A5050644274- Liver Diseases and Immunity
- Pediatric Hepatobiliary Diseases and Treatments
- Liver Disease Diagnosis and Treatment
- Liver Disease and Transplantation
- Hepatitis B Virus Studies
- Gallbladder and Bile Duct Disorders
- Celiac Disease Research and Management
- Organ Transplantation Techniques and Outcomes
- Drug Transport and Resistance Mechanisms
- Renal Transplantation Outcomes and Treatments
- Congenital Anomalies and Fetal Surgery
- Systemic Lupus Erythematosus Research
- Hepatitis Viruses Studies and Epidemiology
- Neonatal Health and Biochemistry
- Pancreatitis Pathology and Treatment
- Cytomegalovirus and herpesvirus research
- Microscopic Colitis
- Renal Diseases and Glomerulopathies
- Gastrointestinal disorders and treatments
- Intestinal Malrotation and Obstruction Disorders
- Blood groups and transfusion
- IgG4-Related and Inflammatory Diseases
- Biliary and Gastrointestinal Fistulas
- Immunodeficiency and Autoimmune Disorders
- Blood Coagulation and Thrombosis Mechanisms
Istituto Mediterraneo per i Trapianti e Terapie ad Alta Specializzazione
2015-2025
University of Palermo
2022-2025
Istituti di Ricovero e Cura a Carattere Scientifico
2016-2025
Bambino Gesù Children's Hospital
2024-2025
University of Pittsburgh
2008-2014
Ospedale Vincenzo Cervello
2011
University of Pisa
2004-2011
Inonu University
2010
Atatürk University
2010
Erzurum Technical University
2010
Background & AimsPFIC2 is caused by mutations in ABCB11 encoding BSEP. In most cases affected children need liver transplantation that thought to be curative. We report on two patients who developed recurrent normal GGT cholestasis mimicking primary BSEP disease, after transplantation.MethodsPFIC2 diagnosis was made infancy both absence of canalicular immunodetection and mutation identification. Liver performed at age 9 (patient 1) 2.8 2) years without major complications. Cholestasis with...
The human EBV-transformed lymphoblastoid cell line (LCL), obtained by infecting peripheral blood monocular cells with Epstein–Barr Virus, has been extensively used for genetic, pharmacogenomic, and immunologic studies. Recently, the role of exosomes also indicated as crucial in crosstalk between EBV host microenvironment. Because that LCL exosomal cargo might play maintaining persistent infection, since little is known regarding non-coding RNAs LCL, aim our work was comprehensive...
The current pandemic SARS-CoV-2 has required an unusual allocation of resources that can negatively impact chronically ill patients and high-complexity procedures. Across the European Reference Network on Pediatric Transplantation (ERN TransplantChild), we conducted a survey to investigate COVID-19 outbreak pediatric transplant activity healthcare practices in both solid organ transplantation (SOT) hematopoietic stem cell (HSCT). replies 30 professionals from 18 centers Europe were...
Celiac disease (CD) is common in patients with autoimmune liver (AILD); however, the long-term response to treatment of AILDs coexistent CD has not been explored detail. The aim present study was analyze features and immunosuppressive children hepatitis (AIH) associated CD.Retrospective prospective evaluation followed at a single center.Among 79 AIH, 15 (19%) had (9 type 1, 3 2, seronegative). In group AIH CD, female sex significantly more represented than alone; also, former group,...
Abstract Background and rationale Progressive familial intrahepatic cholestasis (PFIC) associated with myosin 5B deficiency is a rare liver disease characterised by elevated serum bile acids (sBAs) severe pruritus. The objective of this study was to evaluate treatment the ileal acid transporter inhibitor odevixibat in affected children. Methods This retrospective analysis five children diagnosis PFIC pruritus refractory rifampicin ursodeoxycholic acid, starting (37.2–120 µg/kg.day) between...
ABSTRACT As pediatric liver transplantation comes of age, experts gathered to discuss current paradigms and define gaps in knowledge warranting research further improve patient graft outcomes. Identified areas ripe for collaborative include understanding the molecular cellular mechanisms tolerance role donor‐specific antibodies, considering ways expand donor pool, minimizing long‐term side effects immunosuppression, fine‐tuning surgical techniques minimize biliary vascular complications.
Abstract Background and Aims In paediatrics, porto‐sinusoidal vascular disease (PSVD) is relatively unknown probably underdiagnosed. We aimed to describe clinical phenotypes, histology outcome of children diagnosed with PSVD. Methods Retrospective multicentre study Diagnosis PSVD was based on histopathology reports; liver specimens were re‐evaluated by two expert pathologists. Results Sixty (M/F = 36/26, median age 6.6 years, range 3.3–10.6), from 7 centres, included. Thirty‐six presented...
Conventional treatment for autoimmune hepatitis results in a significant percentage of failures and several, poorly tolerated, side-effects. Therapy cholangitis giant cell associated with haemolysis is documented. Ciclosporin promising all these diseases.We reviewed the records 12 patients treated our unit between 1987 2001. Eight had hepatitis, two hepatitis. Indications ciclosporin were failure (four patients) contraindications to/refusal steroids (eight patients). was administered five...