EPHB4-related lymphatic-related hydrops fetalis
Findings
No curated finding names EPHB4-related lymphatic-related hydrops fetalis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
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.
Definition from the Mondo Disease Ontology (MONDO:0035475), read 2026-09-29. CC BY 4.0.
Where it sits
- A kind of
Other names
5 names
Resolves to: EPHB4-related lymphatic-related hydrops fetalis
- Also called
- EPHB4-related generalised lymphatic dysplasia with atrial septal defectEPHB4-related generalised lymphatic dysplasia with non-immune hydrops fetalisEPHB4-related generalized lymphatic dysplasia with atrial septal defectEPHB4-related generalized lymphatic dysplasia with non-immune hydrops fetalisEPHB4-related LRHF/GLD