Fajans S S
Adv Exp Med Biol. 1985;189:65-87. doi: 10.1007/978-1-4757-1850-8_5.
The heterogeneity within Type II diabetes (NIDDM) and within Maturity-Onset type Diabetes of Young people (MODY), a subset of NIDDM which is inherited in an autosomal dominant fashion, is discussed. Aspects of the definition and phenotypic expression of MODY are reviewed. Within NIDDM there are differences in patterns of inheritance between subgroups. HLA antigen associations are not found in most NIDDM populations but exist in three specific population groups with Type II diabetes. Within NIDDM and within MODY there are differences in the magnitude of insulin responses to glucose, differences in target tissue responsiveness to insulin in vivo, and differences in receptor and post-receptor effects of insulin. Structurally abnormal variant and biologically defective insulin molecules have been found in some Type II diabetic patients and in members of certain MODY families. The presence or absence of obesity may mark heterogeneous groups of Type II diabetic patients, in addition to the importance of obesity in uncovering an insulin secretory defect by causing insulin resistance. There is heterogeneity in susceptibility to vascular disease within NIDDM and MODY. The natural history of carbohydrate metabolism and of insulin secretory responses to glucose in early Type I diabetes and in MODY with low insulin secretory responses are illustrated and similarities and dissimilarities compared and contrasted. Failure to recognize young patients with MODY may contribute to incorrect diagnosis, management, and assignment of prognosis of this form of diabetes in the young by many practicing physicians. The recognition that Type I or insulin-dependent diabetes (IDDM) and Type II or noninsulin-dependent (NIDDM) differ from each other not only phenotypically but also in etiology and pathogenesis led the National Diabetes Data Group (NDDG) to devise the present nomenclature and classification of diabetes mellitus. These were adopted by the World Health Organization. As suggested by the NDDG report, the classification should be reexamined periodically to reflect improved understanding of the disease, to stimulate further research, and to be of help to practicing physicians.
本文讨论了II型糖尿病（非胰岛素依赖型糖尿病，NIDDM）以及青年发病的成年型糖尿病（MODY，NIDDM的一个子集，以常染色体显性方式遗传）内部的异质性。回顾了MODY的定义和表型表达方面。在NIDDM中，各亚组之间的遗传模式存在差异。大多数NIDDM人群中未发现HLA抗原关联，但在三个特定的II型糖尿病患者群体中存在。在NIDDM和MODY内部，对葡萄糖的胰岛素反应强度存在差异，体内靶组织对胰岛素的反应性存在差异，胰岛素的受体及受体后效应也存在差异。在一些II型糖尿病患者和某些MODY家族成员中发现了结构异常的变异胰岛素分子和生物学功能缺陷的胰岛素分子。肥胖的存在与否可能标志着II型糖尿病患者的异质群体，此外，肥胖通过引起胰岛素抵抗在揭示胰岛素分泌缺陷方面也很重要。在NIDDM和MODY中，对血管疾病的易感性存在异质性。阐述了早期I型糖尿病以及胰岛素分泌反应低的MODY中碳水化合物代谢和对葡萄糖的胰岛素分泌反应的自然史，并对异同点进行了比较和对照。许多执业医生未能识别患有MODY的年轻患者，可能导致对这种年轻型糖尿病的错误诊断、治疗和预后判断。认识到I型或胰岛素依赖型糖尿病（IDDM）和II型或非胰岛素依赖型糖尿病（NIDDM）不仅在表型上不同，而且在病因和发病机制上也不同，促使国家糖尿病数据组（NDDG）设计了目前的糖尿病命名法和分类。这些被世界卫生组织采用。正如NDDG报告所建议的，应定期重新审视该分类，以反映对疾病的更好理解，促进进一步研究，并帮助执业医生。
