. Urology, medicine.disease, female genital diseases and pregnancy complications, Albuminuria, Diabetic kidney disease, GFR, Nephrology, Diabetes Mellitus, Type 1, medicine.anatomical_structure, Italy, Disease Progression, Female, medicine.symptom, business, Research Article, Follow-Up Studies, Glomerular Filtration Rate, Kidney disease
Abstract: Background Albuminuria has been classically considered a marker of kidney damage progression in diabetic patients and it is routinely assessed to monitor kidney function. However, the role of a mild GFR reduction on the development of stage ≥3 CKD has been less explored in type 1 diabetes mellitus (T1DM) patients. Aim of the present study was to evaluate the prognostic role of kidney disease measures, namely albuminuria and reduced GFR, on the development of stage ≥3 CKD in a large cohort of patients affected by T1DM. Methods A total of 4284 patients affected by T1DM followed-up at 76 diabetes centers participating to the Italian Association of Clinical Diabetologists (Associazione Medici Diabetologi, AMD) initiative constitutes the study population. Urinary albumin excretion (ACR) and estimated GFR (eGFR) were retrieved and analyzed. The incidence of stage ≥3 CKD (eGFR 30% from baseline was evaluated. Results The mean estimated GFR was 98 ± 17 mL/min/1.73m2 and the proportion of patients with albuminuria was 15.3% (n = 654) at baseline. About 8% (n = 337) of patients developed one of the two renal endpoints during the 4-year follow-up period. Age, albuminuria (micro or macro) and baseline eGFR 90 ml/min/1.73m2 and normoalbuminuria, those with albuminuria at baseline had a 1.69 greater risk of reaching stage 3 CKD, while patients with mild eGFR reduction (i.e. eGFR between 90 and 60 mL/min/1.73 m2) show a 3.81 greater risk that rose to 8.24 for those patients with albuminuria and mild eGFR reduction at baseline. Conclusions Albuminuria and eGFR reduction represent independent risk factors for incident stage ≥3 CKD in T1DM patients. The simultaneous occurrence of reduced eGFR and albuminuria have a synergistic effect on renal function worsening. Electronic supplementary material The online version of this article (10.1186/s12882-018-1136-6) contains supplementary material, which is available to authorized users.
Published: 2018

8. Bleeding symptoms at disease presentation and prediction of ensuing bleeding in inherited FVII deficiency

Author: Di Minno, M., Dolce, A., Mariani, G., STER Study Group, Auerswald, G, Batorova, A, Bernardi, F, Di Minno, G, M. N. Di Minno, Dolce, A, Giansilly Balizot, M, Ingerslev, J, Mariani, G, Martinowitz ,U, Morfini, M, Pinotti, M, Shapiro, A, Schved, JF, Sørensen, B., NAPOLITANO, Mariasanta, Di Minno, M., Dolce, A., Mariani, G.,STER Study Group, Auerswald, G, Batorova, A, Bernardi, F, Di Minno, G, M N Di Minno, Dolce, A, Giansilly-Balizot, M, Ingerslev, J, Mariani, G, Martinowitz ,U, Morfini, M, Napolitano, M, Pinotti, M, Shapiro, A, Schved, JF, Sørensen, B., DI MINNO, Matteo, Dolce, Alberto, and Mariani, Guglielmo
Subjects: Registrie, Male, Pediatrics, Factor VII Deficiency, International Cooperation, Asymptomatic individual, 030204 cardiovascular system & hematology, chemistry.chemical_compound, 0302 clinical medicine, Retrospective Studie, Age Factor, Registries, Young adult, Child, Clinical Trials as Topic, Hematology, Factor VII, Age Factors, Middle Aged, Phenotype, Treatment Outcome, Child, Preschool, Female, Presentation (obstetrics), medicine.symptom, Human, Adult, Quality Control, Risk, medicine.medical_specialty, Adolescent, Hemorrhage, Asymptomatic, Major bleed, 03 medical and health sciences, Young Adult, Internal medicine, medicine, Humans, Retrospective Studies, Models, Statistical, business.industry, Minor bleed, Infant, Retrospective cohort study, chemistry, Disease Presentation, Relative risk, business, 030215 immunology
Abstract: SummaryIndividuals with inherited factor VII (FVII) deficiency display bleeding phenotypes ranging from mild to severe, with 30% of patients having always been asymptomatic (non-bleeding). In 626 FVII-deficient individuals, by analysing data from the International Factor VII (IF7) Registry and the Seven Treatment Evaluation Registry (STER), we determined whether bleeding type at disease presentation and FVII coagulant activity (FVIIc) predict ensuing bleeds. At disease presentation/diagnosis, 272 (43.5%) individuals were non-bleeding, 277 (44.2%) had minor bleeds, and 77 (12.3%) had major bleeds. During a median nine-year index period (IP) observation, 87.9% of non-bleeding individuals at presentation remained asymptomatic, 75.1% of minor-bleeders had new minor bleeds, and 83.1% of major-bleeders experienced new major bleeds. After adjusting for FVIIc levels and other clinical and demographic variables, the relative risk (RR) for ensuing bleedings during the IP was 6.02 (p
Published: 2012

9. Demographic and clinical data in acquired hemophilia A

Author: Knoebl, P, Marco, P, Baudo, F, Collins, P, Huth Kühne, A, Nemes, L, Pellegrini, F, Tengborn, L, Lévesque, H, Aspoeck G, Heistinger M, Knöbl P, Makipernaa A, André H, Aouba A, Bellucci S, Beurrier P, Borg JY, Darnige L, Devignes J, D'Oiron R, Gautier P, Gay V, Girault S, Gruel Y, Guerin V, Hézard N, Khellaf M, Koenig M, Lévesque H, Lifermann F, Marlu R, Peynet J, Quéméneur T, Rothschild C, Schleinitz N, Sigaud M, Trouillier S, Voisin S, Giebl A, Holstein K, Huth Kühne A, Loreth RM, Steigerwald U, Tiede A, Theodossiades G, Nemes L, Radvanyi G, Schlammadinger A, Barillari G, Pasca S, Baudo F, Caimi T, Contino L, Di Minno G, Cerbone AM, Di Minno D, D'incà M, Falanga A, Maggioni A, Lerede T, Franchini M, Gaidano G, De Paoli L, Gamba G, Ghirardi R, Girotto M, Tasca D, Grandone E, Tiscia G, Imberti D, Iorio A, Landolfi R, Di Gennaro L, Novarese L, Mariani G, Lapecorella M, Marietta M, Pedrazzi P, Mazzucconi MG, Santoro C, Morfini M, Linari S, Moratelli S, Paolini R, Piseddu G, Poggio R, POGLIANI, ENRICO MARIA, Carpenedo M, Remiddi C, Santagostino E, Santoro R, Papaleo G, Schinco P, Borchiellini A, Scortechini AR, Siragusa S, Sottilotta G, Squizzato A, Sartori R, Tagariello G, Tagliaferri AR, Di Perna C, Rivolta GF, Testa S, Paoletti O, Toschi V, Zanon E, Brandolini B, Hamulyák K, Kamphuisen P, Laros van Gorkom B, Leebeek FW, Marten N, Novakova I, Schutgens R, van der Linden PW, van Esser J, van der Meer J, Ypma P, Campos M, Aguilar C, Altisent C, Bermejo N, Del Campo R, Ferreiro Argüelles M, González Boullosa R, Gutiérrez Pimentel MJ, Jiménez Yuste V, Jose Felix L, Pascual M, Mingot ME, Perez Garrido R, Perez Gonzale Nz, Prieto Garcia M, Rodriguez Huerta AM, Sedano C, Tolosa Munoz A, Baghaei F, Tengborn L, Boehlen F, Korte W, Chowdary P, Collins P, Evans G, Pavord S, Rangarajan S, Wilde J., Knoebl, P, Marco, P, Baudo, F, Collins, P, Huth Kühne, A, Nemes, L, Pellegrini, F, Tengborn, L, Lévesque, H, Group Author: Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, Jy, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lifermann, F, Marlu, R, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Loreth, Rm, Steigerwald, U, Tiede, A, Theodossiades, G, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Caimi, T, Contino, L, DI MINNO, Giovanni, Cerbone, Am, DI MINNO, matteo nicola dario, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, Mg, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Scortechini, Ar, Siragusa, S, Sottilotta, G, Squizzato, A, Sartori, R, Tagariello, G, Tagliaferri, Ar, Di Perna, C, Rivolta, Gf, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Hamulyák, K, Kamphuisen, P, Laros van Gorkom, B, Leebeek, Fw, Marten, N, Novakova, I, Schutgens, R, van der Linden, Pw, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, Mj, Jiménez Yuste, V, Jose Felix, L, Pascual, M, Mingot, Me, Perez Garrido, R, Perez Gonzale, Nz, Prieto Garcia, M, Rodriguez Huerta, Am, Sedano, C, Tolosa Munoz, A, Baghaei, F, Boehlen, F, Korte, W, Chowdary, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J., Aspoeck, G, Borg, JY, Huth-Kühne, A, Loreth, RM, Di Minno, G, Cerbone, AM, Di Minno, D, D'incà, M, Mazzucconi, MG, Scortechini, AR, Tagliaferri, AR, Rivolta, GF, Laros-van Gorkom, B, Leebeek, FW, van der Linden, PW, Gutiérrez Pimentel, MJ Jiménez-Yuste, V, Jose-Felix, L, Pascual, M, Mingot, ME, Rodriguez-Huerta, AM, Wilde, J, Faculteit Medische Wetenschappen/UMCG, Cardiovascular Centre (CVC), Vascular Ageing Programme (VAP), Huth Kühne, A, Lévesque, H, Borg, J, Loreth, R, Cerbone, A, Mazzucconi, M, Scortechini, A, Tagliaferri, A, Rivolta, G, Brandolini, B, Leebeek, F, van der Linden, P, Gutiérrez Pimentel, M, Mingot, M, Perez Gonzale, N, Rodriguez Huerta, A, and Other departments
Subjects: Registrie, Male, Pediatrics, diagnosis, medicine.medical_treatment, Hemostatic Technique, Kaplan-Meier Estimate, registry, THERAPY, Settore MED/15 - Malattie Del Sangue, Immunosuppressive Agent, IMMUNOADSORPTION, Risk Factors, Pregnancy, 80 and over, Prospective Studies, Registries, Prospective cohort study, health care economics and organizations, Aged, 80 and over, treatment, Immunosuppression, Hematology, Middle Aged, FACTOR-VIII INHIBITOR, Acquired hemophilia, Demographics, Diagnosis, Outcome, Registry, Treatment, Aged, Autoantibodies, Chi-Square Distribution, Europe, Factor VIII, Female, Hemostatic Techniques, Humans, Immunosuppressive Agents, Risk Assessment, Treatment Outcome, Hemophilia A, Hemorrhage, Autoantibodie, acquired hemophilia, demographics, outcome, INTRAVENOUS GAMMA-GLOBULIN, Human, medicine.medical_specialty, Malignancy, hemophilia, registry, medicine, METAANALYSIS, Autoimmune disease, business.industry, Risk Factor, Autoantibody, medicine.disease, Surgery, Prospective Studie, Hemostasis, business, Chi-squared distribution
Abstract: Summary. Background: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA. Objectives: The European Acquired Hemophilia Registry (EACH2) was established to generate a prospective, large-scale, pan-European database on demographics, diagnosis, underlying disorders, bleeding characteristics, treatment and outcome of AHA patients. Results: Five hundred and one (266 male, 235 female) patients from 117 centers and 13 European countries were included in the registry between 2003 and 2008. In 467 cases, hemostasis investigations and AHA diagnosis were triggered by a bleeding event. At diagnosis, patients were a median of 73.9 years. AHA was idiopathic in 51.9%; malignancy or autoimmune diseases were associated with 11.8% and 11.6% of cases. Fifty-seven per cent of the non-pregnancy-related cases were male. Four hundred and seventy-four bleeding episodes were reported at presentation, and hemostatic therapy initiated in 70.5% of patients. Delayed diagnosis significantly impacted treatment initiation in 33.5%. Four hundred and seventy-seven patients underwent immunosuppression, and 72.6% achieved complete remission. Conclusions: Representing the largest collection of consecutive AHA cases to date, EACH2 facilitates the analysis of a variety of open questions in AHA.
Published: 2012
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10. Immunosuppression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2)

Author: Collins, Peter, Baudo, Francesco, Knoebl, Paul, Lévesque, Hervé, Nemes, László, Pellegrini, Fabio, Marco, Pascual, Tengborn, Lilian, Huth Kühne, Angela, Group Author: Aspoeck G, Heistinger M, Knöbl P, Makipernaa A, André H, Aouba A, Bellucci S, Beurrier P, Borg JY, Darnige L, Devignes J, d'Oiron R, Gautier P, Gay V, Girault S, Gruel Y, Guerin V, Hézard N, Khellaf M, Koenig M, Lévesque H, Lifermann F, Marlu R, Ninet J, Peynet J, Quéméneur T, Rothschild C, Schleinitz N, Sigaud M, Trouillier S, Voisin S, Giebl A, Holstein K, Huth Kühne A, Loreth RM, Steigerwald U, Tiede A, Theodossiades G, Nemes L, Radvanyi G, Schlammadinger A, Barillari G, Pasca S, Baudo F, Caimi T, Contino L, D'Angelo Armando CL, Fattorini A, Cerbone AM, D'incà M, Falanga A, Maggioni A, Lerede T, Franchini M, Gaidano G, De Paoli L, Gamba G, Ghirardi R, Girotto M, Tasca D, Grandone E, Tiscia G, Imberti D, Iorio A, Landolfi R, Di Gennaro L, Novarese L, Mariani G, Lapecorella M, Marietta M, Pedrazzi P, Mazzucconi MG, Santoro C, Morfini M, Linari S, Moratelli S, Paolini R, Piseddu G, Poggio R, Pogliani E, Carpenedo M, Remiddi C, Santagostino E, Mancuso ME, Santoro R, Papaleo G, Schinco P, Borchiellini A, Valeri F, Scortechini AR, Siragusa S, Sottilotta G, Squizzato A, Tagariello G, Sartori R, Tagliaferri AR, Di Perna C, Rivolta GF, Testa S, Paoletti O, Toschi V, Zanon E, Brandolin B, Hamulyák K, Kamphuisen P, Laros van Gorkom B, Leebeek FW, Marten N, Novakova I, Schutgens R, van der Linden PW, van Esser J, van der Meer J, Ypma P, Campos M, Aguilar C, Altisent C, Bermejo N, Del Campo R, Ferreiro Argüelles M, González Boullosa R, Gutiérrez Pimentel MJ, Jiménez Yuste V, Jose Felix L, Marco P, Mingot ME, Perez Garrido R, Perez Gonzale NZ, Prieto Garcia M, Rodriguez Huerta AM, Sedano C, Tolosa Munoz A, Baghaei F, Tengborn L, Boehlen F, Korte W, Chowdary P, Collins P, Evans G, Pavord S, Rangarajan S, Wilde J., DI MINNO, GIOVANNI, DI MINNO, MATTEO, Interne Geneeskunde, Foundations and methods of Law, RS: CARIM School for Cardiovascular Diseases, Faculteit Medische Wetenschappen/UMCG, Cardiovascular Centre (CVC), Vascular Ageing Programme (VAP), Collins, Peter, Baudo, Francesco, Knoebl, Paul, Lévesque, Hervé, Nemes, László, Pellegrini, Fabio, Marco, Pascual, Tengborn, Lilian, Huth Kühne, Angela, Group Author: Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, Jy, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lévesque, H, Lifermann, F, Marlu, R, Ninet, J, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Huth Kühne, A, Loreth, Rm, Steigerwald, U, Tiede, A, Theodossiades, G, Nemes, L, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Baudo, F, Caimi, T, Contino, L, D'Angelo Armando, Cl, Fattorini, A, DI MINNO, Giovanni, Cerbone, Am, DI MINNO, Matteo, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, Mg, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Mancuso, Me, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Valeri, F, Scortechini, Ar, Siragusa, S, Sottilotta, G, Squizzato, A, Tagariello, G, Sartori, R, Tagliaferri, Ar, Di Perna, C, Rivolta, Gf, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Brandolin, B, Hamulyák, K, Kamphuisen, P, Laros van Gorkom, B, Leebeek, Fw, Marten, N, Novakova, I, Schutgens, R, van der Linden, Pw, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, Mj, Jiménez Yuste, V, Jose Felix, L, Marco, P, Mingot, Me, Perez Garrido, R, Perez Gonzale, Nz, Prieto Garcia, M, Rodriguez Huerta, Am, Sedano, C, Tolosa Munoz, A, Baghaei, F, Tengborn, L, Boehlen, F, Korte, W, Chowdary, P, Collins, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J., and Other departments
Subjects: Registrie, Male, Clinical Trials and Observations, medicine.medical_treatment, THERAPY, Biochemistry, Gastroenterology, Immunosuppressive Agent, Antibodies, Monoclonal, Murine-Derived, Adult, Aged, Aged, 80 and over, Autoantibodies, Cyclophosphamide, Cyclosporine, Europe, Factor VIII, Female, Follow-Up Studies, Hemophilia A, Humans, Immunosuppressive Agents, Middle Aged, Registries, Rituximab, Secondary Prevention, Steroids, Treatment Outcome, FACTOR-VIII INHIBITORS, Monoclonal, 80 and over, UNITED-KINGDOM, Hematology, RECLASSIFICATION, Immunosuppression, Autoantibodie, Human, medicine.drug, Murine-Derived, medicine.medical_specialty, Immunology, DIAGNOSIS, Antibodies, Follow-Up Studie, Internal medicine, MANAGEMENT, medicine, Steroid, Autoimmune disease, business.industry, Settore MED/09 - MEDICINA INTERNA, Autoantibody, Cell Biology, Odds ratio, CENTER DOCTORS ORGANIZATION, medicine.disease, Surgery, DISCRIMINATION, Etiology, business
Abstract: Acquired hemophilia A (AHA) is an autoimmune disease caused by an autoantibody to factor VIII. Patients are at risk of severe and fatal hemorrhage until the inhibitor is eradicated, and guidelines recommend immunosuppression as soon as the diagnosis has been made. The optimal immunosuppressive regimen is unclear; therefore, data from 331 patients entered into the prospective EACH2 registry were analyzed. Steroids combined with cyclophosphamide resulted in more stable complete remission (70%), defined as inhibitor undetectable, factor VIII more than 70 IU/dL and immunosuppression stopped, than steroids alone (48%) or rituximab-based regimens (59%). Propensity score-matched analysis controlling for age, sex, factor VIII level, inhibitor titer, and underlying etiology confirmed that stable remission was more likely with steroids and cyclophosphamide than steroids alone (odds ratio = 3.25; 95% CI, 1.51-6.96; P < .003). The median time to complete remission was approximately 5 weeks for steroids with or without cyclophosphamide; rituximab-based regimens required approximately twice as long. Immunoglobulin administration did not improve outcome. Second-line therapy was successful in approximately 60% of cases that failed first-line therapy. Outcome was not affected by the choice of first-line therapy. The likelihood of achieving stable remission was not affected by underlying etiology but was influenced by the presenting inhibitor titer and FVIII level.
Published: 2012

11. Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry

Author: Baudo, Francesco, Collins, Peter, Huth Kühne, Angela, Lévesque, Hervé, Marco, Pascual, Nemes, László, Pellegrini, Fabio, Tengborn, Lilian, Knoebl, Paul, Group Author: Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, Jy, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lévesque, H, Lifermann, F, Marlu, R, Ninet, J, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Huth Kühne, A, Loreth, Rm, Steigerwald, U, Tiede, A, Theodossiades, G, Nemes, L, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Baudo, F, Caimi, T, Contino, L, D'Angelo Armando, Cl, Fattorini, A, Cerbone, Am, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, Mg, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Mancuso, Me, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Valeri, F, Scortechini, Ar, Siragusa, S, Sottilotta, G, Squizzato, A, Tagariello, G, Sartori, R, Tagliaferri, Ar, Di Perna, C, Rivolta, Gf, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Brandolin, B, Hamulyák, K, Kamphuisen, P, Laros van Gorkom, B, Leebeek, Fw, Marten, N, Novakova, I, Schutgens, R, van der Linden, Pw, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, Mj, Jiménez Yuste, V, Jose Felix, L, Marco, P, Mingot, Me, Perez Garrido, R, Perez Gonzale, Nz, Prieto Garcia, M, Rodriguez Huerta, Am, Sedano, C, Tolosa Munoz, A, Baghaei, F, Tengborn, L, Boehlen, F, Korte, W, Chowdary, P, Collins, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J., DI MINNO, GIOVANNI, DI MINNO, MATTEO, Other departments, Baudo, Francesco, Collins, Peter, Huth Kühne, Angela, Lévesque, Hervé, Marco, Pascual, Nemes, László, Pellegrini, Fabio, Tengborn, Lilian, Knoebl, Paul, Group Author: Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, Jy, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lévesque, H, Lifermann, F, Marlu, R, Ninet, J, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Huth Kühne, A, Loreth, Rm, Steigerwald, U, Tiede, A, Theodossiades, G, Nemes, L, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Baudo, F, Caimi, T, Contino, L, D'Angelo Armando, Cl, Fattorini, A, DI MINNO, Giovanni, Cerbone, Am, DI MINNO, Matteo, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, Mg, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Mancuso, Me, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Valeri, F, Scortechini, Ar, Siragusa, S, Sottilotta, G, Squizzato, A, Tagariello, G, Sartori, R, Tagliaferri, Ar, Di Perna, C, Rivolta, Gf, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Brandolin, B, Hamulyák, K, Kamphuisen, P, Laros van Gorkom, B, Leebeek, Fw, Marten, N, Novakova, I, Schutgens, R, van der Linden, Pw, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, Mj, Jiménez Yuste, V, Jose Felix, L, Marco, P, Mingot, Me, Perez Garrido, R, Perez Gonzale, Nz, Prieto Garcia, M, Rodriguez Huerta, Am, Sedano, C, Tolosa Munoz, A, Baghaei, F, Tengborn, L, Boehlen, F, Korte, W, Chowdary, P, Collins, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J., Faculteit Medische Wetenschappen/UMCG, Cardiovascular Centre (CVC), Vascular Ageing Programme (VAP), Huth Kühne, A, Lévesque, H, Pellegrini, F, Knoebl, P, Aspoeck, G, Borg, J, Loreth, R, D'Angelo Armando, C, Di Minno, G, Cerbone, A, Di Minno, D, Mazzucconi, M, Mancuso, M, Scortechini, A, Tagliaferri, A, Rivolta, G, Leebeek, F, van der Linden, P, Gutiérrez Pimentel, M, Mingot, M, Perez Gonzale, N, Rodriguez Huerta, A, and Wilde, J
Subjects: Registrie, Male, SURGERY, Biochemistry, THERAPY, Hemostatics, Hemostatic, FACTOR-VIII INHIBITORS, 80 and over, Deamino Arginine Vasopressin, Registries, Desmopressin, UNITED-KINGDOM, Factor IX, Aged, 80 and over, Hematology, biology, Incidence, FEIBA, Recombinant Protein, Middle Aged, Blood Coagulation Factors, Recombinant Proteins, Europe, Treatment Outcome, Coagulation, Female, medicine.drug, Blood Coagulation Factor, Human, Adult, medicine.medical_specialty, Adolescent, Immunology, Aged, Factor VIII, Factor VIIa, Hemophilia A, Hemorrhage, Humans, Young Adult, DIAGNOSIS, Internal medicine, BYPASSING ACTIVITY, medicine, business.industry, Settore MED/09 - MEDICINA INTERNA, RECOMBINANT FACTOR VIIA, Retrospective cohort study, Cell Biology, FACTOR-IX, CENTER DOCTORS ORGANIZATION, Surgery, Recombinant factor VIIa, Propensity score matching, biology.protein, business
Abstract: Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation FVIII. Bleeding episodes at presentation are spontaneous and severe in most cases. Optimal hemostatic therapy is controversial, and available data are from observational and retrospective studies only. The EACH2 registry, a multicenter, pan-European, Web-based database, reports current patient management. The aim was to assess the control of first bleeding episodes treated with a bypassing agent (rFVIIa or aPCC), FVIII, or DDAVP among 501 registered patients. Of 482 patients with one or more bleeding episodes, 144 (30%) received no treatment for bleeding; 31 were treated with symptomatic therapy only. Among 307 patients treated with a first-line hemostatic agent, 174 (56.7%) received rFVIIa, 63 (20.5%) aPCC, 56 (18.2%) FVIII, and 14 (4.6%) DDAVP. Bleeding was controlled in 269 of 338 (79.6%) patients treated with a first-line hemostatic agent or ancillary therapy alone. Propensity score matching was applied to allow unbiased comparison between treatment groups. Bleeding control was significantly higher in patients treated with bypassing agents versus FVIII/DDAVP (93.3% vs 68.3%; P = .003). Bleeding control was similar between rFVIIa and aPCC (93.0%; P = 1). Thrombotic events were reported in 3.6% of treated patients with a similar incidence between rFVIIa (2.9%) and aPCC (4.8%).
Published: 2012

12. Invasive procedures and minor surgery in factor VII deficiency

Author: Mariani,G, Dolce, A, Ingerslev, J, Giansily Blaizot, M, DI Minno, MD, Auerswald, G, DE Saez, AR, Tagliaferri, A, Batorova, A., NAPOLITANO, Mariasanta, Mariani, Guglielmo, Dolce, A., Napolitano, M., Ingerslev, J., Giansily Blaizot, M., DI MINNO, Matteo, Auerswald, G., De Saez, A. R., Tagliaferri, A., Batorova, A., Mariani,G, Dolce, A, Napolitano, M, Ingerslev, J, Giansily-Blaizot, M, DI Minno, MD, Auerswald, G, DE Saez, AR, Tagliaferri, A, and Batorova, A
Subjects: Adult, Male, MINOR SURGERY, INVASIVE PROCEDURES, FACTOR VII DEFICIENCY, Adolescent, Factor VII Deficiency, Blood Loss, Surgical, Infant, Hemorrhage, Hematology, Factor VII, Hemostasi, Middle Aged, Settore MED/15 - Malattie Del Sangue, Minor Surgical Procedure, Young Adult, Coagulant, Child, Preschool, Surgical Procedures, Operative, Female, Child, Genetics (clinical), Aged, Human
Published: 2012

13. Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry

Author: Tengborn L, Baudo F, Huth-Kühne A, Knoebl P, Lévesque H, Marco P, Pellegrini F, Nemes L, Collins P, EACH2 registry contributors, Aspoeck G, Heistinger M, Knöbl P, Makipernaa A, André H, Aouba A, Bellucci S, Beurrier P, Borg JY, Darnige L, Devignes J, d'Oiron R, Gautier P, Gay V, Girault S, Gruel Y, Guerin V, Hézard N, Khellaf M, Koenig M, Lifermann F, Marlu R, Ninet J, Peynet J, Quéméneur T, Rothschild C, Schleinitz N, Sigaud M, Trouillier S, Voisin S, Giebl A, Holstein K, Loreth RM, Steigerwald U, Tiede A, Theodossiades G, Radvanyi G, Schlammadinger A, Barillari G, Pasca S, Caimi T, Contino L, D'Angelo Armando CL, Fattorini A, Di Minno G, Cerbone AM, Di Minno D, D'incà M, Falanga A, Maggioni A, Lerede T, Franchini M, Gaidano G, De Paoli L, Gamba G, Ghirardi R, Girotto M, Tasca D, Grandone E, Tiscia G, Imberti D, Iorio A, Landolfi R, Di Gennaro L, Novarese L, Mariani G, Lapecorella M, Marietta M, Pedrazzi P, Mazzucconi MG, Santoro C, Morfini M, Linari S, Moratelli S, Paolini R, Piseddu G, Poggio R, Pogliani E, Carpenedo M, Remiddi C, Santagostino E, Mancuso ME, Santoro R, Papaleo G, Schinco P, Borchiellini A, Valeri F, Scortechini AR, Siragusa S, Sottilotta G, Squizzato A, Tagariello G, Sartori R, Tagliaferri AR, Di Perna C, Rivolta GF, Testa S, Paoletti O, Toschi V, Zanon E, Hamulyák K, Kamphuisen P, Laros-van Gorkom B, Leebeek FW, Marten N, Novakova I, Schutgens R, van der Linden PW, van Esser J, van der Meer J, Ypma P, Campos M, Aguilar C, Altisent C, Bermejo N, Del Campo R, Ferreiro Argüelles M, González Boullosa R, Gutiérrez Pimentel MJ, Jiménez-Yuste V, Jose-Felix L, Mingot ME, Perez Garrido R, Perez Gonzale NZ, Prieto Garcia M, Rodriguez-Huerta AM, Sedano C, Tolosa Munoz A, Baghaei F, Boehlen F, Korte W, Chowdary P, Evans G, Pavord S, Rangarajan S, Wilde J, Tengborn, L, Baudo, F, Huth-Kühne, A, Knoebl, P, Lévesque, H, Marco, P, Pellegrini, F, Nemes, L, Collins, P, EACH2 registry, C, Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, J, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lifermann, F, Marlu, R, Ninet, J, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Loreth, R, Steigerwald, U, Tiede, A, Theodossiades, G, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Caimi, T, Contino, L, D'Angelo Armando, C, Fattorini, A, Di Minno, G, Cerbone, A, Di Minno, D, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, M, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Mancuso, M, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Valeri, F, Scortechini, A, Siragusa, S, Sottilotta, G, Squizzato, A, Tagariello, G, Sartori, R, Tagliaferri, A, Di Perna, C, Rivolta, G, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Hamulyák, K, Kamphuisen, P, Laros-van Gorkom, B, Leebeek, F, Marten, N, Novakova, I, Schutgens, R, van der Linden, P, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, M, Jiménez-Yuste, V, Jose-Felix, L, Mingot, M, Perez Garrido, R, Perez Gonzale, N, Prieto Garcia, M, Rodriguez-Huerta, A, Sedano, C, Tolosa Munoz, A, Baghaei, F, Boehlen, F, Korte, W, Chowdary, P, Evans, G, Pavord, S, Rangarajan, S, and Wilde, J
Subjects: Adult, Acquired haemophilia A, diagnosis, pregnancy, treatment, Antifibrinolytic Agents, Blood Coagulation Factors, Drug Therapy, Combination, Europe, Factor VIIa, Female, Follow-Up Studies, Hemostatics, Humans, Immunosuppressive Agents, Kaplan-Meier Estimate, Pregnancy, Prospective Studies, Recombinant Proteins, Registries, Treatment Outcome, Hemophilia A, Pregnancy Complications, Hematologic, Drug Therapy, Hematologic, Acquired Haemophilia, Pregnancy, Pregnancy Complications, Combination
Abstract: Objective The European Acquired Haemophilia registry (EACH2) collected data on the demographics, diagnosis, underlying disorders, bleeding characteristics, treatment, and outcome of women with acquired haemophilia A (AHA), a rare and often severe bleeding disorder caused by autoantibodies directed against coagulation factor VIII. Design Prospective, multi-centre, large-scale, pan-European registry. Setting A total of 117 haemophilia centres in 13 European countries. Population Pregnancy-associated AHA. Methods Data were reported using a web-based electronic case report form. Diagnosis was based on the presence of a prolonged activated partial thromboplastin time, reduced coagulation Factor VIII level and positive inhibitor assay. Main outcome measures Presenting characteristics, time to diagnosis, haemostatic treatment and outcome, immunosuppressive treatment and outcome. Results The EACH2 registry (n = 501) documented 42 (8.4%) cases of AHA associated with the peripartum period, a median Factor VIII level at diagnosis of 2.5 (range 0-25) IU/dl and inhibitor titre of 7.8 (range 0.7-348) BU/ml. Antepartum inhibitors were evident in eight women. Time to diagnosis of AHA after delivery was 89 (range 21-120) days. First-line haemostatic treatment was successful in 20/23 (87%) women treated. Bleeding episodes resolved in 17/18 (94%) women treated with a bypassing agent and 29/39 (74%) women achieved complete remission with first-line immunosuppressive treatment. Two babies experienced postnatal bleeding, suggesting transplacental transfer of the antibody. All women were alive at last follow-up. Conclusions Although rare, pregnancy-associated AHA may cause severe bleeding-related morbidity. Once diagnosed, women respond well to haemostatic treatment with bypassing agents and immunosuppression. Awareness of peripartum AHA requires improvement to facilitate rapid and appropriate management.
Published: 2012

14. Pregnancy-associated acquired haemophilia A: Results from the European Acquired Haemophilia (EACH2) registry

Author: Tengborn, L, Baudo, F., Huth Kühne, A., Knoebl, P., Lévesque, H., Marco, P., Pellegrini, F., Nemes, L., Collins, P, Group Author: Aspoeck G, Heistinger M, Knöbl P, Makipernaa A, André H, Aouba A, Bellucci S, Beurrier P, Borg JY, Darnige L, Devignes J, d'Oiron R, Gautier P, Gay V, Girault S, Gruel Y, Guerin V, Hézard N, Khellaf M, Koenig M, Lévesque H, Lifermann F, Marlu R, Ninet J, Peynet J, Quéméneur T, Rothschild C, Schleinitz N, Sigaud M, Trouillier S, Voisin S, Giebl A, Holstein K, Huth Kühne A, Loreth RM, Steigerwald U, Tiede A, Theodossiades G, Nemes L, Radvanyi G, Schlammadinger A, Barillari G, Pasca S, Baudo F, Caimi T, Contino L, D'Angelo Armando CL, Fattorini A, Cerbone AM, D'incà M, Falanga A, Maggioni A, Lerede T, Franchini M, Gaidano G, De Paoli L, Gamba G, Ghirardi R, Girotto M, Tasca D, Grandone E, Tiscia G, Imberti D, Iorio A, Landolfi R, Di Gennaro L, Novarese L, Mariani G, Lapecorella M, Marietta M, Pedrazzi P, Mazzucconi MG, Santoro C, Morfini M, Linari S, Moratelli S, Paolini R, Piseddu G, Poggio R, Pogliani E, Carpenedo M, Remiddi C, Santagostino E, Mancuso ME, Santoro R, Papaleo G, Schinco P, Borchiellini A, Valeri F, Scortechini AR, Siragusa S, Sottilotta G, Squizzato A, Tagariello G, Sartori R, Tagliaferri AR, Di Perna C, Rivolta GF, Testa S, Paoletti O, Toschi V, Zanon E, Hamulyák K, Kamphuisen P, Laros van Gorkom B, Leebeek FW, Marten N, Novakova I, Schutgens R, van der Linden PW, van Esser J, van der Meer J, Ypma P, Campos M, Aguilar C, Altisent C, Bermejo N, Del Campo R, Ferreiro Argüelles M, González Boullosa R, Gutiérrez Pimentel MJ, Jiménez Yuste V, Jose Felix L, Marco P, Mingot ME, Perez Garrido R, Perez Gonzale NZ, Prieto Garcia M, Rodriguez Huerta AM, Sedano C, Tolosa Munoz A, Baghaei F, Tengborn L, Boehlen F, Korte W, Chowdary P, Collins P, Evans G, Pavord S, Rangarajan S, Wilde J., DI MINNO, GIOVANNI, DI MINNO, MATTEO, Tengborn, L, Baudo, F., Huth Kühne, A., Knoebl, P., Lévesque, H., Marco, P., Pellegrini, F., Nemes, L., Collins, P, Group Author: Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, Jy, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lévesque, H, Lifermann, F, Marlu, R, Ninet, J, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Huth Kühne, A, Loreth, Rm, Steigerwald, U, Tiede, A, Theodossiades, G, Nemes, L, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Baudo, F, Caimi, T, Contino, L, D'Angelo Armando, Cl, Fattorini, A, DI MINNO, Giovanni, Cerbone, Am, DI MINNO, Matteo, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, Mg, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Mancuso, Me, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Valeri, F, Scortechini, Ar, Siragusa, S, Sottilotta, G, Squizzato, A, Tagariello, G, Sartori, R, Tagliaferri, Ar, Di Perna, C, Rivolta, Gf, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Hamulyák, K, Kamphuisen, P, Laros van Gorkom, B, Leebeek, Fw, Marten, N, Novakova, I, Schutgens, R, van der Linden, Pw, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, Mj, Jiménez Yuste, V, Jose Felix, L, Marco, P, Mingot, Me, Perez Garrido, R, Perez Gonzale, Nz, Prieto Garcia, M, Rodriguez Huerta, Am, Sedano, C, Tolosa Munoz, A, Baghaei, F, Boehlen, F, Korte, W, Chowdary, P, Evans, G, Pavord, S, Rangarajan, S, and Wilde, J.
Subjects: Adult, Registrie, treatment, Pregnancy Complications, Hematologic, Obstetrics and Gynecology, Factor VIIa, Kaplan-Meier Estimate, Recombinant Protein, Antifibrinolytic Agent, Hemophilia A, Follow-Up Studie, Europe, diagnosi, Immunosuppressive Agent, Prospective Studie, Hemostatic, Treatment Outcome, Pregnancy, Drug Therapy, Combination, Female, Acquired haemophilia A, Blood Coagulation Factor, Human
Published: 2012

15. Consumption of clotting factors in severe haemophilia patients undergoing prophylaxis and on-demand treatment in Italy

