A child with acute pancreatitis and recurrent hypoglycemia due to 3-hydroxy-3-methylglutaryl-CoA lyase deficiency

W. G. Wilson, M. B. Cass, O. Søvik, K. M. Gibson, L. Sweetman

Research output: Contribution to journalArticlepeer-review

37 Scopus citations

Abstract

A five-year-old-girl with a history of recurrent hypoglycemia presented with acidosis, intractable vomiting, and abdominal tenderness; the diagnosis of acute pancreatitis was made by abdominal ultrasonography and supportive biochemical studies. Urinary organic acid analysis revealed metabolites suggestive of HMG-CoA lyase deficiency, and subsequent enzyme assays of lymphocytes and fibroblasts confirmed this diagnosis. Acute pancreatitis, an uncommon condition in childhood, is seen with increased frequency in patients with Reye syndrome, a metabolic disorder with which HMG-CoA lyase deficiency may be confused. The pathogenesis of pancreatitis in Reye syndrome or in HMG-CoA lyase deficiency has not been determined.

Original languageEnglish (US)
Pages (from-to)289-291
Number of pages3
JournalEuropean Journal of Pediatrics
Volume142
Issue number4
DOIs
StatePublished - Sep 1984
Externally publishedYes

Keywords

  • 3-hydroxy-3-methylglutaryl
  • CoA lyase
  • Organic acid-uria
  • Pancreatitis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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