Pediatric Hepatic Venous Outflow Tract Obstruction: Experience from a Transplant Center.

We performed a review of case records of children diagnosed with hepatic venous outflow tract obstruction at our center in last 10 years. Out of 11 cases identified, 6 had variable blocks in the hepatic venous system and 4 had combined hepatic venous and inferior vena cava (IVC) block. One child with paroxysmal nocturnal hemoglobinuria (PNH) had isolated IVC involvement. Angioplasty was attempted in 3 patients; among them 2 had successful outcome. Seven children with advanced liver disease underwent transplantation, which was successful in six. With availability of modalities like interventional radiology and transplantation, the overall prognosis of hepatic venous outflow tract obstruction seems to be good when managed in a well-equipped center.

Celiac disease: What the Indian pediatricians know about the disease.

To ascertain the knowledge, awareness, and practices pertaining to celiac disease (CD) among the Indian pediatricians. A survey link containing a questionnaire was shared through electronic mail using a pediatric database. The survey was kept active for 6 months; all responses received at the end of the survey were analyzed. Two hundred and seventy one pediatricians out of more than 10,000 chose to respond to the survey. Most pediatricians agreed that more patients with CD are being diagnosed than earlier. The reasons for higher detection of CD were perceived to be higher index of clinical suspicion by pediatricians (86.7%) followed by increased awareness among parents (45.8%). Most pediatricians opined that clinical manifestations which prompted to a diagnosis of CD were failure to thrive (96.2%) and chronic diarrhea (81.4%). Knowledge about atypical manifestations of celiac disease was low.  Though knowledge about the common association of CD with type 1 diabetes (62.1%) and autoimmune hepatitis (55.8%) was there, awareness about its association with other uncommon conditions was lacking. Though 68% of the pediatricians were of the opinion that the confirmation of diagnosis by a mucosal biopsy is necessary, 26.5% of respondents believed that only a positive serology was sufficient for a diagnosis. A trial of gluten-free diet (GFD) was thought to be a logical step if serology was positive by 31.3% of respondents. While 87.7% of pediatricians advocated lifelong adherence to GFD, 12.3% felt that GFD could be discontinued in the future. This web-based survey revealed that though pediatricians are seeing increasing number of celiac disease patients, there is a need to increase awareness regarding the disease, its associated conditions, the need for mucosal biopsy to confirm the diagnosis and the necessity of lifelong adherence to GFD.

Prevalence of Nonalcoholic Fatty Liver Disease in Normal-weight and Overweight Preadolescent Children in Haryana, India.

OBJECTIVE: To document the prevalence of non-alcoholic fatty liver disease (NAFLD) and metabolic parameters among normal-weight and overweight schoolchildren. STUDY DESIGN: Cross-sectional study. SETTING: Thirteen private schools in urban Faridabad, Haryana. PARTICIPANTS: 961 school children aged 5-10 years. METHODS: Ultrasound testing was done, and 215 with fatty liver on ultrasound underwent further clinical, biochemical and virological testing. OUTCOME MEASURES: Prevalence of fatty liver on ultrasound, and NAFLD and its association with biochemical abnormalities and demographic risk factors. RESULTS: On ultrasound, 215 (22.4%) children had fatty liver; 18.9% in normal-weight and 45.6% in overweight category. Presence and severity of fatty liver disease increased with body mass index (BMI) and age. Among the children with NAFLD, elevated SGOT and SGPT was observed in 21.5% and 10.4% children, respectively. Liver enzyme derangement was significantly higher in overweight children (27% vs 19.4% in normal-weight) and severity of fatty liver (28% vs 20% in mild fatty liver cases). Eleven (8.1%) children with NAFLD had metabolic syndrome. Higher BMI (OR 35.9), severe fatty liver disease (OR 1.7) and female sex (OR 1.9) had strong association with metabolic syndrome. CONCLUSION: 22.4% of normal-weight and overweight children aged 5-10 years had fatty liver. A high proportion (18.9%) of normal-weight children with fatty liver on ultrasound indicates the silent burden in the population.

Pediatric Inflammatory Bowel Disease.

