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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. 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

3. 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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4. 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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5. 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

6. 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. 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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

7. 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

8. 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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9. The prothrombin time/international normalized ratio (PT/INR) Line: derivation of local INR with commercial thromboplastins and coagulometers – two independent studies

Author

POLLER L, IBRAHIM S, KEOWN M, PATTISON A, JESPERSEN J, EUROPEAN ACTION ON A.N.T.I.C.O.A.G.U.L.A.T.I.O.N. COLLABORATORS: MARUN S, MUNTEAN W, ASPÖCK G, BAILLEUL E, WIJNS W, MICHEL T, JOCHMANS K, CHATELAIN B, MARCELIS L, NIJS AN, JOHNSTON M, KYNDE K, SYRÄJLÄ M, SAVOLAINEN ER, IVASKA K, RAJAMÄKI A, VANHARANTA R, JUHAN I, AILLAUD MF, GUILLIN MC, HUISSE MG, SIE P, LECOMPTE T, SCHMITT Y, HARENBERG J, PLESCH W, ARONIS S, THEODOSSIADES G, TSOUKANAS B, MARKALA D, DITSA M, CAHILL M, MADDEN M, FITZGERALD H, MCCARTHY D, COLLINS B, ROONEY S, NAPARSTEK E, MARONGIU F, ERBA N, TESTA S, MARIANI G, POTÌ R, MANNUCCI PM, TRIPODI A, BADER R, PENGO V, GRESELE P, D'INCÀ M, AGENO W, TOSETTO A, HENSGENS HE, IDEMA RN, OOSTERHOUT DH, MULDER AB, HAMULYAK K, VD DUSSEN H, MUSIAL J, GAGO T, PALMEIRO A, CAMPOS M, MARQUES DIAS DA, CUNHA MONTEIRO Á, ANGELA CUNHA M, DE GAIA WN, PETERNEL P, STEGNAR M, FONTCUBERTA J, BORRELL M, REVERTER JC, SANTASUSANA PD, LOPEZ FERNANDEZ MF, MONTERO J, RIBERA CASADO C, VICENTE V, EDLUND B, EGBERG N, LÄMMLE B, ALBERIO L, DE MOERLOOSE P, REBER G, MACHIN SJ, MACKIE I, LAWRIE A, LUCKIT J, SHANKS D, DOLAN G, FLAHERTY T, LUDLAM CA, WALKER ID, LAWSON DE, TRISCOTT M, MARLAR RA, HEIT JA, WATZKE H, WEISSER B, GALLUS AS, OSMOND J, CORNET I, MUNSTER AM, LEED B, CHILLOU C, DEFARD M, HORELLOU MH, HALKIN H, LOEBSTEIN R, KIRGNER I, DENTALI F, TRAPANI LOMBARDO V, SOTTILOTTA G, CONSIGLIO P, DE MICHELE S, MOIA M, CARPENEDO M, OLIVIERO B, MANOTTI C, TASSONI MI, PALARETI G, BRUSI C, LEGNANI C, PEGORARO C, ANDRIANI C, GRAZIA CIRIGLIANO M, RIBALDI E, ABBATE R, POLI D, PERRICONE C, SCHIAVULI M, CASIERA C, LUPONE MR, ROSSI E, CRIVELLI S, BIROLINI A, PARENTE F, MIGLIETTA AM, CAMPOBASSO M, SYDOR W, KREVEL B, FERNANDEZ MA, LOZANO M, SHIACH C, BOWYER C, CHARLES F., PALARETI, GUALTIERO, COSMI, BENILDE, POLLER L, IBRAHIM S, KEOWN M, PATTISON A, JESPERSEN J, EUROPEAN ACTION ON ANTICOAGULATION. COLLABORATORS: MARUN S, MUNTEAN W, ASPÖCK G, BAILLEUL E, WIJNS W, MICHEL T, JOCHMANS K, CHATELAIN B, MARCELIS L, NIJS AN, JOHNSTON M, KYNDE K, SYRÄJLÄ M, SAVOLAINEN ER, IVASKA K, RAJAMÄKI A, VANHARANTA R, JUHAN I, AILLAUD MF, GUILLIN MC, HUISSE MG, SIE P, LECOMPTE T, SCHMITT Y, HARENBERG J, PLESCH W, ARONIS S, THEODOSSIADES G, TSOUKANAS B, MARKALA D, DITSA M, CAHILL M, MADDEN M, FITZGERALD H, MCCARTHY D, COLLINS B, ROONEY S, NAPARSTEK E, PALARETI G, MARONGIU F, ERBA N, TESTA S, MARIANI G, POTÌ R, MANNUCCI PM, TRIPODI A, BADER R, PENGO V, GRESELE P, D'INCÀ M, AGENO W, TOSETTO A, HENSGENS HE, IDEMA RN, OOSTERHOUT DH, MULDER AB, HAMULYAK K, VD DUSSEN H, MUSIAL J, GAGO T, PALMEIRO A, CAMPOS M, MARQUES-DIAS DA, CUNHA MONTEIRO Á, ANGELA CUNHA M, DE GAIA WN, PETERNEL P, STEGNAR M, FONTCUBERTA J, BORRELL M, REVERTER JC, SANTASUSANA PD, LOPEZ-FERNANDEZ MF, MONTERO J, RIBERA CASADO C, VICENTE V, EDLUND B, EGBERG N, LÄMMLE B, ALBERIO L, DE MOERLOOSE P, REBER G, MACHIN SJ, MACKIE I, LAWRIE A, LUCKIT J, SHANKS D, DOLAN G, FLAHERTY T, LUDLAM CA, WALKER ID, LAWSON DE, TRISCOTT M, MARLAR RA, HEIT JA, WATZKE H, WEISSER B, GALLUS AS, OSMOND J, CORNET I, MUNSTER AM, LEED B, CHILLOU C, DEFARD M, HORELLOU MH, HALKIN H, LOEBSTEIN R, KIRGNER I, DENTALI F, TRAPANI LOMBARDO V, SOTTILOTTA G, CONSIGLIO P, DE MICHELE S, MOIA M, CARPENEDO M, OLIVIERO B, MANOTTI C, TASSONI MI, COSMI B, BRUSI C, LEGNANI C, PEGORARO C, ANDRIANI C, GRAZIA CIRIGLIANO M, RIBALDI E, ABBATE R, POLI D, PERRICONE C, SCHIAVULI M, CASIERA C, LUPONE MR, ROSSI E, CRIVELLI S, BIROLINI A, PARENTE F, MIGLIETTA AM, CAMPOBASSO M, SYDOR W, KREVEL B, FERNANDEZ MA, LOZANO M, SHIACH C, BOWYER C, CHARLES F., Interne Geneeskunde, Biochemie, and RS: CARIM School for Cardiovascular Diseases

