Acute Pancreatitis with Rapid Clinical Improvement in a Child with Isovaleric Acidemia
Open Access
- 4 February 2013
- journal article
- case report
- Published by Hindawi Limited in Case Reports in Pediatrics
- Vol. 2013, 1-3
- https://doi.org/10.1155/2013/721871
Abstract
Isovaleric acidemia is a rare branched-chain organic acidemia. The authors describe a 3.5-year-old girl with isovaleric acidemia and acute abdominal pain associated with bilious emesis. Elevated serum amylase and abdominal ultrasonography demonstrating an enlarged and edematous pancreas, along with the presence of peripancreatic exudates, confirmed the presence of acute pancreatitis. The patient recovered quickly with intravenous hydration, pancreatic rest, and administration of intravenous L-carnitine. Pancreatitis should be ruled out in the context of vomiting in any patient with isovaleric acidemia. Conversely, branched-chain organic acidemias should be included in the differential diagnosis of any child with pancreatitis of unknown origin.Keywords
This publication has 15 references indexed in Scilit:
- Clinical variability of isovaleric acidemia in a genetically homogeneous populationJournal of Inherited Metabolic Disease, 2012
- Advances and challenges in the treatment of branched‐chain amino/keto acid metabolic defectsJournal of Inherited Metabolic Disease, 2011
- Chronic pancreatitis in branched-chain organic acidurias—a case of methylmalonic aciduria and an overview of the literatureEuropean Journal of Pediatrics, 2010
- Recurrent Acute Pancreatitis Associated with Propionic AcidemiaJournal of Pediatric Gastroenterology and Nutrition, 2008
- ‘Classical’ organic acidurias, propionic aciduria, methylmalonic aciduria and isovaleric aciduria: Long‐term outcome and effects of expanded newborn screening using tandem mass spectrometryJournal of Inherited Metabolic Disease, 2006
- Fatty Acid Ethyl Esters Cause Pancreatic Calcium Toxicity via Inositol Trisphosphate Receptors and Loss of ATP SynthesisGastroenterology, 2006
- Branched-chain organic aciduriasSeminars in Neonatology, 2002
- Acute pancreatitis in a patient with glutaric acidemia type IThe Journal of Pediatrics, 1996
- Pancreatitis in patients with organic acidemiasThe Journal of Pediatrics, 1994
- Pancreatic disorders in infancy and childhood: Experience with 92 casesJournal of Pediatric Surgery, 1989