Anomalies of renal development are well know for patients treated for MODY-5 diabetes due to TCF2 mutation.A recent study confirms the existence of pediatric patients having TCF2 mutation but presenting renal anomalies alone.Endocrine and renal evolution of these patients is unknown.The aim of this study is to follow a cohort of patients with TCF2 mutation and initially presenting renal anomalies alone.

Biologic analysis and renal ultrasonography once a year.

After puberty or before kidney transplantation

• Abdominal and pelvic MRI
• Intravenous Glucose Tolerance Test

Inclusion Criteria:

• Patients presenting an anomaly of renal development due to TCF2 mutation
• Age<18 years old

Exclusion Criteria:

• Anomaly of renal development without TCF2 mutation
• Age≥18 years old
• Parents or patients refusing to participate to the study

pediatric patients presenting an abnormality of kidney developmentdue to TCF2 mutation or deletion.