10 results on '"Daniele Zama"'
Search Results
2. Immune cytopenias as a continuum in inborn errors of immunity: An in‐depth clinical and immunological exploration
- Author
-
Daniele Zama, Francesca Conti, Mattia Moratti, Maria E. Cantarini, Elena Facchini, Beatrice Rivalta, Roberto Rondelli, Arcangelo Prete, Simona Ferrari, Marco Seri, and Andrea Pession
- Subjects
autoimmune hemolytic anemia ,autoimmune lymphoproliferative syndrome ,autoimmune neutropenia ,common variable immune deficiency ,DiGeorge syndrome ,immune cytopenias ,Immunologic diseases. Allergy ,RC581-607 - Abstract
Abstract Background Immune thrombocytopenia (ITP), autoimmune hemolytic anemia (AIHA), and autoimmune neutropenia (AIN) are disorders characterized by immune‐mediated destruction of hematopoietic cell lineages. A link between pediatric immune cytopenias and inborn errors of immunity (IEI) was established in particular in the combined and chronic forms. Objective Aim of this study is to provide clinical‐immunological parameters to hematologists useful for a prompt identification of children with immune cytopenias deserving a deeper immunological and genetic evaluation. Methods We retrospectively collected 47 pediatric patients with at least one hematological disorder among which persistent/chronic ITP, AIHA, and AIN, aged 0–18 years at onset of immune cytopenias and/or immune‐dysregulation. The cohort was divided into two groups (IEI+ and IEI−), based on the presence/absence of underlying IEI diagnosis. IEI+ group, formed by 19/47 individuals, included: common variable immune deficiency (CVID; 9/19), autoimmune lymphoproliferative syndrome (ALPS; 4/19), DiGeorge syndrome (1/19), and unclassified IEI (5/19). Results IEI prevalence among patients with ITP, AIHA, AIN, and Evans Syndrome was respectively of 42%, 64%, 36%, and 62%. In IEI+ group the extended immunophenotyping identified the presence of statistically significant (p and the compound heterozygous TNFRSF13B variants p.Ser144Ter (pathogenic) and p.Cys193Arg (variant of uncertain significance), the other one carrying the likely pathogenic monoallelic variant TNFRSF13B:p.Ile87Asn. Conclusion The synergy between hematologists and immunologists can improve and fasten diagnosis and management of patients with immune cytopenias through a wide focused clinical/immunophenotypical characterization, which identifies children worthy of IEI‐related molecular analysis, favouring a genetic IEI diagnosis and potentially unveiling new targeted‐gene variants responsible for IEI phenotype.
- Published
- 2021
- Full Text
- View/download PDF
3. <scp>SARS‐CoV</scp> ‐2 infection and treatment in a cohort of patients with inborn errors of immunity
- Author
-
Francesca Conti, Lucia Pacillo, Donato Amodio, Beatrice Rivalta, Mattia Moratti, Caterina Campoli, Daniele Zama, Ilaria Corsini, Carmela Giancotta, Stefania Bernardi, Samuele Naviglio, Maria Pia Cicalese, Marco Rabusin, Alessandro Aiuti, Caterina Cancrini, Marcello Lanari, Pierluigi Viale, Paolo Palma, Andrea Pession, and Andrea Finocchi
- Subjects
inborn errors of immunity ,treatment ,SARS-CoV-2 ,Immunology ,COVID-19 ,Antibodies, Viral ,Settore MED/38 ,Cohort Studies ,antiviral drugs ,Pediatrics, Perinatology and Child Health ,SARS-COV2 ,Humans ,Immunology and Allergy ,monoclonal antibodies ,primary immunodeficiencies - Published
- 2022
- Full Text
- View/download PDF
4. Autoimmune hemolytic anemia in children with COVID‐19
- Author
-
Andrea Pession, Francesca Gottardi, Daniele Zama, Riccardo Masetti, Pierluigi Viale, Fiorentina Guida, Nicola Dentale, Francesco Baccelli, Fabio Esposito, Livia Pancaldi, Zama D., Pancaldi L., Baccelli F., Guida F., Gottardi F., Dentale N., Esposito F., Masetti R., Viale P., and Pession A.
- Subjects
COVID 19, children, autoimmune hemolytic anemia ,2019-20 coronavirus outbreak ,Coronavirus disease 2019 (COVID-19) ,business.industry ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,Hematology ,medicine.disease ,Oncology ,hemic and lymphatic diseases ,Pediatrics, Perinatology and Child Health ,Immunology ,Medicine ,Autoimmune hemolytic anemia ,business ,Letter to the Editor - Abstract
Pediatric 2019 novel coronavirus disease (COVID-19) is characterized by a wide clinical spectrum, including hematological manifestations.1 Anemia and thrombocytopenia occur mainly in severe forms of the disease and in multisystem inflammatory syndrome2 but are rare in asymptomatic and mildly symptomatic patients. Only few reports of autoimmune hemolytic anemia (AIHA), rarely associated with immune thrombocytopenia (ITP), have been described in children with COVID-19.3–5 Here, we present two pediatric cases of severe cold agglutinin disease and a brief review of the literature
- Published
- 2021
- Full Text
- View/download PDF
5. The gut microbiome in pediatric patients undergoing allogeneic hematopoietic stem cell transplantation
- Author
-
Daniele Zama, Riccardo Masetti, Andrea Pession, Arcangelo Prete, Edoardo Muratore, Silvia Turroni, Patrizia Brigidi, Davide Leardini, Masetti R., Zama D., Leardini D., Muratore E., Turroni S., Prete A., Brigidi P., and Pession A.
