• Medicina clinica · Apr 2019

    Review

    Medical management of haemorrhagic hereditary telangiectasia in adult patients.

    • Antoni Riera-Mestre, Jesús Ribas, and José Castellote.
    • Unidad de Telangiectasia Hemorrágica Hereditaria, Hospital Universitari de Bellvitge-IDIBELL, L'Hospitalet de LLobregat, Barcelona, España; Servicio de Medicina Interna, Hospital Universitari de Bellvitge-IDIBELL, L'Hospitalet de LLobregat, Barcelona, España; Facultad de Medicina y Ciencias de la Salud, Universitat de Barcelona, Barcelona, España. Electronic address: ariera@bellvitgehospital.cat.
    • Med Clin (Barc). 2019 Apr 5; 152 (7): 274-280.

    AbstractHereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant inherited Rare Disease that causes a systemic anomalous vascular overgrowth. The approach and follow-up of these patients should be from multidisciplinary units. Its diagnosis is carried out according to Curaçao clinical Criteria. Telangiectasia in the nasal mucosa cause recurrent epistaxis, the main symptom of HHT and difficult to control. The three types of hepatic shunting, hepatic artery to hepatic vein, hepatic artery to portal vein or to portal vein to hepatic vein, can cause high-output heart failure, portal hypertension or porto-systemic encephalopathy, respectively. These types of vascular involvement can be established using computerised tomography. Pulmonary arteriovenous fistula should be screened for all HHT patients by contrast echocardiography. The main objective is to review the management of epistaxis, liver and lung involvement of the adult patient with HHT.Copyright © 2018 Elsevier España, S.L.U. All rights reserved.

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