Renal agenesis, unilateral

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Renal agenesis, unilateral

ORPHA:93100Clinical subtype

What it is

A form of renal agenesis characterized by the complete absence of development of one kidney accompanied by an absent ureter.

Key facts

Prevalence
1-5 / 10 000 (at birth)
Age of onset
All ages, Antenatal
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.

Genes

BMP4Disease-causing germline mutation(s)
DSTYKDisease-causing germline mutation(s)
FRAS1Disease-causing germline mutation(s)
FREM1Disease-causing germline mutation(s)
FREM2Disease-causing germline mutation(s)
GREB1LDisease-causing germline mutation(s) (loss of function)
RETDisease-causing germline mutation(s)
UPK3ADisease-causing germline mutation(s)

ICD-10 codes

Q60.0ICD-10 names this disease exactly — shared with 1 other rare disease

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.

Cross-references

MEDDRA 10053624MONDO 0019636OMIM 617805UMLS C0266294

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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