Hereditary haemorrhagic telangiectasia

Description

Rendu-Osler-Weber disease, also called hereditary haemorrhagic telangiectasia (HHT), is a genetic disorder of angiogenesis leading to arteriovenous dilatations: cutaneo-mucosal haemorrhagic telangiectasias and visceral shunting.

Index terms

Hereditary haemorrhagic telangiectasia
HHT - [hereditary haemorrhagic telangiectasia]
Osler haemorrhagic telangiectasia syndrome

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Osler-Weber-Rendu disease
Rendu-Osler-Weber disease

Exclusions from above levels

LD2F.1Y – Macrocephaly - Cutis Marmorata Telangiectatica Congenita
2E81 – haemangioma and lymphangioma
LA13.5 – Congenital retinal aneurysm

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LA8B.1 – Congenital anomaly of pulmonary arterial tree
LA8C – Congenital anomaly of coronary artery
http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

LA90.0Y – Other specified capillary malformations
LA90.0Z – Capillary malformations, unspecified

Foundation entity

Hereditary haemorrhagic telangiectasia – http://id.who.int/icd/entity/714406192

Part of: LA90.0: Capillary malformations › LA90: Structural developmental anomalies of the peripheral vascular system › 20: Developmental anomalies