Ketosis-prone diabetes (KPD) is a widespread, emerging, heterogeneous syndrome characterized by patients who present with diabetic ketoacidosis or unprovoked ketosis but do not necessarily have the typical phenotype of autoimmune type 1 diabetes. Multiple, severe forms of beta-cell dysfunction appear to underlie the pathophysiology of KPD. Until recently, the syndrome has lacked an accurate, clinically relevant and etiologically useful classification scheme. We have utilized a large, longitudinally followed, heterogeneous, multiethnic cohort of KPD patients to identify four clinically and pathophysiologically distinct subgroups that are separable by the presence or absence of beta-cell autoimmunity and the presence or absence of beta-cell functional reserve. The resulting "Abeta" classification system of KPD has proven to be highly accurate and predictive of such clinically important outcomes as glycemic control and insulin dependence, as well as an aid to biochemical and molecular investigations into novel causes of beta-cell dysfunction. In this review, we describe the current state of knowledge in regard to the natural history, pathophysiology, and treatment of the subgroups of KPD, with an emphasis on recent advances in understanding their immunological and genetic bases.

中文翻译：

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酮症倾向糖尿病综合征。

酮症倾向糖尿病 (KPD) 是一种广泛存在的、新兴的、异质性综合征，其特征是患者表现为糖尿病酮症酸中毒或无端酮症，但不一定具有自身免疫性 1 型糖尿病的典型表型。多种严重形式的 β 细胞功能障碍似乎是 KPD 病理生理学的基础。直到最近，该综合征还缺乏准确的、临床相关的和病因学上有用的分类方案。我们利用一个大型的、纵向跟踪的、异质的、多民族的 KPD 患者队列来确定四个临床和病理生理学上不同的亚组，这些亚组可通过存在或不存在 β 细胞自身免疫以及存在或不存在 β 细胞功能储备来区分。由此产生的“Abeta” KPD 分类系统已被证明是高度准确的，可以预测血糖控制和胰岛素依赖等临床重要结果，并有助于对 β 细胞功能障碍的新原因进行生化和分子研究。在这篇综述中，我们描述了关于 KPD 亚群的自然史、病理生理学和治疗的当前知识状态，重点是在了解其免疫学和遗传基础方面的最新进展。