Endocrinology Unit, Te Whatu Ora Waikato, Hamilton 3204, New Zealand.
Waikato Clinical Campus, University of Auckland, Hamilton 3204, New Zealand.
J Clin Endocrinol Metab. 2024 Mar 15;109(4):936-943. doi: 10.1210/clinem/dgad467.
Type B insulin resistance syndrome is a rare autoimmune disorder affecting glucose homeostasis, characterized by serum autoantibodies to the insulin receptor (AIRAbs). Patients typically present with severe insulin resistance. A mixed hyper- and hypoglycemia phenotype may also occur, as may isolated hypoglycemia. The classic biochemical pattern comprises elevated insulin levels despite hypoglycemia; however, a small proportion of cases demonstrate "isolated hypoglycemia with low insulin." The primary objectives of this systematic review were to identify the clinical characteristics and outcome of this subgroup.
Systematic review of cases with hypoglycemia with suppressed insulin. Exclusions: hyperglycemia, elevated insulin, AIRAbs not confirmed.
PubMed, Medline, and Embase databases were searched up until February 2023 and complemented by manual citation search. The Joanna Briggs Institute critical appraisal checklist for case reports was used to assess bias.
A total of 5342 articles were identified after duplicate removal. Eleven, all case reports, met all inclusion criteria and were included. Cases belonging to this subgroup were more diverse in sex, age, and ethnicity when compared with type B insulin resistance as a whole. Of the 11 cases, 3 developed lymphoma. High-dose corticosteroid therapy appeared to be effective therapy for the hypoglycemia, with often rapid response.
Isolated hypoglycemia with low insulin forms a rare subgroup of type B insulin resistance. These patients lack the common characteristics of hyperinsulinemic hypoglycemia and hyperglycemia/insulin resistance. Furthermore, while coexisting autoimmune disease is commonly observed, there is potentially an association with aggressive lymphoma, the onset of which may be delayed.
B 型胰岛素抵抗综合征是一种罕见的自身免疫性疾病，影响葡萄糖稳态，其特征是血清中存在针对胰岛素受体的自身抗体（AIRAbs）。患者通常表现为严重的胰岛素抵抗。也可能出现混合性高血糖和低血糖表型，也可能出现孤立性低血糖。经典的生化模式包括尽管低血糖但胰岛素水平升高；然而，一小部分病例表现为“孤立性低血糖伴低胰岛素”。本系统评价的主要目的是确定这一亚组的临床特征和结局。
对伴有低血糖和胰岛素抑制的病例进行系统评价。排除标准：高血糖、胰岛素升高、未确认 AIRAbs。
检索 PubMed、Medline 和 Embase 数据库，检索截至 2023 年 2 月，并通过手动引文搜索进行补充。使用 Joanna Briggs 研究所病例报告的批判性评估清单评估偏倚。
去除重复项后共确定了 5342 篇文章。所有符合所有纳入标准的病例报告均符合纳入标准，并被纳入。与 B 型胰岛素抵抗作为一个整体相比，属于该亚组的病例在性别、年龄和种族方面更加多样化。在 11 例中，有 3 例发展为淋巴瘤。大剂量皮质类固醇治疗似乎对低血糖有效，且反应通常迅速。
低胰岛素性孤立性低血糖是 B 型胰岛素抵抗的一个罕见亚组。这些患者缺乏高胰岛素性低血糖和高血糖/胰岛素抵抗的常见特征。此外，尽管常伴有自身免疫性疾病，但可能与侵袭性淋巴瘤有关，其发病可能会延迟。
