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Pulmonary Arterial Hypertension and Hereditary Haemorrhagic Telangiectasia.

Abstract：

:Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant inherited disease characterised by multisystemic vascular dysplasia. Heritable pulmonary arterial hypertension (HPAH) is a rare but severe complication of HHT. Both diseases can be the result of genetic mutations in ACVLR1 and ENG encoding for proteins involved in the transforming growth factor-beta (TGF-β) superfamily, a signalling pathway that is essential for angiogenesis. Changes within this pathway can lead to both the proliferative vasculopathy of HPAH and arteriovenous malformations seen in HHT. Clinical signs of the disease combination may not be specific but early diagnosis is important for appropriate treatment. This review describes the molecular mechanism and management of HPAH and HHT.

journal_name

Int J Mol Sci

journal_title

International journal of molecular sciences

authors

Vorselaars VMM,Hosman AE,Westermann CJJ,Snijder RJ,Mager JJ,Goumans MJ,Post MC

doi

10.3390/ijms19103203

subject

Has Abstract

pub_date

2018-10-17 00:00:00

issue

10

issn

1422-0067

pii

ijms19103203

journal_volume

19

pub_type

杂志文章,评审

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