BACKGROUND: The incidence of inflammatory bowel disease is increasing in the pediatric population worldwide. NEED AND PURPOSE OF REVIEW: There is paucity of high quality scientific data regarding pediatric inflammatory bowel disease. Most of the guidelines are offshoots of work done in adults, which have been adapted over time to diagnose and treat pediatric patients. This is in part related to the small numbers in pediatric inflammatory bowel disease and less extensive collaboration for multicentric trials both nationally and internationally. METHODS: A literature search was performed using electronic databases i.e. Pubmed and OVID, using keywords: pediatric, inflammatory bowel disease, Crohns disease, Ulcerative colitis, epidemiology and guidelines. This article amalgamates the broad principles of diagnosing and managing a child with suspected inflammatory bowel disease. MAIN CONCLUSIONS: 25% of the patients with inflammatory bowel disease are children and and young adolescents. The primary concern is its impact on growth velocity, puberty and quality of life, including psychosocial issues. Treatment guidelines are being re-defined as the drug armamentarium is increasing. The emphasis will be to achieve mucosal healing and normal growth velocity with minimal drug toxicity.

Prevalence of functional gastrointestinal disorders among adolescents in Delhi based on Rome III criteria: A school-based survey.

BACKGROUND: Functional gastrointestinal diseases (FGIDs) are emerging as an important cause of morbidity in adolescents globally. The prevalence of FGIDs among Indian children or adolescents is not clear. METHODS: A cross-sectional school-based survey conducted in 1115 children aged 10-17 years attending four semi urban government schools of National capital territory (NCT) of Delhi. Rome III questionnaire was translated into Hindi and was filled by the students under supervision. Prevalence of FGIDs was calculated. RESULTS: Ten percent (112) adolescents had FGIDs. Out of 112, 52 % (58) were boys, and 48 % (54) were girls. 2.7 % (30) had functional dyspepsia, 1.3 % (15) had irritable bowel syndrome, 1.4 % (16) had abdominal migraine, 1.5 % (17) had aerophagia, 0.4 % (5) had functional abdominal pain syndrome, and 0.3 % (4) had functional abdominal pain. Prevalence of functional constipation, adolescent rumination syndrome, cyclical vomiting syndrome, and non-retentive fecal incontinence were 0.5 % (6), 0.3 % (4), 0.3 % (3), 0.4 % (5), respectively. Functional abdominal pain-related FGID were present in 6.3 % (70) children (35 boys and 35 girls). Functional constipation (4 vs. 2) and functional abdominal pain syndrome (4 vs. 1, p < 0.05) were significantly more in females. CONCLUSIONS: The prevalence of functional gastrointestinal disorders in our study was 10 %. The most frequent FGID noted was functional dyspepsia.

Living Related Liver Transplantation for Biliary Atresia in the Last 5 years: Experience from the First Liver Transplant Program in India.

OBJECTIVE: To study the clinical, biochemical profile and outcome of patients with biliary atresia (BA) who underwent living related liver transplantation (LRLT) at authors' institute in the last 5 y (2008-2013). METHODS: Case records of the 20 patients diagnosed with biliary atresia who had undergone living related liver transplantation at authors' centre in the last 5 y were analysed. RESULTS: Eighteen patients with BA with a failed Kasai procedure and 2 without a prior Kasai's portoenterostomy received a liver transplant. At a median follow up of 2 y and 6 mo, both the patient and graft survival rates were 90 %. The median age of the recipients at the time of LRLT was 8 mo and 12 (60 %) of the transplanted children were less than or equal to 1 y of age. The male-female ratio was 1.8:1. The median weight was 7.3 kg (5.8-48 kg); two thirds were less than 10 kg. The median pre-transplant total serum bilirubin (TSB) and international normalized ratio (INR) were 12.98 (0.5-48.3) mg/dl and 1.3 (1.0-3.9) respectively. All patients received a living related graft and there was no donor mortality. The median duration of postoperative ventilation was 14 h. The post-operative complications were infection (30 %), vascular complications (20 %) and acute rejection (20 %). The median duration of postoperative hospital stay was 21 d (17-42). Two patients died of combined hepatic and portal vein thrombosis in the early postoperative period. Late rejection was encountered in 1 patient and another developed chronic kidney disease necessitating a renal transplant. There were no late vascular occlusions or development of post transplant lymphoproliferative disease. CONCLUSIONS: Thus, LRLT for BA with or without a prior portoenterostomy, is a feasible and successful treatment modality with good outcomes attained despite the challenges of age and size. This treatment modality is now well established in India.