Subjects
INR correction, endocrine system, medicine.medical_specialty, coagulometers, Prothrombin Time/*standard, Reproducibility of Result, PT, World Health Organization, International Normalized Ratio/*standard, Thromboplastin, Automation, Laboratory/standard, ECAA plasmas, Predictive Value of Tests, health services administration, medicine, Animals, Humans, heterocyclic compounds, International Normalized Ratio, cardiovascular diseases, thromboplastins, Blood Coagulation, Reference standards, Mathematics, Automation, Laboratory, Observer Variation, Prothrombin time, Analysis of Variance, medicine.diagnostic_test, business.industry, fungi, Reproducibility of Results, Hematology, Reference Standards, Surgery, INR Line, Multicenter study, Calibration, Linear Models, Prothrombin Time, Cattle, Rabbits, Observer variation, Nuclear medicine, business
Abstract

The WHO scheme for prothrombin time (PT) standardization has been limited in application, because of its difficulties in implementation, particularly the need for mandatory manual PT testing and for local provision of thromboplastin international reference preparations (IRP).The value of a new simpler procedure to derive international normalized ratio (INR), the PT/INR Line, based on only five European Concerted Action on Anticoagulation (ECAA) calibrant plasmas certified by experienced centres has been assessed in two independent exercises using a range of commercial thromboplastins and coagulometers. INRs were compared with manual certified values with thromboplastin IRP from expert centres and in the second study also with INRs from local ISI calibrations.In the first study with the PT/INR Line, 8.7% deviation from certified INRs was reduced to 1.1% with human reagents, and from 7.0% to 2.6% with rabbit reagents. In the second study, deviation was reduced from 11.2% to 0.4% with human reagents by both local ISI calibration and the PT/INR Line. With rabbit reagents, 10.4% deviation was reduced to 1.1% with both procedures; 4.9% deviation was reduced to 0.5% with bovine/combined reagents with local ISI calibrations and to 2.9% with the PT/INR Line. Mean INR dispersion was reduced with all thromboplastins and automated systems using the PT/INR Line.The procedure using the PT/INR Line provides reliable INR derivation without the need for WHO ISI calibration across the range of locally used commercial thromboplastins and automated PT systems included in two independent international studies.? 2010 International Society on Thrombosis and Haemostasis.

Published
2011
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10. Inhibitors to factor VII in congenital factor VII deficiency

Author

Batorova, A, Mariani, G, Kavakli, K, de Saez AR, Caliskan, U, Karimi, M, Pinotti, Mirko, Napolitano, M, Dolce, A, Sørensen, B, Ingerslev, J, STER Study Group, Batorova A, Mariani G, Kavakli K, de Saez AR, Caliskan U, Karimi M, Pinotti M, Napolitano M, Dolce A, Sørensen B, and Ingerslev J.

Subjects
medicine.medical_specialty, Pediatrics, Factor VII, business.industry, Inhibitors, Factor VII Deficiency, MEDLINE, rVIIa, Hematology, General Medicine, Inhibitors, Factor VII deficiency, Gastroenterology, Antibodies, chemistry.chemical_compound, chemistry, Internal medicine, FVII deficiency, medicine, Humans, Factor VII deficiency, business, Genetics (clinical)
Published
2014

11. 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

12. 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

13. 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

14. 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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15. Factors Influencing Depression Endpoints Research (FINDER): baseline results of Italian patients with depression

Author

Grassi, L, Rossi, A, Barraco, A, Italian Finder Group, Aguglia, E, Ambrosio, LA, Rossi, NB, Bellomo, A, Belloni, G, Biza, M, Bucci, N, Cappuccio, R, Carboni, MG, Cesari, G, Ciappi, F, Cipriani, AM, Cordioli, L, Delcuratolo, V, Di Cello, A, Di Fiorino, M, Di Lauro, A, Falabella, V, Falavolti, S, Farina, G, Federico, T, Gabrielli, F, Gazzera, G, Mariani, G, Minnai, G, Nano, D, Nicolo, G, Parnetti, L, Pierri, G, Puoti, M, Riccio, A, Romeo, A, Veneto, V, Lugo, I, Prodi, PR, Serrano, M, Sorbi, S, Toniolo, E, Venanzini, R, Vender, S, Venuta, M, Volpe, M., FERRARESE, CARLO, Grassi, L, Rossi, A, Barraco, A, Italian Finder, G, Aguglia, E, Ambrosio, L, Rossi, N, Bellomo, A, Belloni, G, Biza, M, Bucci, N, Cappuccio, R, Carboni, M, Cesari, G, Ciappi, F, Cipriani, A, Cordioli, L, Delcuratolo, V, Di Cello, A, Di Fiorino, M, Di Lauro, A, Falabella, V, Falavolti, S, Farina, G, Federico, T, Ferrarese, C, Gabrielli, F, Gazzera, G, Mariani, G, Minnai, G, Nano, D, Nicolo, G, Parnetti, L, Pierri, G, Puoti, M, Riccio, A, Romeo, A, Veneto, V, Lugo, I, Prodi, P, Serrano, M, Sorbi, S, Toniolo, E, Venanzini, R, Vender, S, Venuta, M, and Volpe, M

Subjects
Sertraline, medicine.medical_specialty, education.field_of_study, Visual analogue scale, business.industry, lcsh:RC435-571, Population, Depression Outcome, Depression, HRQoL, Hospital Anxiety and Depression Scale, Psychiatry and Mental health, Psychiatric history, lcsh:Psychiatry, depression, medicine, Escitalopram, Anxiety, medicine.symptom, Primary Research, business, Psychiatry, education, Depression (differential diagnoses), medicine.drug
Abstract

