Practice Recommendations for Metabolic Dysfunction-Associated Steatotic Liver Disease by the Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition (ISPGHAN).

JUSTIFICATION: There has been an alarming increase in metabolic dysfunction-associated steatotic liver disease (MASLD) and it is now the most common chronic liver disease worldwide, in both adult and pediatric populations. The lack of regional guidelines has hampered the formulation of national policies for prevention and management of MASLD in children. Therefore, we formulated recommendations for steatotic liver disease in children. OBJECTIVES: To review the existing literature on the burden and epidemiology of pediatric MASLD and formulate recommendations for diagnostic evaluation, prevention, and management strategies. PROCESS: The Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition invited national and international stakeholders to participate in a consensus meeting held on April 20, 2024, in Mumbai, Maharashtra, India. Various aspects of pediatric steatotic liver disease were deliberated upon and a consensus document and recommendations were formulated after several rounds of discussion. RECOMMENDATIONS: Metabolic dysfunction associated steatotic liver disease (MASLD) should be used as the preferred term in place of non-alcoholic fatty liver disease (NAFLD). There is a high prevalence of steatotic liver disease (SLD) among Indian children and adolescents, especially those who are overweight or obese. This condition may be progressive in childhood and associated with increased morbidity and mortality in adulthood. Various lifestyle, dietary, and genetic factors may predispose individuals to MASLD, including an increased intake of calorie-dense processed foods, sweetened sugar beverages, excessive screen time, higher sedentary time and lack of moderate to vigorous physical activity. MASLD is usually asymptomatic or presents with mild, non-specific symptoms and therefore, a high degree of suspicion is required for early diagnosis. MASLD is usually associated with cardiometabolic factors (hypertension, insulin resistance/diabetes mellitus, and/or dyslipidemia) and secondary causes should be excluded in all cases, particularly in the presence of red flag signs. Screening for MASLD should be considered in all obese children (body mass index or BMI ≥ 95th percentile) and in all overweight children (BMI ≥ 85th and <95thpercentile) with additional risk factors, such as prediabetes/diabetes, dyslipidemia, positive family history of metabolic syndrome, obstructive sleep apnea, and hypopituitarism. Abdominal ultrasound in combination with alanine aminotransferase (ALT) levels should be used as a screening test for MASLD in Indian children as per the proposed algorithm. Diet (any hypocaloric diet) and exercise (aerobic, resistance, or a combination of both; moderate to high intensity; regular in frequency) remain the cornerstones of pediatric MASLD management. Pharmacotherapy and/or endoscopic/surgical techniques for obesity should be considered as adjuncts and should be considered only after a failed adequate trial of lifestyle modifications.

Diagnosis and management of pediatric acute liver failure: consensus recommendations of the Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition (ISPGHAN).

Timely diagnosis and management of pediatric acute liver failure (PALF) is of paramount importance to improve survival. The Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition invited national and international experts to identify and review important management and research questions. These covered the definition, age appropriate stepwise workup for the etiology, non-invasive diagnosis and management of cerebral edema, prognostic scores, criteria for listing for liver transplantation (LT) and bridging therapies in PALF. Statements and recommendations based on evidences assessed using the modified Grading of Recommendations Assessment, Development and Evaluation (GRADE) system were developed, deliberated and critically reappraised by circulation. The final consensus recommendations along with relevant published background information are presented here. We expect that these recommendations would be followed by the pediatric and adult medical fraternity to improve the outcomes of PALF patients.

Unusual case of immune haemolytic disease causing severe neonatal cholestasis in a newborn.

Neonatal hyperbilirubinaemia is a very common entity witnessed in most of the newborns. Rarely are there events where the bilirubin levels reach extreme values mandating invasive therapy. Unconjugated hyperbilirubinaemia when solely present is easy to manage and diagnose the common aetiological factors associated with it. The issue arises when we come across a mixed picture of conjugated with unconjugated hyperbilirubinaemia and puts us in a dilemma as to what are we treating. Our case highlights a similar picture where we witnessed the highest documented levels of total bilirubin but to our surprise the major component of which was direct bilirubin. This report takes us through the differentials which were ruled out and our management strategies for solving this rare mystery.

Natural history, risk factors, and outcome of hepatopulmonary syndrome in pediatric liver diseases.

BACKGROUND: Limited pediatric literature is available regarding hepatopulmonary syndrome (HPS) especially in subjects with biliary atresia (BA) despite its proven prognostic significance. Thus, we aimed to study the natural history, risk factors, and outcome of HPS in BA and other chronic liver disease (CLD) subjects. METHODS: All children (BA and other non-BA CLDs) older than 6 months of age were included in the study. HPS was diagnosed on the basis of standard international criteria. Also, fractional exhaled nitric oxide (FeNO) was measured at baseline. RESULTS: During the study period from January 2017 to December 2018, there were 42 children in BA and 62 in the CLD group. The overall prevalence of HPS was 42.3%: 57.1% in the BA group and 32.2% in the CLD group. Median age at HPS diagnosis was 14.4 months and 90 months in the BA and non-BA CLD groups, respectively. By the end of study period, the prevalence of HPS in the BA group further increased to 73.8% at 0.7% per month. Lower serum albumin (p < 0.05) in BA and higher splenic Z scores (p 0.013) in other CLDs were found to be significant risk factors for HPS. FeNO measurement did not reach diagnostic significance. CONCLUSION: Prevalence of HPS is higher and also develops at an earlier age in the BA group compared to other CLDs. Also, risk of HPS development increases with increasing disease duration in BA. Lower serum albumin in BA and higher splenic Z scores in other CLDs may predict risk for HPS development.