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GB82 Autosomal dominant tubulointerstitial disease

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

Nonglomerular, autosomal dominant kidney diseases characterised by progressive tubulointerstitial fibrosis and progression to end-stage renal disease. Currently there are 4 known genetic defects - in uromodulin, mucin-1, renin and hepatocyte nuclear factor 1-beta. The last is associated with Maturity-Onset Diabetes of the Young (MODY) and thus is classified as MODY-5

code elsewhere

• Medullary sponge kidney  (LB30.8)
• MODY 5 syndrome  (5A13.6)

synonyms

• Autosomal dominant tubulointerstitial disease
• medullary cystic kidney disease
• ADTKD - [autosomal dominant tubulointerstitial disease] NOS
• autosomal dominant tubulointerstitial kidney disease NOS
• MCKD [medullary cystic kidney disease]
• medullary cystic disease
• medullary cystic kidney
• Autosomal dominant tubulointerstitial disease, Type 1
• autosomal recessive medullary cystic disease
• nephronopthisis
• medullary cystic kidney disease, Type 1
• Mucin-1 Kidney disease
• ADTKD-MUC1 [autosomal dominant tubulointerstitial kidney disease MUC1]
• MCKD - [medullary cystic kidney disease] type 1
• Autosomal dominant tubulointerstitial disease Type 2
• medullary cystic kidney disease, Type 2
• ATDKD-UMOD - [Autosomal dominant tubulointerstitial disease-uromodulin]
• MCKD - [medullary cystic kidney disease] type 2
• Uromodulin associated kidney disease
• Uromodulin kidney disease

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