Unilateral multicystic dysplastic kidney

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Unilateral multicystic dysplastic kidney

ORPHA:97363Clinical subtype

Also called Unilateral MCDK · Unilateral multicystic renal dysplasia

What it is

A rare form of multicystic dysplastic kidney (MCDK), a congenital anomaly of the kidney and urinary tract (CAKUT), in which one kidney is large, distended by multiple cysts, and non-functional.

Key facts

Prevalence
1-5 / 10 000 (at birth)
Age of onset
All ages
Inheritance
Autosomal dominant
Classified as
Clinical subtype

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Gene

HNF1BDisease-causing germline mutation(s)

ICD-10 codes

Q61.4filed under a broader ICD-10 category — shared with 5 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MONDO 0019981UMLS C1567426

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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