- Subjects
Oncology ,medicine.medical_specialty ,medicine.medical_treatment ,Population ,Graft vs Host Disease ,gut microbiome ,bloodstream infection ,Context (language use) ,Hematopoietic stem cell transplantation ,03 medical and health sciences ,0302 clinical medicine ,Immune system ,Immunity ,Internal medicine ,graft-versus-host disease ,Humans ,Transplantation, Homologous ,Medicine ,education ,education.field_of_study ,business.industry ,Incidence (epidemiology) ,fecal microbiota transplantation ,Hematology ,medicine.disease ,Gastrointestinal Microbiome ,pediatric ,Graft-versus-host disease ,Hematologic Neoplasms ,030220 oncology & carcinogenesis ,hematopoietic stem cell transplantation ,Pediatrics, Perinatology and Child Health ,business ,030215 immunology ,Cohort study - Abstract
The gut microbiome (GM) has been associated with different clinical outcomes in the context of allogeneic hematopoietic stem cell transplantation (HSCT). Large multicenter cohort studies in adults have found significant correlations with overall survival, relapse, and incidence of complications. Moreover, GM is already a promising target for therapeutic interventions. However, few data are available in children, a population presenting unique features and challenges. During childhood, the GM evolves rapidly with large structural fluctuations, alongside with the maturation of the immune system. Furthermore, the HSCT procedure presents significant differences in children. These considerations underline the importance of a specific focus on the pediatric setting, and the role of GM and its age-dependent trajectory in influencing the immunity reconstitution and clinical outcomes. This review provides a comprehensive overview of the available evidence in the field of GM and pediatric HSCT, highlighting age-specific issues and discussing GM-based therapeutic approaches.
- Published
- 2020
- Full Text
- View/download PDF
6. Longitudinal evaluation of liver stiffness in three pediatric patients with veno‐occlusive disease
- Author
-
Daniele Zama, Federico Ravaioli, Arcangelo Prete, Andrea Pession, Davide Festi, Gianluca Bossù, Riccardo Masetti, Zama D., Bossu G., Ravaioli F., Masetti R., Prete A., Festi D., and Pession A.
- Subjects
fibroscan ,Transplantation ,medicine.medical_specialty ,business.industry ,Liver fibrosis ,Ultrasound ,030232 urology & nephrology ,Urology ,Disease ,sinusoidal obstructive syndrome ,030230 surgery ,Hepatic Complication ,transient elastography ,sinusoidal obstruction syndrome ,03 medical and health sciences ,0302 clinical medicine ,Liver stiffness ,Pediatrics, Perinatology and Child Health ,Medicine ,Veno-Occlusive Disease ,In patient ,veno-occlusive disease ,business ,Transient elastography - Abstract
Liver stiffness measurement by transient elastography is a non-invasive ultrasound-based technique used mainly for the evaluation of liver fibrosis in patients with chronic liver diseases. Some authors have suggested that it could be useful in the assessment of hepatic complications during hematopoietic stem cell transplant (HSCT), especially veno-occlusive disease (VOD) or sinusoidal obstructive syndrome (SOS). Here, we present the evaluation of liver stiffness in three patients who developed severe hepatic VOD/SOS after HSCT. Liver stiffness measurement values were normal before transplant and afterward until the diagnosis of VOD/SOS when a dramatic increase was observed. After the VOD/SOS specific treatment, the values of stiffness showed a similar pattern of progressive reduction in all the three patients, with normalization after two weeks. In this report, we discuss the role of LSM in the management of patients after the diagnosis of VOD/SOS.
