Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare entity that mimics various inflammatory strictures of the small intestine. Pediatric literature is scarce. We analyzed the clinical, radiological, endoscopic and histopathological features of children with CMUSE that differentiate it from small bowel Crohn’s disease (SBCD) and gastrointestinal tuberculosis (GITB). CMUSE was diagnosed by the following criteria: (1) unexplained small bowel strictures with superficial ulcers, (2) chronic/relapsing ulcers of small bowel after resection, (3) no signs of systemic inflammation, (4) absence of other known etiologies of small bowel ulcers. SBCD and GITB were diagnosed based on standard criteria. The clinical features, laboratory parameters, radioimaging, endoscopy (including video capsule endoscopy [VCE], intra-operative endoscopy), histopathological features and treatment outcome were noted. Out of 48, CMUSE was diagnosed in 13 (27

Hereditary fructose intolerance (HFI) is a rare autosomal recessive inherited disorder that occurs due to the mutation of enzyme aldolase B located on chromosome 9q22.3. A fructose load leads to the rapid accumulation of fructose 1-phosphate and manifests with its downstream effects. Most commonly children are affected with gastrointestinal symptoms, feeding issues, aversion to sweets and hypoglycemia. Liver manifestations include an asymptomatic increase of transaminases, steatohepatitis and rarely liver failure. Renal involvement usually occurs in the form of proximal renal tubular acidosis and may lead to chronic renal insufficiency. For confirmation, a genetic test is favored over the measurement of aldolase B activity in the liver biopsy specimen. The crux of HFI management lies in the absolute avoidance of foods containing fructose, sucrose, and sorbitol (FSS). There are many dilemmas regarding tolerance, dietary restriction and occurrence of steatohepatitis. Patients with HFI who adhere strictly to FSS free diet have an excellent prognosis with a normal lifespan. This review attempts to increase awareness and provide a comprehensive review of this rare but treatable disorder.

Delayed referral of neonatal cholestasis (NC) can result in significant morbidity and mortality. In this multi-center study, we aimed to evaluate the reliability of the stool card in the Indian population and develop an integrated NC card with (a) urine color identification and (b) stool color for early referral. Consecutive children with NC were enrolled and divided into two groups (biliary atresia [BA] and non-BA). Normal healthy children at 6–8 weeks of age served as controls. Each photograph of stool and urine samples of every child was evaluated by 6 parents, 6 paramedical staff, and 4 trainee doctors using a stool color card as a reference for stool samples. Of 319 children (BA [n = 58], non-BA [n = 62], and controls [n = 199]), parents correctly detected dark yellow urine in all NC. Stool samples of 50 (86%) children with BA were unanimously labeled as pale by all observers. The average inter-item correlation showed good correlation between parents and trainee doctors of 0.77 and 0.64 with paramedical staff. The integrated NC card proposes to recognize neonatal cholestasis at an early stage irrespective of etiology. It is a major step towards public health benefit both at the community as well as physicians’ levels to enable early detection and timely referral and management.

Background: Late-onset hepatic failure (LOHF) is a distinct entity of intractable liver diseases with limited pediatric experience. We aimed to identify the etiology and risk factors that determine the poor outcome (PO) of pediatric LOHF. Methods: LOHF was defined as liver failure occurring 5–24 weeks after onset of jaundice and without any evidence of underlying chronic liver disease. PO (death or liver transplantation within 160 days) was compared with spontaneous recovery (SR; complete normalization of liver functions in the native liver). Pediatric end-stage liver disease (PELD) score and King’s College Criteria (KCC) were applied to investigate their prognostic value. Results: We enrolled 47 children (6 [2–16] years) with LOHF. Hepatitis A was the most common etiology (15, 32%) and 64% complicated with infections. Twelve children (25%) had SR over 6 (1–24) months, while 28 (60%) children had PO. Univariate analysis showed indeterminate etiology, hepatic encephalopathy (HE), infection, acute kidney injury, and high PELD score determined PO. On multivariate regression analysis, only PELD score with a cutoff 32 (area under curve 0.833, sensitivity 68%, specificity 92%) predicted PO. KCC showed a sensitivity of 85.7%, specificity of 41.7% to determine PO in our cohort. Conclusion: Indeterminate etiology, presence of HE, occurrence of infection at any site, and acute kidney injury lead to the PO. PELD score ≥32 can be utilized to optimize the listing for liver transplantation. A significant proportion survives with the native liver.

