European Journal of Pediatrics

, Volume 170, Issue 2, pp 241–245 | Cite as

Chronic pancreatitis in branched-chain organic acidurias—a case of methylmalonic aciduria and an overview of the literature

  • Jan Marquard
  • Tarik el Scheich
  • Dirk Klee
  • Marcus Schmitt
  • Thomas Meissner
  • Ertan Mayatepek
  • Jun Oh
Short Report
First Online:
Received:
Accepted:

Abstract

A severe rare complication in patients with branched-chain organic acidurias (BCOA) is pancreatitis with a limited number of patients published so far. Here, we report on a patient with methylmalonic aciduria (MMA) who developed chronic pancreatitis after several episodes of acute pancreatitis. In addition, an overview is given about some previous published cases with BCOA who developed pancreatitis in the course of the disease. In half of the published MMA patients with pancreatitis, an acute pancreatitis was reported while the rest suffered from a chronic form of this disease. Acute pancreatitis in BCOA patients can clinically present in the context of recurrent vomiting and an impaired general physical condition even without typical signs of pancreatitis. Any form of pancreatitis should be ruled out in the assessment of acutely ill patients with BCOA.

Keywords

Branched-chain organic acidurias Pancreatitis Methylmalonic aciduria Propionic aciduria 
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Notes

Conflicts of interest

The authors report no conflicts of interest. This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors. All authors have no financial relationship with any organization.

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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Jan Marquard
    • 1
  • Tarik el Scheich
    • 2
  • Dirk Klee
    • 3
  • Marcus Schmitt
    • 4
  • Thomas Meissner
    • 1
  • Ertan Mayatepek
    • 1
  • Jun Oh
    • 1
    • 5
  1. 1.Department of General PediatricsUniversity Children’s Hospital DüsseldorfDüsseldorfGermany
  2. 2.Department of Pediatric CardiologyUniversity Children’s Hospital DüsseldorfDuesseldorfGermany
  3. 3.Institute of Radiology, Department of Pediatric RadiologyUniversity Hospital DüsseldorfDuesseldorfGermany
  4. 4.Department of Gastroenterology, Hepatology and InfectiologyUniversity Hospital DüsseldorfDuesseldorfGermany
  5. 5.Department of Pediatric NephrologyUniversity Medical Centre Hamburg-EppendorfHamburgGermany

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