Author: Giampaolo A, Abbonizio F, Puopolo M, Arcieri R, Mannucci PM, Hassan HJ, Accorsi A, Ettorre PC, Schiavoni M, Palareti G, Rodorigo G, Valdré L, Amaddii G, Billio A, Notarangelo L, Iannacaro P, Muleo P, Biasioli C, Rossi V, Alatri A, Testa S, Vincenzi D, Scapoli G, Morfini M, Molinari AC, Lapecorella M, Mariani G, Baudo F, Caimi MT, Federici AB, Gringeri A, Santagostino E, Marietta M, Coppola A, Perricone C, Schiavulli M, Miraglia E, Rocino A, Zanon E, Gagliano F, Mancuso G, Siragusa S, Rivolta F, Tagliaferri A, Gamba G, Iorio A, Oliovecchio E, Dragani A, Arbasi MC, Albertini P, Mancino A, Lombardo VT, Latella C, D'Incà M, Landolfi Raffaele, Biondo Francesca, Mazzucconi MG, Santoro Cristina, Mameli AL, Piseddu G, Schinco PC, Messina M, Rossetti G, Barillari G, Feola G, Franchini M, Gandini G, Castaman G, Rodeghiero F, Italian Association of Haemophilia Centres, DI MINNO, GIOVANNI, Giampaolo, A, Abbonizio, F, Puopolo, M, Arcieri, R, Mannucci, Pm, Hassan, Hj, Accorsi, A, Ettorre, Pc, Schiavoni, M, Palareti, G, Rodorigo, G, Valdré, L, Amaddii, G, Billio, A, Notarangelo, L, Iannacaro, P, Muleo, P, Biasioli, C, Rossi, V, Alatri, A, Testa, S, Vincenzi, D, Scapoli, G, Morfini, M, Molinari, Ac, Lapecorella, M, Mariani, G, Baudo, F, Caimi, Mt, Federici, Ab, Gringeri, A, Santagostino, E, Marietta, M, Coppola, A, DI MINNO, Giovanni, Perricone, C, Schiavulli, M, Miraglia, E, Rocino, A, Zanon, E, Gagliano, F, Mancuso, G, Siragusa, S, Rivolta, F, Tagliaferri, A, Gamba, G, Iorio, A, Oliovecchio, E, Dragani, A, Arbasi, Mc, Albertini, P, Mancino, A, Lombardo, Vt, Latella, C, D'Incà, M, Landolfi, Raffaele, Biondo, Francesca, Mazzucconi, Mg, Santoro, Cristina, Mameli, Al, Piseddu, G, Schinco, Pc, Messina, M, Rossetti, G, Barillari, G, Feola, G, Franchini, M, Gandini, G, Castaman, G, Rodeghiero, F, and Italian Association of Haemophilia, Centres
Subjects: Clotting factor, Male, Pediatrics, medicine.medical_specialty, Factor VIII, business.industry, Haemophilia A, Hematology, Disease, medicine.disease, Haemophilia, Hemophilia A, Hemophilia B, Factor IX, On demand treatment, Coagulation, Italy, Surveys and Questionnaires, medicine, Humans, Female, National registry, business, Coagulation Disorder
Abstract: Dear Sir, Treatment of haemophilia is costly, and it must be carefully monitored by physicians and health authorities, so as to improve the planning of the supply of factor concentrates obtained from the fractionation of human plasma or by recombinant technology. Therapy with coagulation factors can be used either prophylactically to prevent bleeding, which entails multiple weekly infusions, or on-demand, through infusion of the amount of factors determined by the severity of the disease and patient’s weight, to stop ongoing bleeding. According to a recent study, factor VIII (FVIII) consumption among persons with haemophilia A (HA) has increased in countries with high-income economies (Stonebraker et al., 2010). In Italy, haemophiliacs and other persons with congenital coagulation disorders are monitored by the National Registry of Congenital Coagulopathies (NRCC) established at the Istituto Superi=
Published: 2011

16. Thrombosis in inherited factor VII deficiency

Author: Mariani, G, Herrmann, Fh, Schulman, S, Batorova, A, Wulff, K, Etro, Daniela, Dolce, A, Auerswald, G, Astermark, J, Schved, Jf, Ingerslev, J, Bernardi, Francesco, and INTERNATIONAL FACTOR VII DEFICIENCY STUDY GROUP
Subjects: Adult, Male, Heterozygote, medicine.medical_specialty, Pathology, Time Factors, Adolescent, Factor VII Deficiency, Gene mutation, Compound heterozygosity, Thrombophilia, Gastroenterology, Internal medicine, medicine, Humans, Aged, Venous Thrombosis, Disseminated intravascular coagulation, biology, business.industry, Homozygote, Factor V, Thrombosis, Hematology, Congenital FVII deficiency, Replacement therapy, Surgery, Disseminated Intravascular Coagulation, Middle Aged, medicine.disease, Zygosity, Venous thrombosis, Phenotype, Databases as Topic, Factor Xa, Mutation, biology.protein, Female, Prothrombin, business
Abstract: Thrombosis in congenital factor (F) VII deficiency was investigated through extensive phenotypic and molecular-genetic studies. Patients with a history of thrombosis among 514 entries in the FVII Deficiency Study Group database were evaluated. Thrombotic events were arterial in one case, disseminated intravascular coagulation in another and venous in seven. Gene mutations were characterized in eight patients: three were homozygous, three compound heterozygous and two heterozygous. FXa and IIa generation assays were consistent with the genetic lesions. One patient was heterozygous for the FV Leiden and one for the FIIG20210A mutation. In seven patients, surgical interventions and/or replacement therapies had a close temporal relationship with thrombosis, while in the remaining, events were apparently spontaneous. Thromboses were not associated with any specific age, phenotype, mutation zygosity or thrombophilic abnormalities. In particular, severe FVII deficiency did not seem to offer protection from strong thrombosis risk factors such as surgery and replacement therapy.
Published: 2003
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17. Evidence-based recommendations on the treatment of von Willebrand disease in Italy

Author: Mannucci, Pm, Franchini, M, Castaman, G, Federici, Ab, Contino, L, Accorsi, A, Ciavarella, N, Schiavoni, M, Scaraggi, Fa, Rodorigo, G, Valdre, L, Targhetta, R, Tagariello, G, Radossi, P, Musso, R, Cultrera, D, Muleo, G, Iannacaro, P, Biasioli, C, Testa, S, Alatri, A, Vincenzi, D, Scapoli, G, Morfini, M, Molinari, Ac, Boeri, E, Caprino, D, Delios, G, Girotto, M, Mariani, G, Lapecorella, M, Carloni, Mt, Cantori, I, Santagostino, E, Gringeri, A, Marietta, M, Pedrazzoli, P, Di Minno, G, Coppola, A, Perricone, C, Schiavulli, M, Rocino, A, Berrettini, M, Zanon, E, Mancuso, G, Siragusa, S, Malato, A, Saccullo, G, Tagliaferri, A, Rivolta, F, Iorio, A, Oliovecchio, E, Ferrante, F, Dragani, A, Rossi, A, Mancino, A, Albertini, P, Macchi, S, D'Incà, M, De Rossi, G, Luciani, M, Landolfi, R, Mazzucconi, Maria Gabriella, Santoro, Cristina, Piseddu, G, Schinco, Pc, Rossetti, G, Barillari, G, Feola, G, Gandini, G., Mannucci, Pm, Franchini, M, Castaman, G, Federici, Ab, Contino, L, Accorsi, A, Ciavarella, N, Schiavoni, M, Scaraggi, Fa, Rodorigo, G, Valdre, L, Targhetta, R, Tagariello, G, Radossi, P, Musso, R, Cultrera, D, Muleo, G, Iannacaro, P, Biasioli, C, Testa, S, Alatri, A, Vincenzi, D, Scapoli, G, Morfini, M, Molinari, Ac, Boeri, E, Caprino, D, Delios, G, Girotto, M, Mariani, G, Lapecorella, M, Carloni, Mt, Cantori, I, Santagostino, E, Gringeri, A, Marietta, M, Pedrazzoli, P, DI MINNO, Giovanni, Coppola, A, Perricone, C, Schiavulli, M, Rocino, A, Berrettini, M, Zanon, E, Mancuso, G, Siragusa, S, Malato, A, Saccullo, G, Tagliaferri, A, Rivolta, F, Iorio, A, Oliovecchio, E, Ferrante, F, Dragani, A, Rossi, A, Mancino, A, Albertini, P, Macchi, S, D'Incà, M, De Rossi, G, Luciani, M, Landolfi, R, Mazzucconi, Mg, Santoro, C, Piseddu, G, Schinco, Pc, Rossetti, G, Barillari, G, Feola, G, and Gandini, G.
Subjects: Male, desmopressin, Evidence-Based Medicine, Factor VIII, Hemostatics, von willebrand disease, von Willebrand Diseases, von willebrand factor, Treatment Outcome, Italy, von Willebrand Factor, Humans, Deamino Arginine Vasopressin, Drug Therapy, Combination, Female, Original Article
Abstract: von Willebrand disease (VWD) is the most common hereditary bleeding disorder affecting both males and females. It arises from quantitative or qualitative defects of von Willebrand factor (VWF) and causes bleeding of mucous membranes and soft tissues. The aim of treatment is to correct the dual defect of haemostasis caused by the abnormal/reduced VWF and the concomitant deficiency of factor VIII (FVIII).This document contains evidence-based recommendations for the management of VWD compiled by AICE (the Italian Association of Haemophilia Centres). All the evidence supporting these recommendations are based on non-randomised comparative studies or case series, because randomised controlled clinical trials or meta-analyses are not available for this disease.Desmopressin (DDAVP) is the treatment of choice for patients with type 1 VWD with FVIII and VWF levels of 10 U/dL or more, while VWF/FVIII concentrates are indicated for those who are unresponsive or insufficiently responsive to DDAVP (severe type 1, type 2 and 3 VWD). VWF concentrates devoid of FVIII, not yet licensed in Italy, may be considered for short-term prophylaxis in elective surgery or for long-term secondary prophylaxis.
Published: 2009

18. Sentinel lymph node mapping in breast cancer: a critical reappraisal of the internal mammary chain issue

Author: Manca, G., DUCCIO VOLTERRANI, Mazzarri, S., Duce, V., Svirydenka, A., Giuliano, A., and Mariani, G.
Subjects: Tomography, Emission-Computed, Single-Photon, Image-Guided Biopsy, Male, Evidence-Based Medicine, Sentinel Lymph Node Biopsy, Carcinoma, Reproducibility of Results, Breast Neoplasms, Breast, Female, Humans, Lymph Nodes, Lymphatic Metastasis, Multimodal Imaging, Sensitivity and Specificity, Tomography, X-Ray Computed, X-Ray Computed, Emission-Computed, Tomography, Single-Photon
Abstract: Although, like the axilla, the internal mammary nodes (IMNs) are a first-echelon nodal drainage site in breast cancer, the importance of their treatment has long been debated. Seminal randomized trials have failed to demonstrate a survival benefit from surgical IMN dissection, and several retrospective studies have shown that IMNs are rarely the first site of recurrence. However, the recent widespread adoption of sentinel lymph node (SLN) biopsy has stimulated a critical reappraisal of such early results. Furthermore, the higher proportion of screening-detected cancers, improved imaging and techniques (i.e., lymphoscintigraphy for radioguided SLN biopsy) make it possible to visualize lymphatic drainage to the IMNs. The virtually systematic application of adjuvant systemic and/or loco-regional radiotherapy encourages re-examination of the significance of IMN metastases. Moreover, randomized trials testing the value of postmastectomy irradiation and a meta-analysis of 78 randomized trials have provided high levels of evidence that local-regional tumor control is associated with long-term survival improvements. This benefit was limited to trials that used systemic chemotherapy, which was not routinely administered in the earlier studies. However, the contribution from IMN treatment is unclear. Lymphoscintigraphic studies have shown that a significant proportion of breast cancers have primary drainage to the IMNs, including approximately 30% of medial tumors and 15% of lateral tumors. In the few studies where IMN biopsy was performed, 20% of sentinel IMNs were metastatic. The risk of IMN involvement is higher in patients with medial tumors and positive axillary nodes. IMN metastasis has prognostic significance, as recognized by its inclusion in the American Joint Committee on Cancer staging criteria, and seems to have similar prognostic importance as axillary nodal involvement. Although routine IMN evaluation might be indicated, it has not been routinely performed, perhaps because IMN drainage with lymphoscintigraphy is more difficult to demonstrate than axillary drainage. This difference is due to technical reasons and not the absence of lymphatics to the IMN. Recent anatomical studies have confirmed a model of breast lymphatic drainage that comprises superficial, deep and perforating systems. The superficial system drains to the axilla, usually to a lymph node posterior to the pectoralis minor muscle. The deep system drains to the axilla and also anastomoses with the perforating system which drains to the IMNs. The perforating system does not connect with the superficial system. The prevalence of IMN drainage tends to reflect the method of lymphoscintigraphy, where peritumoral (deep lymphatic system) injections have a much higher likelihood of IMN drainage than subareolar or subdermal (superficial lymphatic system) injections. The fused SPECT/CT images represent a further technical solution to increase the identification of IMNs and consequently can significantly reduce the false negative rate of sentinel lymph node biopsy. Before mature results from current and future randomized trials assessing the benefit of IMN irradiation become available, lymphoscintigraphy and IMNs biopsy may be used to guide decisions regarding systemic and local-regional treatment. However, even in patients with visualized primary IMN drainage, the potential benefit of treatment should be balanced against the risk of added morbidity.
Published: 2014

19. Italian Registry of Haemophilia and Allied Disorders. Objectives, methodology and data analysis

Author: Iorio, A, Oliovecchio, E, Morfini, M, Mannucci, Pm, Contino, L, Accorsi, A, Ciavarella, N, Schiavoni, M, Scaraggi, Fa, Rodorigo, G, Valdré, L, Targhetta, R, Tagariello, G, Radossi, P, Musso, R, Cultrera, D, Muleo, G, Iannacaro, P, Biasioli, C, Testa, S, Alatri, A, Vincenzi, D, Scapoli, G, Molinari, Ac, Boeri, E, Caprino, D, Mariani, G, Lapecorella, M, Carloni, Mt, Cantori, I, Santagostino, E, Gringeri, A, Federici, Ab, Marietta, M, Pedrazzoli, P, Di Minno, G, Coppola, A, Perricone, C, Schiavulli, M, Rocino, A, Berrettini, M, Zanon, E, Mancuso, G, Siragusa, S, Malato, A, Saccullo, G, Tagliaferri, A, Rivolta, F, Ferrante, F, Dragani, A, Rossi, A, Mancino, A, Albertini, P, Macchi, S, Hassan, J, D'Inca, M, De Rossi, G, Luciani, M, Landolfi, R, Mazzucconi, Maria Gabriella, Santoro, Cristina, Piseddu, G, Carla Schinco, P, Rossetti, G, Barillari, G, Feola, G, Gandini, G, Franchini, M, Castaman, G., Iorio, A, Oliovecchio, E, Morfini, M, Mannucci, Pm, Contino, L, Accorsi, A, Ciavarella, N, Schiavoni, M, Scaraggi, Fa, Rodorigo, G, Valdré, L, Targhetta, R, Tagariello, G, Radossi, P, Musso, R, Cultrera, D, Muleo, G, Iannacaro, P, Biasioli, C, Testa, S, Alatri, A, Vincenzi, D, Scapoli, G, Molinari, Ac, Boeri, E, Caprino, D, Mariani, G, Lapecorella, M, Carloni, Mt, Cantori, I, Santagostino, E, Gringeri, A, Federici, Ab, Marietta, M, Pedrazzoli, P, DI MINNO, Giovanni, Coppola, A, Perricone, C, Schiavulli, M, Rocino, A, Berrettini, M, Zanon, E, Mancuso, G, Siragusa, S, Malato, A, Saccullo, G, Tagliaferri, A, Rivolta, F, Ferrante, F, Dragani, A, Rossi, A, Mancino, A, Albertini, P, Macchi, S, Hassan, J, D'Inca, M, De Rossi, G, Luciani, M, Landolfi, R, Mazzucconi, Mg, Santoro, C, Piseddu, G, Carla Schinco, P, Rossetti, G, Barillari, G, Feola, G, Gandini, G, Franchini, M, and Castaman, G.
Subjects: Adult, Male, Pediatrics, medicine.medical_specialty, Adolescent, Haemophilia A, MEDLINE, Haemophilia, Severity of Illness Index, haemophilia, registry, von willebrand's disease, von willebrand’s disease, Blood Coagulation Disorders, Inherited, Von willebrand, Severity of illness, Health care, Computer software, medicine, Humans, Haemophilia B, Registries, Age of Onset, Child, Genetics (clinical), Societies, Medical, Aged, business.industry, Infant, Hematology, General Medicine, Middle Aged, medicine.disease, Databases as Topic, Italy, Child, Preschool, Data Interpretation, Statistical, Female, Medical emergency, business
Abstract: National haemophilia registries are powerful instruments to support health care and research. A national registry was established in Italy by the Ministry of Health until 1999. Since 2003 the Italian Association of Haemophilia Centres (AICE) started a new programme aiming at building up the Italian Registry of Haemophilia and Allied Disorders. The AICE identified an expert panel to steer the registry. A computer software to assist patient management was developed and all the AICE-affiliated haemophilia treatment centres (HTC) were prompted to adopt it. Twice a year a predefined set of anonymized data is centralized and merged into a national database. Duplicated entries are managed through a confidentiality sparing mechanism. The database covers sociodemographic, clinical, laboratory and treatment data. A subset of data are shared with the Ministry of Health (Istituto Superiore di Sanita,ISS).Overall, data were collected six times by 43 of 49 HTC; 41 centres updated their patients' records up to December 2006. The database contains 6632 unique records, 442 of them referring to dead patients. Database growth and missing data clearance showed a constantly positive trend over time. The database has collected records of the following alive patients - haemophilia A: 1364 severe, 398 moderate and 935 mild; haemophilia B: 231 severe, 138 moderate and 204 mild; von Willebrand's disease: 1208 type 1, 346 type 2 and 96 type 3. Inhibitor patients were 296 (of which 194 high responders and 65 low responders).The Italian registry run by AICE adds to the list of the available national haemophilia registries and is intended to establish treatment guidelines and foster research projects in Italy.
Published: 2008

