CONGENITAL PERMANENT DIABETES - A DIFFERENT TYPE OF DIABETES

Objective: To investigate two related children with congenital permane nt diabetes, Subjects and methods: Two related male patients of Arab o rigin with congenital permanent diabetes were studied for immunologica l and genetic markers, insulin and glucagon secretion, thiamine levels , pancreatic ultrasounds studies and developmental characteristics. Re sults: Both patients developed normally. The immunological markers wer e negative, there was no ketosis at diagnosis, and both patients were negative for diabetes susceptibility alleles at the HLA locus. Insulin levels were undetectable, glucagon secretion, thiamine levels and pan creatic ultrasound studies were normal. Conclusions: Congenital perman ent diabetes is an extremely rare entity that differs from type I or t ype II diabetes. Our patients have a unique disorder with isolated bet a cell defect and no additional manifestations. Autosomal recessive in heritance is suggested.