Background Factors Influencing Depression Endpoints Research (FINDER) is a 6-month, prospective, observational study carried out in 12 European countries aimed at investigating health-related quality of life (HRQoL) in outpatients receiving pharmacological treatment for a first or new depressive episode. Baseline characteristics of patients enrolled in Italy are presented. Methods All treatment decisions were at the discretion of the investigator. Data were collected at baseline and after 3 and 6 months of treatment. Baseline evaluations included demographics, medical and psychiatric history, and medications used in the last 24 months and prescribed at enrolment. The Hospital Anxiety and Depression Scale (HADS), was adopted to evaluate depressive symptoms, while somatic and painful physical symptoms were assessed by using the Somatic Symptom Inventory (SSI) and a 0 to 100 mm visual analogue scale (VAS), HRQoL via 36-item Short Form Health Survey (SF-36), and the European Quality of Life 5-Dimensions (EQ-5D) instrument. Results A total of 513 patients were recruited across 38 sites. The mean ± standard deviation (SD) age at first depressive episode was 38.7 ± 15.9 years, the mean duration of depression 10.6 ± 12.3 years. The most common psychiatric comorbidities in the previous 24 months were anxiety/panic (72.6%) and obsessive/compulsive disorders (13.4%), while 35.9% had functional somatic syndromes. Most patients (65.1%) reported pain from any cause. Monotherapy with selective serotonin reuptake inhibitors (SSRIs) and tricyclic antidepressants (TCAs) was prescribed at enrolment in 64.5% and 6.4% of the cases, respectively. The most commonly prescribed agents were sertraline (17.3%), escitalopram (16.2%), venlaflaxine (15.6%) and paroxetine (14.8%). The mean HADS subscores for depression and anxiety were 13.3 ± 4.2 and 12.2 ± 3.9, respectively; 76.4% of patients could be defined as being 'probable cases' for depression and 66.2% for anxiety. The mean total score of VAS-pain in the last week was 42.9 ± 27.1, with highest scores reported in the 'interference of pain with daily activities' and in 'amount of time patient was awake and had pain'. From SF-36, the worst health status was found for role limitations due to emotional problem, mental health and social functioning. A mean score < 50 (that is, below the standardised population norm) was also found in all remaining domains. The SF-36 summary scores and EQ-5D (health status and VAS) were lower in patients with moderate/severe pain than in those with no or mild pain. Conclusion The baseline results of patients enrolled in the FINDER study in Italy show clinical and functional impairments, and poor HRQoL. The results obtained after 6 months of therapy will permit better understanding the effects of different variables on clinical outcomes and HRQoL.

Published
2009

16. Comparison of the rates of joint arthroplasty in patients with severe factor VIII and IX deficiency: an index of different clinical severity of the 2 coagulation disorders

Author

Tagariello G, Iorio A, Santagostino E, Morfini M, Bisson R, Innocenti M, Mancuso ME, Mazzucconi MG, Pasta GL, Radossi P, Rodorigo G, Santoro C, Sartori R, Scaraggi A, Solimeno LP, Mannucci PM, Ciavarella N, Valdrè L, Targhetta R, Biasioli C, Vincenzi D, Molinari C, Boeri E, Mariani G, Lapecorella M, Coppola A, Schiavulli M, Rocino A, Mancuso G, Siragusa S, Malato A, Tagliaferri A, Rivolta F, Oliovecchio E, Marcucci M, Dragani A, Mancino A, D'Incà M, De Rossi G, Luciani M, Landolfi R, Piseddu G, Schinco PC, Rossetti G, Barillari G, Feola G, Gandini G, Giuffrida A, Castaman G., DI MINNO, GIOVANNI, Tagariello, G, Iorio, A, Santagostino, E, Morfini, M, Bisson, R, Innocenti, M, Mancuso, ME, Mazzucconi, MG, Pasta, GL, Radossi, P, Rodorigo, G, Santoro, C, Sartori, R, Scaraggi, A, Solimeno, LP, Mannucci, PM, Ciavarella, N, Valdrè, L, Targhetta, R, Biasioli, C, Vincenzi, D, Molinari, C, Boeri, E, Mariani, G, Lapecorella, M, di Minno, G, Coppola, A, Schiavulli, M, Rocino, A, Mancuso, G, Siragusa, S, Malato, A, Tagliaferri, A, Rivolta, F, Oliovecchio, E, Marcucci, M, Dragani, A, Mancino, A, D'Incà, M, De Rossi, G, Luciani, M, Landolfi, R, Piseddu, G, Schinco, PC, Rossetti, G, Barillari, G, Feola, G, Gandini, G, Giuffrida, A, Castaman, G, Mancuso, Me, Mazzucconi, Mg, Pasta, Gl, Solimeno, Lp, Mannucci, Pm, DI MINNO, Giovanni, Schinco, Pc, and Castaman, G.

Subjects
Adult, medicine.medical_specialty, Adolescent, medicine.medical_treatment, Immunology, Population, macromolecular substances, Gene mutation, Hemophilia A, Hemophilia B, Severity of Illness Index, Biochemistry, Arthroplasty, Settore MED/15 - Malattie Del Sangue, Cohort Studies, Young Adult, Risk Factors, hemic and lymphatic diseases, Internal medicine, Severity of illness, medicine, Coagulopathy, Humans, Child, education, Retrospective Studies, education.field_of_study, Hematology, hamophilia, arthroplasty, business.industry, Retrospective cohort study, Cell Biology, Odds ratio, Blood Coagulation Disorders, Middle Aged, medicine.disease, Surgery, Italy, Joint Diseases, business, Algorithms
Abstract

Data from the Italian Hemophilia Centres were collected to perform a retrospective survey of joint arthroplasty in patients with severe hemophilia. Twenty-nine of 49 hemophilia centers reported that 328 of the 347 operations were carried out in 253 patients with severe hemophilia A (HA) and 19 in 15 patients with severe hemophilia B (HB). When results were normalized to the whole Italian hemophilia population (1770 severe HA and 319 severe HB), patients with HA had a 3-fold higher risk of undergoing joint arthroplasty (odds ratio [OR], 3.38; 95% confidence interval [CI], 1.97-5.77; P < .001). These results were confirmed after adjustment for age, HIV, hepatitis C virus (HCV), and inhibitor in a Cox regression model (HR, 2.65; 95% CI, 1.62-4.33; P < .001). The survival analysis of time to joint arthroplasty in the subset of patients with severe HA was not affected by the severity of factor VIII (FVIII) gene mutations. A systematic review of literature articles reporting joint arthroplasties in HA and HB showed that the proportion of HA patients who had undergone arthroplasties was higher than that of HB patients, in agreement with the findings in our Italian cohort. These data suggest that the 2 inherited coagulation disorders have a different severity of clinical phenotype.

Published
2009

17. 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

18. 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

19. 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

20. 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

21. Outbreak of hepatitis A among Italian patients with haemophilia

Author

Gringeri, A., Colombo, M., Zanetti, A., Rodeghiero, F., Tagariello, G., Gamba, G., De Rosa, V., Morfini, M., Berrettini, M., Mariani, G., Mazzucconi, Maria Gabriella, and Schinaia, N.

