Background

Diabetes mellitus is the most commonly encountered endocrinopathy in patients with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), which manifests as multisystemic organ failure. Whether the management of diabetes mellitus in MELAS requires special consideration is not fully clarified.

Methods

In this single-center study, we retrospectively reviewed the medical records of patients with MELAS to elucidate the clinical characteristics of MELAS–associated diabetes mellitus.

Results

Four patients among a total of 25 individuals with MELAS who were treated in the study institution developed diabetes mellitus. One patient had well-controlled diabetes mellitus, whereas the remaining three patients experienced hyperglycemic crisis as the first manifestation of diabetes mellitus. Two of the three patients were children aged four and six years. The hyperglycemic events occurred after surgery, infection, and status epilepticus, respectively. None of the three patients had diabetes mellitus previously based on randomly measured serum glucose levels that were within the normal range before the hyperglycemic crisis. Glycated hemoglobin levels measured during the hyperglycemic crisis indicated prediabetes in two patients and diabetes mellitus in one patient. Two patients recovered, whereas one patient died after developing multiorgan failure.

Conclusions

Fulminant-onset diabetes mellitus occurring in patients with MELAS underscore the importance of routine measurement for glycated hemoglobin and more intense evaluation of glucose intolerance regardless of the patient age and lack of symptoms. Clinicians should be aware of the potential acute onset of hyperglycemic crisis in patients with MELAS, especially in individuals with aggravating factors.

中文翻译：

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线粒体脑病、乳酸酸中毒和中风样发作 (MELAS) 患者的高血糖危象

背景

糖尿病是线粒体脑病、乳酸性酸中毒和卒中样发作 (MELAS) 患者中最常见的内分泌疾病，表现为多系统器官衰竭。MELAS 中糖尿病的管理是否需要特别考虑尚未完全阐明。

方法

在这项单中心研究中，我们回顾性审查了 MELAS 患者的医疗记录，以阐明 MELAS 相关糖尿病的临床特征。

结果

在研究机构接受治疗的总共 25 名 MELAS 患者中，有 4 名患者发展为糖尿病。一名患者的糖尿病控制良好，而其余三名患者则以高血糖危象为糖尿病的首发表现。三名患者中有两名是四岁和六岁的儿童。高血糖事件分别发生在手术、感染和癫痫持续状态之后。根据随机测量的血糖水平在高血糖危象前的正常范围内，这三名患者之前均未患有糖尿病。在高血糖危象期间测量的糖化血红蛋白水平表明两名患者患有前驱糖尿病，一名患者患有糖尿病。两名患者康复，

结论

MELAS 患者发生暴发性糖尿病强调了常规测量糖化血红蛋白和更深入地评估葡萄糖耐受不良的重要性，而不管患者的年龄和无症状。临床医生应该意识到 MELAS 患者可能会急性出现高血糖危象，尤其是有加重因素的个体。