Moinak Sen Sarma

Extrahepatic Portal Venous Obstruction: What Should be the Mainstay of Treatment?

The two cornerstones of management for Extrahepatic portal vein obstruction (EHPVO) are endotherapy and surgery [Porto-systemic shunts (PSS)/Mesorex bypass (MRB)]. Endotherapy is the mainstay of treatment for acute variceal bleed control and has also been used extensively for secondary prophylaxis till variceal eradication is achieved. However, long-term follow-up beyond endoscopic eradication of esophageal varices (EEEV) indicates that there are numerous delayed bleed and non bleed sequelae of EHPVO, which merit surgery as a definitive procedure to decompress the hypertensive portal venous system. While endotherapy obliterates natural porto-systemic collaterals in the gastroesophageal region, persistently raised portal pressures manifest as an increase in secondary isolated gastric varices, ectopic varices, portal hypertensive vasculopathy, issues related to massive splenomegaly, portal biliopathy, growth retardation and hence impaired quality of life (QOL). An ideal management strategy should address both bleed and non-bleed consequences of EHPVO and translate into a near normal QOL. Further, MRB has opened up new dimensions to the management philosophy of EHPVO. This review article critically evaluates the role of surgery and endotherapy based on available literature and authors’ own experience.Surgery and endotherapy are complementary. However, with increasing duration of follow-up post EEEV, it is evident that there is resurgence in the role of surgery (PSS/MRB) as a single one time definitive procedure for alleviating all bleed and delayed non bleed sequelae of EHPVO.Surgery for EHPVO (PSS/MRB) should not be allowed to become a dying art and future generations of surgeons should continue to receive training in this specialized area of surgery.

Classical galactosemia among Indian children: Presentation and outcome from a Pediatric Gastroenterology center

ObjectiveTo analyze the presentation and predictors of outcome of children with galactosemia.MethodsAnalysis of clinical, laboratory, microbiological profile and outcome of patients fulfilling the diagnostic criteria: i) clinical setting; ii) reduced erythrocyte Gal-1-PUT enzyme activity; and iii) unequivocal response to lactose-free diet.Results24 patients; median age of symptom onset and diagnosis: 10 (3-75) d and 55 (15-455) days, respectively. 71% had uncorrectable coagulopathy; 71% systemic infections; and 54% had ascites. Outcome: consisted of 87.5% survival with normalization of liver function tests at 5.5 (1-24) months follow-up.ConclusionDespite delayed referral, high Pediatric end-stage liver disease scores and systemic infections, long-term outcome in galactosemia is rewarding. A subset of children have developmental delay.

Prognostic scoring systems and outcome of endovascular radiological intervention of chronic Budd-Chiari syndrome in children

Prognostic scoring systems (PSS) have not been validated in children with chronic Budd‐Chiari syndrome (BCS). We aimed to analyse the long‐term outcome of radiological intervention (RI) and validate the PSS in children.

Cholangiopathy in children with extrahepatic portal venous obstruction

Portal cavernoma cholangiopathy (PCC), a surgical‐endoscopic dilemma, has not been studied comprehensively, more so in children. Our study aimed to evaluate PCC in children using a combination of magnetic resonance cholangiography‐portovenography (MRC‐MRPV) and endoscopic ultrasonography (EUS).

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

JustificationManagement 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.ProcessA 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.ObjectiveTo formulate practice guidelines for the management of childhood functional constipation that are relevant to Indian children.RecommendationsFunctional 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.

Risk of Pediatric Celiac Disease According to HLA Haplotype and Country

SummaryThis multi-centric cohort study [1] from four countries followed a group of infants with the HLA haplotype DR3-DQ2 or DR4-DQ8, from birth through the first few years of life; seeking the appearance of antibodies to tissue transglutaminase (tTG) (labeled as celiac disease autoimmunity), and development of celiac disease. This was part of a larger study evaluating the development of type 1 diabetes in a cohort of infants with genetic susceptibility (based on carrying the HLA haplotype DR3-DQ2 or DR4-DQ8) [2]. Over a median follow-up duration of nearly five years, the investigators reported 12% prevalence of celiac disease autoimmunity and 3% prevalence of celiac disease. They also identified that the respective risks of these two outcomes varied by the HLA genotype: 26% and 11% with homozygosity for DR3-DQ2 haplotype; 11% and 3% with DR3-DQ2/DR4-DQ8 haplotype; 8% and 3% with DR4-DQ8 homozygosity; and 3% and <1% among those with DR4-DQ8/DR8-DQ4 haplotype. There was statistically significant higher risk of celiac disease autoimmunity and celiac disease in infants from Europe (highest risk in Sweden), female gender, and those with family history of celiac disease.