Jan 1, 1977

Studies on families with Osler's disease

Folia haematologica : internationales Magazin für klinische und morphologische Blutforschung
C Hadnagy, P Kovalszky

Abstract

The authors examined 143 members of a family, where they found 37 (= 25.87%) of Osler patients. It was only in 30% of these patients that the symptoms occurred before the tenth year of age. As in one patient the symptoms did not appear until the age of 58 years, the possibility cannot be excluded that symptoms of the disease will become manifest even in other, sill younger members of the family in the course of time. Epistaxis was observed in 93% of the cases, nephrorrhagia in no case, hepatopathy and gastrorhagy were found only once in each case. The X-ray examination revealed arteriovenous pulmonary aneurysm in 5 cases. As a rule, oestrogen treatment led to good results. A case of death occurred during an influenza epidemic in a severe anaemic patient. Clinical main symptoms of Osler's disease were epistaxis and arteriovenous fistulae which could be roentgenologically identified in the lung. Teleangiectasia could be detected during the autopsy besides vessel anomalies on the surface even in the bronchi, oesophagus, trachea, stomach, kidneys, small intestine and particularly in the large intestines. Conditions of iron deficiency may very often occur in osler Patients; they require a substituting treatment.

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Mentioned in this Paper

Influenza
Iron Deficiency Anemia
Tracheomalacia
Hereditary Hemorrhagic Telangiectasia
Asiderotic Anemia
Estrogen Effect
Epistaxis
Iron
Genealogical Tree

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