C F Munns1, R B McCrossin, M J Thomsett, J Batch. 1. Department of Endocrinology, Royal Children's Hospital, Herston, Queensland, Australia. Munnsc@health.qld.gov.au
Abstract
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.
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.
Authors: Stefania Giordano; Antonio Martocchia; Lavinia Toussan; Manuela Stefanelli; Francesca Pastore; Antonio Devito; Marcello G Risicato; Luigi Ruco; Paolo Falaschi Journal: World J Diabetes Date: 2014-12-15
Authors: Abdulrahman A Al-Hussaini; Nimer M Sulaiman; Musa D Alzahrani; Ahmed S Alenizi; Mannan Khan Journal: BMC Pediatr Date: 2012-10-06 Impact factor: 2.125