Mahévas T, Gobert D, Gatfossé M, Mekinian A, Fain O
Service de médecine interne, DHU i2B, hôpital Saint-Antoine, université Paris 6, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France.
Service de médecine interne, DHU i2B, hôpital Saint-Antoine, université Paris 6, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France.
Rev Med Interne. 2017 Mar;38(3):201-203. doi: 10.1016/j.revmed.2016.05.001. Epub 2016 Oct 13.
Hepatic glycogenosis is a rare syndrome, which includes poorly controlled diabetes mellitus, hepatomegaly, delayed puberty, and growth delay. Insulin edema is sometimes associated.
An 18-year-old woman presented with diffuse edema, hepatomegaly, amenorrhea, uncontrolled diabetes, and elevated transaminases and cholestasis. Hepatic ultrasonography and abdominal computed tomographic scan confirmed the hepatomegaly. The liver biopsy showed a massive glycogenosis and the diagnosis of hepatic glycogenosis was confirmed. Too large doses of insulin were responsible of diffuse edema. Diabetes equilibration and diminution of insulin intakes allow correction of this disorder.
Excess of insulin can lead to excessive hepatic glycogen storage by activation of glycogenosis enzymes. Biological manifestations consist on elevated liver enzymes and hyperlactatemia. There is a link between administration of high dose of insulin and edema. Hepatic glycogenosis should be suspected when diabetes is uncontrolled and be considered as a differential diagnosis of steatosis. It may be associated and revealed by insulin edema directly related to excessive insulin intakes.
肝糖原累积症是一种罕见综合征，包括糖尿病控制不佳、肝肿大、青春期延迟和生长发育迟缓。有时会伴有胰岛素性水肿。
一名18岁女性出现弥漫性水肿、肝肿大、闭经、糖尿病控制不佳、转氨酶升高和胆汁淤积。肝脏超声检查和腹部计算机断层扫描证实了肝肿大。肝活检显示大量糖原累积，肝糖原累积症诊断得以证实。过量胰岛素导致了弥漫性水肿。糖尿病病情得到控制以及胰岛素摄入量减少后，这种病症得以纠正。
胰岛素过量可通过激活糖原累积酶导致肝脏糖原过度储存。生物学表现为肝酶升高和高乳酸血症。高剂量胰岛素给药与水肿之间存在关联。当糖尿病控制不佳时，应怀疑肝糖原累积症，并将其视为脂肪变性的鉴别诊断。它可能与因胰岛素摄入过多直接导致的胰岛素性水肿相关并由其揭示。
