Department of Pediatrics and Communicable Diseases, C.S. Mott Children's Hospital and the University of Michigan, Ann Arbor, MI, USA.
Pediatr Diabetes. 2012 Dec;13(8):652-5. doi: 10.1111/j.1399-5448.2012.00884.x. Epub 2012 Jul 3.
Insulin autoimmune syndrome (IAS) or Hirata's disease is a rare disorder characterized by hypoglycemia secondary to insulin autoantibodies (IAb). Over 200 patients have been described from Japan with significantly less numbers being reported from outside the Orient. IAS is more common in patients older than 40 yr of age with reports in the pediatric age group being notably rarer. Exposure to sulfhydryl group containing medications is implicated in the pathogenesis of this syndrome. In this report, we describe a case of IAS in an African-American adolescent. A 16-yr-old healthy African-American male was diagnosed with Graves' disease and started on Methimazole. Four weeks later, he was found unconscious and hypoglycemic (blood sugar 1.5 mmol/L). Evaluation was negative for insulinoma. Insulin antibodies were positive. Oral glucose tolerance test revealed elevated free insulin concentrations with disproportionately elevated total insulin levels. The patient was started on prednisone, diazoxide, and propranolol for management of IAS and hyperthyroidism. Thyroid radio-ablation was subsequently undertaken. The doses of prednisone and diazoxide were tapered and these medications discontinued after 9 months. The insulin antibody levels decreased gradually and became undetectable in 6 months with resolution of the hypoglycemia.
胰岛素自身免疫综合征（IAS）或平田病是一种罕见的疾病，其特征是由于胰岛素自身抗体（IAb）导致的低血糖。日本已经描述了超过 200 例患者，而在东方以外的地区报告的数量明显较少。IAS 更常见于年龄大于 40 岁的患者，儿科年龄组的报告则明显较少。含巯基的药物暴露与该综合征的发病机制有关。在本报告中，我们描述了一例非裔美国青少年的 IAS 病例。一名 16 岁的健康非裔美国男性被诊断患有 Graves 病，并开始服用甲巯咪唑。四周后，他被发现昏迷并低血糖（血糖 1.5mmol/L）。排除了胰岛素瘤的可能性。胰岛素抗体阳性。口服葡萄糖耐量试验显示游离胰岛素浓度升高，而总胰岛素水平不成比例升高。患者开始接受泼尼松、二氮嗪和普萘洛尔治疗 IAS 和甲状腺功能亢进症。随后进行了甲状腺放射性碘治疗。泼尼松和二氮嗪的剂量逐渐减少，这些药物在 9 个月后停止使用。胰岛素抗体水平逐渐下降，6 个月后降至不可检测水平，低血糖得到缓解。
