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.

COVID-19 pandemic and inflammatory bowel disease from patients' perspective: A survey from COVID epicenter in India.

BACKGROUND AND AIM: The COVID pandemic and countrywide lockdown has had significant impact on patients with inflammatory bowel disease (IBD), with delay in diagnosis, difficulty in access to healthcare and unavailability of drugs. We conducted a telephonic survey to assess this impact. METHODS: Out of 350, 302 participated in the survey. Demographic data, disease severity at the time of survey, extent of disease, details of therapy, and adherence were noted. A validated questionnaire addressing information source, perception of COVID-19 situation, contact with healthcare, and adherence to standard precautions was administered telephonically. RESULTS: Out of 350 contacted patients, 302 (86.28%) patients participated in the survey. Median age of cohort was 39 years. Ulcerative colitis (UC) constituted 79%, 16% Crohn's disease (CD), and 5% IBD-unclassified. At the time of survey, 86.98% patients with UC were in clinical remission and 75.75% of CD patients were generally well. A total of 115 (38%) cases were nonadherent to therapy due to unavailability of medicines (66.38%), financial constraints (25.21%) and inability to reach healthcare facility (3.6%). Disease flare was seen in 14.2% and correlated well with nonadherence. Existing drug therapy was switched to alternative drug in 70 (23.17%) cases due to unavailability (74%). Social media (52.3%) and television (40.4%) were the common sources of information about the pandemic. Telemedicine platforms (WhatsApp and telephone) were used by 180 (59.6%) patients for consultation with good acceptance (81.6%). 87 (28.8%) patients failed to contact healthcare. Apprehension regarding severe COVID infection was noted in 80% while 29% thought that IBD therapy could increase infection risk. Adherence to wearing mask, hand washing, and social distancing was 100%. CONCLUSION: Pandemic resulted in disruption of healthcare visits and medication supply. Majority were concerned about increased risk of COVID-19 infection and adhered to standard precautions. Mobile phone-based formats for patient care may be an alternative due to patient acceptance and convenience.