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Hereditary Haemorrhagic Telangiectasia (hereditary + haemorrhagic_telangiectasia)

Distribution by Scientific Domains
Medical Sciences100%

Selected Abstracts

Bosentan therapy for pulmonary arterial hypertension associated with hereditary haemorrhagic telangiectasia

EUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 2006
D. Bonderman
Abstract Hereditary haemorrhagic telangiectasia (HHT) is a disorder of arteriovenous malformations and telangiectases. In rare cases affected individuals may develop typical pulmonary arterial hypertension (PAH). Vasodilator therapy has not been recommended because of a potential increase in arteriovenous shunt volume. This report is on two patients with severe HHT-associated PAH who were treated with bosentan, an oral endothelin ETA/ETB receptor antagonist. After 1 year, symptomatic and functional improvements were confirmed by haemodynamic evaluation demonstrating a significant decrease of mean pulmonary artery pressures and an increase in cardiac index, without evidence for an increase in effective shunt volume. [source]

Hereditary haemorrhagic telangiectasia involving the bone marrow and liver

BRITISH JOURNAL OF HAEMATOLOGY, Issue 2 2009
Jason Willis
No abstract is available for this article. [source]

Hereditary haemorrhagic telangiectasia associated with vitiligo, autoimmune thyroiditis, iridocyclitis and a myelodysplastic syndrome

CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 4 2005
A. Grapsa
No abstract is available for this article. [source]

Bosentan therapy for pulmonary arterial hypertension associated with hereditary haemorrhagic telangiectasia

EUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 2006
D. Bonderman
Abstract Hereditary haemorrhagic telangiectasia (HHT) is a disorder of arteriovenous malformations and telangiectases. In rare cases affected individuals may develop typical pulmonary arterial hypertension (PAH). Vasodilator therapy has not been recommended because of a potential increase in arteriovenous shunt volume. This report is on two patients with severe HHT-associated PAH who were treated with bosentan, an oral endothelin ETA/ETB receptor antagonist. After 1 year, symptomatic and functional improvements were confirmed by haemodynamic evaluation demonstrating a significant decrease of mean pulmonary artery pressures and an increase in cardiac index, without evidence for an increase in effective shunt volume. [source]

The diagnostic quandary of hereditary haemorrhagic telangiectasia vs.

BRITISH JOURNAL OF DERMATOLOGY, Issue 4 2001
CREST syndrome
The distribution and clinical appearance of the telangiectasia in the CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal involvement, sclerodactyly, telangiectasia) and hereditary haemorrhagic telangiectasia (HHT) are very similar. Several previously reported cases of the CREST syndrome simulating HHT illustrate this diagnostic quandary. We report a patient who met the diagnostic criteria for both the CREST syndrome and HHT, and discuss the distinguishing features of the two diseases, including the distinctive histopathological findings of telangiectasia in HHT. [source]