Hermansky-Pudlak syndrome

Orpha code: 79430OMIM code: 614171

Definicja

Hermansky-Pudlak syndrome (HSP) is a multi-system disorder characterized by oculocutaneous albinism, bleeding diathesis and, in some cases, neutropenia, pulmonary fibrosis, or granulomatous colitis. HPS comprises eight known disorders (HPS-1 to HPS-8), the majority of which present with the same clinical phenotype to varying degrees of severity.

Disease data
Klasyfikacja

Disease

Synonimy
HPS
HPS
Kod ORPHA
79430
Kod OMIM
614171
Kod ICD10
E70.3
Kod ICD11
EC23.20

No additional description.

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