[Rendu-Osler-Weber disease. Descriptive study of 17 cases]. / La enfermedad de Rendu-Osler-Weber. Estudio descriptivo de 17 casos.
Rev Clin Esp
; 201(11): 638-41, 2001 Nov.
Article
en Es
| MEDLINE
| ID: mdl-11786130
OBJECTIVE: To study the prevalence and presentation forms of hereditary hemorrhagic telangiectasia (HHT) among the population in our hospital area. MATERIALS AND 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.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Telangiectasia Hemorrágica Hereditaria
Tipo de estudio:
Diagnostic_studies
/
Observational_studies
/
Prevalence_studies
/
Screening_studies
Límite:
Adult
/
Aged
/
Aged80
/
Female
/
Humans
/
Male
/
Middle aged
Idioma:
Es
Revista:
Rev Clin Esp
Asunto de la revista:
MEDICINA
Año:
2001
Tipo del documento:
Article
País de afiliación:
España
Pais de publicación:
España