Permanent insulin-dependent diabetes mellitus after congenital temporary diabetes mellitus.

Congenital temporary diabetes mellitus is a rare syndrome occurring in the first.six weeks of life. It differs from classical juvenile-onset diabetes mellitus in infants in that it is transient, with complete recovery after three to four months. There is usually no family history of diabetes mellitus. The affected infants are underweight, yet full-term, with severe dehydration. The neonate has a "peculiar pallor and lined, aged appearance associated with a remarkably open-eyed alert facies."' Two to three weeks after birth glycosuria and hyperglycaemia, often severe, are noted; but ketosis is usually absent. Such children are insulin sensitive, requiring on average 8 units per day. Some patients have been followed-up for as long as 25 years2 with no evidence of recurrence of the diabetes mellitus. This report describes for the first time the development of permanent insulin-dependent diabetes mellitus in a young woman who had had congenital temporary diabetes mellitus as an infant.'