diabetes mellitus, ketosis-prone
diabetes mellitus, ketosis-proneA form of diabetes (OMIM:612227), which presents as a classic type-1, type-2 or atypical presentation, most common in those of African ancestry. Insulin may be required, but patients can be weaned from it and have prolonged remission with metabolic control. Insulin resistance may be seen in obese patients.
Defects in PAX4, which encodes a transcription factor that plays critical roles in foetal development (e.g., of pancreatic islets), cause ketosis-prone diabetes.