Recurrent acute pancreatitis in a Saudi child with isovaleric acidemia

Isovaleric acidemia (IVA), also known as the sweaty foot syndrome, is an autosomal recessive organic acid disorder due to a defect in the mitochondrial Flavin Adenine Dinucleotide (FAD)-dependent enzyme isovaleryl CoA dehydrogenase. Acute pancreatitis as a complication has been reported. We are pres...

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Bibliographic Details
Published inAnnals of tropical medicine and public health Vol. 9; no. 4; pp. 276 - 278
Main Author Satti, Satti
Format Journal Article
LanguageEnglish
Published Accra Wolters Kluwer - Medknow Publications 01.07.2016
Medknow Publications and Media Pvt. Ltd
Medknow Publications & Media Pvt. Ltd
Subjects
Care and treatment
Case studies
Diagnosis
Isovaleric acidemia
Pancreatitis
Saudi Arabia
isovaleric acidemia
pancreatitis
diabetic ketoacidosis
Child
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Summary:Isovaleric acidemia (IVA), also known as the sweaty foot syndrome, is an autosomal recessive organic acid disorder due to a defect in the mitochondrial Flavin Adenine Dinucleotide (FAD)-dependent enzyme isovaleryl CoA dehydrogenase. Acute pancreatitis as a complication has been reported. We are presenting a rare case of recurrent acute pancreatitis complicating a 9-year-old girl with IVA. She presented with repeated attacks of vomiting, severe abdominal pain, and fever since the age of 18 months. These were diagnosed as attacks of pancreatitis with high serum amylase presenting clinically as diabetic ketoacidosis. Later, she developed chronic pancreatitis which is proved by lapratomy. In the last attack, she was comatosed with fulminant sepsis and sooner the patient died. Blood sample taken during this last attack confirmed the diagnosis of IVA. An inborn error of organic acid metabolism should be considered in children with pancreatitis of unknown origin.
ISSN:1755-6783
0974-6005
DOI:10.4103/1755-6783.184807