Experience of 100 solid organ transplants over a five-yr period from the first successful pediatric multi-organ transplant program in India.

To analyze the clinical profile and outcome of pediatric patients who had undergone a liver and/or RT at our center over a five yr period, case records of all the patients who had undergone a liver or RT were analyzed retrospectively. One hundred solid organ transplants were performed at our center between January 2007 and January 2012. These included 50 liver, 44 renal, one sequential liver and renal, and two CLKT. BA was the most common indication for an LT (38%). At a median follow-up of two yr three months, the patient survival was 88%. The most common indication for an RT was chronic glomerulonephritis (54.5%). At a median follow-up of three yr, the survival was 91%. The CLKT were performed for hyperoxaluria. Two yr post LT, a sequential RT was performed for ESRD resulting from transplant associated microangiopathy. All patients received a living related graft. The common post-operative complications were infections, vascular complications, and graft dysfunction. Survival rates for liver and RT at our center are comparable to those in the established centers in the West.

Validation of point-of-care testing for coeliac disease in children in a tertiary hospital in north India.

OBJECTIVE: Some of the conventional serological tests for coeliac disease (CD) are expensive, time-consuming and not readily available in developing countries, leading to a delay in diagnosis. Recently, point-of-care tests (POCT) have been manufactured and tested in Europe but have not been validated in our setting. We therefore aimed to study the diagnostic accuracy of the POCT 'Biocard' test in diagnosing CD in Indian children. DESIGN: Cross-sectional study. SETTING: Tertiary care centre in north India. PATIENTS: Children, aged 2-18 years, with chronic diarrhoea, short stature or refractory anaemia underwent serological testing for CD with antiendomysial antibodies (AEA), antitissue transglutaminase (tTG) antibodies and Biocard test followed by duodenal biopsy irrespective of serological results. CD was diagnosed with positive AEA and duodenal biopsy showing >grade 2 changes using modified Marsh criteria. Those who were both AEA negative and had normal histology were considered CD negative. RESULTS: Of 319 children who underwent the serological testing, 170 agreed for biopsy. Of these, 110 were diagnosed with CD and 30 were found to be CD negative. Remaining 30 had discordant AEA and histology results and were not included in analysis. Biocard test agreed with 92/110 positive and 27/30 negative diagnoses based on reference tests (83.6% sensitivity and 90% specificity). tTG was found to be 93.8% sensitive and 96.4% specific. CONCLUSIONS: We successfully validated the POCT for CD in our setting. It could be used to increase case detection rates in developing countries with a large undiagnosed CD burden.

Alagille syndrome with a previously undescribed mutation.

BACKGROUND: Alagille Syndrome is a rare genetic disease characterized by abnormalities of the intrahepatic biliary ducts with cholestasis along with multisystem anomalies. CASE CHARACTERISTICS: An 8-year old child with persisting jaundice, severe itching and failure to thrive. OBSERVATION: Diagnosis of Alagille syndrome was made on the basis of clinical features, typical facies and liver biopsy showing bile duct paucity. Genetic analysis revealed a novel de novo mutation in the JAG 1 gene. OUTCOME: The child was started on ursodeoxycholic acid following which the itching improved. MESSAGE: A novel de novo mutation in JAG 1 gene is described in this child with Alagille Syndrome.

Management of neonatal cholestasis: consensus statement of the Pediatric Gastroenterology Chapter of Indian Academy of Pediatrics.