20. Hernia repair in the Lombardy region in 2000: preliminary results

Author: Ferrante, F., Rusconi, A., Galimberti, A., Grassi, M., Beluffi, L., Salamina, G., Casati, A., Cassinelli, G. B., Colpani, L., Colledan, M., Forloni, B., Geroni, P., Longoni, M., Morelli, A., Novelino, L., ENRICO OPOCHER, Pecis, C., Perrone, G., Baroni, C., Capretti, P. L., Cazzaniga, R., Giovannetti, M., Puccio, F., Marcianò, P., Sbarbada, V., Scheda Ricciotti, G., Tambussi, U., Zola, C., Formenti, A., Rumi, A., Savio, S., Tommasini Degna, C., Alquati, P., Brown, P., Gafà, M., Martinotti, A., Costa, M., Confalonieri, G. M., Crema, G., Erba, R., Frega, G., Olivari, N., Pricolo, R., Benati, L., Campo, S., Mariani, G., Bruni, T., Fontanili, M., Arisi, G. P., Beltramo, M., Bocca, M., Bragherio, G., Brambilla, A., Cacioli, D., Calzoni, D., Magnoni, E., Castoldi, M., Ceriani, V., Ballabio, R., Confalonieri, F., Contessini Avesani, E., Campanelli, G. P., Trivellini, G., Corsi, C., Croce, E., Creperio, G., Di Carlo, V., Familiari, G., Ferla, F., Ferrari, G., Forti, D., Greco, D., Fox, U., Gherardi, G., Lunghi, C., Maggioni, P., Mantovani, G., Maruotti, R., Mascia, G., Mentasti, A., Mezzeti, M., Monestiroli, U., Nespoli, A., Potestio, M., Rolandi, P., Rosati, R., Roviaro, G. C., Sampietro, R., Scorza, R., Staudacher, C., Stefanoni, G., Taschieri, A., Tenchini, P., Trabattoni, P., Trabucchi, E., Uggeri, F., Bottani, G., Cebrelli, C., Fossati, G., Ferrante, F, Rusconi, A, Galimberti, A, Grassi, M, Beluffi, L, Salamina, G, Casati, A, Cassinelli, G, Colpani, L, Colledan, M, Forloni, B, Geroni, P, Longoni, M, Morelli, A, Novelino, L, Opocher, E, Pecis, C, Perrone, G, Baroni, C, Capretti, P, Cazzaniga, R, Giovannetti, M, Puccio, F, Marciano, P, Sbarbada, V, Scheda Ricciotti, G, Tambussi, U, Zola, C, Formenti, A, Rumi, A, Savio, S, Tommasini Degna, C, Alquati, P, Brown, P, Gafa, M, Martinotti, A, Costa, M, Confalonieri, G, Crema, G, Erba, R, Frega, G, Olivari, N, Pricolo, R, Benati, L, Campo, S, Mariani, G, Bruni, T, Fontanili, M, Arisi, G, Beltramo, M, Bocca, M, Bragherio, G, Brambilla, A, Cacioli, D, Calzoni, D, Magnoni, E, Castoldi, M, Ceriani, V, Ballabio, R, Confalonieri, F, Contessini Avesani, E, Campanelli, G, Trivellini, G, Corsi, C, Croce, E, Creperio, G, Di Carlo, V, Familiari, G, Ferla, F, Ferrari, G, Forti, D, Greco, D, Fox, U, Gherardi, G, Lunghi, C, Maggioni, P, Mantovani, G, Maruotti, R, Mascia, G, Mentasti, A, Mezzeti, M, Monestiroli, U, Nespoli, A, Potestio, M, Rolandi, P, Rosati, R, Roviaro, G, Sampietro, R, Scorza, R, Staudacher, C, Stefanoni, G, Taschieri, A, Tenchini, P, Trabattoni, P, Trabucchi, E, Uggeri, F, Bottani, G, Cebrelli, C, and Fossati, G
Subjects: Male, Reoperation, medicine.medical_specialty, Hernia, medicine.medical_treatment, Risk Assessment, Severity of Illness Index, Postoperative Complications, Recurrence, Surveys and Questionnaires, medicine, Humans, Anesthesia, Herniorrhaphy, Surgical repair, Mesh, Recurrent Inguinal Hernia, Hospital stay, Laparotomy, Surgical approach, business.industry, General surgery, Surgical Mesh, medicine.disease, Hernia repair, Surgery, Treatment Outcome, Female, Follow-Up Studies, Health Care Surveys, Italy, Laparoscopy, Anterior approach, business, Abdominal surgery
Abstract: Hernia repair is the most common surgical procedure in general surgery in Italy and in the Lombardy region. In the last decade, the use of mesh, the concept of a tension-free technique, and the postoperative rate of recurrences after Bassini or Shouldice operations have completely changed the surgical approach to hernia repair. For this reason, we sent a questionnaire to 148 surgical departments in the Lombardy region to investigate about total hernia operations performed in 2000 in Lombardy, the surgical approach, the surgical techniques used, the type of anesthesia and the hospital stay. One hundred five out of 148 surgical departments returned the questionnaire, and we collected information on a total of 16,935 surgical operations for hernia: 16,494 were performed using tension-free techniques. The inguinal anterior approach is the one of choice for primary and recurrent inguinal hernia, whereas the open preperitoneal and laparoscopic approaches are limited to bilateral and recurrent hernias. The majority of cases were treated under locoregional anesthesia and with a hospital stay of two nights.
Published: 2002

21. Ramus marginalis mandibulae nervus facialis palsy in hemifacial microsomia

Author: Silvestri, A., Mariani, G., and ROBERTO ANTONIO VERNUCCI
Subjects: Male, Facial Asymmetry, Electromyography, Facial Paralysis, Facial Muscles, Humans, Female, Mandible, Organ Size, Facial Nerve Diseases, Child, Maxillofacial Development
Abstract: The paralysis of the ramus marginalis mandibulae nervus facialis may occur in Hemifacial Microsomia (HM); the combination of both HM and palsy contributes to an elongation of the mandibular body. This study explores a possible correlation between neurological deficit, muscular atony, and structural deficiency.Of 58 patients with HM who had come to the University of Rome (Sapienza) Pre-surgical Orthodontics Unit, 4 patients were afflicted with Hemifacial Microsomia and ramus marginalis mandibulae nervus palsy; these patients underwent physical, neurological, opthamologic and systemic examinations. The results were then analysed in order to determine a possible correlation between neuro-muscular and structural deficit.Electroneurographic and electromyographic examinations were performed to estimate facial nerve and muscles involvement.Neuroelectrographic exam showed a damage of the nervous motor fibres of the facial nerve ipsilateral to HM, with an associated damage of the muscular function, while neuro-muscular functions on the healthy side were normal.The peripheral nervous and muscular deficits affect the function of facial soft tissues and the growth of mandibular body with an asymmetry characterised by a hypodevelopment of the ramus (due to the HM) and by an elongation of the mandibular body (due to ramus marginalis mandibulae nerve palsy), so that the chin deviation is contralateral to HM. In these forms, a neurological examination is necessary to assess the neurological damage on the HM side. Neuromuscular deficiency can also contribute to a relapse tendency after a surgical-orthodontic treatment.
Published: 2008

22. Minimally invasive radio-guided parathyroidectomy on a group of 452 primary hyperparathyroid patients - Refinement of preoperative imaging and intraoperative procedure

Author: Rubello, D, Mariani, G, and Pelizzo, MARIA ROSA
Subjects: Adenoma, Adult, Male, Parathyroidectomy, Thyroid nodules, medicine.medical_specialty, Adolescent, medicine.medical_treatment, Radiosurgery, Scintigraphy, Monitoring, Intraoperative, Preoperative Care, Humans, Minimally Invasive Surgical Procedures, Medicine, Radiology, Nuclear Medicine and imaging, Aged, Retrospective Studies, Parathyroid adenoma, Aged, 80 and over, Hyperparathyroidism, medicine.diagnostic_test, business.industry, General Medicine, Middle Aged, medicine.disease, Radiography, Parathyroid Neoplasms, Concomitant, Female, Radiology, business, Complication, Gamma probe
Abstract: SummaryThe aim of this study was to investigate the efficacy of minimally invasive radio-guided parathyroidectomy (MIRP) in primary hyperparathyroid (PHPT) patients. Patients, methods: 452 consecutive PHPT patients were evaluated. Inclusion criteria for MIRP were (a) evidence at scintigraphy of a solitary parathyroid adenoma (PA); (b) a clear sestamibi uptake in the PA; (c) the absence of concomitant thyroid nodules; (d) no history of familial HPT or MEN; (e) no history of previous neck irradiation. Intra-operative protocol consisted of the injection of a low 37 MBq sestamibi dose in the operating suite 10 min before surgery. A hand held 11-mm collimated gamma probe was used. Quick PTH (QPTH) was routinely measured. Results: 344 out of the 452 patients met the inclusion criteria, and MIRP was successfully performed in 321 of them (93.3%). No major intra-operative complication was recorded. MIRP required a mean operative time of 32 min, and a mean hospital stay of 1.2 d. The parathyroid to background ratio (P/B) calculated by the probe was well correlated with the P/B calculated by sestamibi SPECT (r = 0.91; p
Published: 2007

23. Clinical phenotypes and factor VII genotype in congenital factor VII deficiency

Author: Mariani, G, Herrmann, Fh, Dolce, A, Batorova, A, Etro, Daniela, Peyvandi, F, Wulff, K, Schved, Jf, Auerswald, G, Ingerslev, J, Bernardi, Francesco, and INTERNATIONAL FACTOR VII DEFICIENCY STUDY GROUP
Subjects: Adult, Male, medicine.medical_specialty, Pathology, Adolescent, Genotype, Factor VII Deficiency, Hemorrhage, Gene mutation, Gastroenterology, Asymptomatic, chemistry.chemical_compound, Sex Factors, Polymorphism (computer science), Interquartile range, Internal medicine, medicine, Coagulopathy, Humans, Expressivity (genetics), Child, factor VII deficiency, clinical phenotype, factor VII genotype, Aged, Aged, 80 and over, Factor VII, business.industry, Genetic Variation, Infant, Hematology, Middle Aged, medicine.disease, Phenotype, chemistry, Child, Preschool, Factor Xa, Mutation, Female, medicine.symptom, business
Abstract: SummaryTo investigate the relationship between clinical phenotype, clotting activity (FVIIc) and FVII genotype, a multi-center study of factor VII (FVII) congenital deficiency with centralized genotyping and specific functional assays was carried out. FVII mutations characterized in patients (n=313) were extremely heterogeneous (103 different, 22 novel). Clinical phenotypes ranged from asymptomatic condition, including 15 homozygotes and 14 double heterozygotes, to patients with a severe disease char-acterized by life-threatening and disabling symptoms (CNS, GI bleeding and hemarthrosis) strongly associated with an early age of presentation. Based on type and number of symptoms we classified 90 'severe' (median FVIIc 1.4%, IQR [Interquartile Range] 0.9–3.8), 83 'moderate' (FVIIc 3%, IQR 1–21.7), and 140 'mild' bleeders (FVIIc 14%, IQR 3–31). The significantly different FVIIc levels, and the decreasing prevalence of homozygotes or double heterozygotes among severe (98%), moderate (84%) and mild (56%) bleeders, further support our classification. The excess of females among moderate bleeders (female/male ratio =2.6) is attributable to menorrhagia. There was no evidence for modulation of clinical features by frequent functional polymorphisms. Homozygotes for the same mutation (Ala294Val; 11125del C) with similar FVIIc and FXa generation levels, showed striking differences in clinical phenotypes. Our study depicts the ample clinical picture of this rare disorder, proposes a severity classification and provides arguments for the early management of the disease in the severe cases. Genotype-phenotype relationships indicate the presence of major environmental and/or extragenic components modulating expressivity of FVII deficiency.
Published: 2005

24. Positron Emission Tomography (PET) in the staging of head neck cancer: comparison between PET and CT

Author: Bruschini, P., Giorgetti, A., Bruschini, L., Nacci, A., Volterrani, D., Mirco Cosottini, Ursino, F., Mariani, G., and Fattori, B.
Subjects: Male, Fluorodeoxyglucose F18, Head and Neck Neoplasms, Humans, Female, Middle Aged, Radiopharmaceuticals, Tomography, X-Ray Computed, Sensitivity and Specificity, Aged, Neoplasm Staging, Tomography, Emission-Computed
Abstract: Standard pre-treatment clinical staging (TNM) of head and neck squamous cell carcinoma includes clinical and instrumental objective examination of primary tumour and of the cervical lymph nodes (inspection, palpation of neck, panendoscopy, biopsy of tumour, fine needle aspiration of nodes) and computed tomography or magnetic resonance imaging. Albeit, this procedure presents diagnostic limitations in the identification of approximately 1/3 of T1, of small sized nodes and in the diagnosis of metastases. Positron emission tomography-fluorodesoxy-glucose imaging, in the diagnostic workup of these cases, appears to offer an important contribution, however, its use is limited due to poor availability of this equipment and the high cost of the examination. In the present study, a comparison is made of results of standard clinical staging and positron emission tomography-fluorodesoxy-glucose in 22 patients with head and neck carcinoma prior to surgical treatment, with the results of pathological staging (pTNM) carried out on surgical specimens. In the staging of the tumour, computed tomography shows a sensitivity of 71% and positron emission tomography of 81%. In the staging of nodes, computed tomography imaging shows a sensitivity of 73%, a specificity of 57% and an accuracy of 68%, whereas positron emission tomography shows a sensitivity of 93%, a specificity of 100% and an accuracy of 95%. Furthermore, positron emission tomography identified 1/5 occult tumours and one tumour revealed at objective endoscopic examination, but not by computed tomography. The risk of occult nodes following positron emission tomography was found to be 7%. Overall, these results are in keeping with those reported in the literature, thus confirming the usefulness of positron emission tomography-fluorodesoxy-glucose in identifying occult tumours and nodes, in which computed tomography appears to be limited. Indications of positron emission tomography-fluorodesoxy-glucose may play a role in the choice of therapeutic options for the clinically N0 neck.
Published: 2003

25. [Total thyroidectomy in the surgical treatment of thyroid disease a retrospective clinical study]

Author: Perri, S., Mariani, G., Trunfio, A., Antonellis, M., Procacciante, Fabio, Pietrangeli, F., Nardi, M. J., Lotti, R., Gabrielli, F., and Citone, G.
Subjects: Adult, Male, Hypocalcemia, Thyroidectomy, Humans, Female, Middle Aged, Thyroid Diseases, Aged, Retrospective Studies
Abstract: Thyroid diseases constitute a group of benign and malignant affections, among which the most represented is multinodular goitre. Nowadays, an important question regards routine total thyroidectomy as the treatment of choice for all thyroid diseases. The aim of the present study is to verify if total thyroidectomy can always represent an option for modern surgery of thyroid disease.One hundred-thirty-seven patients underwent operations for thyroid disease during an 11-year period. Fifty-three patients underwent total thyroidectomy, 27 affected by a benign pathology and 26 by a malignant one.Both short term and long term results have been analysed.In conclusion, the authors affirm that a complete resection of the gland is mandatory for the surgeon in the treatment of malignant diseases because the primary aim for oncologic surgery of the thyroid is the reduction of local recurrence and the increase of survival. As far as benign diseases are concerned, some surgeons affirm that morbility of non-total operations is lower than total thyroidectomy; anyway, the authors affirm that the most important factor to prevent morbility after total thyroidectomy is an appropriate surgical technique. In this way, both the identification of parathyroid glands and the identification preparation of recurrent nerve are considered the most valid method to prevent lesions. Another factor in favour of total thyroidectomy is represented by the risk of carcinoma on the residual tissue.
Published: 2001

26. [Treatment of mandibular condylar hyperplasia in developmental age. Clinical case]

Author: Silvestri, Alessandro, Incisivo, V., and Mariani, G.
Subjects: Radiography, Adolescent, Facial Asymmetry, Mandibular Condyle, Humans, Female, Hypertrophy, Radionuclide Imaging, Malocclusion, Follow-Up Studies
Abstract: A case of hyperplasia of the mandibular condyle in a growing-up subject, observed at the Department of Maxillo-Facial Surgery of the University of Rome "La Sapienza", is described. Hyperplasia of the mandibular condyle is a facial asymmetry due to the unilateral overdevelopment of the mandibular bone. In this study the authors underline how bone scintigraphy, 3D tomography and electrognatographic analysis, associated with standard radiography and cephalometry, are important methods of diagnosis in order to make an early diagnosis of hyperplasia of the mandibular condyle and differential diagnosis with other pathologies. In particular, bone scintigraphy is a useful screening procedure to detect if the pathology is in an active phase or not. The 3D tomography is used in pre-surgery to evaluate precisely morphological and structural alterations of the craniofacial bones on a tridimentional base. Finally, the electrognatographic test records the mandibular activity both in physiological and pathological conditions. All these instrumental techniques allow to make a diagnosis and lead to a possible therapeutical approach.
Published: 2001

27. Serum phospholipids are the main environmental determinants of activated factor VII in the most common FVII genotype. European Union Concerted Action 'Clotart'

Author: Mariani G, Bernardi F, Bertina R, Vv, Vicente, Prydz H, Samama M, Pm, Sandset, Gd, Di Nucci, Mg, Testa, Bendz B, Flavia Chiarotti, Mv, Ciarla, and Strom R
Subjects: Adult, Male, Polymorphism, Genetic, Sex Factors, Age Factors, Humans, Female, Factor VIIa, Factor VII, Middle Aged, Alleles, Phospholipids, Aged
Abstract: Numerous studies have emphasized the role of triglyceride-rich lipoproteins and of Factor VII (FVII) polymorphisms in determining levels of FVII activity.This study was undertaken to evaluate the role of other lipid fractions and the interaction between lipids and FVII in subjects with recognised genotypes. Volunteer subjects (n=459) from 5 European countries were studied. Blood samples were drawn irrespective of the time of day or fasting status. Levels of FVII activity (FVIIc), activated FVII (FVIIa) and FVII antigen (FVIIAg) were evaluated with reference to a number of lipid parameters (HDL-, LDL- and total cholesterol, triglycerides, phospholipids, lipoprotein(a), and apoliproptein A1). The two most common FVII polymorphisms were analyzed in combination (353R/Q and 5'F7; alleles M1/M2 and A1/A2, respectively).Homozygotes for the A1 and M1 alleles (M11/A11) had significantly higher FVII levels. At multiple regression analysis the strongest predictor of FVIIa and FVIIc was the concentration of phospholipids. This interaction was confined to the A11M11 genotype subjects.These data indicate that lipids contribute mainly to FVIIa levels through their phospholipid content, and that the degree of this contribution is strictly dependent on FVII genotypes.
Published: 1999

28. Hodgkin's disease presenting below the diaphragm. The experience of the Gruppo Italiano Studio Linfomi (GISL)

Author: Iannitto, E., Accurso, V., Federico, M., Daniele Vallisa, Pieresca, C., Gravina, S. F., Di Costanzo, F., Di Trapani, R., Silingardi, V., and Mariani, G.
Subjects: Adult, Male, Adolescent, Hodgkin's disease, clinical stage, prognostic factors, Splenic Neoplasms, Diaphragm, Middle Aged, Hodgkin Disease, Italy, Abdominal Neoplasms, Humans, Female, Prospective Studies, Retroperitoneal Neoplasms, Aged
Abstract: Infradiaphragmatic Hodgkin's disease is rare, making up 5-12% of cases in clinical stages I and II; consequently, several questions concerning prognosis and treatment strategy remain to be answered. The aim of this study was to analyze the clinical and prognostic characteristics and outcome of this condition.A series of 282 patients with CS I-II Hodgkin's disease (HD) was investigated. In 31 patients the disease was confined below the diaphragm (BDHD), and in the remaining above the diaphragm (ADHD). The presenting features and outcomes were compared in the two groups.The BDHD group was older (p0.0002), had a higher frequency of males (p0.08) and a different histological subtype group distribution (p0.0001). Stage II BDHD patients had a worse overall survival rate (OS) than stage II ADHD patients (68.8% vs 86.6% at 8 years, p0.01) if age is not considered; patients with more than 40 years of age, in fact, had the same survival rates as those with ADHD. BDHD patients with intra-abdominal disease alone had worse prognostic factors and OS (p = 0.12) than patients with inguinal-femoral nodes.Although BDHD patients present distinct features, they have the same OS and relapse-free survival rate as age-adjusted ADHD patients. According to our experience patients with stage I peripheral BDHD respond well to radiotherapy-based regimens. Those with stage II and or intra-abdominal disease are more challenging; chemotherapy or a combined therapy seem to be more suitable approaches for these patients.
Published: 1998

