A Case of Myotonic Dystrophy (MD) Associated with Glucose-Induced Hyperinsulinemia Followed by Reactive Hypoglycemia and Increased Number of Cytosine-Thymine-Guanine (CTG) Trinucleotide Repeats in MD Gene

Endocrine Journal

KIMIKO YAMAUCHI¹  KUNIO KOSHIMURA¹  MOTOI SOHMIYA¹  YUZURU KATO¹ 

[1] First Division, Department of Medicine, Shimane Medical University

关键词: Myotonic dystrophy;    Reactive hypoglycemia;    Oral glucose administration;    Proinsulin;    Hyperinsulinemia;   

DOI  :  10.1507/endocrj.47.277

学科分类：内分泌与代谢学

来源: Japan Endocrine Society

【 摘 要 】

References(29)Cited-By(1)A 39-year-old man with myotonic dystrophy consulted our hospital for nausea, vomiting and dizziness that occurred after 75g oral glucose tolerance test (OGTT). Reexamination of OGTT revealed remarkable hyperinsulinemia (622μU/ml) followed by reactive hypoglycemia (50mg/dl) and such hypoglycemic symptoms as nausea, vomiting, dizziness and palpitation. DNA analysis of the circulating lymphocytes revealed increased (1, 500 times) number of cytosine-thymine-guanine (CTG) trinucleotide repeats in myotonic dystrophy protein kinase (DM kinase) gene. Gel chromatographic analysis of the plasma in combination with sensitive enzyme immunoassay of insulin revealed that the ratio of proinsulin to total immunoreactive insulin was elevated at fasting (12.9%), and was decreased to 8.9% at 60min after glucose administration. These findings may indicate that biologically active authentic insulin was predominantly secreted after glucose administration in the present case. This is the first case report of myotonic dystrophy with hyperinsulinemia associated with reactive hypoglycemia induced by oral glucose administration.

【 授权许可】

Unknown