Sridhar Aarthi, Renzu Mahvish, Mehta Vidhi, Hubers Carly, Ruby Edward
Mercy Catholic Medical Center, Darby, PA 19023, USA.
Trinity Health Oakland, Pontiac, MI 48341, USA.
JCEM Case Rep. 2024 Jul 12;2(7):luae129. doi: 10.1210/jcemcr/luae129. eCollection 2024 Jul.
Insulin antibody syndrome (IAS), also known as Hirata disease, is a rare condition characterized by spontaneous hypoglycemic episodes unrelated to exogenous insulin exposure. It is caused by elevated serum levels of insulin autoantibodies (IAA). IAS typically occurs when a triggering factor, such as medication or viral infection, interacts with a predisposing genetic background. Diagnosing IAS is challenging due to its rarity and the presence of multiple potential causes for hyperinsulinemic hypoglycemia. The presence of Whipple triad-symptoms of hypoglycemia, low plasma glucose concentration, and relief of symptoms after raising plasma glucose-strongly supports the diagnosis of IAS. However, the detection of IAA is considered the most reliable test. Timely diagnosis can facilitate appropriate treatment and prevent unnecessary imaging studies and invasive procedures, thereby reducing costs. Currently, no definitive guidelines exist for managing IAS. Most management strategies involve supportive measures due to the high rate of spontaneous remission, with hypoglycemia often managed through dietary interventions. However, a few medications have shown benefit. Although predominantly observed in the Japanese population, IAS cases have been reported in other ethnicities, including Caucasians. This report presents a unique case of IAS in an African American male.
胰岛素抗体综合征(IAS),也称为平田病,是一种罕见的病症,其特征为出现与外源性胰岛素暴露无关的自发性低血糖发作。它由血清胰岛素自身抗体(IAA)水平升高引起。IAS通常在诸如药物或病毒感染等触发因素与易感遗传背景相互作用时发生。由于IAS罕见且高胰岛素血症性低血糖存在多种潜在病因,因此诊断具有挑战性。Whipple三联征(低血糖症状、低血糖血浆葡萄糖浓度以及提高血浆葡萄糖后症状缓解)的存在强烈支持IAS的诊断。然而,IAA的检测被认为是最可靠的检查。及时诊断有助于进行适当治疗,并避免不必要的影像学检查和侵入性操作,从而降低成本。目前,对于IAS的管理尚无明确的指南。由于自发缓解率高,大多数管理策略都涉及支持性措施,低血糖通常通过饮食干预进行管理。然而,一些药物已显示出益处。虽然IAS主要在日本人群中观察到,但在包括白种人在内的其他种族中也有病例报告。本报告介绍了一名非裔美国男性患IAS的独特病例。