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Acute pancreatitis with rapid clinical improvement in a child with isovaleric acidemia

Elpis Mantadakis1, Ioannis Chrysafis, Emmanouela Tsouvala

  • 1Department of Pediatrics, Democritus University of Thrace Faculty of Medicine, University General Hospital of Alexandroupolis, 68100 Alexandroupolis, Greece.

Case Reports in Pediatrics
|February 23, 2013
PubMed

Insights

Isovaleric acidemia can cause acute pancreatitis in children. Early diagnosis and treatment, including L-carnitine, are crucial for recovery and managing this rare metabolic disorder.

Area of Science:

  • Biochemistry
  • Pediatrics
  • Gastroenterology

Background:

  • Isovaleric acidemia is a rare inherited metabolic disorder affecting branched-chain amino acid metabolism.
  • Gastrointestinal symptoms, including abdominal pain and vomiting, are common in isovaleric acidemia.

Purpose of the Study:

  • To report a case of isovaleric acidemia presenting with acute pancreatitis.
  • To highlight the importance of considering isovaleric acidemia in the differential diagnosis of pediatric pancreatitis.

Main Methods:

  • Case report of a 3.5-year-old girl.
  • Clinical presentation, laboratory findings (elevated serum amylase), and imaging (abdominal ultrasonography) were analyzed.
  • Treatment included intravenous hydration, pancreatic rest, and L-carnitine administration.

Main Results:

  • The patient presented with acute abdominal pain and bilious emesis, indicative of acute pancreatitis.
  • Diagnostic findings confirmed pancreatitis, including elevated amylase and pancreatic edema.
  • The patient showed rapid improvement with supportive care and L-carnitine.

Conclusions:

  • Acute pancreatitis is a potential complication of isovaleric acidemia.
  • Pancreatitis should be considered in patients with isovaleric acidemia presenting with vomiting.
  • Branched-chain organic acidemias should be included in the differential diagnosis of unexplained pediatric pancreatitis.

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