Most of those who develop diabetes show atrophy of the entire pancreas, with mild or subclinical deficiency of exocrine as well as endocrine function.

Kidney and genitourinary malformation and diseases may occur, but inconsistently even within a family, and the specific conditions include a range of apparently unrelated anomalies and processes.

Kidney disease may develop before or after hyperglycemia, and a significant number of people with MODY5 are discovered in renal clinics.

Male defects have included epididymal cysts, agenesis of the vas deferens, or infertility due to abnormal spermatozoa.

[citation needed] Renal cysts and diabetes syndrome is caused by mutations in or deletions of the HNF1B gene.