JUSTIFICATION: Neonatal cholestasis is an important cause of chronic liver disease in young children. Late referral and lack of precise etiological diagnosis are reasons for poor outcome in substantial number of cases in India. There is a need to create better awareness among the pediatricians, obstetricians and primary care physicians on early recognition, prompt evaluation and referral to regional centers. PROCESS: Eminent national faculty members were invited to participate in the process of forming a consensus statement. Selected members were requested to prepare guidelines on specific issues, which were reviewed by two other members. These guidelines were then incorporated into a draft statement, which was circulated to all members. A round table conference was organized; presentations, ensuing discussions, and opinions expressed by the participants were incorporated into the final draft. OBJECTIVES: To review available published data on the subject from India and the West, to discuss current diagnostic and management practices in major centers in India, and to identify various problems in effective diagnosis and ways to improve the overall outcome. Current problems faced in different areas were discussed and possible remedial measures were identified. The ultimate aim would be to achieve results comparable to the West. RECOMMENDATIONS: Early recognition, prompt evaluation and algorithm-based management will improve outcome in neonatal cholestasis. Inclusion of stool/urine color charts in well baby cards and sensitizing pediatricians about differentiating conjugated from the more common unconjugated hyperbilirubinemia are possible effective steps. Considering the need for specific expertise and the poor outcome in sub- optimally managed cases, referral to regional centers is warranted.

Management of acute liver failure in infants and children: consensus statement of the pediatric gastroenterology chapter, Indian academy of pediatrics.

PROCESS: Selected members were requested to prepare guidelines on specific issues, which were reviewed by two other members. These guidelines were then incorporated into a draft statement, which was circulated to all members. On 17th December 2011, Kunwar Viren Oswal round table conference was organized by the Apollo Center for Advanced Pediatrics, Indraprastha Apollo Hospital, New Delhi and the Sub-specialty Chapter of Pediatric Gastroenterology, Indian Academy of Pediatrics. Presentations, ensuing discussions, and opinions expressed by the participants were incorporated into the final draft. OBJECTIVES: To formulate comprehensive evidence based guidelines for management of acute liver failure in India. RECOMMENDATIONS: Viral hepatitis is the leading cause of acute liver failure (ALF) in India. Search for metabolic etiology, particularly in infants and neonates, and in apparently idiopathic cases needs to be done. Planning for early transfer is important as the risks involved with patient transport may increase or even preclude transfer at later stages. Management should be in an intensive care setting in select situations. There is currently insufficient evidence to routinely prescribe branched-chain amino acids, non-absorbable antibiotics or lactulose. Group recommends use of N-acetyl cysteine routinely in patients with ALF. Administration of antibiotics is recommended where infection is present or the likelihood of impending sepsis is high. Enteral nutrition is preferred to parenteral nutrition. Protein restriction is not recommended. An international normalized ratio >4 or Factor V concentration of <25% are the best available criteria for listing for liver transplantation. Overall 40-50% of ALF patients survive without transplantation. Survival in patients fulfilling criteria for liver transplantation and not transplanted is 10-20%. Liver transplantation is a definite treatment for ALF with high one-and five-year survival rates.

Antituberculosis therapy-associated cutaneous leukocytoclastic vasculitis.

Antituberculosis therapy-associated cutaneous leukocytoclastic vasculitis (CLV) has been rarely reported. We describe a case of CLV induced by rifampicin and pyrazinamide. A 14-year-old male diagnosed with disseminated tuberculosis developed purpuric lesions after 1.5 months of treatment. Histopathology was consistent with leukocytoclastic vasculitis. Skin lesion improved after cessation of the two drugs and treatment with corticosteroids.

Urinary potassium loss in children with acute liver failure and acute viral hepatitis.

