ICD-11 classes
05 Endocrine, nutritional or metabolic diseases
Metabolic disorders
Inborn errors of metabolism
5C56 — Lysosomal diseases
5C56.0 — Sphingolipidosis
5C56.01 — Fabry disease
ICD-11 5C56.01 — Fabry disease
Fabry disease (FD) is a progressive, inherited, multisystemic lysosomal storage disease characterised by specific neurological, cutaneous, renal, cardiovascular, cochleo-vestibular and cerebrovascular manifestations.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere:
- Sphingolipidoses with skin manifestations #16079
- Syndromes with structural anomalies due to inborn errors of metabolism #23408
The diagnosis is included in 1 another class.
- Glomerular disease associated with Fabry disease (GB4Z)