Clinical profile of adult and pediatric patients with hepatic Wilson's disease.

BACKGROUND: The clinical profile varies in patients with Wilson's disease (WD). There is paucity of data regarding adult and pediatric patients with hepatic WD. METHODS: As many as 140 consecutive patients diagnosed with hepatic WD between December 2006 and January 2021 were included in the study. Data was collected regarding the demographic parameters, clinical presentation, extrahepatic organ involvement, liver histology and laboratory investigations. Adult and children (0-14 years) with hepatic WD were compared regarding these features. RESULT: Eighty-eight adults and 52 children were included in the study. The median age of presentation was 17 years (range: 1.1-42 years). Male preponderance was seen (adult 68/88, 69%; children 40/52, 77%). Adults as compared to children presented more commonly as cirrhosis (52/88 vs. 15/52, p = 0.0005) and with hepatic decompensation (35/88 vs. 9/52, p = 0.005). Presentation with acute-on-chronic liver failure (ACLF) was more common in children (10/52 vs. 2/88, p = 0.0005). Twenty-eight-day mortality was 50% (5/10) in children and none in adults presenting with ACLF. Nazer's Prognostic Index (≥ 7) and New Wilson Index were more accurate in predicting mortality among children with ACLF with AUROC 1, while AARC (APASL ACLF Research Consortium) was less accurate with AUROC 0.45. Liver histology findings were similar in adults and children. Extrahepatic involvement was also similar. (8/88 in adults vs. 3/52 children, p value 0.48). CONCLUSION: Most patients with WD present as cirrhosis in adulthood. ACLF is more common in children. Nazer's prognostic index and new Wilson Index score are accurate in predicting mortality in children with ACLF.

Anomalous Pancreatobiliary Ductal Union Presenting as Recurrent Acute and Chronic Pancreatitis in Children and Adolescents With Response to Endotherapy.

Introduction Anomalous pancreaticobiliary duct union (APBDU) is defined by the abnormal position of the junctional union of the common bile duct and the pancreatic duct, outside the duodenal wall above the influence of sphincter of Oddi, associated with choledochal cysts and biliary malignancies. APBDU may rarely present as recurrent acute pancreatitis (RAP) or chronic pancreatitis (CP). We aimed to study the prevalence of patients with APBDU presenting as RAP or CP and their response to endotherapy. Methods A retrospective audit of the prospectively maintained endoscopy database at our institute between January 2018 and November 2020 was conducted to identify cases of APBDU presenting as RAP or CP. Details of investigations, endoscopic retrograde cholangiopancreatography (ERCP) findings, and follow-up till six months were noted. Results We identified 26 cases of APBDU, of which five (19.2%) cases presented as RAP or CP. Of these five patients, two had RAP, while three presented with CP (median: 11 years; range: 4-25 years). Magnetic resonance cholangiopancreatography (MRCP) showed APBDU in three patients. One patient with RAP had a Komi type IIIB anomaly. Another patient with RAP had a rare anomaly with absent ventral PD, with the bile duct communicating and draining through the dorsal duct. Two patients with CP had a long common channel with Komi IIA anomaly. One patient with CP had IIIC2 anomaly. Pancreas divisum was noted in three patients, all of whom underwent minor-papilla sphincterotomy. Successful pancreatic stent placement was performed in all patients. Over one year of follow-up, patients with CP had a significant decrease in pain as measured by the visual analog scale. Those with RAP had no further episodes of pancreatitis. Conclusion APBDU is a rare cause of RAP and CP in young patients, occasionally missed on MRCP. RAP and CP caused by APBDU show good response to endotherapy.