Recent years have shown a rise in occurrence of multidrug resistant ascitic fluid infection (AFI) including resistant to third generation cephalosporins. Our aim was to find the prevalence, antibiotics resistance and outcome of AFI in children with liver disease. Children (≤ 18 years) with liver disease-related ascites were prospectively enrolled from April 2015 to October 2017. Based on the results of ascitic fluid examination and culture, patients were classified as having AFI [spontaneous bacterial peritonitis (SBP), culture negative neutrocytic ascites (CNNA) and monomicrobial non-neutrocytic bacterascites (MNB)] and no-AFI. AFI diagnosed after 48 h of index hospitalization was considered as nosocomial. We enrolled 194 children with a median age of 85 [2–216] months. Chronic liver disease was the commonest etiology (153, 79%). AFI was present in 60 (31%) children [SBP (n = 13), CNNA (n = 39), MNB (n = 8)] of which 53% were nosocomial and resulted in high in-hospital mortality. Gram-negative bacilli dominated the ascitic fluid culture (12/21, 57%) and 10/12 (83%) of them were extended spectrum beta-lactamases (ESBL) producers. Six (60%) ESBL producers were sensitive to cefoperazone–sulbactam and 70% to carbapenems. Child–Pugh-Turcotte (CPT) score of ≥ 11 independently determined in-hospital mortality in children with AFI. AFI was found in 31% children with liver disease and almost half of them were nosocomial resulting in high mortality. ESBL producing Gram-negative bacteria were the most frequently isolated organisms. Cefoperazone–sulbactam or carbapenems may be useful empirical antibiotics in nosocomial setting. Children with AFI and CPT score ≥ 11 should be evaluated for liver transplantation.

OBJECTIVES:Endoscopic ultrasound (EUS) is an established tool for evaluation of adults with acute recurrent pancreatitis (ARP) whereas data in pediatrics is limited. Our study assessed the role of EUS in identifying etiology including changes of chronic pancreatitis (CP) in children and adolescents with ARP. METHODS:Children with ARP (≥2 episodes of acute pancreatitis [AP]) were prospectively evaluated with a detailed clinical proforma and EUS. Subjects with known etiology of ARP or CP on ultrasonography/computed tomography and magnetic resonance cholangiopancreatography (MRCP, Cambridge grade ≥3) were excluded. Parenchymal and ductal changes on EUS as per minimal standards terminology (MST) features were noted. RESULTS:Thirty-two children (22 boys, age 14 [8-18] years) with ARP (median of 3 [2-5] episodes of AP) were enrolled. EUS was safe and technically successful in all. Gall bladder sludge was found in 1 (3%) case and none had other pancreatobiliary structural abnormalities. EUS diagnosis of CP (≥4 features) was made in 10/32 (31%) cases. Subjects with CP on EUS had a longer disease duration than those without CP (45 [10-97] vs 22 [8-78] months; P = ns). MRCP was normal in 28 and showed pancreas divisum in 1 case. Three cases had equivocal (Cambridge II) changes at initial MRCP and 2 of them had repeat MRCP, which showed definite (Cambridge IV) CP. All these 3 cases had CP on EUS. CONCLUSIONS:EUS diagnosed CP (≥4 features) in 31% and biliary abnormality in 3% children with ARP. EUS is safe, sensitive, and useful for early diagnosis of CP in children with ARP.

