ICD-11 classes
16 Diseases of the genitourinary system
Diseases of the urinary system
Cystic or dysplastic kidney disease
LD2F.13 — Meckel-Gruber syndrome

ICD-11 LD2F.13 — Meckel-Gruber syndrome

Meckel syndrome (MKS) is a monogenic disease characterised by a combination of renal cysts and variably associated features, including developmental anomalies of the central nervous system (usually occipital encephalocele), hepatic ductal dysplasia and cysts, and polydactyly., and a lethal course, with death occurring in the perinatal period.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

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