Evaluation of Clinical Outcomes in Children with Intrahepatic Cholestasis Postpartial External Biliary Diversion: A Single-Center Experience.

Background: Severe pruritus caused by progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (AGS) is refractory to medical treatment. Surgical interruption of the enterohepatic circulation is considered the mainstay of alleviating distressing symptoms and delaying cirrhosis. Aim and Objectives: This study aims to evaluate the short-term effect of partial external biliary diversion (PEBD) on pruritus, liver disease progression, patient's growth, and quality of life. Material and Methods: This prospective cohort study enrolled children with PFIC and AGS from July 2019 to July 2021, whose guardians consented to the PEBD procedure. A standard surgical approach was performed by a single surgeon. Outcomes were measured subjectively and objectively pre- and post-procedure using the pruritus 5-D itching score, Paediatric Quality of Life Inventory scale (PedsQL), growth parameters, bile acids level, and liver function tests. Patients' follow-up period ranged from 6 to 12 months. Results: Seven patients had PEBD procedure; five with PFIC and two with AGS. A significant improvement was detected in the 5-D itching score (p-value < 0.001), PedsQL (p-value < 0.001), and bile acids level (p-value 0.013). The preexisting growth failure was ameliorated. The downward trend in the bilirubin level was not significant. No influential difference in the other liver function tests occurred. No intra-operative complications encountered. Only one case had a post-operative stoma prolapse which was managed surgically. Conclusion: PEBD procedure could be considered as an effective and safe treatment options for intractable pruritus in patients with PFIC or AGS, providing preserved synthetic liver functions.

Methotrexate Hepatotoxicity in Children with Juvenile Idiopathic Arthritis: A Single-Center Study.

BACKGROUND: Juvenile idiopathic arthritis (JIA) could be disabling if left untreated. Methotrexate (MTX) is well known as a cornerstone in management. However, its adverse effects may limit treatment. OBJECTIVE: The objective of this study was to evaluate the frequency of hepatotoxicity based on liver chemistry in JIA children receiving MTX. METHODS: An observational case-control study of children with JIA who attend the Pediatric Rheumatology Unit, Cairo University Pediatric Hospital, Egypt, from January 2018 to December 2018 was carried out. Data were retrieved for 80 children; 50 (62.5%) were prescribed MTX. Their demographic, clinical characteristics, mean dose, duration of MTX therapy and other medications were described. Hepatotoxicity was defined as at least one value above the normal laboratory range of either ALT or AST during the study period. RESULTS: Fourteen patients developed hepatotoxicity, giving an incidence of 28%. Children receiving MTX had higher alanine aminotransferase (ALT) interquartile range (IQR) (26 [21-359] vs. 23[20-32]; p =0.003), higher aspartate aminotransferase (AST) interquartile range (IQR) (31 [22-267] vs. 28[2-35] IU/L; p <0.001), and lower alkaline phosphatase (ALP) mean (±SD) (98±35.5 vs. 256 ± 39.5 IU/L; p <0.001). However, there were no significant differences in age, sex, weight, type of JIA, and duration of MTX treatment (p< 0.05). CONCLUSION: Hepatotoxicity due to MTX, based on liver chemistry, is common among children with JIA.

Predictors of Short-Term Outcome of Kasai Portoenterostomy for Biliary Atresia in Infants: a Single-Center Study.

PURPOSE: The outcome predictors of Kasai portoenterostomy (KPE) for biliary atresia (BA) are controversial. This study aimed to identify possible short-term outcome predictors of KPE for BA in infants. METHODS: This retrospective study included infants with BA who underwent KPE between January 2015 and December 2017 and were followed up for at least 6 months after surgery at the Pediatric Hepatology Unit, Cairo University Pediatric Hospital, Egypt. The short-term outcome was jaundice clearance within 6 months following surgery. All data were compared between the jaundice free group and those with persistent jaundice to identify the predictors of jaundice clearance. RESULTS: The study included 75 infants. The mean age at the time of surgery was 82.43±22.77 days (range, 37-150 days), and 28 (37.3%) infants cleared their jaundice within 6 months postoperative. Age at surgery did not significantly affect the outcome (p=0.518). Infants with persistent jaundice had significantly higher pre-operative levels of aspartate aminotransferase (AST) than those who were jaundice free (p=0.041). Receiver operating characteristic curve analysis showed that preoperative AST ≤180 IU/L was predictive of a successful KPE, with sensitivity 74.5% and specificity 60.7%. Infants with bile plugs in liver biopsy had a 6-fold higher risk of persistent jaundice than those without bile plugs (95% confidence interval: 1.59-20.75, p=0.008). CONCLUSION: Jaundice clearance after KPE for BA can be predicted using preoperative AST and presence of bile plugs in liver biopsy.