Gallbladder abnormalities in children with metachromatic leukodystrophy

Jina Kim, Zhifei Sun, Brian Ezekian, Gary R. Schooler, Vinod K. Prasad, Joanne Kurtzberg, Henry E. Rice, Elisabeth T. Tracy

Research output: Contribution to journalArticlepeer-review

12 Scopus citations

Abstract

Background Metachromatic leukodystrophy (MLD) is a lysosomal storage disease that leads to neurological deterioration and visceral involvement, including sulphatide deposition in the gallbladder wall. Using our institution's extensive experience in treating MLD, we examined the incidence of gallbladder abnormalities in the largest cohort of children with MLD to date. Methods We conducted a retrospective review of all children with MLD, adrenoleukodystrophy (ALD), or Krabbe disease who underwent hematopoietic stem cell transplantation (HSCT) at our institution between 1994 and 2015. Baseline characteristics and unadjusted outcomes were compared using the Kruskal–Wallis test for continuous variables and Pearson χ2 test for categorical variables, with significance defined as P < 0.05. Results In total, 87 children met study criteria: 29 children with MLD and 58 children with ALD or Krabbe disease. Children with MLD were more likely to demonstrate gallbladder abnormalities on imaging, both before HSCT (41.4% versus 5.2%, P < 0.001) and after HSCT (75.9% versus 41.4%, P = 0.002). Consequently, a larger proportion of children with MLD underwent surgical or interventional management of biliary disease (10.3% versus 3.4%, P = 0.03). Conclusions Children with MLD have a significantly greater incidence of gallbladder abnormalities than children with other lysosomal storage diseases. Biliary disease should be considered in children with MLD who develop abdominal pain, and cholecystectomy should be considered for persistent, symptomatic gallbladder abnormalities.

Original languageEnglish (US)
Pages (from-to)187-191
Number of pages5
JournalJournal of Surgical Research
Volume208
DOIs
StatePublished - Feb 1 2017
Externally publishedYes

Keywords

  • Adrenoleukodystrophy
  • Child
  • Gallbladder
  • Krabbe disease
  • Metachromatic leukodystrophy

ASJC Scopus subject areas

  • Surgery

Fingerprint Dive into the research topics of 'Gallbladder abnormalities in children with metachromatic leukodystrophy'. Together they form a unique fingerprint.

Cite this