Subjects
Pediatrics, medicine.medical_specialty, business.industry, MEDLINE, Hepatitis A, Outbreak, General Medicine, medicine.disease, Haemophilia, Hemophilia A, Disease Outbreaks, Italy, Immunology, medicine, Coagulopathy, Humans, Viral disease, Complication, business
Published
1992

22. Serum Creatinine (sCr) Changes Induced by Hemodialysis (HD) with Relation to the Plasma Volume/Hematocrit Ratio (PV/Ht)

Author

G. Fanara, Mariani G, M. Gonella, and Maccheroni M

Subjects
Creatinine, medicine.medical_specialty, medicine.diagnostic_test, 030232 urology & nephrology, Biomedical Engineering, Medicine (miscellaneous), Bioengineering, General Medicine, 030204 cardiovascular system & hematology, Hematocrit, Plasma volume, Biomaterials, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Endocrinology, chemistry, Internal medicine, medicine, Hemodialysis hd
Published
1982
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23. 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

24. 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

25. Prolonged prothrombin time, Factor VII and activated FVII levels in chronic liver disease are partly dependent on Factor VII gene polymorphisms

Author

T. Di Paolantonio, Alessandra Vaccaro, S. Gentile, Stefania Grimaudo, Guglielmo Mariani, Lucio Lo Coco, Antonio Craxì, F. Capone, G. Venezia, A. Usticano, R. Savella, Grimaudo, S, Craxi, A, Gentile, S, Di Paolantonio, T, Vaccaro, A, Venezia, G, Lo Coco, L, Savella, R, Usticano, A, Capone, F, Mariani, G, Gentile, Sandro, and Mariani, G.

Subjects
Liver Cirrhosis, medicine.medical_specialty, Cirrhosis, Genotype, Liver Cirrhosi, Fibrinogen, Chronic liver disease, chemistry.chemical_compound, hemic and lymphatic diseases, Internal medicine, medicine, Humans, Allele, Allele frequency, Alleles, Hepatitis, Chronic, Prothrombin time, Polymorphism, Genetic, Hepatology, Factor VII, medicine.diagnostic_test, business.industry, Gastroenterology, medicine.disease, Endocrinology, chemistry, Case-Control Studies, Prothrombin Time, business, Case-Control Studie, medicine.drug, Human
Abstract

Background. Prothrombin time is a benchmark for functional assessment in cirrhosis and Factor VII levels (FVII), crucial in determining the prothrombin time, are genetically determined. Methods. We have evaluated the prothrombin time, a number of haemostatic variables synthesised by the liver (FII, FV, FVII and activated FVII, AT and fibrinogen) and two polymorphisms of the FVII gene (5′F7 and 353R/Q) in: (a) patients with liver cirrhosis ( n = 118), (b) patients with chronic hepatitis ( n = 102) and (c) controls ( n = 100). Results. By one-way analyses of variance, the prothrombin time and the mean levels of the FII, FV, FVIIc, FVIIa, and AT were statistically different between cirrhotics, chronic hepatitis patients and controls. The allele frequency of the FVII polymorphisms did not differ between the three groups. Those rare patients (4.6%) who were homozygous for the type 2 alleles had markedly reduced FVIIc and FVIIa levels. The analysis carried out taking into account Child class versus FVII genotype showed that the mean FVIIc levels were comparable for different genotypes within each Child's class, with the exception of the patients homozygous for the type 1 allele. Conclusion. Our findings help to explain the not infrequent finding of a severely prolonged prothrombin time in patients who are otherwise in a good functional class.

Published
2005

26. (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

27. Nuclear Medicine Imaging of Non-orthopedic or Cardiovascular Implantable Device Infection

Author

Francesco Bartoli, Martina Sollini, Paola Anna Erba, Roberta Zanca, Lazzeri, E, Signore, A, Erba, PA, Prandini, N, Versari, A, D´Errico, G, Mariani, G, Erba, P, Bartoli, F, Zanca, R, and Sollini, M

Subjects
medicine.medical_specialty, education.field_of_study, Implantable device-related infection, business.industry, Hospitalized patients, PET/CT, Biofilm, Population, Nosocomial pathogens, Biomarker, SPECT/CT, Diagnostic strategy, Scintigraphy with labeled autologous leukocyte, Treatment monitoring, Nuclear medicine imaging, Orthopedic surgery, medicine, Population Risk, [18F]FDG, Intensive care medicine, education, business, Host factor
Abstract

The use of implantable medical devices has increased in “modern” medical and surgical practice, and the rate of their infections is variable, depending on the type of device and the population risk profile. The overall risk of device-related infection across the population is around 1%, but it might be significantly increased in specific populations as vulnerability to microbial pathogens increases in elderly, immuno-compromised, and hospitalized patients. Device-related infection results from the multifaceted interaction of bacterial, device, and host factor. Multiresistant nosocomial pathogens are the most common organisms colonizing the surface of devices, and a full understanding of how a biofilm formation is crucial to understand the pathogenesis of their infection and define the most suitable treatment. This chapter describes the most relevant implantable medical devices of interest to nuclear medicine and the corresponding diagnostic strategy for each of them with particular regard to the role of nuclear medicine procedure in the diagnostic algorithm.

Published
2021

28. Comparison of MRI, [18F]FDG PET/CT, and 99mTc-UBI 29-41 scintigraphy for postoperative spondylodiscitis—a prospective multicenter study

Author

Rodolfo Nunez, Shazia Fatima, Giuliano Mariani, Sunil Puri, Anil Dhal, Diana Paez, Mike Sathekge, Olivier Pellet, Paola Anna Erba, Francesco Giammarile, Rodolfo Ferrando, Paulo Almeida Filho, Hassan Douis, Elena Lazzeri, Olga Morozova, Vincent Peter Magboo, Paez, D, Sathekge, M, Douis, H, Giammarile, F, Fatima, S, Dhal, A, Puri, S, Erba, P, Lazzeri, E, Ferrando, R, Filho, P, Magboo, V, Morozova, O, Nunez, R, Pellet, O, and Mariani, G

Subjects
Spondylodiscitis, [18F]FDG PET/CT, 99m, medicine.medical_specialty, Discitis, Scintigraphy, Sensitivity and Specificity, 030218 nuclear medicine & medical imaging, Tc-UBI 29-41, 03 medical and health sciences, 0302 clinical medicine, Spine surgery, Fluorodeoxyglucose F18, Positron Emission Tomography Computed Tomography, Biopsy, medicine, Medical imaging, Humans, Radiology, Nuclear Medicine and imaging, Prospective Studies, Radionuclide Imaging, medicine.diagnostic_test, Diagnostic imaging, MRI, Postoperative spine infection, SPECT/CT with, [, 18, F]FDG PET/CT, business.industry, General Medicine, medicine.disease, Magnetic Resonance Imaging, Multicenter study, Orthopedic surgery, Original Article, Fdg pet ct, Radiopharmaceuticals, Complication, Nuclear medicine, business, SPECT/CT with 99mTc-UBI 29-41, 030217 neurology & neurosurgery
Abstract

