Pulmonary microvascular architecture in hereditary haemorrhagic telangiectasia
نویسندگان
چکیده
منابع مشابه
Hereditary haemorrhagic telangiectasia.
Hereditary haemorrhagic telangiectasia (Osler-Rendu-Weber syndrome) is an autosomal dominant vascular disorder, manifesting with telangiectases and bleeding in different parts of the body. We report a patient who presented with bleeding from various sites.
متن کاملPrimary pulmonary hypertension in families with hereditary haemorrhagic telangiectasia.
Primary pulmonary hypertension (PPH) is a rare but severe and progressive disease characterised by obstructive lesions of small pulmonary arteries. Patients with PPH often have mutations in the bone morphogenetic protein receptor type II (BMPR2) gene, whereas some carry mutations in the activin receptor-like kinase 1 (ALK-1) gene, generally associated with hereditary haemorrhagic telangiectasia...
متن کامل'Pseudocirrhosis' in hereditary haemorrhagic telangiectasia.
Telangiectasia-associated hepatic fibrosis (TAHF) in a 68-year-old woman with hereditary haemorrhagic telangiectasia (HHT) is described. The patient died of oat-cell carcinoma of the lung. In addition to the structural alterations which have been described previously in HHT, the liver exhibited focal midlobular hepatocytic necrosis and tumour metastases. The possibility that treatment of HHT wa...
متن کاملNeonatal pulmonary arteriovenous malformation in hereditary haemorrhagic telangiectasia.
A 3 week old infant presented with persistent hypoxaemia and was diagnosed with pulmonary arteriovenous malformations. Her family history was positive for hereditary haemorrhagic telangiectasia. She was treated successfully with coil embolotherapy at the age of 4 months. Transcatheter embolisation may be considered the primary treatment for pulmonary arteriovenous malformations in infancy.
متن کاملHereditary haemorrhagic telangiectasia: neuropathological observations.
While the literature pertaining to hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu disease) has been quite extensive, very few reports have dealt with the neurological manifestations of the disease. Occasional anatomical studies have appeared, but reports of neuropathological findings have been rare. The purpose of the present paper is to provide neuropathological observations in a pa...
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ژورنال
عنوان ژورنال: Thorax
سال: 2017
ISSN: 0040-6376,1468-3296
DOI: 10.1136/thoraxjnl-2016-209299