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.

Study on Steroid Induced Ocular Findings in Children with Nephrotic Syndrome.

INTRODUCTION: Long term use of corticosteroids in Nephrotic Syndrome (NS) is associated with ocular complications such as Posterior Subcapsular Cataract (PSC), glaucoma, increased intra-ocular pressure, ptosis, mydriasis, eyelid skin atrophy, keratisis, thinning of cornea and sclera, repeated hordeolum exacerbations. AIM: To determine ocular abnormalities in children with NS and their association with steroid dosing and duration. MATERIALS AND METHODS: Hundred patients with NS in the age group of 2-18 years, with no evidence of other systemic disease, were considered for the study. Group I included cases (66) who took standard steroid regimen for first episode of NS. Group II included cases (34) who took steroid irrationally (daily basis for long time) other than standard regimen. Steroid induced ocular complications were compared in both groups after detailed ophthalmological examination. Statistical analysis was done using Statistical Package for Social Science (SPSS-20.0). RESULTS: Ocular abnormalities were found in 12 patients out of 66 in Group I (18%) and 16 patients out of 34 patients in Group II (47%). Out of 12 patients of Group I who had ocular problems, three had myopic astigmatism, eight patients had PSC and one had temporal disc pallor. While in Group II, out of 16 patients who had ocular complications, two patients had raised intra ocular pressure and two patients had myopic astigmatism and 12 patients had posterior sub capsular cataract. The difference in mean cumulative steroid doses in both groups was statistically significant but no such difference was there between duration of steroid intake in both group. CONCLUSION: Our study concluded that ocular complications were more common in patients with irrationally steroid intake and cumulative steroid dose intake was also significantly higher in same patients.

Progressive Familial Intrahepatic Cholestasis (PFIC) in Indian Children: Clinical Spectrum and Outcome.

OBJECTIVE: To study the clinical and laboratory profile of children with progressive familial intrahepatic cholestasis (PFIC) and evaluate their outcome. METHODS: The study is a retrospective review of all cases diagnosed with PFIC between January 2011 and July 2015. All children underwent histopathological examination and immunostaining. Management was done as per institute's protocol. RESULTS: There were a total of 24 PFIC cases (PFIC 1-2, PFIC 2-19, PFIC 3-3). Eleven presented as neonatal cholestasis, whereas 13 others presented after 6 months of life. Median age of presentation in PFIC 2 was 5.5 months with a time lag of 13 months in diagnosis. PFIC 1 and 2 presented in infancy, whereas PFIC 3 presented late. Familial clustering was seen in 12 of 24 cases. Pruritus resolved with medical management in two-thirds of cases, 3 cases required biliary diversion (BD) with dramatic improvement. One child improved after liver transplantation. CONCLUSIONS: PFIC accounts for 8% of neonatal cholestasis and 34% of cholestasis in older children with PFIC 2 being the commonest subtype. Medical therapy is successful in majority. Partial internal BD should be offered to non-cirrhotic low gamma glutamyl transferase PFIC with intractable pruritus. Progression to cirrhosis may be prevented or delayed by early diagnosis and timely intervention.