Purpose Postoperative infection still constitutes an important complication of spine surgery, and the optimal imaging modality for diagnosing postoperative spine infection has not yet been established. The aim of this prospective multicenter study was to assess the diagnostic performance of three imaging modalities in patients with suspected postoperative spine infection: MRI, [18F]FDG PET/CT, and SPECT/CT with 99mTc-UBI 29-41. Methods Patients had to undergo at least 2 out of the 3 imaging modalities investigated. Sixty-three patients enrolled fulfilled such criteria and were included in the final analysis: 15 patients underwent all 3 imaging modalities, while 48 patients underwent at least 2 imaging modalities (MRI + PET/CT, MRI + SPECT/CT, or PET/CT + SPECT/CT). Final diagnosis of postoperative spinal infection was based either on biopsy or on follow-up for at least 6 months. The MRI, PET/CT, and SPECT/CT scans were read blindly by experts at designated core laboratories. Spine surgery included metallic implants in 46/63 patients (73%); postoperative spine infection was diagnosed in 30/63 patients (48%). Results Significant discriminants between infection and no infection included fever (P = 0.041), discharge at the wound site (P < 0.0001), and elevated CRP (P = 0.042). There was no difference in the frequency of infection between patients who underwent surgery involving spinal implants versus those who did not. The diagnostic performances of MRI and [18F]FDG PET/CT analyzed as independent groups were equivalent, with values of the area under the ROC curve equal to 0.78 (95% CI: 0.64–0.92) and 0.80 (95% CI: 0.64–0.98), respectively. SPECT/CT with 99mTc-UBI 29-41 yielded either unacceptably low sensitivity (44%) or unacceptably low specificity (41%) when adopting more or less stringent interpretation criteria. The best diagnostic performance was observed when combining the results of MRI with those of [18F]FDG PET/CT, with an area under the ROC curve equal to 0.938 (95% CI: 0.80–1.00). Conclusion [18F]FDG PET/CT and MRI both possess equally satisfactory diagnostic performance in patients with suspected postoperative spine infection, the best diagnostic performance being obtained by combining MRI with [18F]FDG PET/CT. The diagnostic performance of SPECT/CT with 99mTc-UBI 29-41 was suboptimal in the postoperative clinical setting explored with the present study.

Published
2020

29. 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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30. Hybrid Imaging and Radionuclide Therapy of Musculoskeletal Diseases

Author

Lesley Flynt, Giuliano Mariani, Elena Lazzeri, Martina Sollini, Roberta Zanca, Roberto Boni, Paola Anna Erba, Torsten Kuwert, Duccio Volterrani, Paola Anna Erba, Ignasi Carrió, H. William Strauss, Giuliano Mariani, Erba, P, Sollini, M, Zanca, R, Boni, R, Flynt, L, Lazzeri, E, Mariani, G, and Kuwert, T

Subjects
Pathology, medicine.medical_specialty, Planar Imaging, MSK, hybrid imaging, medicine.diagnostic_test, business.industry, Connective tissue, Bone tissue, Bone remodeling, Extracellular matrix, medicine.anatomical_structure, Bone scintigraphy, Radionuclide therapy, medicine, Bone marrow, business
Abstract

Bone tissue is a specialized form of connective tissue, to which mineralization of the extracellular matrix confers remarkable hardness and strength. It fulfills three main functions

Published
2019

31. Hybrid Imaging and Radionuclide Therapy in Hemato-oncology

Author

Paola Anna Erba, Roberto Boni, Sara Galimberti, Martina Sollini, Volterrani, D, Erba, PA, Carrió, I, Strauss, HW, Mariani, G, Erba, P, Sollini, M, Boni, R, and Galimberti, S

Subjects
Pathology, medicine.medical_specialty, business.industry, T cell, Cancer, Plasma cell, medicine.disease, Lymphoma, medicine.anatomical_structure, immune system diseases, hemic and lymphatic diseases, Radionuclide therapy, Haematological disease, hybrid imaging, lymphoma, leukemia, MM, medicine, Bone marrow, business, Monoclonal gammopathy of undetermined significance, B cell
Abstract

Lymphomas are hematologic malignancies characterized by abnormal proliferation of lymphoid cells, most commonly arising in lymph nodes, but potentially involving any organ or tissue in the body. Several classification systems have been proposed for lymphomas. Classification of a lymphoma under a certain category can affect treatment and prognosis. Common features considered for classifying lymphomas generally include: (i) whether or not it is a Hodgkin lymphoma; (ii) whether the abnormally replicating cell is a T cell or B cell; and (iii) site where the malignant cell arises.Multiple myeloma (MM) is the most common primary bone cancer. The clonal proliferation of malignant plasma cells in the bone marrow may result both in local growth and in systemic effects due to the overproduction of a monoclonal protein (M-protein). Almost all patients evolve from an asymptomatic premalignant stage, termed monoclonal gammopathy of undetermined significance (MGUS), through the smoldering myeloma (SMM), and eventually to “malignant” MM. MM differs from MGUS and SMM by the presence of end-organ damage associated with a complex syndrome named CRAB. MM, MGUS and smoldering myeloma (SMM) are defined according to the updated version of the criteria for the diagnosis of plasma cell proliferative disorders established by the International Myeloma Working Group.

Published
2019
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32. Hybrid Imaging of the Peripheral Lymphatic System

Author

Roberto Boni, Andrea Marciano, Giuliano Mariani, Martina Sollini, Rossella Di Stefano, Paola Anna Erba, Volterrani, D, Erba, PA, Carrió, I, Strauss, HW, Mariani, G, Erba, P, Boni, R, Sollini, M, Marciano, A, and Di Stefano, R

Subjects
Pathology, medicine.medical_specialty, business.industry, government.form_of_government, Cisterna chyli, Spleen, Thoracic duct, Lymphatic Endothelium, medicine.anatomical_structure, Lymphatic system, medicine, government, Bone marrow, Lymph, business, Lymph node, Lymphatic system, lymphoscintigraphy, hybrid imaging
Abstract

The lymphatic circulation is part of the peripheral circulation, linking blood circulation at its origins (the interstitial space) and at its final drainage point (the thoracic duct). The lymphatic system includes fluid (lymph), the lymphatic vessels, the lymph nodes (stations along the drainage route where fluid and cell exchange between blood and lymph occur), and other lymphoid tissue, particularly the spleen and bone marrow. The lymphatic system has a significant role in maintaining the balance of tissue fluid volume and an appropriate cellular chemical environment (the capillary plasma filtration process). An important role of the lymphatic circulation is the transport of the antigen and immune cells from peripheral tissues to lymph nodes. The interaction between lymphatic endothelial cells and leukocytes controls the immune cell migration and immune responses by regulating lymphatic flow and various secreted molecules such as chemokines and cytokines in tissue. Components of the lymphatic system include cells, vessels, and fluids. The lymphatic system is integrated with the hematopoietic and neurohormonal systems and can be divided into peripheral (from the interstitial space to and within the nearest lymph node) and central (efferent lymphatics, cisterna chyli, and thoracic duct, all lymphatic organs).