Background and Aims: Sub-acute hepatic failure (SAHF) is a grey zone of liver dysfunction with variable course. Pediatric experience of SAHF is poorly highlighted in literature. We aimed to identify the risk factors that determine poor outcome of SAHF in children. Methods: Children admitted with SAHF were analysed. Poor outcome (PO: death or liver transplantation within 90 days) was compared with spontaneous recovery (SR: complete normalisation of liver functions in native liver). Pediatric End stage Liver Disease (PELD) and King's College Criteria (KCC) were applied to investigate their prognostic value. Results: Forty eight children with median age 6 (range, 2–16) years were enrolled. Etiological workup showed hepatitis A as most common etiology (15, 31%), Hepatitis B in 6 (12.5%), Hepatitis E in 1 (2%), Co-infection with hepatitis A and E in 7 (14.5%), Cytomegalovirus in 1 (2%) and Autoimmune hepatitis type 2 in 1 (2%), indeterminate in 15 (31%) while 2 (4%) children had incomplete evaluation. Twelve children (25%) had SR over a median period of 6 months (range, 1–24). Twenty eight (58.3%) children had PO while another 8 (16.7%) were recovering but lost to follow-up and excluded from analysis. Univariate analysis comparing PO and SR is shown in Table 1. On multivariate logistic regression analysis, only PELD score with a cut-off 32 (AUC: 0.833, sensitivity 68%, specificity 92%) predicted PO. King's college criteria (KCC) showed a sensitivity of 85.7%, specificity of 41.7%, positive predictive value of 77.4% and negative predictive value of 55.6% in our cohort. Conclusions: Hepatitis A is the most common etiology of SAHF in chldren. Non-viral etiology, hepatic encephalopathy, occurrence of infection and acute kidney injury determine PO. PELD score with a cut-off 32 has better specificity than KCC to predict PO. A significant proportion (42%) may escape liver transplantation. The authors have none to declare.

Various atypical manifestations have been described in acute viral hepatitis (AVH). We evaluated the prevalence, clinical features, response to treatment and outcome of various atypical manifestations of AVH in children. Consecutive children (≤ 18 years) with AVH due to hepatitis A, B, or E were studied while patients with acute or acute on chronic liver failure were excluded. Diagnosis of atypical manifestations was based on standard criteria. A total of 477 children with AVH (median age 7.0 (5–11) years, 74% boys) were seen; 22% (n = 106) had atypical manifestations. Prolonged cholestasis was the most common (11%), followed by ascites (7%), intravascular hemolysis (3%), relapsing hepatitis (2%), acute pancreatitis (1.3%), and thrombocytopenia (0.7%). Atypical manifestations were more common in HAV as compared to HBV (30% vs. 3%, p = 0.00) and HEV (30% vs. 15%, p = 0.07). Prolonged cholestasis was significantly more common in older children (20% in > 10 years vs. 9% in 6–10 years ; p = 0.009 and 5% in 0–5 years of age [p < 0.000]). Ascites was more common in younger children, although not significant. All patients recovered with supportive treatment.

