ABSTRACT
BACKGROUND: Biliary complications (BCs) remain an important cause of morbidity after pediatric liver transplantation. Technical factors have already been implicated in the development of BCs. Previous reports have associated the use of partial grafts, particularly living donor grafts, with a higher incidence of BCs. Our aim is to study the factors associated with the development of BCs in a large cohort of pediatric liver transplant recipients. METHODS: Retrospective cohort study of 670 children (<18 years of age) who underwent a primary liver transplant between March 2000 and January 2015. Patients who did and did not develop BCs were compared with identify associated factors. Univariate and multivariate analyses were performed. RESULTS: A total of 115 patients (17.2%) developed BCs (83 strictures and 44 leaks). Of the study participants, 594 had living donor liver transplants. Multiple arterial anastomoses was a protective factor for BCs, and a ductoplasty was a risk factor. Living donor grafts and multiple biliary anastomoses were more frequently associated with leaks. Patients with BCs had a higher reoperation rate and longer hospital stays. There was no difference in patient or graft survival. CONCLUSIONS: Technical factors play a major role in the development of BCs, particularly leaks. Strictures are more frequently associated with an inadequate arterial supply to the bile duct, and multiple arterial anastomoses may protect children from this complication. The use of partial grafts was not an independent factor for BCs in high-volume centers that are experienced with this technique.
Subject(s)
Anastomotic Leak/etiology , Cholestasis/etiology , Liver Transplantation/adverse effects , Age Factors , Anastomotic Leak/diagnosis , Anastomotic Leak/surgery , Biliary Tract Surgical Procedures/adverse effects , Chi-Square Distribution , Child, Preschool , Cholestasis/diagnosis , Cholestasis/surgery , Female , Humans , Infant , Kaplan-Meier Estimate , Length of Stay , Liver Transplantation/methods , Living Donors , Male , Multivariate Analysis , Odds Ratio , Proportional Hazards Models , Reoperation , Retrospective Studies , Risk Factors , Time Factors , Treatment Outcome , Vascular Surgical Procedures/adverse effectsABSTRACT
OBJECTIVE: To determine the frequency and significance of diabetes mellitus (DM)-related autoantibodies in children with autoimmune hepatitis (AIH). RESEARCH DESIGN AND METHODS: Anti-islet cell antibodies (ICA), insulin autoantibodies (IAA), and anti-glutamic acid decarboxylase (GAD65) antibodies were assessed in 28 children (25 female) with AIH before and after 3-9 years of therapy with azathioprine and prednisone. RESULTS: There was biochemical and clinical remission of AIH activity in 76% of the children after 1 year of immunosuppressive therapy. Positive ICA and IAA were found in 60.7% and 18.5% of the patients, decreasing to 38.5% and 12% after 3-9 years of therapy. Anti-GAD autoantibodies were present in only one patient who had Graves' disease, high ICA titer, and developed type 1 DM after 3 years. After 3-9 years of follow up, all had normal fasting glycemia, glycosylated hemoglobin (HbA1c), and, with a single exception, normal responses to oral glucose tolerance testing. No increase in the frequencies of HLA antigens was observed in ICA- and IAA-positive patients compared to antibody-negative patients or a control population. The majority of the patients with HLA-DRB1*03 or DRB1*04, however, were positive for ICA (7/10), and three of them had IAA. The frequency of high risk HLA DQB1*0302 or DQB1*02 alleles was low and similar to control frequencies, indicating low-risk for DM despite the presence of DM-related autoimmunity markers. CONCLUSIONS: AIH in childhood is associated with high frequency of ICA and IAA, with less than expected rates of progression to DM. Immunosuppression reduced ICA and IAA frequency and titers.
Subject(s)
Autoantibodies/biosynthesis , Diabetes Mellitus/immunology , Hepatitis, Autoimmune/immunology , Actins/immunology , Adolescent , Animals , Antibodies, Antinuclear/analysis , Autoantibodies/analysis , Child , Child, Preschool , Cytochrome P-450 CYP2D6/immunology , Cytosol/immunology , Diabetes Complications , Diabetes Mellitus, Type 1/complications , Diabetes Mellitus, Type 1/immunology , Diabetes Mellitus, Type 2/complications , Diabetes Mellitus, Type 2/immunology , Female , Fluorescent Antibody Technique, Indirect , Glutamate Decarboxylase/immunology , Hepatitis, Autoimmune/complications , Humans , Insulin/immunology , Islets of Langerhans/immunology , Isoenzymes/immunology , Male , Muscle, Smooth/immunology , Rats , Rats, WistarABSTRACT
Trata-se de uma criança do sexo feminino de 3 anos de idade, com quadro clínico de hepatite aguda, com icterícia, hepatoesplenomegalia, altos níveis de transaminase glutâmico-pirúvica e gamaglobulina. Foi encontrada a presença de altos níveis de anticorpos anti-microssomo fígado rim tipo 1(anti-LKM 1) pelo método de imunofluorescência e confirmado por Elisa e análise de Werstern Blot. A biopsia hepática mostrou hepatite crônica ativa, e a terapia imunossupressora foi iniciada imediatamente
