Indian Journal of Gastroenterology

, Volume 31, Issue 6, pp 343–345

Intractable ascites as a manifestation of Wolman’s disease: report of two sibs

  • Rachana Kathuria
  • Ujjal Poddar
  • Jayanta Ghosh
  • Surender Kumar Yachha
  • V. Gnanapriya
  • Rakesh Pandey
  • Anupriya Kaur
  • Subha Phadke
  • Anshu Srivastava
Case Series

DOI: 10.1007/s12664-012-0253-6

Cite this article as:
Kathuria, R., Poddar, U., Ghosh, J. et al. Indian J Gastroenterol (2012) 31: 343. doi:10.1007/s12664-012-0253-6
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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.

Keywords

ChildhoodDiureticsFamilialLiver storage disorderParacentesis

Copyright information

© Indian Society of Gastroenterology 2012

Authors and Affiliations

  • Rachana Kathuria
    • 1
  • Ujjal Poddar
    • 1
  • Jayanta Ghosh
    • 1
  • Surender Kumar Yachha
    • 1
  • V. Gnanapriya
    • 2
  • Rakesh Pandey
    • 2
  • Anupriya Kaur
    • 3
  • Subha Phadke
    • 3
  • Anshu Srivastava
    • 1
  1. 1.Department of Pediatric GastroenterologySanjay Gandhi Postgraduate Institute of Medical SciencesLucknowIndia
  2. 2.Department of PathologySanjay Gandhi Postgraduate Institute of Medical SciencesLucknowIndia
  3. 3.Department of Medical GeneticsSanjay Gandhi Postgraduate Institute of Medical SciencesLucknowIndia