Published
2019

33. 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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34. 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

35. 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

36. Diagnostic Applications of Nuclear Medicine: Multiple Myeloma

Author

Martina Sollini, Sara Galimberti, Paola Anna Erba, Roberto Boni, Strauss, HW, Mariani, G, Volterrani, D, Larson, SM, Sollini, M, Galimberti, S, Boni, R, and Erba, P

Subjects
Oncology, medicine.medical_specialty, Hematological malignancy, Multiple myeloma, business.industry, Internal medicine, Multimodality imgaging, medicine, business, medicine.disease, Bone tumor
Abstract

Multiple myeloma represents about 1.8% of all cancers and 10% of all hematologic malignancies. Multiple myeloma (MM) is the most common primary bone cancer. The clonal proliferation of malignant plasma cells in the bone marrow may result both in local growth and in systemic effects due to the overproduction of a monoclonal protein (M-protein). Plasma cell proliferation is linked to a variety of clinical presentations of the disease, ranging from simple monoclonal gammopathy of undetermined significance (MGUS) to smoldering myeloma (SMM) to full-blown "malignant" MM. MM differs from MGUS and SMM by the presence of the end-organ damage associated with a complex syndrome named CRAB. However, in the updated version of the criteria for the diagnosis of plasma cell proliferative disorders established by the International Myeloma Working Group (IMWG), more specific criteria have been established to define MM, MGUS, and SMM. Excess bone marrow plasmacells, M-protein, osteolytic bone lesions, renal disease, and immunodeficiency constitute the pathophysiologic bases of the clinical manifestations of MM. Severe bone pain, pathologic fractures, spinal cord compression, and hypercalcemia are caused by lytic bone lesions. The course of MMis highly variable, and the clinical behavior is remarkably heterogeneous. Many studies have identified prognostic factors capable of predicting this heterogeneity in survival (serum β2-microglobulin, albumin, C-reactive protein, and lactate dehydrogenase). The standard workup of MM is based on a number of laboratory tests that are utilized for risk stratification. The International Staging System (ISS) is a powerful and reproducible three-stage classification in which the ISS3 class is associated with the poorest outcome. Imaging studies demonstrate the extent and severity of bone involvement (intramedullary/extramedullary, site and number of lesions) at baseline, including disease- related complications, such as pathologic fractures; they also serve to assess response to treatment and provide follow-up surveillance. The ISS introduced over 25 years ago is mainly based on serum β2-microglobulin and albumin levels, without any reference to the presence of bone lesions or to the methodology employed for their evaluation. Although the ISS serves as a prognostic indicator rather than as an accurate scoring system, it adequately estimates tumor burden and risk stratification, allowing differentiation of MGUS and SMM from MM. In 1975 Durie and Salmon introduced a clinical staging system based on the presence of bone lesions to grade the severity of the disease. While the original Durie and Salmon system was essentially based on the use of planar x-ray for evaluating bones, a newer version, the PLUS system was released in 2005 to improve the accuracy of staging with advanced imaging modalities such as [18F]FDG-PET/CT, and MRI.

Published
2017
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37. Factor VII Deficiency: Clinical Phenotype, Genotype and Therapy

Author

Mariasanta Napolitano, Sergio Siragusa, G. Mariani, Napolitano, M, Siragusa,S, and Mariani,G.

Subjects
Pediatrics, medicine.medical_specialty, Gastrointestinal bleeding, Pathology, Genotype, diagnosis, Inherited Factor VII derficiency, Disease, Review, 030204 cardiovascular system & hematology, Asymptomatic, 03 medical and health sciences, 0302 clinical medicine, Repalcement Therapy, replacement therapy, medicine, Factor VII deficiency, Clinical phenotype, business.industry, Bleeding, General Medicine, Plasma levels, medicine.disease, Phenotype, inherited Factor VII deficiency, medicine.symptom, business, 030215 immunology
Abstract

Factor VII deficiency is the most common among rare inherited autosomal recessive bleeding disorders, and is a chameleon disease due to the lack of a direct correlation between plasma levels of coagulation Factor VII and bleeding manifestations. Clinical phenotypes range from asymptomatic condition—even in homozygous subjects—to severe life-threatening bleedings (central nervous system, gastrointestinal bleeding). Prediction of bleeding risk is thus based on multiple parameters that challenge disease management. Spontaneous or surgical bleedings require accurate treatment schedules, and patients at high risk of severe hemorrhages may need prophylaxis from childhood onwards. The aim of the current review is to depict an updated summary of clinical phenotype, laboratory diagnosis, and treatment of inherited Factor VII deficiency.

Published
2017

38. Diagnostic applications of nuclear medicine: Leukemias

Author

Martina Sollini, Roberto Boni, Sara Galimberti, Paola Anna Erba, Strauss, HW, Mariani, G, Volterrani, D, Larson, SM, Sollini, M, Galimberti, S, Boni, R, and Erba, P

Subjects
medicine.medical_specialty, Leukemia, business.industry, Bone cancer, Medicine, Medical physics, Hematological malignancie, business, Imaging
Abstract

Leukemias are a group of acute and chronic hematological neoplasias characterized by the dissemination of the cancer cells originating in the bone marrow via the bloodstream. In 2016 the estimated number of new leukemia cases was more than 110,000 in all of Europe and 47,000 in the USA. Leukemias, cause of 4% of all cancer deaths and account for 3.6% of all cancers. Historically, leukemias have been divided into four major categories further classified into subtypes based on specific features of cells: acute lymphocytic leukemia (ALL), chronic lymphocytic leukemia (CLL), acute myelogenous leukemia (AML), and chronic myelogenous leukemia (CML). A revised classification of myeloid/lymphoid neoplasms and leukemias has recently been published to better characterize each disease. This updated classification incorporated new scientific and clinical information to refine diagnostic criteria for previously described neoplasms and introduced newly recognized disease entities. In this chapter the main entities of leukemia, with specific regard to imaging for diagnosis, treatment response assessment, and follow-up, will be treated according to what reported in the clinical guidelines.

