Lupsa Beatrice C, Chong Angeline Y, Cochran Elaine K, Soos Maria A, Semple Robert K, Gorden Phillip
From Clinical Endocrinology Branch (BCL, AYC, EKC, PG), National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, United States; and University of Cambridge Metabolic Research Laboratories (MAS, RKS), Institute of Metabolic Science, Addenbrooke's Hospital, Cambridge, United Kingdom.
Medicine (Baltimore). 2009 May;88(3):141-153. doi: 10.1097/MD.0b013e3181a5b42e.
Autoimmune syndromes are a rare cause of hypoglycemia characterized by elevated levels of insulin in the presence of either anti-insulin antibodies (insulin autoimmune syndrome) or anti-insulin receptor antibodies (type B insulin resistance). Insulin autoimmune syndrome is the third leading cause of hypoglycemia in Japan, but has rarely been described in the non-Asian population.In the current study, we report the clinical and biochemical characteristics and clinical course of 2 white patients with insulin autoimmune syndrome, and present a literature review of non-Asian patients reported with insulin autoimmune syndrome. Also, we describe the clinical and biochemical characteristics of patients reported in the literature with type B insulin resistance who manifested hypoglycemia. We compare the clinical and laboratory features of insulin autoimmune syndrome and type B insulin resistance with each other and with other forms of hyperinsulinemic hypoglycemia.Autoimmune forms of hypoglycemia are uncommon. However, they should be considered in any patient with hypoglycemia in the setting of unsuppressed insulin levels associated with anti-insulin or anti-insulin receptor antibodies. Making the correct diagnosis may spare a hypoglycemic patient from an unnecessary pancreatic surgical procedure.
自身免疫综合征是低血糖症的罕见病因,其特征为在存在抗胰岛素抗体(胰岛素自身免疫综合征)或抗胰岛素受体抗体(B型胰岛素抵抗)的情况下胰岛素水平升高。胰岛素自身免疫综合征是日本低血糖症的第三大主要病因,但在非亚洲人群中鲜有报道。在本研究中,我们报告了2例患有胰岛素自身免疫综合征的白人患者的临床和生化特征及临床病程,并对报道的非亚洲胰岛素自身免疫综合征患者进行了文献综述。此外,我们描述了文献中报道的表现为低血糖症的B型胰岛素抵抗患者的临床和生化特征。我们将胰岛素自身免疫综合征和B型胰岛素抵抗的临床及实验室特征相互之间以及与其他形式的高胰岛素血症性低血糖症进行比较。自身免疫性低血糖症并不常见。然而,对于任何在存在与抗胰岛素或抗胰岛素受体抗体相关的未受抑制的胰岛素水平情况下发生低血糖症的患者,都应考虑到自身免疫综合征。做出正确诊断可能使低血糖症患者避免不必要的胰腺外科手术。