29. [Feasibility of a cyclophosphamide methotrexate and 5-fluorouracil regime intravenously administered with and without granulocyte-colony stimulating factor in surgically treated breast carcinoma]

Author: Garrone, O., Del Mastro, L., Mariani, G. L., Ardizzoni, A., Venturini, M., and Rosso, R.
Subjects: Adult, Male, Adolescent, dose intensity, Breast Neoplasms, Middle Aged, Combined Modality Therapy, breast neoplasms, drug therapy, granulocyte colony stimulating factor, Methotrexate, Antineoplastic Combined Chemotherapy Protocols, Granulocyte Colony-Stimulating Factor, Injections, Intravenous, Humans, Female, Fluorouracil, Cyclophosphamide, Aged
Abstract: The aim of this study was to evaluate the feasibility of CMF 1.8-28 regimen, with all three drugs administered intravenously (IV), and to compare the hematologic toxicity of this regimen with or without G-CSF. Patients aged 18 to 65 years with histologically proven breast cancer treated by surgery and without distant metastases were eligible for the study. All patients had to have normal white blood cells (WBC) count (WBCor = 3000/mm3 and/or neutrophilsor = 2000), and platelets (Plt) counts (or = 100,000/mm3), and adequate renal and hepatic function. The toxicity was recorded according to World Health Organization Scale. CMF 1.8 regimen was: cyclophosphamide 600 mg/m2, methotrexate 40 mg/m2, 5-fluorouracil 600 mg/m2. The drugs were given IV on day 1 and 8, and cycles repeated every 28 days. G-CSF (5 micrograms/kg/day) was administered subcutaneously from day 9 to 20, starting from the second cycle of chemotherapy. A complete blood count with white-cell differential and platelet count was obtained twice a week. For each patient bone marrow toxicity variables recorded during the first cycle (without G-CSF) were compared with values during the second cycle (with G-CSF). One of 10 entered patients, 1 was not evaluated due to missing data on hematologic toxicity. All patients received chemotherapy with or without G-CSF at the scheduled 28th day. Treatment with G-CSF after CMF 1.8 regimen resulted in a significantly WBC's earlier nadir (average day of nadir 14 vs 17; p = 0.0005), while there was no difference in the average values of WBC at the nadir. Moreover, the average value of platelets recorded at the nadir was significantly lower with the use of G-CSF (average No. of platelets/mm3; 185,111 vs 116,000; p = 0.001). Complete hematologic recovery without and with G-CSF was reached on day 25 and 20 respectively (p = 0.001). CMF 1.8 with IV cyclophosphamide is a feasible regimen with and without G-CSF and can be used in adjuvant setting instead of "classic" CMF in order to improve the low compliance observed when cyclophosphamide is given by mouth. As reported by others, we observed that after standard chemotherapy G-CSF anticipated the nadir of WBC and hastened hematologic recovery (WBC3000 and Pltor = 100,000).(ABSTRACT TRUNCATED AT 400 WORDS)
Published: 1993

30. Protein charge permselectivity in type 2 and type 1 diabetes

Author: Pietravalle, P., Susanna Morano, Rossi, M. G., Mariani, G., Cristina, G., Ghirlanda, G., Cotroneo, P., Clementi, A., and Di Mario, U.
Subjects: Adult, Anions, Male, Metabolic Clearance Rate, Middle Aged, Sensitivity and Specificity, Molecular Weight, Proteinuria, Diabetes Mellitus, Type 1, Diabetes Mellitus, Type 2, Cations, Immunoglobulin G, Albuminuria, Humans, Female
Published: 1993

31. Risk of iron overload and 'hemochromatosis allele(s)' in patients on maintenance hemodialysis

Author: Taccone Gallucci, M, DI NUCCI, Gian Domenico, Meloni, C, Mariani, G, Valeri, M, Piazza, A, Elia, L, Torromeo, Concetta, Mandelli, Franco, and Casciani, Cu
Subjects: Adult, Ferric Oxide, Saccharated, Male, Risk, Adolescent, Transfusion Reaction, Anemia, Ferric Compounds, Glucaric Acid, Renal Dialysis, Ferritins, Humans, Female, Hemochromatosis, Child, Alleles, Aged, Uremia
Abstract: In the present study, we have evaluated the relationship between serum ferritin (SF) levels, 'hemochromatosis allele(s)', blood transfusions and iron parenteral administration in 69 hemodialysis patients. We demonstrated significantly higher SF levels in patients with hemochromatosis allele(s) (HA+) than in patients without hemochromatosis alleles (HA-). In addition, HA+ patients who had received blood transfusions up to 15 months prior to the study had SF levels even higher than those without blood transfusions. On the other hand, HA- patients had normal levels of SF, independent of blood transfusions. After intravenous administration of 1 g iron saccharate, SF levels were significantly higher only in HA+ transfused patients. In conclusion, our study demonstrated that HA+ patients are at a higher risk of iron overload and therefore the use of transfusional and/or parenteral iron should be strictly limited.
Published: 1987

32. TRAIL decoy receptors mediate resistance of acute myeloid leukemia cells to TRAIL

Author: Riccioni, R., luca pasquini, Mariani, G., Saulle, E., Rossini, A., Diverio, D., Pelosi, E., Vitale, A., Chierichini, A., Cedrone, M., Foà, R., Lo Coco, F., Peschle, C., and Testa, U.
Subjects: Adult, Myeloid, Male, Oncogene Proteins, Fusion, Adolescent, Recombinant Fusion Proteins, Drug Resistance, Antineoplastic Agents, Apoptosis, HL-60 Cells, Tretinoin, Acute, GPI-Linked Proteins, Receptors, Tumor Necrosis Factor, Monocytes, TNF-Related Apoptosis-Inducing Ligand, Leukemia, Promyelocytic, Acute, acute promyelocytic leukemia, apoptosis, leukemia, trail, trail receptors, Receptors, Receptors, Tumor Necrosis Factor, Member 10c, Tumor Cells, Cultured, Humans, Hydroxyurea, Fusion, Tumor Stem Cell Assay, Etoposide, Promyelocytic, Oncogene Proteins, Caspase 8, Leukemia, Cultured, Caspase 3, Cell Membrane, Cytarabine, Cell Differentiation, U937 Cells, Middle Aged, Tumor Cells, Receptors, TNF-Related Apoptosis-Inducing Ligand, Tumor Necrosis Factor Decoy Receptors, Drug Resistance, Neoplasm, Leukemia, Myeloid, Acute Disease, Neoplasm, Female, Tumor Necrosis Factor, Settore MED/15 - Malattie del Sangue, Granulocytes
Abstract: The tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) is regarded as a potential anticancer agent. However, many cancer cells are resistant to apoptosis induction by TRAIL. The present study was designed to evaluate the sensitivity to TRAIL-induced apoptosis in acute myeloblastic leukemias (AML).TRAIL/TRAIL receptor (TRAIL-R) expression and sensitivity to TRAIL-mediated apoptosis were explored in 79 AML patients, including 17 patients with acute promyelocytic leukemia (APL).In non-APL AML we observed frequent expression of TRAIL decoy receptors (TRAIL-R3 and TRAIL-R4), while TRAIL-R1 and TRAIL-R2 expression was restricted to AML exhibiting monocytic features. Total leukemic blasts, as well as AML colony-forming units (AML-CFU), were invariably resistant to TRAIL-mediated apoptosis. APL express membrane-bound TRAIL on their surface and exhibit a pattern of TRAIL-R expression similar to that observed in the other types of AML. Before, during and after retinoic acid treatment APL cells are TRAIL-resistant. The induction of granulocytic maturation of APL cells by retinoic acid was associated with a marked decline of TRAIL expression.The analysis of experimental APL models (i.e., U937 cells engineered to express PML/RAR-Eo and NB4 cells) provided evidence that PML/RAR-Eo expression was associated with downmodulation of TRAIL-R1 and with resistance to TRAIL-mediated apoptosis. We suggest that AML blasts, including APL blasts, are resistant to TRAIL-mediated apoptosis, a phenomenon seemingly related to the expression of TRAIL decoy receptors on these cells. Finally, APL blasts express membrane-bound TRAIL that could confer an immunologic privilege to these cells.

33. Hurthle cell adenoma of the thyroid: In 32 consecutive cases

Author: Stefano Guadagni, Francavilla, S., Agnifili, A., Bernardinis, G., Mariani, G., and Carboni, M.
Subjects: Adult, Male, Adolescent, Hurthle cell adenoma, Biopsy, Needle, Middle Aged, ultrastructure, thyroid, Neoplasms, Multiple Primary, Survival Rate, Microscopy, Electron, Thyroidectomy, Adenoma, Oxyphilic, Humans, Female, Thyroid Neoplasms, Neoplasm Recurrence, Local, Aged, Follow-Up Studies, Forecasting, Retrospective Studies
Abstract: Hürthle cell neoplasms of the thyroid gland are uncommon but potentially lethal lesions, the treatment of which is controversial because of a lack of information about their biological and clinical behaviour. Based on histological criteria Hürthle cell tumours cannot always be separated accurately into benign and malignant. Unexpected evolutions with malignant late recurrence of an adenoma previously labelled as benign have been reported. The authors reviewed the clinical and pathological features of 32 patients with Hürthle cell adenomas. The results suggest that total thyroidectomy 'de principe' is not necessary and that some electron microscopy criteria are useful to understand the nature and to predict the biological deportment of Hürthle cell adenomas.

34. A critical reappraisal of false negative sentinel lymph node biopsy in melanoma

Author: Manca, G., Romanini, A., Rubello, D., Mazzarri, S., Boni, G., Chiacchio, S., Tredici, M., Duce, V., Tardelli, E., DUCCIO VOLTERRANI, and Mariani, G.
Subjects: Image-Guided Biopsy, Male, Tomography, Emission-Computed, Single-Photon, Evidence-Based Medicine, Skin Neoplasms, Sentinel Lymph Node Biopsy, Reproducibility of Results, Multimodal Imaging, Sensitivity and Specificity, X-Ray Computed, Lymphatic Metastasis, Humans, Female, Emission-Computed, Lymph Nodes, Melanoma, Tomography, X-Ray Computed, False Negative Reactions, Tomography, Single-Photon
Abstract: Lymphatic mapping and sentinel lymph node biopsy (SLNB) have completely changed the clinical management of cutaneous melanoma. This procedure has been accepted worldwide as a recognized method for nodal staging. SLNB is able to accurately determine nodal basin status, providing the most useful prognostic information. However, SLNB is not a perfect diagnostic test. Several large-scale studies have reported a relatively high false-negative rate (5.6-21%), correctly defined as the proportion of false-negative results with respect to the total number of "actual" positive lymph nodes. The main purpose of this review is to address the technical issues that nuclear physicians, surgeons, and pathologists should carefully consider to improve the accuracy of SLNB by minimizing its false-negative rate. In particular, SPECT/CT imaging has demonstrated to be able to identify a greater number of sentinel lymph nodes (SLNs) than those found by planar lymphoscintigraphy. Furthermore, a unique definition in the international guidelines is missing for the operational identification of SLNs, which may be partly responsible for this relatively high false-negative rate of SLNB. Therefore, it is recommended for the scientific community to agree on the radioactive counting rate threshold so that the surgeon can be better radioguided to detect all the lymph nodes which are most likely to harbor metastases. Another possible source of error may be linked to the examination of the harvested SLNs by conventional histopathological methods. A more careful and extensive SLN analysis (e.g. molecular analysis by RT-PCR) is able to find more positive nodes, so that the false-negative rate is reduced. Older age at diagnosis, deeper lesions, histologic ulceration, head-neck anatomical location of primary lesions are the clinical factors associated with false-negative SLNBs in melanoma patients. There is still much controversy about the clinical significance of a false-negative SLNB on the prognosis of melanoma patients. Indeed, most studies have failed to show that there is worse melanoma-specific survival for false-negative compared to true-positive SLNB patients.

35. Biodistribution and pharmacokinetic screening in humans of monoclonal antibody AR-3 as a possible immunoscintigraphy agent in patients with pancreatic cancer

Author: Mariani, G., Molea, N., Bacciardi, D., Boggi, U., Bonino, C., Costa, A., Viacava, P., Castagna, M., Bodei, L., Tarditi, L., Giuliano Mariani, and Vitek, F.
Subjects: Male, Antibodies, Monoclonal, Mice, Nude, Pilot Projects, Middle Aged, Iodine Radioisotopes, Pancreatic Neoplasms, Mice, Radioimmunodetection, Biomarkers, Tumor, Animals, Humans, Female, Tissue Distribution, Aged
Abstract: This pharmacokinetic study was performed in order to assess the potential usefulness of the murine monoclonal antibody (MoAb) AR-3-IgG1 as an immunoscintigraphy agent for pancreatic cancer. This MoAb, which defines a mucin-like antigen (CAR-3) expressed by a large fraction of pancreatic cancers, shows in fact favourable in vivo localizing properties in the experimental animal model of human tumor xenograft. 131I-AR-3-IgG1 was injected i.v. into 5 patients with suspected pancreatic cancer. Whole-body maps and spot views of the abdominal area were recorded with a computerized gamma-camera, and specific regions of interest drawn over the liver and spleen helped to define the kinetics of activity in these organs. Blood samples taken from 0.1-144 hours post-injection and daily urine collections over the same interval served to define the kinetics of plama distribution and removal of activity from the body. Different multicompartmental models were tested to fit the experimental data, assuming as the starting hypothesis that there was to be significant nonspecific tracer accumulation in the liver, spleen and bone marrow, as already observed for the majority of radioiodinated murine MoAbs injected into humans. Surgery confirmed pancreatic cancer in 3 out of the 5 patients (chronic pancreatitis and periampullary cancer in one each); in all these 3 patients immunostaining with the MoAb AR-3 demonstrated the presence of the CAR-3 antigen (with a cytoplasmic and endoluminal/secretory pattern of distribution). Nonspecific radioactivity accumulation in the liver, spleen and bone marrow was extremely low, linked essentially to the blood pool effect of circulating activity in these organs.(ABSTRACT TRUNCATED AT 250 WORDS)

36. Evaluation of postoperative blood coagulation changes in elderly patients undergoing major surgery

Author: Caporale A, Mc, Tirindelli, Paolo AURELLO, Mancini F, Giuliani A, Rengo M, Mariani G, and Aureggi A
Subjects: Aged, 80 and over, Male, Antithrombin III, Fibrinogen, Plasminogen, Thrombophlebitis, Blood Coagulation Factors, Risk Factors, Surgical Procedures, Operative, von Willebrand Factor, Humans, Female, Aged, Follow-Up Studies, Protein C
Abstract: 18 elderly patients submitted to major surgery for malignancies or other disease were studied to assess the relationship between changes of blood coagulation factors and inhibitors in the early post-operative period and the appearance of lower limb deep vein thrombosis. A decrease in serum antithrombin III (AT III) Protein C antigen (PC: Ag) and Plasminogen activity (PLG) levels from the second to the fourth postoperative day, together with a simultaneous increase in serum fibrinogen (FG) and von Willebrand Factor (vWF:Ag) antigen levels was observed. In 8 patients, PC:Ag levels dropped below the limit considered at risk to develop DVT (less than 60 U/dl). A patient with the lowest PC:Ag levels had deep vein thrombosis From the analysis of data it was concluded that in the postoperative period, blood coagulation changes occur in elderly patients, predisposing to the risk of deep vein thrombosis.

