Hepatic glycogenosis: reversible hepatomegaly in type 1 diabetes.
OBJECTIVE
To describe the aetiology, clinical features and appropriate treatment for hepatic glycogenosis in poorly controlled type 1 diabetes.


METHODS
A review of three adolescents with poor diabetes control, hepatomegaly and elevated serum liver transaminase concentrations.


RESULTS
Symptoms included abdominal pain, anorexia, nausea and vomiting. All had tender hepatomegaly; two had splenomegaly. Liver biopsy was performed on two patients. Histology revealed hepatic glycogenosis in both; one also demonstrated macrovesicular steatosis. With improved glycaemic control, all three showed resolution of their symptoms, organomegaly and elevated serum liver transaminase concentrations.


CONCLUSIONS
Insulin-reversible hepatic glycogenosis is the most common cause of hepatomegaly and raised serum liver transaminase concentrations in children and adolescents with type 1 diabetes. Having excluded other causes of hepatic dysfunction, a 4 week therapeutic trial of improved glycaemic control is recommended prior to more invasive investigations.
Munns
C F
CF
Department of Endocrinology, Royal Children's Hospital, Herston, Queensland, Australia. Munnsc@health.qld.gov.au
McCrossin
R B
RB
Thomsett
M J
MJ
Batch
J
J
D000293:Adolescent; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003922:Diabetes Mellitus, Type 1 / Q000150:complications*; D005260:Female; D006008:Glycogen Storage Disease / Q000150:complications* / Q000503:physiopathology; D006529:Hepatomegaly / Q000150:complications*; D006801:Humans; D008099:Liver / Q000201:enzymology / Q000473:pathology / Q000503:physiopathology*; D008297:Male; D000637:Transaminases / Q000097:blood
D000637:Transaminases