RESUMO
Thirty-two patients (17 men and 15 women) are presented in whom the diagnosis Rendu-Osler-Weber disease was established between 1980 and 1990 in the Leeward Islands of the Netherlands Antilles during hospitalization. Among these patients, there were eight families, each of which contained two patients. Estimated point-prevalence rate of the disease in 1991 was 19.4/100,000, which is ten times higher than has been reported so far. Epistaxis, gastrointestinal blood loss and cerebrovascular accidents (CVA) were the three main presenting symptoms. Mean age at diagnosis was 53.2 years, while the delay in reaching the diagnosis averaged 8.3 years. Arteriovenous fistulae in the lung developed in 11 patients (34 percent). Eleven patients experienced a total of twelve CVAs at a mean age of 48 years; four of these patients had an ischaemic CVA in the presence of pulmonary fistulae, while three patients had an haemorrhagic stroke in the presence of a cerebral arteriovenous malformation. Thus, Morbus Rendu-Osler-Weber is a prevalent, but rather under-diagnosed disease and frequently presents with cerebrovascular accidents at an early age in inhabitants of the Leeward Islands of the Netherlands Antilles (AU)
