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Table 1: 
Patient population and prevalence of vascular lesions
Patients with Definite HHT No (%)
No. of patients376
Mean age (SD) (yr)53.0 (43.4)
Sex
    Male151 (40.1)
    Female225 (59.8)
Imaging
    CTA/MRA140 (37.2)
    DSA46 (12.2)
    MRI365 (97.1)
Curacao criteria
    Epistaxis334 (88.8)
    Mucocutaneous telangiectasia327 (87.0)
    Visceral AVMs321 (85.4)
    Family history324 (86.2)
Mutation analysis
    ENG61 (49.6)
    ACVRL148 (39.0)
    SMAD414 (11.4)
Chest CT311 (82.7)
    Pulmonary AVMs176 (56.6)
Abd/pelv CT344 (91.5)
    Hepatic vascular malformations185 (53.8)
    Other GI vascular malformations8 (2.3)
Neuroimaging findings
    Cerebral AVM48 (12.8)
    DVA45 (12.0)
    Cavernoma/focal hemosiderin deposition13 (3.5)
    Capillary telangiectasia9 (2.4)
    Cerebral aneurysm8 (2.1)
  • Note:—Abd indicates abdominal; pelv, pelvic; GI, gastrointestinal.