OBJECTIVES: The aim of the present study was to determine urinary potassium (K⁺) loss (as measured by fractional excretion of K⁺ [FEK] and transtubular K⁺ gradient [TTKG]) in children with acute liver failure (ALF) and acute viral hepatitis (AVH) at the time of presentation to the hospital and day 45 of follow-up. METHODS: Twenty-five patients with ALF and 84 patients with AVH were worked up for clinical features, liver function tests, and hepatitis viral infections and monitored for outcome. All of the patients with ALF were hospitalized. FEK and TTKG were estimated on the day patients were first seen in the hospital or hospitalized and later on day 45 of follow-up. RESULTS: Sixty percent (15/25) of patients with ALF were hypokalemic (serum K⁺ <3.5 mEq/L) as compared with only 12% (10/84) in the AVH group (P = 0.000) at the time of presentation in the hospital. Inappropriate kaliuresis was present in 80% to 100% of hypokalemic children compared with 0% to 30% of normokalemic individuals at the time of first contact in either the ALF or AVH group. Inappropriate urinary K⁺ loss and serum K⁺ levels in the hypokalemic individuals improved as the hepatic functions recovered by day 45 of follow-up (P = 0.014-0.000). No significant change in kaliuresis was observed among normokalemic subjects between first contact and later on day 45 of follow-up (P = 0.991-0.228). Despite different physiologic mechanisms, appropriateness of kaliuresis measured by FEK and TTKG showed results in the same direction. CONCLUSIONS: Hypokalemia and inappropriate kaliuresis observed during the acute phase of ALF and AVH reversed with clinical and biochemical recovery. In the absence of major gastrointestinal losses and renal abnormalities, there is need to investigate the contributory role of factors like hyperaldosteronism and food intake, which may have therapeutic implications.

Transposed intrathoracic stomach: functional evaluation.

BACKGROUND: To study the functional aspects of the transposed stomach in the thoracic cavity and its effects on other organ systems. PATIENTS AND METHODS: Children who had undergone gastric transposition more than 5 years ago were evaluated for symptoms, anthropometry, anaemia, duodenogastric reflux, pulmonary function, gastric emptying, gastric pH, gastroesophageal reflux and stricture, gastric motility, and gastritis and atrophy on histological examination of gastric mucosa. RESULTS: Ten children were evaluated at a median follow-up of 90.5 months. On evaluation of symptoms, nine children were satisfied with the overall outcome. All patients had their weight and 7 patients had height less than 3 rd percentile for their respective age. Anaemia was present in 7/10 children. On evaluation with hepatobiliary scintigraphy, duodenogastric reflux was present in only 1 patient. Mass contractions of the transposed stomach were present in two thirds of the children. The mean gastric emptying t1/2 was 39.1 minutes. Pulmonary function tests were suggestive of restrictive lung disease in all the patients. Forced vital capacity (FVC) and forced expiratory volume in 1 sec (FEV1) were worse in children who underwent transposition or diversion following oesophageal anastomotic leak. Acid secretion was preserved in most patients with episodes of high gastric pH during sleep in nearly half. Mild gastritis was present in all patients where as mild atrophy of the gastric mucosa was observed in only 1child. Helicobacter pylori were positive in 3/ 8 children. Barium swallow demonstrated reflux in 2 children. CONCLUSIONS: Most children with transposed stomach remain asymptomatic on follow up. However, subclinical abnormalities are detected on investigations, which need close observation as they can manifest later in life.

Prolonged cholestasis due to hepatitis A virus infection.

We present a 12-year old boy with jaundice for 2 weeks. The child was deeply icteric and had hepatomegaly. IgM antibodies for hepatitis A virus were positive. However this child had prolonged cholestasis and cholestyramine was started. The child responded only after prednisolone was started.

Upper gastrointestinal bleeding.

Upper gastrointestinal bleeding is a life threatening condition in children. Common sources of upper gastrointestinal bleeding in children include variceal hemorrhage (most commonly extra-hepatic portal venous obstruction in our settings) and mucosal lesions (gastric erosions and ulcers secondary to drug intake). While most gastrointestinal bleeding may not be life threatening, it is necessary to determine the source, degree and possible cause of the bleeding. A complete and thorough history and physical examination is therefore vital. Esophagogastroduodenoscopy and colonoscopy are currently considered the first-line diagnostic procedures of choice for upper and lower GI bleeding, respectively. The goals of therapy in a child with GI bleeding should involve hemodynamic resuscitation, cessation of bleeding from source and prevention of future episodes of GI bleeding. Antacids supplemented by H2- receptor antagonists and proton pump inhibitors are the mainstay in the treatment of bleeding from mucosal lesion. For variceal bleeds, therapeutic emergency endoscopy is the treatment of choice after initial hemodynamic stabilization of the patient. Independent prognostic factors are presence of shock and co-morbidities. Underlying diagnosis, coagulation disorder, failure to identify the bleeding site, anemia and excessive blood loss are other factors associated with poor prognosis.