Irritable Bowel Syndrome: Is it Rare in Children?

Irritable bowel syndrome (IBS) is one of the most common and best studied disorders among the group of functional gastrointestinal disorders. It is a functional bowel disorder in which abdominal pain or discomfort is associated with defecation or a change in bowel habit. Visceral hypersensitivity and increased GIT motility are the main patho-physiological mechanism for developing IBS. IBS present with diarrhea and constipation or both. Investigation is least needed for diagnosis of IBS rather done to exclude differential diagnosis. Diagnosis is done on the basis of Rome-III criteria. Proper counseling, dietary management, anti-spasmotic and antidepressant are the mainstay of treatment.

Clinical and Laboratory Predictors of Esophageal Varices in Children with Chronic Liver Disease.

Esophageal varices are a serious consequence of portal hypertension in patients with chronic liver diseases. Several studies have evaluated possible noninvasive markers of esophageal varices to reduce the number of unnecessary endoscopies in patients with chronic liver disease. Aim of this study was to identify clinical and laboratory predictors of esophageal varices in children with chronic liver disease. This cross sectional observational study was done at Pediatric Gastroenterology and Nutrition Department of Bangabandhu Sheikh Mujib Medical University, Dhaka, over a period of 6 months. Fifty (50) consecutive cases of chronic liver disease patient under 15 years of age were evaluated clinically, biochemically and endoscopically. Esophageal varices were classified into 4 grades according to Conn's grading system. Based on endoscopic findings (presence/absence of esophageal varices) all the patients were categorized into two groups; Group I: Chronic liver disease with esophageal varices and Group II: Chronic liver disease without esophageal varices. Most (51.5%) of the patients in Group I belong to age 10-12 years and majority 35.3% of Group II patients belong to ≤2 years. More than half of the (51.5%) patients had grade III varices followed by 24.2% grade II, 21.2% grade I and only 3.0% had grade IV. All patients had splenomegaly in Group I and 70.6% of Group II, which was significantly (p<0.05) higher in Group I. Jaundice, spider angioma, hepatomegaly and ascites werealmost alike between two groups. Platelet count <1,50,000/mm³ in 72.73% patients of Group I and 41.18% in Group II, which was significantly (p<0.05) higher in Group I. Similarly, Serum albumin <3.5mg/dl in 78.79% patients of Group I and 47.6% of Group II, was also significantly (p<0.05) higher in Group I. Serum bilirubin, serum alanine aminotransferase and International normalized ratio (INR) were almost similar between two groups. Wilson's disease was found in 42.4% of chronic liver disease with esophageal varices and 35.3% of Chronic liver disease without esophageal varices. Hepatitis B virus infections were 6.1% and 5.9% in Group I and Group II respectively. Unknown cause was found 42.4% and 17.6% in Group I and Group II respectively. Splenomegaly, Platelet count <1,50,000/mm³ and serum albumin <3.5mg/dl are important predictors of esophageal varices. These three parameters can be used independently to predict esophageal varices in children with chronic liver disease.

Childhood Wilson Disease: Bangladesh Perspective.

Wilson's disease (WD) is an autosomal recessive disorder affecting copper metabolism causing copper induced damage to various organs. In children liver is commonly involved. Central nervous system, eyes, RBC, kidneys, brain and bones may also be affected. Aim of the study is to evaluate clinical & laboratory profile of Wilson's disease in children. This cross sectional descriptive study was conducted at the department of Paediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh, from January 2011 to December, 2013. One hundred consecutive children of WD between 3 to 18 years of age were evaluated for clinical & biochemical profile. Mean age of studied children was 8.5±1.5 years. Male female ratio was 2:1. Ninety one percent patients were Muslim and nine percent Hindu. Consanguinity of marriage was found in 30% cases. Seven parents were first degree cousin. Family history of chronic liver disease was present in 15% of patients. Most (53%) cases of the hepatic WD presented between 5 to 10 years of age and most of the neurologic WD manifested in 10-15 years age group. Among 100 patients of WD, 69 children presented only with hepatic manifestations, 6 only with neurological manifestations, 14 with both hepatic & neurological manifestation, 10 children was asymptomatic and 1 patient presented with psychiatric features. WD presented as chronic liver disease (CLD) in 42%, CLD with portal hypertension in 34%, acute hepatitis in 20% and fulminant hepatic failure in 4% cases. Stigmata of chronic liver disease were found in 18% patients. Commonest stigmata was thenar and hypothenar wasting (n=8). Keiser- Fleischser ring (K-F ring) was found in 76% of the total patients. K-F ring was present in 84% ( 58 out of 69) of the hepatic only Wilsonian patients and in 90% (18 out of 20) of all neurologic Wilsonian patients. Asymptomatic and psychiatric patient had no K-F ring. About 26% of the WD patients had Coombs negative hemolytic anemia in PBF. Most of the WD patients had altered liver function. Elevated serum transaminase was found in 85% of all cases, prolonged prothrombin time in 59% cases & low serum albumin in 53% cases. Seventy three percent patients had low serum ceruloplasmin, basal urinary copper of >100µgm/day was found in 81% cases and urinary copper following penicillamine challenge of >1200µgm/day was found in 92% cases. In 28 cases with hepatic presentation esophageal varices were identified by upper gastrointestinal endoscopy. WD patient with hepatic presentations were given zinc sulphate along with penicillamine. All patients with neurological manifestation as well as asymptomatic cases were maintained on zinc therapy. WD is a treatable metabolic cause of liver disease. Majority of studied WD children presented with hepatic manifestation of which 76% presented with CLD. Any child presented with jaundice after the age of 3 years should be investigated for WD.