One Japanese MODY family with severe and progressive microangiopathies.

Recently we investigated maturity-onset diabetes of the young (MODY) with severe and progressive microangiopathies in one Japanese family. The proband was a female who was diagnosed as having diabetes when she was 11 years old, and was controlled without insulin for 9 years. She is now 30 years old and has been suffering from proliferative retinopathy since the age of 20. Her maternal grandfather, mother, uncle, and younger sister are also diabetic with severe microangiopathies. MODY is a concept which was first put forward by Tattersall and Fajans in 1975. It was defined as a form of diabetes diagnosed before the age of 25 years, controlled without insulin for more than 2 years and demonstrating dominant heredity over more than three generations. Most Western papers have reported few microangiopathies in the MODY patient, but our findings run counter to this description.