37. Evidence of jak2 val617phe positive essential thrombocythemia with splanchnic thrombosis during estroprogestinic treatment

Author: Marianna Colagrande, Guglielmo Mariani, Antonio Tabilio, Mauro Di Ianni, Mariasanta Napolitano, Mario Lapecorella, Alessandro Lucchesi, Lapecorella M., Tabilio A., Lucchesi A., Napolitano M., Colagrande M., Di Ianni M., Mariani G., Lapecorella, M, Tabilio, A, Lucchesi,A, Napolitano, M, Colagrande, M, Di Ianni,M, and Mariani, G
Subjects: Adult, medicine.medical_specialty, Mutation, Missense, Oral contraceptive, Essential thrombocythemia, Gastroenterology, Contraceptives, Oral, Hormonal, Pathogenesis, Mesenteric Veins, Portal thrombosis, Myeloproliferative Disorders, Internal medicine, medicine, Humans, Platelet, Splanchnic Circulation, Janus kinase 2, biology, essential thrombocythemia, Janus kinase 2, oral contraceptives, portal thrombosis, Kinase, Vascular disease, business.industry, Thrombosis, Hematology, General Medicine, medicine.disease, Thrombocytopenia, Endocrinology, biology.protein, Female, business
Abstract: The discovery of the Janus kinase 2 Val617Phe mutation has brought new insights into the development of myeloproliferative disorders; however, the pathogenesis of essential thrombocythemia and its related thrombotic complications has not been completely understood. Although the Janus kinase 2 Val617Phe mutation confirms the initially suspected clonal character of the disease, factors influencing clonal transformation and expansion in the bone marrow have not been fully detected. Furthermore, patients affected by essential thrombocythemia who are carriers of the Janus kinase 2 Val617Phe mutation show a higher incidence of venous thromboembolism both before, and at the time of diagnosis, compared with noncarriers, and recent evidence of splanchnic and cerebral vein thrombosis in carriers of the Janus kinase 2 Val617Phe mutation has been reported. The intake of oral contraceptives is a strong and independent risk factor for venous thromboembolism. In addition, in-vitro tests showed both an altered primary haemostatic plug formation and enhanced platelet aggregation in patients taking such drugs. Little is known, though, about the influence of steroid hormones on both megakaryopoiesis and platelet function in patients with the Janus kinase 2 Val617Phe mutation. Herewith, we report the case of a 30-year-old woman who took a third generation oral contraceptive for 5 months and developed an essential thrombocythemia with splenog-portal axis and superior mesenteric vein thrombosis. She was found to carry the kinase gene Janus kinase 2 mutation. © 2008 Wolters Kluwer Health | Lippincott Williams & Wilkins.
Published: 2008

38. Sequential FDG-PET/CT reliably predicts response of locally advanced rectal cancer to neo-adjuvant chemo-radiation therapy

Author: Stefano Fanti, Giorgio Crepaldi, Elena Banti, Domenico Rubello, Carlo Capirci, Marcello Gava, Lucia Rampin, Pier Carlo Muzzio, Fabrizio Galeotti, Paola Anna Erba, Giuliano Mariani, Capirci, C, Rampin, L, Erba, P, Galeotti, F, Crepaldi, G, Banti, E, Gava, M, Fanti, S, Mariani, G, Muzzio, P, Rubello, D, Capirci C., Rampin L., Erba P.A., Galeotti F., Crepaldi G., Banti E., Gava M., Fanti S., Mariani G., Muzzio P.C., and Rubello D.
Subjects: Adult, Male, medicine.medical_specialty, Colorectal cancer, PET/CT, medicine.medical_treatment, Sensitivity and Specificity, Fluorodeoxyglucose F18, Humans, Medicine, Radiology, Nuclear Medicine and imaging, Myocardial infarction, Rectal cancer, Prospective cohort study, Aged, Aged, 80 and over, PET-CT, Chemotherapy, Rectal Neoplasms, business.industry, Reproducibility of Results, General Medicine, Middle Aged, Prognosis, medicine.disease, Clinical trial, Radiation therapy, Treatment Outcome, Chemotherapy, Adjuvant, Positron-Emission Tomography, Orthopedic surgery, Female, Radiotherapy, Adjuvant, Radiology, Neoplasm Recurrence, Local, Radiopharmaceuticals, Tomography, X-Ray Computed, business, Nuclear medicine, Chemo-radiation, Pathological response
Abstract: Purpose: Prediction of rectal cancer response to preoperative, neo-adjuvant chemo-radiation therapy (CRT) provides the opportunity to identify patients in whom a major response is expected and who may therefore benefit from alternative surgical approaches. Traditional morphological imaging techniques are effective in defining tumour extension in the initial diagnostic and staging work-up, but perform poorly in distinguishing residual neoplastic tissue from scarring post CRT, when restaging the patient before surgery. Fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET) is a promising tool for monitoring the effect of anti-tumour therapy. The aim of this study was to prospectively assess the value of sequential FDG-PET scans in predicting the response of locally advanced rectal cancer to neo-adjuvant CRT. Methods: Forty-four consecutive patients with locally advanced (cT3-4) primary rectal cancer and four patients with pelvic recurrence of rectal cancer were enrolled in this prospective study. Treatment consisted of external beam intensified radiotherapy (50 Gy to the posterior pelvis, 56 Gy to the tumour), chemotherapy (in most cases PVI 5-FU at 300 mg/m2 per day) and, 8-10 weeks later, surgery with curative intent. All patients underwent FDG-PET/CT both before CRT and 5-6 weeks after completing CRT. One patient died before surgery because of acute myocardial infarction, and was therefore excluded from further analysis. Semi-quantitative measurements of FDG uptake (SUVmax), absolute difference (ΔSUVmax) and percent SUVmax difference (Response Index, RI) between pre- and post-CRT PET scans were considered. Results were correlated with pathological response, assessed both by histopathological staging of the surgical specimens (pTNM) and by the tumour regression grade (TRG) according to Mandard's criteria (patients with TRG1-2 being defined as responders and patients with TRG3-5 as non-responders). Results: Following neo-adjuvant CRT, of the 45 patients submitted to surgery, 23 (51.1%) were classified as responders according to Mandard's criteria (8 TRG1 and 15 TRG2), while the remaining 22 (48.9%) were non-responders (9 TRG3 and 13 TRG4-5). Considering all patients, the mean pre-CRT SUVmax was 15.6, significantly higher than the mean value of 5.4 post CRT (p < 0.001). Nevertheless, when stratifying patients according to response to CRT (using Mandard's criteria), the mean RI was significantly higher in responders than in non-responders (75.9% versus 46.9%,p = 0.0015). Using a 66.2% SUVmax decrease as the cut-off value (identified by ROC analysis) for defining response to therapy, the following parameters were obtained: 79.2% specificity, 81.2% sensitivity, 77% positive predictive value, 89% negative predictive value and 80% overall accuracy. Conclusion: The results suggest the potential utility of FDG-PET as a complementary diagnostic and prognostic procedure in the assessment of neo-adjuvant CRT response of locally advanced rectal cancer. ΔSUV max and RI seem the best predictors of CRT response.
Published: 2007

39. (18)F-FDG PET/CT in the evaluation of recurrent ovarian cancer: a prospective study on forty-one patients

Author: Stefano Fanti, Lucia Rampin, Paola Anna Erba, Giuliano Mariani, Maddalena Sansovini, D. Rubello, M. Farsad, C. Nanni, P. De Iaco, Nanni, C, Rubello, D, Farsad, M, De Iaco, P, Sansovini, M, Erba, P, Rampin, L, Mariani, G, Fanti, S, Nanni C., Rubello D., Farsad M., De Iaco P., Sansovini M., Erba P., Rampin L., Mariani G., and Fanti S.
Subjects: Adult, medicine.medical_specialty, Sensitivity and Specificity, Fluorodeoxyglucose F18, Ovarian cancer, medicine, Humans, Recurrent disease, False Positive Reactions, Prospective Studies, Prospective cohort study, False Negative Reactions, Aged, Ovarian Neoplasms, medicine.diagnostic_test, business.industry, Ca125 serum level, Ultrasound, 18F-FDG PET/CT, Magnetic resonance imaging, General Medicine, Middle Aged, medicine.disease, Conventional radiologic imaging, Oncology, CA125 SERUM LEVELS, Recurrent Ovarian Cancer, Positron emission tomography, Positron-Emission Tomography, Female, Surgery, Fdg pet ct, Radiology, Neoplasm Recurrence, Local, Radiopharmaceuticals, Tomography, X-Ray Computed, business, Nuclear medicine
Abstract: Aim Many patients with ovarian cancer are at high risk of recurrence especially in the 2 years following first-line therapy. CA125 serum levels measurement associated to computed tomography (CT), ultrasound (US) and magnetic resonance imaging (MRI) are currently used during follow-up to detect recurrent disease. Unfortunately, in a relevant percentage of cases all of these traditional imaging techniques provide a significant number of doubtful/equivocal results or turn out negative even in presence of elevated Ca125 levels. Aim of our study was to evaluate sensitivity, specificity and accuracy of 18 F-FDG PET/CT in a group of patients with suspicion of ovarian cancer recurrence. Methods We prospectively evaluated 41 patients with a mean age of 59.4 years who had been previously treated for ovarian cancer with surgery and radio-chemotherapy or radio-chemotherapy alone. Following the performance of traditional radiologic imaging (US, CT, MRI) and Ca125 measurement, all patients underwent additional 18 F-FDG PET/CT. PET/CT results were compared with histologic findings or clinical, laboratory and repeated traditional imaging techniques during subsequent follow-up data. Results Of 41 patients 32 had a positive PET-CT (30 true positive, two false positive) whereas nine a negative PET/CT (five true negative, four false negative). Overall, in our experience 18 F-FDG PET/CT provided a good sensitivity (88.2%), specificity (71.4%) and accuracy (85.4%), superior to that reported in literature for traditional radiologic imaging. Conclusions It can be concluded that 18 F-FDG PET/CT appears to be a useful and accurate tool in disclosing early recurrent ovarian cancer.
Published: 2005

40. Improving women’s health in low-income and middle-income countries. Part II

Author: Miriam Mikhail, Giuliano Mariani, Dina H. Salama, Diana Paez, Paola Anna Erba, Neerja Bhatla, Joanna Kasznia-Brown, Ravi Kashyap, Mariani, G, Kasznia-Brown, J, Paez, D, Mikhail, M, Salama, D, Bhatla, N, Erba, P, and Kashyap, R
Subjects: Diagnostic Imaging, Women health, Low income countries, medicine.medical_specialty, business.industry, Editorials, Low income and middle income countries, General Medicine, 03 medical and health sciences, 0302 clinical medicine, Nursing, Nuclear Medicine and Imaging, 030220 oncology & carcinogenesis, Family medicine, Medical imaging, Humans, Women's Health, Medicine, Female, Radiology, Nuclear Medicine and imaging, 030212 general & internal medicine, Radiology, business, Developing Countries, Poverty
Published: 2017
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41. Safety of everolimus plus exemestane in patients with hormone-receptor–positive, HER2–negative locally advanced or metastatic breast cancer progressing on prior non-steroidal aromatase inhibitors: primary results of a phase IIIb, open-label, single-arm, expanded-access multicenter trial (BALLET)

Author: Miguel Martin, Gabriella Mariani, Daniele Generali, István Láng, Vivianne C. G. Tjan-Heijnen, Andrea Michelotti, Edda Simoncini, Guy Jerusalem, Jozef Mardiak, Eva Ciruelos, Filippo Montemurro, Joaquín Gavilá, S. Bianchetti, Pierfranco Conte, Katia Lorizzo, M. Camozzi, Patrick Neven, Bjørn Naume, RS: GROW - R3 - Innovative Cancer Diagnostics & Therapy, MUMC+: MA Medische Oncologie (9), Interne Geneeskunde, Jerusalem, G., Mariani, G., Ciruelos, E. M., Martin, M., Tjan Heijnen, V. C. G., Neven, P., Gavila, J. G., Michelotti, A., Montemurro, F., Generali, Daniele, Simoncini, E., Lang, I., Mardiak, J., Naume, B., Camozzi, M., Lorizzo, K., Bianchetti, S., and Conte, Pietro
Subjects: Male, 0301 basic medicine, Receptor, ErbB-2, chemistry.chemical_compound, Elderly, 0302 clinical medicine, Exemestane, Antineoplastic Combined Chemotherapy Protocols, Medicine, Neoplasm Metastasis, Aged, 80 and over, advanced breast cancer, Advanced breast cancer, BMI, Everolimus, Hormone-receptor positive, Stomatitis, Hematology, Oncology, Aromatase Inhibitors, Incidence (epidemiology), Metastatic breast cancer, ErbB Receptors, Postmenopause, Everolimu, Receptors, Estrogen, 030220 oncology & carcinogenesis, Female, medicine.drug, medicine.medical_specialty, Drug-Related Side Effects and Adverse Reactions, Breast Neoplasms, elderly, Disease-Free Survival, 03 medical and health sciences, Multicenter trial, Internal medicine, Humans, Adverse effect, Aged, Sirolimus, hormone-receptor positive, Gynecology, business.industry, everolimus, medicine.disease, Stomatiti, stomatitis, Androstadienes, Clinical trial, 030104 developmental biology, chemistry, Expanded access, Neoplasm Recurrence, Local, business
Abstract: Background This European phase IIIb, expanded-access multicenter trial evaluated the safety of EVE plus EXE in a patient population similar to BOLERO-2. Patients and methods Post-menopausal women aged ≥18 years with hormone receptor-positive, human epidermal growth factor-receptor-2–negative advanced breast cancer (ABC) recurring/progressing during/after prior non-steroidal aromatase inhibitors were enrolled. The primary objective was safety of EVE plus EXE based on frequency of adverse events (AEs), and serious AEs (SAEs). The secondary objective was to evaluate AEs of grade 3/4 severity. Results The median treatment duration was 5.1 months [95% confidence interval (CI) 4.8–5.6] for EVE and 5.3 months (95% CI 4.8–5.6) for EXE. Overall, 2131 patients were included in the analysis; 81.8% of patients experienced EVE- or EXE-related or EVE/EXE-related AEs (investigator assessed); 27.2% were of grade 3/4 severity. The most frequently reported non-hematologic AEs were (overall %, % EVE-related) stomatitis (52.8%; 50.8%) and asthenia (22.8%; 14.6%). The most frequently reported hematologic AEs were (overall %, % EVE-related) anemia (14.4%; 8.1%) and thrombocytopenia (5.9%; 4.6%). AE-related treatment discontinuations were higher in elderly (≥70 years) versus non-elderly patients (23.8% versus 13.0%). The incidence of EVE-related AEs in both elderly and non-elderly patients appeared to be lower in first-line ABC versus later lines. The incidence of AEs (including stomatitis/pneumonitis) was independent of BMI status (post hoc analysis). Overall, 8.5% of patients experienced at least one EVE-related SAE. Of the 121 on-treatment deaths (5.7%), 66 (3.1%) deaths were due to disease progression and 46 (2.2%) due to AEs; 4 deaths were suspected to be EVE-related. Conclusions This is the largest ever reported safety dataset on a general patient population presenting ABC treated with EVE plus EXE and included a sizeable elderly subset. Although the patients were more heavily pretreated, the safety profile of EVE plus EXE in BALLET was consistent with BOLERO-2. Clinical trial registration EudraCT Number: 2012-000073-23. This article has been accepted for publication in Annals of Oncology. Published by Oxford University Press.
Published: 2016
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42. Role of clinical and laboratory parameters for treatment choice in patients with inherited FVII deficiency undergoing surgical procedures: evidence from the STER registry

Author: Di Minno, Matteo N. D., Napolitano, Mariasanta, Dolce, Alberto, Mariani, Guglielmo, Auerswald, Guenter, Batorova, Angelica, Bernardi, Francesco, Di Minno, Giovanni, Giansilly-Balizot, Muriel, Ingerslev, Jorgen, Martinowitz, Uri, Morfini, Massimo, Pinotti, Mirko, Shapiro, Amy, Siragusa, Sergio, Schved, Jean Francois, Sørensen, Benny, Di Minno, Matteo N. D., Napolitano, Mariasanta, Dolce, Alberto, Mariani, Guglielmo, DI MINNO, Giovanni, Di Minno, M., Napolitano, M., Dolce, A., Mariani, G., and Siragusa, S.
Subjects: Adult, Male, medicine.medical_specialty, Adolescent, Clinical Decision-Making, Socio-culturale, Hemorrhage, 030204 cardiovascular system & hematology, Independent predictor, Gastroenterology, Asymptomatic, surgery, 03 medical and health sciences, chemistry.chemical_compound, Young Adult, 0302 clinical medicine, Internal medicine, medicine, Humans, In patient, Registries, Factor VII deficiency, bleeding disorders, bleeding disorder, Surgical Procedures, Factor VII, business.industry, Disease Management, Perioperative, Hematology, Surgical procedures, Middle Aged, Combined Modality Therapy, Operative, factor VII deficiency, Treatment Outcome, chemistry, 030220 oncology & carcinogenesis, Surgical Procedures, Operative, Factor VII Deficiency, Female, Symptom Assessment, medicine.symptom, business, Major bleeding
Abstract: Perioperative bleeding is a major concern in patients with factor VII (FVII) deficiency. Evaluating data of 95 FVII-deficient patients undergoing 110 surgical procedures (61 major, 49 minor), we assessed the impact of type of surgery, bleeding phenotype and FVII coagulant activity (FVII:C) levels on perioperative replacement therapy (RT). Compared to those with higher FVII:C levels, patients with
Published: 2018

43. Improving women's health in low-income and middle-income countries. Part I: Challenges and priorities

Author: Dina H. Salama, Diana Paez, Neerja Bhatla, Miriam Mikhail, Paola Anna Erba, Joanna Kasznia-Brown, Ravi Kashyap, Giuliano Mariani, Mariani, G, Kasznia-Brown, J, Paez, D, Mikhail, M, Salama, D, Bhatla, N, Erba, P, and Kashyap, R
Subjects: Radiology, Nuclear Medicine and Imaging, business.industry, Maternal Health, Editorials, MEDLINE, Low income and middle income countries, General Medicine, Developing Countrie, 03 medical and health sciences, Reproductive Health, 0302 clinical medicine, 030220 oncology & carcinogenesis, Environmental health, Nuclear Medicine and Imaging, Humans, Women's Health, Medicine, Female, 030212 general & internal medicine, Socioeconomics, business, Radiology, Developing Countries, Poverty, Human
Published: 2017

44. Safety and tolerability of subcutaneous trastuzumab for the adjuvant treatment of human epidermal growth factor receptor 2-positive early breast cancer: SafeHer phase III study's primary analysis of 2573 patients

