Recurrent pancreatitis in ornithine transcarbamylase deficiency.
Ornithine transcarbamylase (OTC) deficiency is a urea cycle defect with varying frequency and severity of episodes of hyperammonemia. We report three patients with OTC deficiency with recurrent pancreatitis. The pathogenesis of acute pancreatitis in this patient population requires further elucidation. Pancreatitis significantly affected dietary/metabolic management and increased frequency of hospitalizations.
Division of Human Genetics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, USA.
Carlos E
CE
Prada
Ajay
A
Kaul
Robert J
RJ
Hopkin
Kimberley I
KI
Page
Jaimie D
JD
Nathan
Dennis W
DW
Bartholomew
Mitchell B
MB
Cohen
James E
JE
Heubi
Nancy D
ND
Leslie
T Andrew
TA
Burrow
D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D020163:Ornithine Carbamoyltransferase Deficiency Disease / Q000150:complications*; D010195:Pancreatitis / Q000150:complications*; D012008:Recurrence; D055815:Young Adult