| efrinereceptor B4-gerelateerde lymfatische hydrops foetalis (aandoening) | | efrinereceptor B4-gerelateerde lymfatische hydrops foetalis | | EphB4-gerelateerde lymfatische hydrops foetalis
EPHB4-gerelateerde lymfatische dysplasie met niet-immune hydrops foetalis
| | EPHB4-related lymphatic-related hydrops fetalis | | EPHB4-related generalized lymphatic dysplasia with non-immune hydrops fetalis
EPHB4-related generalized lymphatic dysplasia with atrial septal defect
Ephrin receptor B4-related lymphatic-related hydrops fetalis
| | A rare primary lymphedema characterized by a highly variable lymphatic phenotype ranging from severe lymphatic-related hydrops fetalis, which may cause perinatal demise or fully resolve to become completely asymptomatic, to a mild presentation in older patients with persistent varicose veins, peripheral edema and impaired lymph drainage in the lower limbs. Atrial septal defect has been described in association and may be the only anomaly in some patients. |
| | Id | 1222666002 | | Status | Primitive |
| SNOMED CT to Orphanet simple map | 568065 |
| SNOMED CT to ICD-10 extended map | | Target | I89.0 | | Rule | TRUE | | Advice | ALWAYS I89.0 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | | Correlation | SNOMED CT source code to target map code correlation not specified |
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