Author: Jose Chacon, Katja Ziegler-Löhr, Kamran Rashid, Stanley Madretsma, Hortense Laharie Mineur, Soo Hyeon Lee, Bohuslav Melichar, Jasna Pesic, Julia Hall, Jörg Schilling, Paola Morales Espinosa, Wendy Taylor, Francesco Cognetti, Doris Augustin, Ines Sandri, Laura Murillo Jaso, Alejandro Juarez Ramiro, Nora Artagaveytia, Rocio Reategui, Nataliia Voitko, Teresa Gamucci, Lisa H Barraclough, Jérôme Alexandre, Mohammed Butt, Frank Priou, A.J. van de Wouw, Cristina Marinela Oprean, Isabel Alonso, Suzana Vasovic, Fernando Roque, Marc Thill, Viktoria Dvornichenko, K. Bouzid, Idris Yucel, Andrea Stefek, Jose Manuel Lopez Vega, Daniil Stroyakovskiy, R. Chiara Forcignanò, Mohammed Harb, Andrzej Mruk, Jana Prausová, Lydia Dreosti, Prabir Chakraborti, Armando Santoro, Lee Wei Ching, Anna Tuczek, Jane Beith, Larisa Ciule, Hakan Bozcuk, Antonino Musolino, Hartmut Kristeleit, Clare Crowley, T.C. Kok, Dhurata Koroveshi, Natasha Mithal, Laura Garcia Sanchis, Stephan Henschen, Carmen Cañabate Arias, A. Contu, Antoaneta Tomova, Alper Sevinc, Helga Droogendijk, Gustavo Fernando Ismael, Konstantinos Papazisis, Laurent Gasnault, Sandra Bakker, Judit Kocsis, Bernd Christensen, Stephen Kelly, Rosana Jarcau, Christian Jackisch, George Fountzilas, Cyril Foa, Annebeth W. Haringhuizen, Silvia Neciosup, Juan Matos Santos, Finlander Rosales Osegueda, Robinson Rodriguez, Marcus Schmidt, Bart de Valk, Kathryn Wright, A.S. Dhadda, Elizabeth Sherwin, Sabino De Placido, Luigi Cavanna, Joelle Egreteau, Shazza Rehman, Giacomo Allegrini, Doerte Luedders, Poovandren Govender, Hugues Barletta, Iztok Takač, Yuraima Garcia, Michael Green, Geneviève Jolimoy, Marcela Urrego, Chanyoot Bandidwattanawong, Vito Lorusso, Annette van der Velden, Rene Muñoz, Djumhana Atmakusuma, Christos Papandreou, Craig Macmillan, Hassan Errihani, Iris Schrader, Isabelle Desmoulins, Jean-Marc Ferrero, Mohamed Idris, B. Ataseven, Andre Farrokh, Isabelle Moullet, Iain R. Macpherson, N. Al-Sakaff, Stephen Chia, Blanca Hernando Fernandez De Aranguiz, Lorena Lion, Alexandros Ardavanis, Ani Zlatareva-Petrova, Ernesto Pablo Korbenfeld, Hugo Castro, Mirta Garcia, Heike Passmann-Kegel, Lionel Uwer, Gary Richardson, Marion Paul, Georgia Demetriou, Andreas Köhler, V. Kovcin, Eliot Sims, Gerasimos Aravantinos, Adriana Dominguez, Daniel Rauch, Greta Beyer, Laurence J. C. van Warmerdam, Roberto Bordonaro, Raymond Ng, David Coeffic, Rostislav Vyzula, Bernard Leduc, Jozef Mardiak, Andrea Pigi, Ingo Runnebaum, Jose Angel Garcia Saenz, Areewan Somwangprasert, Cristina Llorca Ferrandiz, Coskun Hasan Senol, Martin Griesshammer, Friedrich Overkamp, Suzanne Nguyen, Maria Turdean, Udaiveer Panwar, Zsuzsanna Nagy, Francesco Giotta, Andreas Schneeweiss, Teresa Ramon y Cajal Asensio, Jae Hong Seo, Joohyuk Sohn, Jean-Philippe Jacquin, Daniela Grecea, Jasmina Nedovic, Arrate Plazaola Alcibar, Tadeusz Pienkowski, Jetske M. Meerum Terwogt, Elmar Stickeler, Hazem I. Assi, Vadim Shirinkin, Grzegorz Slomian, Etela Mišurová, Roberto Hegg, K. Friedrichs, Corinne Dagada, Jean-François Berdah, Fulden Yumuk, Alexandru Eniu, Amit Chakrabarti, Mathias Fehr, Christoph Salat, Dan Lungulescu, Heinrik Martin Strebel, Antonio Llombart Cussac, Rémy Largillier, Stefan Curescu, Albert von der Assen, Emmanuel Guardiola, Andras Csejtei, Tamas Hickish, Krzysztof Krzemieniecki, Yaroslav Shparyk, Ramon Perez Carrion, Michela Donadio, Purificacion Martinez del Prado, Sandra Franco, J.J. Braun, Michael Friedlander, Suhail Anwar, Thierry Petit, Sarah Smith, Rafael Gutierrez Pilarte, Laia Garrigos Cubells, Frans L. G. Erdkamp, Jedzada Maneechavakajorn, Mastura Yusof, Jocelyn Adams, Diana Cascallar, Luis Antonio Fernandez Morales, Max S. Mano, Simon Waters, Carlos Beato, Philippe Martin, Martin Hogg, Isabelle Sillet Bach, Monica Casalnuovo, Klara Mezei, Alexey Manikhas, Margarida Damasceno, Sergey Emelyanov, Gabriella Mariani, Kecman Gordana, Gianfilippo Bertelli, Ignacio Pelaez Fernandez, Damir Vrbanec, Maria Wagnerova, Johannes Petrus Jordaan, Marina Cazzaniga, Mustafa Deryal, Ruth Davis, Abdurrahman Isikdogan, Sanjay Raj, José Juan Illarramendi Mañas, Vinod Ganju, Maria Dolores Torregrosa Maicas, Glenda Ramos, Nugroho Prayogo, H. Orfeuvre, Filipovic S, Joke Tio, Andrew Redfern, M. Shing, Eduardo Yanez, Khalil Zaman, Jin-Seok Ahn, Dino Amadori, Bahriye Aktas, Miriam O'Connor, Uta Ringsdorf, Christophe Desauw, J. Gligorov, Jorge Corona, Michele De Laurentiis, Arthur Wischnik, Paolo Pedrazzoli, Katalin Boér, Caroline Archer, Anne Kendall, Ori Freedman, Maya Tsakova, Dana Lucia Stanculeanu, Kevin Patterson, Cathy Kelly, Nellie Lay Chin Cheah, X. Artignan, Anil A. Joy, Steffi Busch, Monica Nave, Bryan Hennessy, Lorenzo Livi, X. Pivot, R.J.B. Blaisse, Adolfo Murias Rosales, Juan Carlos Alcedo, Dalila Marcano, Emmanuel Beguier, Andreas Müller, László Csaba Mangel, Christina Schlatter, Fernando Gaion, Tjoung-Won Park-Simon, Sebastian Wojcinski, Ute Bückner, Florinel Badulescu, Cynthia Mayte Villarreal Garza, Rozenn Allerton, Mikhail Lichinitser, Damir Gugić, Manuela Rabaglio, Jens Kisro, Iris Scheffen, Vincent Phua, Marc A. Bollet, Giampaolo Biti, M. Verrill, Adrien Melis, Andrew M Wardley, Ali Arican, Hamdy A. Azim, Lelia-Eveline Bauer, Tsai-Wang Chang, Nik Hauser, René Lazaro González Mendoza, Dominique Jaubert, Samreen Ahmed, Mazhar Shah, János Szántó, Kunibert Latos, Xavier Pivot, Helen Gogas, Elona Juozaityte, Luca Moscetti, Helene Simon, Giacomo Carterni, Dan-Corneliu Jinga, Olivia Pagani, Elena Rota Caremoli, Esther Arbona, Cornelia Liedtke, Stylianos Kakolyris, Abdulla Alhasso, Omalkhair Abulkhair, Jose Ponce Lorenzo, Julian Singer, Tony Branson, Claudia Hänle, Ingvild Mjaaland, Chiun-Sheng Huang, Heri Fadjari, Jonathan Joffe, Laetitia Stefani, Dieter Lampe, Franck Burki, S. Lauer, Sabine Schmatloch, Gracieux Fernando, Dina Sakaeva, Christina Balser, Michael Martin, Nora Bittner, Andrea Heider, Antonio Frassoldati, Serafin Morales Murillo, Hakan Akbulut, Saad Tahir, Tilmann Lantzsch, Christine Brezden-Masley, Vanessa Helena, Tran Van Thuan, F.E. de Jongh, Roger K.C. Ngan, Elke Faust, Hugues Bourgeois, Flora Li Tze Chong, Nehal Masood, Keun Seok Lee, J. Bishop, Mathias Warm, Dimitris Mavroudis, Petrosian Veersamy, Judith Fraser, Andres Garcia-Palomo Perez, Heiko Graf, Vanesa Quiroga Garcia, Jyh-Cherng Yu, Maria Jose Villanueva Silva, Elke Simon, Diana Aleman, Kazim Uygun, Cosima Brucker, Michael Weigel, Volkmar Müller, Djohan Kurnianda, Duncan Wheatley, Amr Abdel Aziz, Benno Lex, Laura G. Estévez, Darren Teoh, María Isabel León, Noemia Afonso, Frances Yuille, Amelia Tienghi, Gernot Seipelt, Jose Alberto Nogueira, Dumitru Filip, Zafar Malik, Fatima Cardoso, Giorgio Cruciani, Winnie Yeo, Luis Vera, Santiago Gonzalez Santiago, Richard North, M.W. Dercksen, Zsolt Horváth, Noelia Martinez Jañez, Marta Mion, Marcela Ferrari, Natalia Valdiviezo, Oana Zveltlana Cojocarasu, Alessandra Morelle, Medy Tsalic, Sonia Pernas Simon, Christoph Maintz, Daniele Farci, Alvaro Edson Lessa, Jeremy Monge, Joseph Gligorov, Anthony Neal, Norberto Batista Lopez, Piotr Tomczak, Yesim Eralp, Kasan Seetalarom, Thitiya (Sirisinha) Dejthevaporn, Jamal Zekri, Steven John Proctor, Saira Nasim, Muireann Kelleher, Eftal Yucel, Quirine Clementine van Rossum-Schornagel, Linda Coate, Paolo Marchetti, Theresa Howe, Carlos Alberto Hernandez, Roberto Torres, Konstanta Timcheva, Evaristo Maiello, Anita Prechtl, Jamil Asselah, Branislav Bystricky, Kate Scatchard, Zeba Aziz, Jaroslava Leskova, Sherko Kuemmel, Paolo Bidoli, Richard Ashford, Piotr Sawrycki, Claude Bressac, Alberto Bottini, Pilar Lopez Alvarez, Nadine Dohollou, Alejandro Andres Acevedo Gaete, M. De Laurentiis, T.J. Smilde, Andrew Proctor, Catherine Prady, Michele Aieta, Jan Henry Svensson, Reda Garidi, Erik Wist, Antonia Perello Martorell, Mohammed Jaloudi, Graeme Lumsden, Eva-Maria Grischke, Ali Youssef, Annemieke van der Padt, Kadri Altundag, Christina Bechtner, Mireille Mousseau, Heba El Zawahry, Maartje Los, Alvydas Česas, Alfredo Falcone, Salima Hamizi, Franchette W P J van den Berkmortel, Cesar Estuardo Hernandez-Monroy, K.H. Jung, Swati Kulkarni, R.K. Agrawal, Hwei Chung Wang, Hany Eldeeb, Fredrika Killander, Jose Luis Alonso Romero, Antonio Pazzola, Daan ten Bokkel Huinink, Mario Campone, Beena C.R. Devi, Florence Dalenc, Pedro Jimenez Gallego, Mawin Vongsaisuwon, Timur Ceric, Chantal Bernard Marty, R. A. Popescu, J. van den Bosch, Luis Matamala, Sylvia Ruth, Maria Litwiniuk, Maria Lomas Garrido, Mark Churn, Christian Kersten, Francesco Del Piano, Eddie Herman Tanggo, Antonio Fernandez Aramburo, Kyung Hae Jung, Christos Papadimitriou, Hamdy Abdel Azeem, Patricia Bastick, Tobias Hesse, Maree Colosimo, Lucia Gonzalez Cortijo, Mark Verrill, Gligorov, J, Ataseven, B, Verrill, M, De Laurentiis, M, Jung, K. H, Azim, H. A, Al-sakaff, N, Lauer, S, Shing, M, Pivot, X., de Laurentiis, M, Jung, K, Azim, H, Al-Sakaff, N, Pivot, X, Koroveshi, D, Bouzid, K, Casalnuovo, M, Cascallar, D, Korbenfeld, E, Bastick, P, Beith, J, Colosimo, M, Friedlander, M, Ganju, V, Green, M, Patterson, K, Redfern, A, Richardson, G, Ceric, T, Gordana, K, Beato, C, Ferrari, M, Hegg, R, Helena, V, Ismael, G, Lessa, A, Mano, M, Morelle, A, Nogueira, J, Timcheva, K, Tomova, A, Tsakova, M, Zlatareva-Petrova, A, Asselah, J, Assi, H, Brezden-Masley, C, Chia, S, Freedman, O, Harb, M, Joy, A, Kulkarni, S, Prady, C, Gaete, A, Matamala, L, Torres, R, Yanez, E, Franco, S, Urrego, M, Gugic, D, Vrbanec, D, Melichar, B, Prausova, J, Vyzula, R, Pilarte, R, Leon, M, Munoz, R, Ramos, G, Azeem, H, Aziz, A, El Zawahry, H, Osegueda, F, Alexandre, J, Artignan, X, Barletta, H, Beguier, E, Berdah, J, Marty, C, Bollet, M, Bourgeois, H, Bressac, C, Burki, F, Campone, M, Coeffic, D, Cojocarasu, O, Dagada, C, Dalenc, F, Del Piano, F, Desauw, C, Desmoulins, I, Dohollou, N, Egreteau, J, Ferrero, J, Foa, C, Garidi, R, Gasnault, L, Guardiola, E, Hamizi, S, Jarcau, R, Jacquin, J, Jaubert, D, Jolimoy, G, Mineur, H, Largillier, R, Leduc, B, Martin, P, Melis, A, Monge, J, Moullet, I, Mousseau, M, Nguyen, S, Orfeuvre, H, Petit, T, Priou, F, Bach, I, Simon, H, Stefani, L, Uwer, L, Youssef, A, Aktas, B, von der Assen, A, Augustin, D, Balser, C, Bauer, L, Bechtner, C, Beyer, G, Brucker, C, Buckner, U, Busch, S, Christensen, B, Deryal, M, Farrokh, A, Faust, E, Friedrichs, K, Graf, H, Griesshammer, M, Grischke, E, Hanle, C, Heider, A, Henschen, S, Hesse, T, Jackisch, C, Kisro, J, Kohler, A, Kuemmel, S, Lampe, D, Lantzsch, T, Latos, K, Lex, B, Liedtke, C, Luedders, D, Maintz, C, Muller, V, Overkamp, F, Park-Simon, T, Paul, M, Prechtl, A, Ringsdorf, U, Runnebaum, I, Ruth, S, Salat, C, Scheffen, I, Schilling, J, Schmatloch, S, Schmidt, M, Schneeweiss, A, Schrader, I, Seipelt, G, Simon, E, Stefek, A, Stickeler, E, Thill, M, Tio, J, Tuczek, A, Warm, M, Weigel, M, Wischnik, A, Wojcinski, S, Ziegler-Lohr, K, Aravantinos, G, Ardavanis, A, Fountzilas, G, Gogas, H, Kakolyris, S, Mavroudis, D, Papadimitriou, C, Papandreou, C, Papazisis, K, Castro, H, Hernandez-Monroy, C, Ngan, R, Yeo, W, Bittner, N, Boer, K, Csejtei, A, Horvath, Z, Kocsis, J, Mangel, L, Mezei, K, Nagy, Z, Szanto, J, Atmakusuma, D, Fadjari, H, Kurnianda, D, Prayogo, N, Tanggo, E, Coate, L, Hennessy, B, Kelly, C, Martin, M, Nasim, S, O'Connor, M, Aieta, M, Allegrini, G, Amadori, D, Bidoli, P, Biti, G, Bordonaro, R, Bottini, A, Carterni, G, Cavanna, L, Cazzaniga, M, Cognetti, F, Contu, A, Cruciani, G, Donadio, M, Falcone, A, Farci, D, Forcignano, R, Frassoldati, A, Gaion, F, Gamucci, T, Giotta, F, Livi, L, Lorusso, V, Maiello, E, Marchetti, P, Mariani, G, Mion, M, Moscetti, L, Musolino, A, Pazzola, A, Pedrazzoli, P, Pigi, A, de Placido, S, Caremoli, E, Santoro, A, Tienghi, A, Ahn, J, Lee, K, Lee, S, Seo, J, Sohn, J, Cesas, A, Juozaityte, E, Cheah, N, Chong, F, Devi, B, Phua, V, Teoh, D, Ching, L, Yusof, M, Corona, J, Dominguez, A, Mendoza, R, Hernandez, C, Ramiro, A, Santos, J, Espinosa, P, Villarreal Garza, C, Errihani, H, Bakker, S, van den Berkmortel, F, Blaisse, R, Huinink, D, van den Bosch, J, Braun, J, Dercksen, M, Droogendijk, H, Erdkamp, F, Haringhuizen, A, de Jongh, F, Kok, T, Los, M, Madretsma, S, Terwogt, J, van der Padt, A, van Rossum-Schornagel, Q, Smilde, T, de Valk, B, van der Velden, A, van Warmerdam, L, van de Wouw, A, North, R, Kersten, C, Mjaaland, I, Wist, E, Aziz, Z, Masood, N, Rashid, K, Shah, M, Alcedo, J, Aleman, D, Neciosup, S, Reategui, R, Valdiviezo, N, Vera, L, Fernando, G, Roque, F, Strebel, H, Krzemieniecki, K, Litwiniuk, M, Mruk, A, Pienkowski, T, Sawrycki, P, Slomian, G, Tomczak, P, Afonso, N, Cardoso, F, Damasceno, M, Nave, M, Badulescu, F, Ciule, L, Curescu, S, Eniu, A, Filip, D, Grecea, D, Jinga, D, Lungulescu, D, Oprean, C, Stanculeanu, D, Turdean, M, Dvornichenko, V, Emelyanov, S, Lichinitser, M, Manikhas, A, Sakaeva, D, Shirinkin, V, Stroyakovskiy, D, Abulkhair, O, Zekri, J, Filipovic, S, Kovcin, V, Nedovic, J, Pesic, J, Vasovic, S, Ng, R, Bystricky, B, Leskova, J, Mardiak, J, Misurova, E, Wagnerova, M, Takac, I, Demetriou, G, Dreosti, L, Govender, P, Jordaan, J, Veersamy, P, Romero, J, Lopez, N, Arias, C, Chacon, J, Aramburo, A, Morales, L, Garcia, M, Estevez, L, Garcia-Palomo Perez, A, Garcia Saenz, J, Garcia Sanchis, L, Cubells, L, Cortijo, L, Santiago, S, De Aranguiz, B, Manas, J, Gallego, P, Cussac, A, Ferrandiz, C, Garrido, M, Alvarez, P, Vega, J, Del Prado, P, Janez, N, Murillo, S, Rosales, A, Jaso, L, Fernandez, I, Martorell, A, Carrion, R, Simon, S, Alcibar, A, Lorenzo, J, Garcia, V, Asensio, T, Maicas, M, Villanueva Silva, M, Killander, F, Svensson, J, Fehr, M, Hauser, N, Muller, A, Pagani, O, Passmann-Kegel, H, Popescu, R, Rabaglio, M, Rauch, D, Schlatter, C, Zaman, K, Chang, T, Huang, C, Wang, H, Yu, J, Bandidwattanawong, C, Maneechavakajorn, J, Seetalarom, K, Dejthevaporn, T, Somwangprasert, A, Vongsaisuwon, M, Akbulut, H, Altundag, K, Arican, A, Bozcuk, H, Eralp, Y, Idris, M, Isikdogan, A, Senol, C, Sevinc, A, Uygun, K, Yucel, E, Yucel, I, Yumuk, F, Shparyk, Y, Voitko, N, Jaloudi, M, Adams, J, Agrawal, R, Ahmed, S, Alhasso, A, Allerton, R, Anwar, S, Archer, C, Ashford, R, Barraclough, L, Bertelli, G, Bishop, J, Branson, T, Butt, M, Chakrabarti, A, Chakraborti, P, Churn, M, Crowley, C, Davis, R, Dhadda, A, Eldeeb, H, Fraser, J, Hall, J, Hickish, T, Hogg, M, Howe, T, Joffe, J, Kelleher, M, Kelly, S, Kendall, A, Kristeleit, H, Lumsden, G, Macmillan, C, Macpherson, I, Malik, Z, Mithal, N, Neal, A, Panwar, U, Proctor, A, Proctor, S, Raj, S, Rehman, S, Sandri, I, Scatchard, K, Sherwin, E, Sims, E, Singer, J, Smith, S, Tahir, S, Taylor, W, Tsalic, M, Wardley, A, Waters, S, Wheatley, D, Wright, K, Yuille, F, Alonso, I, Artagaveytia, N, Rodriguez, R, Arbona, E, Garcia, Y, Lion, L, Marcano, D, and Van Thuan, T
Subjects: 0301 basic medicine, Oncology, Cancer Research, Receptor, ErbB-2, medicine.medical_treatment, Medizin, Antineoplastic Agent, 0302 clinical medicine, Breast cancer, Trastuzumab, Adjuvant, Aged, 80 and over, education.field_of_study, Middle Aged, HER2/neu, Tolerability, Chemotherapy, Adjuvant, 030220 oncology & carcinogenesis, Herceptin, Subcutaneous, subcutaneous, Female, Survival Analysi, Breast Neoplasm, medicine.drug, Human, Adult, medicine.medical_specialty, Injections, Subcutaneous, Population, Socio-culturale, Antineoplastic Agents, Breast Neoplasms, Injections, Subcutaneou, 03 medical and health sciences, Young Adult, Internal medicine, medicine, Adjuvant therapy, Humans, Subcutaneou, education, Adverse effect, Aged, Chemotherapy, Adjuvant, breast cancer, HER2/neu, herceptin, trastuzumab, business.industry, medicine.disease, Survival Analysis, Surgery, Discontinuation, 030104 developmental biology, business
Abstract: Aim To assess the safety and tolerability of adjuvant subcutaneous trastuzumab (Herceptin ® SC, H SC), delivered from an H SC Vial via hand-held syringe (Cohort A) or single-use injection device (Cohort B), with or without chemotherapy, for human epidermal growth factor receptor 2 (HER2)-positive stage I to IIIC early breast cancer (EBC) in the phase III SafeHer study (NCT01566721). Methods Patients received 600 mg fixed-dose H SC every 3 weeks for 18 cycles. The chemotherapy partner was at the investigators' discretion (H SC monotherapy was limited to ≤10% of the population). Data from the first H SC dose until 28 days (plus a 5-day window) after the last dose are presented. Results are descriptive. Results In the overall population, 2282/2573 patients (88.7%) experienced adverse events (AEs). Of the above, 128 (5.0%) patients experienced AEs leading to study drug discontinuation; 596 (23.2%) experienced grade ≥ 3 AEs and 326 (12.7%) experienced serious AEs. Grade ≥ 3 cardiac disorders were reported in 24 patients (0.9%), including congestive heart failure in eight (0.3%). As expected, the AE rates varied according to the timing of chemotherapy in both cohorts, with higher rates in concurrent versus sequential chemotherapy subgroups. In the concurrent chemotherapy subgroup, AEs were more common during the actual period of concurrent chemotherapy compared with the period when patients did not receive concurrent chemotherapy. Conclusion SafeHer confirms the safety and tolerability of the H SC 600 mg fixed dose for 1 year (every 3 weeks for 18 cycles) as adjuvant therapy with concurrent or sequential chemotherapy for HER2-positive EBC. These primary analysis results are consistent with the known safety profile for intravenous H and H SC.
Published: 2017

