Celiac disease is a form of enteropathy affecting the (small) intestine in genetically predisposed children and adults, precipitated by the ingestion of gluten-containing foods. It is also referred to as celiac sprue, glutensensitive enteropathy, and nontropical sprue. Though its presentation is commonly associated with the gastrointestinal system other systemic associations like hepatobiliary system, nervous system, hematologic system, cardiovascular system are not uncommon. We hereby present a case of a 14 year old girl with a history of global growth retardation. Systemic examination suggested severe anemia, massive pleural and pericardial effusions subsequently confirmed by chest x ray and echocardiography which also revealed an underlying atrial septal defect. Raised levels of serum anti-transglutaminase antibody (IgA) arose suspicion of Celiac disease which was confirmed after the histopathological study of the duodenal biopsy. We are reporting this case to highlight a rare association of celiac disease with a congenital heart disease.
BACKGROUND An enterocutaneous fistula (ECF) is an abnormal communication between the small or large bowel and the skin. It is one of the serious complications of gastrointestinal surgery and has a mortality rate of up to 20%. Management involves dealing with malnutrition, sepsis, metabolic derangement and electrolyte imbalance. METHODS This is a combined prospective and retrospective study conducted at SVMC & RC from June 2017 to May 2019. Thirty patients with ECF were enrolled in this study. Patients with biliary, pancreatic and anal fistulas were excluded. Factors influencing spontaneous closure of fistula such as fistula characteristics, organ of origin, sepsis etc. were studied. Initial management for all patients was conservative with restoration of fluid and electrolyte imbalance, and were initially started on parenteral nutrition using dextrose 10%, Astymin, Intralipid, human albumin and total parenteral nutrition (TPN). RESULTS Fistula closed spontaneously in 22 patients. Operative closure was done in 5. Death occurred in 3 (mortality rate-10%). Out of 30 fistulas, 28 were post-operative fistulas. One was due to trauma and one was due to Crohn's disease. Among the former, 83% developed a fistula following emergency surgery. Albumin levels after 3 weeks of treatment, were predictive of both spontaneous closure and mortality. CONCLUSIONS Post-operative causes account for majority of enterocutaneous fistulas. Anatomical origin of fistula, associated factors like sepsis, malnutrition, hypoalbuminemia and abdominal wall defect, influence the spontaneous closure. Total parenteral nutrition increases the spontaneous closure rate of fistulas and decrease mortality rate.
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