HEREDITARY FAMILIAL CONGENITAL HAEMORRHAGIC NEPHRITIS

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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.

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This report describes the renal pathology in three siblings with hereditary nephritis. All three cases showed combined features of chronic glomerulonephritis, pyelonephritis, and interstitial nephritis. Foam cells were seen in only one case. These findings support the contention of Krickstein, Gloor, and Balogh (1966) that the renal changes in hereditary nephritis are those of a mixed nephritis.

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Hereditary 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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'Pseudocirrhosis' in hereditary haemorrhagic telangiectasia.

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Hereditary haemorrhagic telangiectasia: a clinical analysis.

Data from 98 patients with hereditary haemorrhagic telangiectasia (HHT) are presented. All were symptomatic by 40 years of age and 62% by 16 years. Nose bleeding was the first symptom of disease in 90% of cases with mucocutaneous telangiectases appearing 5 to 20 years later. Complications of HHT are discussed and an age of onset curve given.

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ژورنال

عنوان ژورنال: BMJ

سال: 1927

ISSN: 0959-8138,1468-5833

DOI: 10.1136/bmj.1.3454.504