All issues > Volume 37(4); 1994

Case Report

J Korean Pediatr Soc. 1994;37(4):565-572. Published online April 15, 1994.

A Case of Prader-Willi Syndrome with Diabetes Mellitus

Hee Ran HR Choi¹, Eun Ha EH Choi¹, Seong Hee SH Jang¹, Young Min YM Ahn¹

¹Department of Pediatrics, Kangnam Hospital Public Corporation, Seoul, Korea

Abstract

Prader-Willi syndrome is characterized by infantile hypotonia, mental retardation, hyperhagia, hypogonadism and obestiy. Approximately 60% of all PLW syndrome show an interstitial deletion of chromosome 15, 37% have apparently normal chromosome, and 3.6% have a variety of other abnormalities involving chromosome 15. Diabetes mellitus has been considered a component of PLW syndrome and the incidence is about 7%. We experienced a 17-year-old female who revealed mental retardation, hypogonadism, obesity, and non-insulin dependent type DM, compatible with Prader-Willi syndrome.

Keywords :Prader-Willi syndrome, Diabetes mellitus