Renal cysts and diabetes syndrome
Renal cysts and diabetes syndrome
Definition
Renal cysts and diabetes syndrome (RCAD) is a rare form of maturity-onset diabetes of the young (MODY) characterized clinically by heterogeneous cystic renal disease and early-onset familial non-autoimmune diabetes. Pancreatic atrophy, liver dysfunction and genital tract anomalies are also features of the syndrome.
Also known as CAKUT with diabetes, congenital anomalies of the kidney and urinary tract with diabetes, hepatocyte nuclear Factor 1-beta-associated monogenic diabetes, HNF1B-MODY, HNF1B-related renal cysts and diabetes syndrome, maturity onset diabetes of the Young, type 5, MODY5, RCAD, RCAD syndrome, renal cysts and diabetes syndrome, renal cysts-maturity-onset diabetes of the young syndrome, renal dysfunction-early-onset diabetes syndrome — per MONDO
Also identified as
- DOID 0111101 per MONDO
- MESH C535520 per MONDO
- NCIT C123018 per MONDO
- OMIM 137920 per MONDO
- Orphanet 93111 per MONDO
- SCTID 446641003 per MONDO
- UMLS C0431693 per MONDO
Affected Organs
| Organ | Involvement | Source |
|---|---|---|
| Alimentary part of gastrointestinal system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Alimentary part of gastrointestinal system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Endocrine gland | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Pancreas | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Pancreas | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |