Munns C F, McCrossin R B, Thomsett M J, Batch J
Department of Endocrinology, Royal Children's Hospital, Herston, Queensland, Australia.
J Paediatr Child Health. 2000 Oct;36(5):449-52. doi: 10.1046/j.1440-1754.2000.00547.x.
To describe the aetiology, clinical features and appropriate treatment for hepatic glycogenosis in poorly controlled type 1 diabetes.
A review of three adolescents with poor diabetes control, hepatomegaly and elevated serum liver transaminase concentrations.
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.
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.
描述1型糖尿病控制不佳时肝糖原累积症的病因、临床特征及适当治疗方法。
回顾3例糖尿病控制不佳、肝肿大且血清肝转氨酶浓度升高的青少年病例。
症状包括腹痛、厌食、恶心和呕吐。所有人都有压痛性肝肿大；2人有脾肿大。对2例患者进行了肝活检。组织学检查显示两人均有肝糖原累积症；其中一人还表现为大泡性脂肪变性。随着血糖控制改善，三人的症状、器官肿大及血清肝转氨酶浓度均恢复正常。
胰岛素可逆性肝糖原累积症是1型糖尿病儿童和青少年肝肿大及血清肝转氨酶浓度升高的最常见原因。在排除其他肝功能障碍原因后，建议在进行更具侵入性的检查之前，先进行为期4周的血糖控制改善治疗试验。
