Clinical and laboratory data in pediatric autoimmune hepatitis: study from referral center in Iran.

BACKGROUND: Autoimmune hepatitis (AIH) is an auto-inflammatory liver disease of children and adults, affecting patients of any age, sex, race or ethnicity, with more prevalence in females. OBJECTIVE: The aim of this study was to evaluate clinical manifestation, laboratory findings, and outcome of children with autoimmune hepatitis. MATERIALS AND METHODS: We evaluated 86 patients treated and followed with final diagnosis of AIH between years 2010 to 2018. Physical findings (including jaundice, hepatomegaly, splenomegaly and encephalopathy), liver enzymes, liver histology and autoantibodies (including ANA, Anti LKM-1 and ASMA) were extracted from medical files. Then the patients were followed for their final outcome (including response to medical treatment or successful treatment withdrawal, liver transplantation or death). RESULTS: Among 86 patients with AIH with mean age 9.10±4.36 years old, 66.27% were females. Jaundice (75.6%) and hepatomegaly (46.5%) were the most frequent physical findings, followed by splenomegaly (32.6%) and encephalopathy (17.4%). Aminotransferases including AST and ALT were elevated at least 3 times more than upper limit of normal in most of the patients (61.6% and 55.81%, respectively). Autoantibodies were available in 53 of 86 patients, 24.5% had AIH-1, 3.8% had AIH-II and 67.9% were seronegative. Medical treatment including prednisolone and azathioprine was started for patients, 53 of 86 cases (61.6%) had remission and 11 of 86 (13.7%) tolerated medication withdrawal successfully. Among all cases, 26 (30.2%) patients needed liver transplantation. Mortality rate was 9 among 86 cases (10.5%). CONCLUSION: Jaundice and hepatomegaly was the most frequent clinical findings. Mortality rate was 10.5.

Clinical and laboratory data in pediatric autoimmune hepatitis: study from referral center in Iran / Datos clínicos y de laboratorio en hepatitis autoinmune pediátrica: estudio del centro de referencia en Irán

ABSTARCT Background : Autoimmune hepatitis (AIH) is an auto-inflammatory liver disease of children and adults, affecting patients of any age, sex, race or ethnicity, with more prevalence in females. Objective : The aim of this study was to evaluate clinical manifestation, laboratory findings, and outcome of children with autoimmune hepatitis. Materials and methods : We evaluated 86 patients treated and followed with final diagnosis of AIH between years 2010 to 2018. Physical findings (including jaundice, hepatomegaly, splenomegaly and encephalopathy), liver enzymes, liver histology and autoantibodies (including ANA, Anti LKM-1 and ASMA) were extracted from medical files. Then the patients were followed for their final outcome (including response to medical treatment or successful treatment withdrawal, liver transplantation or death). Results : Among 86 patients with AIH with mean age 9.10±4.36 years old, 66.27% were females. Jaundice (75.6%) and hepatomegaly (46.5%) were the most frequent physical findings, followed by splenomegaly (32.6%) and encephalopathy (17.4%). Aminotransferases including AST and ALT were elevated at least 3 times more than upper limit of normal in most of the patients (61.6% and 55.81%, respectively). Autoantibodies were available in 53 of 86 patients, 24.5% had AIH-1, 3.8% had AIH-II and 67.9% were seronegative. Medical treatment including prednisolone and azathioprine was started for patients, 53 of 86 cases (61.6%) had remission and 11 of 86 (13.7%) tolerated medication withdrawal successfully. Among all cases, 26 (30.2%) patients needed liver transplantation. Mortality rate was 9 among 86 cases (10.5%). Conclusion : Jaundice and hepatomegaly was the most frequent clinical findings. Mortality rate was 10.5%.