Published
2017

39. 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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40. 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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41. 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

42. Hemopoietic and angiogenetic progenitors in healthy athletes: different responses to endurance and maximal exercise

Author

Veca M, Ugo Testa, Eleonora Petrucci, Giuseppe Morici, Laura Chimenti, Paolo Palange, Gioia M, Maria R. Bonsignore, Alice Huertas, Anna Bonanno, Roberta Riccioni, Gualtiero Mariani, Bonsignore, MR, Morici, G, Riccioni, R, Huertas, A, Petrucci, E, Veca, M, Mariani, G, Bonanno, A, Chimenti, L, Gioia, M, Palange, P, and Testa, U

Subjects
Adult, Male, medicine.medical_specialty, Physiology, Neovascularization, Physiologic, Antigens, CD34, Physical exercise, Hematopoietic Cell Growth Factors, Settore BIO/09 - Fisiologia, Running, angiopoietin, marathon, circulating progenitors, growth factors, Antigens, CD, Endurance training, Physiology (medical), Internal medicine, medicine, Humans, AC133 Antigen, Progenitor cell, Glycoproteins, Erythroid Precursor Cells, biology, Athletes, business.industry, Healthy subjects, Endothelial Cells, circulating progenitor, Middle Aged, Cadherins, Hematopoietic Stem Cells, biology.organism_classification, Haematopoiesis, Endocrinology, Physical Endurance, Cytokines, Angiogenesis Inducing Agents, adult, angiogenesis inducing agents, antigens, athletes, blood, cadherins, cd, cd34, cytokines, endothelial cells, erythroid precursor cells, glycoproteins, granulocytes, hematopoietic cell growth factors, hematopoietic stem cells, humans, male, middle aged, neovascularization, peptides, physical endurance, physiologic, physiology, running, AC133 antigen, Maximal exercise, Peptides, business, Granulocytes
Abstract

J Appl Physiol. 2010 Jul;109(1):60-7. Epub 2010 May 6. Hemopoietic and angiogenetic progenitors in healthy athletes: different responses to endurance and maximal exercise. Bonsignore MR, Morici G, Riccioni R, Huertas A, Petrucci E, Veca M, Mariani G, Bonanno A, Chimenti L, Gioia M, Palange P, Testa U. SourceBiomedical Department, Internal and Specialistic Medicine (DIBIMIS), Section of Pneumology, University of Palermo, Via Trabucco, 180, 90146 Palermo, Italy. marisa@ibim.cnr.it Abstract The effects of endurance or maximal exercise on mobilization of bone marrow-derived hemopoietic and angiogenetic progenitors in healthy subjects are poorly defined. In 10 healthy amateur runners, we collected venous blood before, at the end of, and the day after a marathon race (n = 9), and before and at the end of a 1.5-km field test (n = 8), and measured hemopoietic and angiogenetic progenitors by flow cytometry and culture assays, as well as plasma or serum concentrations of several cytokines/growth factors. After the marathon, CD34(+) cells were unchanged, whereas clonogenetic assays showed decreased number of colonies for both erythropoietic (BFU-E) and granulocyte-monocyte (CFU-GM) series, returning to baseline the morning post-race. Conversely, CD34(+) cells, BFU-E, and CFU-GM increased after the field test. Angiogenetic progenitors, assessed as CD34(+)KDR(+) and CD133(+)VE-cadherin(+) cells or as adherent cells in culture expressing endothelial markers, increased after both endurance and maximal exercise but showed a different pattern between protocols. Interleukin-6 increased more after the marathon than after the field test, whereas hepatocyte growth factor and stem cell factor increased similarly in both protocols. Plasma levels of angiopoietin (Ang) 1 and 2 increased after both types of exercise, whereas the Ang-1-to-Ang-2 ratio or vascular endothelial growth factor-A were little affected. These data suggest that circulating hemopoietic progenitors may be utilized in peripheral tissues during prolonged endurance exercise. Endothelial progenitor mobilization after exercise in healthy trained subjects appears modulated by the type of exercise. Exercise-induced increase in growth factors suggests a physiological trophic effect of exercise on the bone marrow. PMID:20448032[PubMed - indexed for MEDLINE]

Published
2010
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43. 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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44. 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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45. 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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46. Deferral of assessment of pulmonary embolism

Author

Maria Antonietta Bressan, Antonino Giarratano, Guglielmo Mariani, Fernando Porro, Maria Cristina Buonanno, Elena Maggi, Raffaela Anastasio, Alessandra Malato, Francesco Falaschi, Sergio Siragusa, Lucio Lo Coco, SIRAGUSA S, MALATO A, FALASCHI F, PORRO F, ANASTASIO R, GIARRATANO A, LO COCO L, BUONANNO MC, MAGGI E, BRESSAN MA, and MARIANI G

Subjects
Adult, Male, Risk, medicine.medical_specialty, Time Factors, medicine.drug_class, Low molecular weight heparin, Thrombophilia, Ventilation/perfusion ratio, Fibrin Fibrinogen Degradation Products, Predictive Value of Tests, Thromboembolism, D-dimer, Prevalence, Ventilation-Perfusion Ratio, medicine, Humans, Aged, Aged, 80 and over, Venous Thrombosis, business.industry, Respiratory disease, Anticoagulants, Hematology, Middle Aged, medicine.disease, Pulmonary embolism, Surgery, Hospitalization, Venous thrombosis, Early Diagnosis, Treatment Outcome, Predictive value of tests, Female, Pulmonary Embolism, business, Tomography, Spiral Computed, Algorithms
Abstract

We evaluated a simplified algorithm for safely postponing diagnostic imaging for pulmonary embolism (PE). At the index visit, patients were identified as being at high or low risk of PE; the former received full dosage low molecular weight heparin while the latter were left untreated until performance of diagnostic imaging (max 72 hours). During this period, no thromboembolic events occurred in low-risk patients (0/211, 0.% [upper 95% CI 0.9%]); only one event occurred in those at high-risk (1/125, 0.8% [upper 95% CI, 1.2]). Our study demonstrates that diagnostic imaging for PE can be safely deferred for up to 3 days.

Published
2007
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47. Replacement therapy in inherited factor VII deficiency: occurrence of adverse events and relation with surgery

Author

Angelika Batorova, M. N. D. Di Minno, Kaan Kavakli, G. Mariani, Alberto Dolce, Pimlak Charoenkwan, Muriel Giansily-Blaizot, N B Mirbehbahani, M.-F. López Fernández, Jørgen Ingerslev, Mehran Karimi, Mariasanta Napolitano, Département d'hématologie biologique[Montpellier], Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-CHU Saint-Eloi, Université de Montpellier (UM), Napolitano, M, Dolce, A., Batorova, A., Giansily Blaizot, M., Ingerslev, J., Mirbehbahani, N., DI MINNO, Matteo, Lopez Fernandez, M. F., Karimi, M., Charoenkwan, P., Kavakli, K., Mariani, G., Napolitano, M., Giansily-Blaizot, M., Di Minno, M., and Lopez Fernandez, M.