- Published
- 2019
- Full Text
- View/download PDF
7. Hematopoietic stem cell transplantation for curing children with severe autoimmune diseases: Is this a valid option?
- Author
-
Daniele Zama, Andrea Pession, Arcangelo Prete, Riccardo Masetti, and Pietro Gasperini
- Subjects
Transplantation ,Type 1 diabetes ,medicine.medical_specialty ,business.industry ,medicine.medical_treatment ,Incidence (epidemiology) ,Hematopoietic stem cell transplantation ,Disease ,medicine.disease ,medicine.disease_cause ,Autoimmunity ,immune system diseases ,Pediatrics, Perinatology and Child Health ,Severity of illness ,Cohort ,Immunology ,medicine ,business ,Intensive care medicine ,Juvenile rheumatoid arthritis - Abstract
The cure of children with severe AD, especially patients with severe, progressive, and therapy-resistant autoimmunity, represents a challenge for current medical practice. The idea of HSCT as a promising therapeutic opportunity was borne accidentally from finding patients who, after undergoing HSCT for a hematological indication, were cured of a concomitant AD. Thus, over the last two decades, HSCT has been extensively investigated, and it has become an appealing therapy for rheumatological (juvenile rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis) and hematological diseases (immune cytopenias). Recently, interesting results have been also described in type 1 diabetes mellitus and Crohn's disease. Although the use of HSCT has been steadily rising in the last few years, many questions are still open, especially after the discoveries of many new biological agents. Given the low incidence of ADs in children, most of the data about the use of the HSCT for these diseases are taken from a mixed cohort of adults and children. The aim of this review is to summarize the published studies and to try to answer the question as to whether this procedure can be considered a promising approach.
- Published
- 2012
- Full Text
- View/download PDF
8. Oral Presentations
- Author
-
Giacomo Faldella, Luigi Corvaglia, M. Spizzichino, Rosina Alessandroni, Arianna Aceti, Elisa Mariani, and Daniele Zama
- Subjects
medicine.medical_specialty ,business.industry ,Internal medicine ,Pediatrics, Perinatology and Child Health ,medicine ,Reflux ,Apnea ,General Medicine ,medicine.symptom ,business ,Gastroenterology - Published
- 2009
- Full Text
- View/download PDF
9. Inflammatory disease of the central nervous system induced by anti-GD2monoclonal antibody in a patient with high risk neuroblastoma
- Author
-
Duccio Maria Cordelli, Erika Massaccesi, Andrea Pession, Arcangelo Prete, William Morello, Daniele Zama, and Riccardo Masetti
- Subjects
medicine.medical_specialty ,Pathology ,Hematology ,medicine.drug_class ,business.industry ,Central nervous system ,Inflammation ,Disease ,medicine.disease ,Monoclonal antibody ,medicine.anatomical_structure ,Oncology ,Antibodies monoclonal ,Internal medicine ,Neuroblastoma ,Pediatrics, Perinatology and Child Health ,Immunology ,medicine ,High risk neuroblastoma ,medicine.symptom ,business - Published
- 2014
- Full Text
- View/download PDF
10. The frequency of apneas in very preterm infants is increased after non-acid gastro-esophageal reflux
- Author
-
Giacomo Faldella, Daniele Zama, Arianna Aceti, M. Spizzichino, Elisa Mariani, Silvia Galletti, Maria Grazia Capretti, and Luigi Corvaglia
- Subjects
medicine.medical_specialty ,medicine.diagnostic_test ,Endocrine and Autonomic Systems ,Physiology ,business.industry ,fungi ,Gastroenterology ,Reflux ,Apnea ,Polysomnography ,medicine.disease ,Ph monitoring ,Gastro esophageal reflux ,Very preterm ,Anesthesia ,Internal medicine ,Medicine ,medicine.symptom ,business ,Esophageal pH monitoring ,Apnea of prematurity - Abstract
Background To evaluate whether physical and/or chemical features of gastro-esophageal reflux (GER) influence its relationship with apnea of prematurity (AOP). Methods Fifty-eight preterm newborns (GA ≤33 weeks) with recurrent apneas were studied by simultaneous polysomnography and combined impedance and pH monitoring, to analyze whether the correlation between GER and AOP varies according to the acidity, duration and height of GERs. Key Results The frequency of apnea (number apnea/min) occurring after-GER [median (range) 0.07 (0–0.25)] was higher than the one detected in GER-free period [0.06 (0.04–0.13), P = 0.015], and also than the one detected before-GER [0 (0–0.8), P = 0.000]. The frequency of apneas detected in the 30’’ after pH-GER [median (range), 0 min−1 (0–1.09)] was higher than the frequency detected in the 30’’ before [0 (0–0.91), P = 0.04]; even more, the frequency of apneas detected after non-acid MII-GER episodes [0 (0–2)] was significantly higher than the one detected before [0 (0–1), P = 0.000], whereas the frequency of apneas detected before acid MII-GER episodes [0 (0–0.67)] did not differ from the one detected after [0 (0–2), P = 0.137]. The frequency of pathological apneas detected in the 30’’ after-GER (0 min−1, range 0–0.55) was higher than the frequency detected before (0, range 0–0.09; P = 0.001). No difference in mean height or in mean duration was found between GERs correlated and those non-correlated to apnea. Conclusions & Inferences Non-acid GER is responsible for a variable amount of AOP detected after-GER: this novel finding must be taken into consideration when a therapeutic strategy for this common problem is planned.
- Published
- 2010
- Full Text
- View/download PDF
Catalog
Discovery Service for Jio Institute Digital Library
For full access to our library's resources, please sign in.