45. Iron-dependent erythropoiesis in women with excessive menstrual blood losses and women with normal menses

Author: Gaspare Carta, Maria Grazia Perilli, G. Mariani, Elvira Grandone, Giuseppe Celenza, Sergio Siragusa, Alberto Dolce, Mariasanta Napolitano, Assunta Orecchioni, Napolitano, M, Dolce, A, Celenza, G, Grandone, E, Perilli, MG, Siragusa, S, Carta,G, Orecchioni, A, and Mariani, G
Subjects: Adult, medicine.medical_specialty, Anemia, Iron, Iron deficient anemia, Physiology, Iron deficiency, Excessive mesntrual blood losses, Young Adult, Iron losses, Internal medicine, Humans, Medicine, Erythropoiesis, Prospective Studies, Young adult, Prospective cohort study, Menorrhagia, Menstrual blood, Gynecology, Hematology, Anemia, Iron-Deficiency, business.industry, Iron deficiency, Menstrual blood losses, General Medicine, Middle Aged, medicine.disease, Menstruation, Cohort, Female, business
Abstract: In women of fertile age, iron loss consequent to excessive menstrual discharge is by far the most frequent cause of iron-deficient anemia. However, the relationship between menstrual discharge and iron loss is poorly understood. In this prospective study, total menstrual and iron losses were assayed in a large cohort of non-anemic women and women with excessive menstrual blood losses (menorrhagia) in order to provide data useful for intervention. One hundred and five Caucasian women aged 20–45 years were recruited. Blood cell count and serum ferritin (SF) levels were determined in each case before menses. Menstrual fluid losses (MFL) were determined using a standardized pads’ weight method. Hematin concentration was assayed by a variant of the Alkaline Hematin Method from which iron concentration was calculated. Mean SF levels were 36.2 (range 8.6–100) ng/ml in healthy women and 6.4 (range 5–14) ng/ml in patients with menorrhagia. Median values of iron lost/cycle were 0.87 mg in healthy women and 5.2 mg in patients with menorrhagia (ranges 0.102–2.569 and 1.634–8.665 mg, respectively, p < 0.001). In women with menorrhagia, iron lost/cycle strongly correlated (0.789, p < 0.001) with MFL. In conclusion, healthy women with normal menses lose, on average, 1 mg iron/cycle. Average iron losses in patients with menorrhagia are, at least in our cohort, on average, five-to-six times higher than normal. Most women with menorrhagia had totally depleted iron stores. Indirect, quantitative evaluation of iron lost with menses may be useful to assess the risk of developing iron-deficient anemia in individual patients.
Published: 2013
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46. Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies

Author: Alberto Dolce, G. Mariani, Gili Kenet, Muriel Giansily-Blaizot, A. Ruiz De Sáez, Guenter Auerswald, Angelika Batorova, J.-F. Schved, Marie-Anne Bertrand, Ampaiwan Chuansumrit, Mehran Karimi, M. N. D. Di Minno, Roya Dolatkhah, Mariasanta Napolitano, Tahir Shamsi, Jørgen Ingerslev, Département d'hématologie biologique[Montpellier], Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-CHU Saint-Eloi, Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Napolitano, M., Di Minno, M., Batorova, A., Dolce, A., Giansily-Blaizot, M., Ingerslev, J., Schved, J., Auerswald, G., Kenet, G., Karimi, M., Shamsi, T., Ruiz de Sáez, A., Dolatkhah, R., Chuansumrit, A., Bertrand, M., Mariani, G., Napolitano, M, DI MINNO, Matteo, Giansily Blaizot, M., Schved, J. F., and Bertrand, M. A.
Subjects: Male, Pediatrics, Factor VII Deficiency, 030204 cardiovascular system & hematology, Cohort Studies, chemistry.chemical_compound, 0302 clinical medicine, Antifibrinolytic agent, gynaecological bleeding, Registries, Child, Genetics (clinical), Aged, 80 and over, Factor VII, Incidence (epidemiology), Hazard ratio, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Hematology, General Medicine, Middle Aged, Antifibrinolytic Agents, Recombinant Proteins, 3. Good health, Phenotype, Treatment Outcome, Child, Preschool, Cohort, Female, women, inherited factor VII deficiency, recombinant activated factor VII, Cohort study, Adult, medicine.medical_specialty, Adolescent, Mucocutaneous zone, Hemorrhage, Factor VIIa, Young Adult, 03 medical and health sciences, medicine, Humans, Menorrhagia, Aged, Proportional Hazards Models, Coagulants, business.industry, Proportional hazards model, Infant, Surgery, ROC Curve, chemistry, business, 030215 immunology
Abstract: Introduction A paucity of data exists on the incidence, diagnosis and treatment of bleeding in women with inherited factor VII (FVII) deficiency. Aim Here we report results of a comprehensive analysis from two international registries of patients with inherited FVII deficiency, depicting the clinical picture of this disorder in women and describing any gender-related differences. Methods A comprehensive analysis of two fully compatible, international registries of patients with inherited FVII deficiency (International Registry of Factor VII deficiency, IRF7; Seven Treatment Evaluation Registry, STER) was performed. Results In our cohort (N = 449; 215 male, 234 female), the higher prevalence of mucocutaneous bleeds in females strongly predicted ensuing gynaecological bleeding (hazard ratio = 12.8, 95% CI 1.68–97.6, P = 0.014). Menorrhagia was the most prevalent type of bleeding (46.4% of patients), and was the presentation symptom in 12% of cases. Replacement therapies administered were also analysed. For surgical procedures (n = 50), a receiver operator characteristic analysis showed that the minimal first dose of rFVIIa to avoid postsurgical bleeding during the first 24 hours was 22 μg kg−1, and no less than two administrations. Prophylaxis was reported in 25 women with excellent or effective outcomes when performed with a total weekly rFVIIa dose of 90 μg kg−1 (divided as three doses). Conclusion Women with FVII deficiency have a bleeding disorder mainly characterized by mucocutaneous bleeds, which predicts an increased risk of ensuing gynaecological bleeding. Systematic replacement therapy or long-term prophylaxis with rFVIIa may reduce the impact of menorrhagia on the reproductive system, iron loss and may avoid unnecessary hysterectomies.
Published: 2016
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47. Standardization of MRI and Scintigraphic Scores for Assessing the Severity of Bone Marrow Involvement in Adult Patients With Type 1 Gaucher Disease

Author: Simona Ortori, Marzio Perri, Silvia Linari, Fiorina Giona, Giuliano Mariani, Federica Guidoccio, Maria Domenica Cappellini, Fabrizio Minichilli, Paola Anna Erba, Maja Di Rocco, Mariani, G, Perri, M, Minichilli, F, Ortori, S, Linari, S, Giona, F, Di Rocco, M, Cappellini, M, Guidoccio, F, and Erba, P
Subjects: Adult, Male, Technetium Tc 99m Sestamibi, medicine.medical_specialty, bone marrow, Bone marrow infiltration, Gaucher disease, Scintigraphy, Severity of Illness Index, Pelvis, 030218 nuclear medicine & medical imaging, 03 medical and health sciences, symbols.namesake, 0302 clinical medicine, Cohen's kappa, medicine, Humans, scintigraphy, Radiology, Nuclear Medicine and imaging, Femur, Radionuclide Imaging, Retrospective Studies, Observer Variation, Lumbar Vertebrae, medicine.diagnostic_test, Adult patients, business.industry, Nonparametric statistics, Reproducibility of Results, Type 1 Gaucher Disease, General Medicine, Middle Aged, Magnetic Resonance Imaging, MRI, Bonferroni correction, medicine.anatomical_structure, 030220 oncology & carcinogenesis, symbols, Female, Radiology, Bone marrow, Nuclear medicine, business
Abstract: OBJECTIVE. MRI and 99mTc-sestamibi scintigraphy are used to estimate bone marrow infiltration in patients with Gaucher disease (GD), but comparison of data obtained at different institutions is difficult because different scores are employed for semiquantitative assessment. We developed normalized scores for comparing data both within a single method (MRI) and between different methods (MRI versus scintigraphy). MATERIALS AND METHODS. We evaluated 51 patients with type 1 GD (26 women, 25 men; mean age ± SD, 36.3 ± 10.9 years old). T1- and T2-weighted turbo spin-echo sequences at 1.5 T served to derive the bone marrow burden score (0-16), the vertebra-disk ratio (VDR), the Terk score (0-3), and the Spanish-MRI score (S-MRI, 0-24). Scintigraphy was scored between 0 and 8. Each score was normalized into four categories: 0 = normal, 1 = mild, 2 = intermediate, 3 = severe involvement. Interobserver and intraobserver agreements were evaluated by kappa statistics; nonparametric statistics with Bonferroni correction assessed correlations among the various original and normalized scores. RESULTS. Interobserver agreement was excellent for the original scores (κ = 0.730- 0.843) and even more so for the normalized scores (κ = 0.775-0.940). Intraobserver agreement kappa values ranged from 0.753 to 0.937 for the original scores and 0.851 to 1.000 for the normalized scores. Highly significant correlations were found among the various original scores (r = 0.42-0.86, p values between 0.0296 and < 0.0001), except for VDR versus S-MRI and Terk. Normalization generally induced marginal reductions in statistical significance, whereas S-MRI versus VDR reached statistical significance with the normalized scores. CONCLUSION. Our data indicate no significant loss of statistical information is caused by the normalization we employed. Our approach therefore facilitates comparison of different scores obtained in different institutions with different imaging modalities.
Published: 2016

48. Correlations between progression of coronary artery disease and circulating endothelial progenitor cells

Author: Cesare Peschle, Natalia V. Rivera, Eleonora Petrucci, Ugo Testa, Davide D’Andrea, Roberta Riccioni, Antonio Colombo, Gerolama Condorelli, Gianluigi Condorelli, Carlo Briguori, Francesca De Micco, Annibale Alessandro Puca, Gualtiero Mariani, Briguori, C., Testa, U., Riccioni, R., Colombo, A., Petrucci, E., Condorelli, Gerolama, Mariani, G., D'Andrea, Davide, De Micco, F., Rivera, N. V., Puca, A. A., Peschle, C., and Condorelli, G.
Subjects: Male, Vascular Endothelial Growth Factor A, medicine.medical_specialty, Coronary Artery Disease, Biochemistry, Cohort Studies, Colony-Forming Units Assay, Coronary artery disease, Risk Factors, Internal medicine, Genetics, Humans, Medicine, Progenitor cell, Molecular Biology, Cells, Cultured, business.industry, Stem Cells, Middle Aged, Flow Cytometry, Prognosis, medicine.disease, Pathophysiology, Survival Rate, ROC Curve, Disease Progression, cardiovascular system, Cardiology, Female, Endothelium, Vascular, business, Biotechnology, Cohort study
Abstract: The pathophysiology of coronary artery disease (CAD) progression is not well understood. Endothelial progenitor cells (EPCs) may have an important role. In the present observational cohort study we assessed the number of circulating EPCs in 136 patients undergoing elective percutaneous coronary intervention and who had at least one major epicardial vessel with a nonsignificant stenosis [15% DS increase of the objective vessel at follow-up. At 24 mo, 57 patients (42%) experienced significant progression. Independent predictors of disease progression were LDL cholesterol > 100 mg/dl (OR=1.03; 95% CI 1.01-1.04; P=0.001), low plasma levels of CFU-ECs (OR=3.99; 95% CI 1.54-10.37; P=0.005), and male sex (OR=3.42; 95% CI 1.15-10.22; P=0.027). Circulating levels of EPCs are significantly lower in patients with angiographic CAD progression.
Published: 2010
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49. Residual vein thrombosis to establish duration of anticoagulation after a first episode of deep vein thrombosis: the Duration of Anticoagulation based on Compression UltraSonography (DACUS) study

Author: Raffaela Anastasio, Sergio Siragusa, Maria Teresa Attanzio, Alessandra Casuccio, Valeria Cigna, Glauco Milio, Alessandra Malato, Alberto Dolce, Amato C, Guido Bajardi, Bellisi M, Oreste Cormaci, Massimo Pellegrino, Guglielmo Mariani, SIRAGUSA S, MALATO A, ANASTASIO R, CIGNA V, MILIO G, AMATO C, BELLISI M, ATTANZIO MT, CORMACI O, PELLEGRINO M, DOLCE A, CASUCCIO A, BAJARDI G, and MARIANI G
Subjects: Adult, Male, medicine.medical_specialty, medicine.drug_class, Deep vein, Immunology, Hemorrhage, Biochemistry, Drug Administration Schedule, Settore MED/15 - Malattie Del Sangue, Deep vein thrombosi, oral anticoagulant, Secondary Prevention, medicine, Humans, Aged, Ultrasonography, Venous Thrombosis, First episode, residual vein thrombosis, Vascular disease, business.industry, Anticoagulant, Hazard ratio, Anticoagulants, Cell Biology, Hematology, Middle Aged, medicine.disease, Settore MED/11 - Malattie Dell'Apparato Cardiovascolare, Thrombosis, Confidence interval, Surgery, Venous thrombosis, Treatment Outcome, medicine.anatomical_structure, Female, business
Abstract: Residual vein thrombosis (RVT) indicates a prothrombotic state and is useful for evaluating the optimal duration of oral anticoagulant treatment (OAT). Patients with a first episode of deep vein thrombosis, treated with OAT for 3 months, were managed according to RVT findings. Those with RVT were randomized to either stop or continue anticoagulants for 9 additional months, whereas in those without RVT, OAT was stopped. Outcomes were recurrent venous thromboembolism and/or major bleeding. Residual thrombosis was detected in 180 (69.8%) of 258 patients; recurrent events occurred in 27.2% of those who discontinued (25/92; 15.2% person-years) and 19.3% of those who continued OAT (17/88; 10.1% person-years). The relative adjusted hazard ratio (HR) was 1.58 (95% confidence interval [CI], 0.85-2.93; P = .145). Of the 78 (30.2%) patients without RVT, only 1 (1.3%; 0.63% person-years) had a recurrence. The adjusted HR of patients with RVT versus those without was 24.9 (95% CI, 3.4-183.6; P = .002). One major bleeding event (1.1%; 0.53% person-years) occurred in patients who stopped and 2 occurred (2.3%; 1.1% person-years) in those who continued OAT. Absence of RVT identifies a group of patients at very low risk for recurrent thrombosis who can safely stop OAT. This trial was registered at http://www.ClinicalTrials.gov as no. NCT00438230.
Published: 2008
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50. How do the elderly negotiate a step? A biomechanical assessment

Author: Giulia Adalgisa Mariani, Serena Maselli, Maria Grazia Benedetti, Lisa Berti, Sandro Giannini, Benedetti M.G., Berti L., Maselli S., Mariani G., and Giannini S.
Subjects: Adult, Male, Aging, medicine.medical_specialty, Population, Biophysics, Poison control, medicine, Humans, Orthopedics and Sports Medicine, Range of Motion, Articular, Muscle, Skeletal, education, Postural Balance, Aged, Aged, 80 and over, Leg, education.field_of_study, business.industry, medicine.disease, Trunk, Comorbidity, Biomechanical Phenomena, medicine.anatomical_structure, Climbing, Tripping, Physical therapy, Female, Ankle, business, Range of motion, human activities, Locomotion, Muscle Contraction
Abstract: Background Tripping over a raised surface is considered to be the most common cause of falls in the elderly. The aim of this study was to detect alterations of the motor pattern of elderly subjects while climbing a single step (“one step negotiation”) which may account for tripping and risk of falling. Methods We tested a sample of 32 “healthy” elderly subjects with a mean age of 72.4 years (SD 4.81; range 65–86). The control group consisted of 18 young subjects with a mean age of 26.5 years (SD 2.12; range 24–33). An experimental set-up for kinematic assessment while climbing a single step was provided. The elderly population was characterized clinically and functionally by a comprehensive geriatric assessment including information about comorbidity, disability, depression, motor, and muscular function. Findings Despite the high level of motor ability measured clinically, biomechanical analysis enabled us to demonstrate precise changes in step-climbing strategy in the elderly. A prolonged double stance phase duration, a greater anterior flexion of the trunk, a greater flexion of the hip, and a reduced dorsiflexion of the ankle were detected with respect to controls. All these factors and especially the latter could be determinants in the possible risk of tripping. Interpretation The biomechanical analysis performed on a population of healthy elderly subject has shown precise abnormalities of the trunk, hip and ankle kinematic during the motor task execution. This information can be of relevance in planning physical activity programs adapted for elderly and aimed at reducing the risk of tripping and falling.
Published: 2007
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