[Rendu-Osler-Weber disease. Descriptive study of 17 cases].
OBJECTIVE
To study the prevalence and presentation forms of hereditary hemorrhagic telangiectasia (HHT) among the population in our hospital area.


METHODS
Descriptive, retrospective study of patients with the diagnosis of HHT from to 1999 at La Fe Hospital, Valencia. Family history, repeated epistaxis, mucocutaneous telangiectasis and visceral lesions, as well as administered treatments, and clinical course were evaluated.


RESULTS
Seventeen patients were diagnosed during such time period, with ages ranging from 23 to 80 years. Eight patients had family histories. The most common symptoms included epistaxis and iron-deficiency anemia, followed by muco-cutaneous telangiectasis. Six patients had neurological, five pulmonary, five gastrointestinal, and three hepatic manifestations, usually secondary to vascular malformations at those levels.


CONCLUSIONS
The prevalence of the disease maybe higher than previously reported. This disease should be considered in patients with repeated epistaxis and/or iron-deficiency anemia. The presence of telangiectasis that can suggest the diagnosis should be investigated. An early diagnosis of HHT can be useful for the early control of associated visceral malformations. Treatment of HHT should be tailored according to the clinical manifestations of the patient.
Alonso Estellés
R
R
Servicio de Medicina Interna, Hospital Universitario La Fe, Valencia, Spain.
Campo López
C
C
Todolí Parra
J A
JA
Calabuig Alborch
J R
JR
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology
La enfermedad de Rendu-Osler-Weber. Estudio descriptivo de 17 casos.