RESUMEN Antecedentes : La hepatitis autoinmune (AIH) es una enfermedad hepática autoinflamatoria de niños y adultos, que afecta a pacientes de cualquier edad, sexo, raza u origen étnico, con mayor prevalencia en las mujeres. Objetivo : El objetivo de este estudio fue evaluar la manifestación clínica, los hallazgos de laboratorio y el resultado de los niños con hepatitis autoinmune. Materiales y métodos : Evaluamos 86 pacientes tratados y seguidos con el diagnóstico final de AIH entre los años 2010 a 2018. Los hallazgos físicos (incluyendo ictericia, hepatomegalia, esplenomegalia y encefalopatía), enzimas hepáticas, histología hepática y autoanticuerpos (incluidos ANA, Anti LKM-1 y ASMA) se extrajeron de las historias médicas. Luego, los pacientes fueron seguidos para su resultado final (incluida la respuesta al tratamiento médico o la retirada exitosa del tratamiento, el trasplante de hígado o la muerte). Resultados : Entre 86 pacientes con AIH con una edad media de 9,10 ± 4,36 años, el 66,27% eran mujeres. La ictericia (75,6%) y la hepatomegalia (46,5%) fueron los hallazgos físicos más frecuentes, seguidos de esplenomegalia (32,6%) y encefalopatía (17,4%). Las aminotransferasas que incluyen AST y ALT se elevaron al menos 3 veces más que el límite superior de la normalidad en la mayoría de los pacientes (61,6% y 55,81%, respectivamente). Los autoanticuerpos estaban disponibles en 53 de 86 pacientes, 24,5% tenían AIH-1, 3,8% tenían AIH-II y 67,9% eran seronegativos. Se inició tratamiento médico que incluyó prednisolona y azatioprina, 53 de 86 casos (61,6%) tuvieron remisión y 11 de 86 (13,7%) toleraron el retiro de medicamentos con éxito. Entre todos los casos, 26 (30,2%) pacientes necesitaron un trasplante de hígado. La tasa de mortalidad fue de 9 entre 86 casos (10,5%). Conclusión : la ictericia y la hepatomegalia fueron los hallazgos clínicos más frecuentes. La tasa de mortalidad fue del 10,5%.

Evaluation of Underlying Liver Disease and Its Severity in Children Referred for Liver Transplant: a Single-Center Report From Nemazee Hospital of Shiraz.

OBJECTIVES: Liver transplant has been used as a curative approach for children with end-stage liver diseases. Here, we describe the underlying causes for pediatric liver transplant performed at the Shiraz Organ Transplantation Center, Nemazee Hospital, Shiraz, Iran. MATERIALS AND METHODS: In this cross-sectional descriptive study, children < 18 years old who were candidates for liver transplant from 2007 to 2010 at the Shiraz Organ Transplantation Center were included. Patients were evaluated for their underlying diseases leading to liver failure. Disease severity was assessed and compared with Pediatric End-Stage Liver Disease model and the Model for End-Stage Liver Disease scores. RESULTS: Of 107 patients, 60.8% were males and 39.2% were females. The mean age was 11.6 ± 4.9 years. Thirteen patients (12.5%) were < 2 years old, 26 (24%) were 2 to 6 years old, 33 (30.8%) were 6 to 12 years old, and 35 (32.7%) were 12 to 18 years old. Underlying liver diseases comprised biliary atresia (27.1%), cryptogenic cirrhosis (21.5%), autoimmune cirrhosis (13.1%), familial intrahepatic cholestasis (11.2%), Wilson disease (9.3%), tyrosinemia (7.4%), neonatal hepatitis (4.7%), congenital hepatic fibrosis (3.7%), and Caroli disease (1.9%). Jaundice (83.2%), ascites (57%), and esophageal varices (43%) were the most common clinical findings. Mean serum direct bilirubin, total bilirubin, international normalized ratio, and serum creatinine values were 3.6 ± 0.8 mg/dL, 9.3 ± 9.1 mg/dL, 2.1 ± 1.1, and 0.6 ± 0.2 mg/dL, respectively. The mean Pediatric End-Stage Liver Disease score in children < 12 years old was 11.4 ± 9.1. The mean Model for EndStage Liver Disease score in children > 12 years old was 13.7 ± 5.9. There were no differences in scores among sex, age groups, or different etiologies. CONCLUSIONS: Scores for disease severity were not significantly different with regard to different causes of underlying diseases for liver transplant in Iranian children.

Erythropoietin for acute multiple sclerosis in patients with optic neuritis as a first demyelination event.

OBJECTIVE: To investigate the safety, tolerability, and short-term efficacy of treatment with erythropoietin in patients with optic neuritis as a first demyelination event. METHODS: We conducted this randomized double-blind pilot study in the Shiraz University of Medical Sciences, Shiraz, Iran, from March 2007 to January 2009. The participants were patients aged 18-45 years with optic neuritis and at least 3 hyperintense lesions on T2-weighted and FLAIR MRI, but no clinically definite multiple sclerosis (MS). They were randomized into 2 groups. The case group (5 patients) received intravenous methyl prednisolone (1000 mg/24 hours) and intravenous erythropoietin (20,000 unit/24 hours) for 5 consecutive days, and the control group (5 patients) received intravenous methyl prednisolone at the same dose as the case group, and a placebo. The groups were followed for one year and compared for adherence to protocol, adverse drug effects, mean duration of conversion to clinically definite MS, and MRI changes. RESULTS: All patients tolerated the protocol. One patient who received erythropoietin developed cerebral venous sinus thrombosis and anti-cardiolipin antibody positivity. One patient in the control group, but no patients in the case group, fulfilled the McDonald criteria for MS during the follow-up period, but none of the participants in either group developed clinically definite MS according to the Poser criteria. CONCLUSION: Erythropoietin may be effective, but should be used with caution.