title: Intractable ascites as a manifestation of Wolman's disease: report of two sibs.
abstract: Wolman disease (WD) is a rare, inherited, rapidly fatal condition presenting in early infancy. The disease manifests in the first month of life with failure to thrive, vomiting, diarrhea, abdominal distension, hepatosplenomegaly and bilateral adrenal calcification and is nearly always fatal before the age of 1 year. Barring a case report of isolated fetal ascites, there is no report of intractable ascites as the presentation of WD till date. We report two siblings with WD who both had intractable ascites and required therapeutic paracentesis, albumin infusion, and diuretics to control tense ascites. Although rare, WD should be considered in the differential diagnosis of infantile ascites.
authors.affiliation: Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226 014, India.
authors.forename: Rachana
authors.initials: R
authors.lastname: Kathuria
authors.forename: Ujjal
authors.initials: U
authors.lastname: Poddar
authors.forename: Jayanta
authors.initials: J
authors.lastname: Ghosh
authors.forename: Surender Kumar
authors.initials: SK
authors.lastname: Yachha
authors.forename: V
authors.initials: V
authors.lastname: Gnanapriya
authors.forename: Rakesh
authors.initials: R
authors.lastname: Pandey
authors.forename: Anupriya
authors.initials: A
authors.lastname: Kaur
authors.forename: Subha
authors.initials: S
authors.lastname: Phadke
authors.forename: Anshu
authors.initials: A
authors.lastname: Srivastava
mesh_terms: D001201:Ascites / Q000209:etiology* / Q000628:therapy; D005183:Failure to Thrive; D017809:Fatal Outcome; D006801:Humans; D007223:Infant; D008297:Male; D019152:Paracentesis; D015223:Wolman Disease / Q000150:complications* / Q000175:diagnosis / Q000235:genetics