Efficacy and Predictors of Pain Response to Combined Antioxidants in Children with Chronic Pancreatitis.

BACKGROUND: Pain is a major problem in 90% of patients with chronic pancreatitis (CP). Studies evaluating response to antioxidants (AO) are conflicting and no pediatric studies are available. AIMS: To evaluate markers of oxidative stress (OS), and efficacy and predictors of response to AO in improving pain in children with CP. METHODS: Antioxidants were given to CP children for 6 months. Subjects were assessed at baseline and post-therapy for pain and markers of OS [serum thiobarbituric acid reactive substances (TBARS), superoxide dismutase (S-SOD)] and antioxidant levels [vitamin C, selenium, total antioxidant capacity-ferric reducing ability of plasma (FRAP)]. Matched healthy controls were assessed for OS and antioxidant levels. Good response was defined as ≥ 50% reduction in number of painful days/month. RESULTS: 48 CP children (25 boys, age 13 years) and 14 controls were enrolled. 38/48 cases completed 6 months of therapy. CP cases had higher OS [TBARS (7.8 vs 5.2 nmol/mL; p < 0.001)] and lower antioxidant levels [FRAP (231 vs. 381.3 µmol/L; p = 0.003), vitamin C (0.646 vs. 0.780 mg/dL; p < 0.001)] than controls. Significant reduction in TBARS and S-SOD and increase in FRAP, vitamin C, and selenium occurred after 6 months. 10.5% cases had minor side effects. 26(68%) cases had a good response, with 9(24%) becoming pain-free. Subjects with severe ductal changes had lower median BMI (- 0.73 vs 0.10; p = 0.04) and responded less often than those with mild changes (17/29 vs 9/9; p = 0.036). CONCLUSION: CP children have higher OS than healthy controls. Antioxidant therapy is safe. Pain response is seen in 68% cases, less often in patients with severe ductal changes.

Effect of chelation therapy in pediatric Wilson's disease: Liver and endoscopic outcome.

BACKGROUND: As there is paucity of exclusive literature on pediatric hepatic Wilson's disease (WD), this study was undertaken to evaluate the efficacy of chelation on hepatocellular function and portal hypertension in WD. METHODS: Wilson's disease patients with ≥9 months of follow-up were evaluated for response to chelation therapy in the following categories: (a) complete remission, (b) partial remission (c) progression of disease; (d) drug toxicity. Pediatric end-stage liver disease (PELD), Nazar and New Wilson Index scores were compared. Hemodynamically stable patients underwent esophagogastroduodenoscopy (baseline and surveillance) and received prophylaxis (primary or secondary). Endoscopic outcome was assessed at follow-up. RESULTS: Of the 111 WD children (aged 9 [3-15] years; PELD score 16 [-11 to 60]), 65 with follow-up of 3.6 (0.8-12) years on chelation (83% D-penicillamine monotherapy, 17% D-penicillamine and zinc) were analyzed. 81% had severe disease at presentation. Favorable outcome (complete and or partial remission), progression of disease and drug toxicity were seen in 71%, 29% and 10.8%, respectively. Two-thirds had esophageal varices which did not show progression. Large esophageal varices (16%) were effectively downgraded in 3 (2-6) therapeutic endoscopic sessions. Nazar score and PELD score at baseline were independent predictors of outcome with favorable correlation with each other (r = .864, P < .001). PELD cutoff 9.45 (AUC: 71%, sensitivity: 87%, specificity: 50%; P = .009) and Nazar score cut off 3.5 (AUC: 68%, sensitivity: 83%, specificity: 50%; P = .02) were associated with poor prognosis. CONCLUSIONS: Despite severe liver disease, the majority of hepatic WD can be managed on D-penicillamine monotherapy. PELD score and Nazar score effectively determine the outcome.

Management of Childhood Functional Constipation: Consensus Practice Guidelines of Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition and Pediatric Gastroenterology Chapter of Indian Academy of Pediatrics.

JUSTIFICATION: Management practices of functional constipation are far from satisfactory in developing countries like India; available guidelines do not comprehensively address the problems pertinent to our country. PROCESS: A questionnaire-based survey was conducted among selected practising pediatricians and pediatric gastroenterologists in India, and the respondents agreed on the need for an Indian guideline on the topic. A group of experts were invited to present the published literature under 12 different headings, and a consensus was developed to formulate the practice guidelines, keeping in view the needs in Indian children. OBJECTIVE: To formulate practice guidelines for the management of childhood functional constipation that are relevant to Indian children. RECOMMENDATIONS: Functional constipation should be diagnosed only in the absence of red flags on history and examination. Those with impaction and/or retentive incontinence should be disimpacted with polyethylene glycol (hospital or home-based). Osmotic laxatives (polyethylene glycol more than 1 year of age and lactulose/lactitol less than 1 year of age) are the first line of maintenance therapy. Stimulant laxatives should be reserved only for rescue therapy. Combination therapies of two osmotics, two stimulants or two classes of laxatives are not recommended. Laxatives as maintenance therapy should be given for a prolonged period and should be tapered off gradually, only after a successful outcome. Essential components of therapy for a successful outcome include counselling, dietary changes, toilet-training and regular follow-up.

Infant with hepatomegaly and hypoglycemia: A setting for fatty acid oxidation defects.

Fatty acid oxidation defects (FAOD) are one of the commonest metabolic liver diseases (MLDs) that can have varied presentations in different age groups. An infant presented with short history of jaundice and irritability, examination showed soft hepatomegaly. Investigations revealed non-ketotic hypoglycemia suggesting FAOD which was later confirmed as carnitine uptake defect with gas chromatography and mass spectrometry and mutation analysis. Patient improved with acute management of metabolic crisis, carnitine supplementation and corn starch therapy with reversal of encephalopathy, reduction in hepatomegaly, maintenance of euglycemia and improvement in liver function tests and creatine phosphokinase on follow up. Non-ketotic hypoglycemia is a characteristic finding in FAODs. Early diagnosis and appropriate management can result in excellent outcomes in patients with FAODs.