Background and Aim: Prognostic scoring systems (PSI) have not been validated to identify high risk children with chronic Budd-Chiari syndrome (BCS) who undergo radiological intervention (RI). Our aim was to identify which of known PSI predict the outcomes in various subgroups of chronic BCS.Methods: Admitted children with chronic BCS (October 2011–March 2016) were analyzed in three subgroups: (a) SI: successful intervention (patent stent at last follow-up); (b) PO: poor outcome (stent block or death) and (c) NU: naïve unintervened (undergoing endotherapy and awaiting RI). PSI analyzed were PELD, Rotterdam score, BCS-TIPS, Zeitoun and Murad prognostic indices in all and MELD (MELD-Na, iMELD, uMELD) in children >12 years.Results: 68 patients had median age at presentation 10 (1.5–17) years and duration of illness 6 (1–48) months. 23 patients lost to follow-up for >1 year were excluded. 26 of 31 underwent 28 successful primary procedures: HV angioplasty + stenting (n = 17), IVC angioplasty + stenting (n = 3) HV angioplasty (n = 2), IVC angioplasty (n = 3) and modified trans-jugular intrahepatic peritoneal shunt (n = 3). Stent patency restored in 4/9 secondary recanalization. 4/5 stent block and 5 unintervened patients died. Finally 3 groups (SI: n = 21, PO: n = 10, NU: n = 14) were analyzed. PELD cut-off of 11 determined poor outcome (AUC: 0.74, 80% sensitivity, 67% specificity, P = 0.02). Comparisons: PELD [SI: 4 (−8 to 68) vs. PO: 16 (9–67); P = 0.02]; Rotterdam score [NU: 1.08 (0.04–2.45) vs. PO: 1.18 (1.09–3.43), P = 0.04; SI: 1.10 (0.03–3.11) vs. PO: 1.18 (1.09–3.43), P = 0.05]; Murad prognostic index [SI: 1.04 (0.01–3.03) vs. PO: 1.04 (1.04–3.05), P = 0.04; NU: 1.04 (0.01–2.32) vs. PO: 1.04 (1.04–3.05), P = 0.04]. Overall BCS-TIPS index [1.9 (0.93–5.17)] and other scores were not significant among the three groups.Conclusion: Interventional success and survival in pediatric chronic BCS is determined by PELD and Murad prognostic indices. Rotterdam score is helpful only prior to intervention. BCS-TIPS index is not applicable in children.Conflicts of InterestThe authors have none to declare. Background and Aim: Prognostic scoring systems (PSI) have not been validated to identify high risk children with chronic Budd-Chiari syndrome (BCS) who undergo radiological intervention (RI). Our aim was to identify which of known PSI predict the outcomes in various subgroups of chronic BCS. Methods: Admitted children with chronic BCS (October 2011–March 2016) were analyzed in three subgroups: (a) SI: successful intervention (patent stent at last follow-up); (b) PO: poor outcome (stent block or death) and (c) NU: naïve unintervened (undergoing endotherapy and awaiting RI). PSI analyzed were PELD, Rotterdam score, BCS-TIPS, Zeitoun and Murad prognostic indices in all and MELD (MELD-Na, iMELD, uMELD) in children >12 years. Results: 68 patients had median age at presentation 10 (1.5–17) years and duration of illness 6 (1–48) months. 23 patients lost to follow-up for >1 year were excluded. 26 of 31 underwent 28 successful primary procedures: HV angioplasty + stenting (n = 17), IVC angioplasty + stenting (n = 3) HV angioplasty (n = 2), IVC angioplasty (n = 3) and modified trans-jugular intrahepatic peritoneal shunt (n = 3). Stent patency restored in 4/9 secondary recanalization. 4/5 stent block and 5 unintervened patients died. Finally 3 groups (SI: n = 21, PO: n = 10, NU: n = 14) were analyzed. PELD cut-off of 11 determined poor outcome (AUC: 0.74, 80% sensitivity, 67% specificity, P = 0.02). Comparisons: PELD [SI: 4 (−8 to 68) vs. PO: 16 (9–67); P = 0.02]; Rotterdam score [NU: 1.08 (0.04–2.45) vs. PO: 1.18 (1.09–3.43), P = 0.04; SI: 1.10 (0.03–3.11) vs. PO: 1.18 (1.09–3.43), P = 0.05]; Murad prognostic index [SI: 1.04 (0.01–3.03) vs. PO: 1.04 (1.04–3.05), P = 0.04; NU: 1.04 (0.01–2.32) vs. PO: 1.04 (1.04–3.05), P = 0.04]. Overall BCS-TIPS index [1.9 (0.93–5.17)] and other scores were not significant among the three groups. Conclusion: Interventional success and survival in pediatric chronic BCS is determined by PELD and Murad prognostic indices. Rotterdam score is helpful only prior to intervention. BCS-TIPS index is not applicable in children. Conflicts of InterestThe authors have none to declare. The authors have none to declare.