Subjects
Male, medicine.medical_specialty, Pediatrics, Web of science, Factor VII Deficiency, 030204 cardiovascular system & hematology, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Internal medicine, medicine, Humans, Young adult, Adverse effect, Factor VII deficiency, Child, Adverse events, inherited factor VII deficiency replacement therapy, Genetics (clinical), ComputingMilieux_MISCELLANEOUS, Hematology, business.industry, Infant, Newborn, Infant, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, General Medicine, Middle Aged, Infant newborn, 3. Good health, Surgery, 030220 oncology & carcinogenesis, Child, Preschool, Female, business
Abstract

International audience

Published
2015
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48. Diagnostic, therapeutic and healthcare management protocols in parathyroid surgery: II Consensus Conference of the Italian Association of Endocrine Surgery Units (U.E.C. CLUB)

Author

Rocco Domenico Alfonso Bellantone, Giovanni Conzo, Claudio Marcocci, M. L. Brandi, P. G. Nasi, Paolo Miccoli, Giuliano Mariani, Alfredo Pontecorvi, Maria Grazia Chiofalo, Maria Rosa Pelizzo, Raffaele Paragliola, Filomena Cetani, Guido Gasparri, Nadia Innaro, Marco Raffaelli, Alessandro Giordano, M. De Palma, E. Leopaldi, M. Boniardi, Furio Pacini, G. De Toma, Mario Testini, P. L. Marini, Luca Rosato, Nicola Avenia, Rosato, L, Raffaelli, M, Bellantone, R, Pontecorvi, A, Avenia, N, Boniardi, M, Brandi, Ml, Cetani, F, Chiofalo, Mg, Conzo, Giovanni, De Palma, M, Gasparri, G, Giordano, A, Innaro, N, Leopaldi, E, Mariani, G, Marcocci, C, Marini, Pl, Miccoli, P, Nasi, Pg, Pacini, F, Paraglioli, Rm, Pelizzo, Mr, Testini, M, and De Toma, G.

Subjects
medicine.medical_specialty, Consensus, Waiting Lists, Endocrinology, Diabetes and Metabolism, medicine.medical_treatment, Parathyroid Diseases, Time to treatment, protocols, Directive Counseling, Health administration, Time-to-Treatment, surgery, Consent Forms, Parathyroid Glands, Endocrinology, Medicine, Humans, parathyroid, Diagnostic, Parathyroid disease, Parathyroidectomy, business.industry, General surgery, Consensus conference, medicine.disease, hyperparathyroidism, postoperative management, parathyroidectomy, parathyroid disease, preoperative work up, Surgery, Endocrine surgery, Critical Pathways, Delivery of Health Care, Hospitalization, Practice Guidelines as Topic, therapeutic, healthcare management, hyperparathyroidism, parathyroid disease, parathyroidectomy, preoperative work up, postoperative management, Club, Parathyroid surgery, business
Abstract

Aim To update the Diagnostic-Therapeutic-Healthcare Protocol (Protocollo Diagnostico-Terapeutico-Assistenziale, PDTA) created by the U.E.C. CLUB (Association of the Italian Endocrine Surgery Units) during the I Consensus Conference in 2008. Methods In the preliminary phase, the II Consensus involved a selected group of experts; the elaboration phase was conducted via e-mail among all members; the conclusion phase took place during the X National Congress of the U.E.C. CLUB. The following were examined: diagnostic pathway and clinical evaluation; mode of admission and waiting time; therapeutic pathway (patient preparation for surgery, surgical treatment, postoperative management, management of major complications); hospital discharge and patient information; outpatient care and follow-up. Conclusions The PDTA for parathyroid surgery approved by the II Consensus Conference (June 2013) is the official PDTA of the U.E.C. CLUB.

Published
2014

49. Sorafenib as a feasible therapeutic option in haemophiliacs with hepatocellular carcinoma

Author

Gemma Bruera, Aldo Victor Giordano, Enrico Ricevuto, M. Napolitano, Alessandro Lucchesi, Mario Lapecorella, M. Tudini, Guglielmo Mariani, LAPECORELLA,M, NAPOLITANO,M, TUDINI,M, BRUERA, G, LUCCHESI,A, GIORDANO,A V, MARIANI,G, and RICEVUTO,E

Subjects
Sorafenib, Oncology, medicine.medical_specialty, hepatocellular carcinoma, haemophilia, treatment, business.industry, Hematology, General Medicine, medicine.disease, Haemophilia, Hepatocellular carcinoma, Internal medicine, medicine, business, Genetics (clinical), medicine.drug
Abstract

hepatocellular carcinoma, haemophilia, treatment

Published
2010
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50. 99mTc-Sestamibi Scintigraphy to Monitor the Long-Term Efficacy of Enzyme Replacement Therapy on Bone Marrow Infiltration in Patients with Gaucher Disease

Author

Giuliano Mariani, Anna Pierini, James M. Dambrosia, Roscoe O. Brady, Paola Anna Erba, Maja Di Rocco, Fiorina Giona, Ferdinando Buffoni, Silvia Linari, Mirella Filocamo, Fabrizio Minichilli, Erba, P, Minichilli, F, Giona, F, Linari, S, Dambrosia, J, Pierini, A, Filocamo, M, Di Rocco, M, Buffoni, F, Brady, R, and Mariani, G

Subjects
Adult, Male, Technetium Tc 99m Sestamibi, congenital, hereditary, and neonatal diseases and abnormalities, Pathology, medicine.medical_specialty, Time Factors, Bone marrow infiltration, Disease, Gaucher disease, Scintigraphy, Bone marrow disease, Gastroenterology, Internal medicine, Scintigraphic score, medicine, Enzyme replacement therapy, Bone Marrow Diseases, Female, Gaucher Disease, Humans, Radionuclide Imaging, Retrospective Studies, Treatment Outcome, Enzyme Replacement Therapy, Radiology, Nuclear Medicine and imaging, Platelet, medicine.diagnostic_test, business.industry, nutritional and metabolic diseases, 99mTc Sestamibi, medicine.anatomical_structure, Bone marrow, business
Abstract

Assessing the skeletal response to enzyme replacement therapy (ERT) in Gaucher disease (GD) is problematic. We investigated the reliability of (99m)Tc-sestamibi scintigraphy in monitoring changes in bone marrow involvement induced by ERT. Methods In 52 GD patients, the efficacy of ERT on bone marrow disease was monitored using at least 2 sequential (99m)Tc-sestamibi scans; 17 patients were receiving ERT at enrollment, and 35 were ERT-naive. We elaborated a dose-response model by statistical analysis based on linear mixed models. Results Patients whose marrow disease improved had received a significantly higher ERT dose per month than patients who did not improve. Significantly more patients reached near-disappearance of marrow disease if their disease burden at enrollment had been lower and the duration of clinical signs shorter. The response of the marrow scintigraphic score was more pronounced in ERT-naive patients. No relevant effect of ERT on marrow disease was observed until platelet count and splenomegaly had improved. Conclusion Although based on localized evaluation, changes in the (99m)Tc-sestamibi score closely correlated with the main determinants of ERT, with a definite dose-response relationship. The threshold at which ERT induced any improvement in bone marrow disease was 35-36 U/kg/mo; in ERT-naive patients, the scintigraphic score declined by 1 unit after ERT at 28 U/kg/mo.

Published
2013

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