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Table 1 Baseline characteristics

From: Current HHT genetic overview in Spain and its phenotypic correlation: data from RiHHTa registry

 n (%) or mean (SD)
Patients, n215
Sex, n (%)
 Male79 (36.7)
 Female136 (63.3)
Age at HHT diagnosis (years), mean ± SD42.2 ± 17.5
Ethnicity
 Caucasian190 (88.4)
 Hispanic2 (0.9)
 Others9 (4.2)
 No clinical data14 (6.5)
Underlying conditions, n (%)
 Current smoker26 (12.1)
 Hypertension43 (20)
 Diabetes Mellitus11 (5.1)
 Dyslipidemia33 (15.3)
 Venous Thromboembolism12 (5.5)
 Atrial fibrillation8 (3.7)
HHT criteria, n (%)
 4 HHT criteria99 (46)
 3 HHT criteria73 (34)
 2 or less HHT criteria32 (14.9)
 No clinical data11 (5.1)
Family history
 Positive178 (82.8)
 Negative11 (5.1)
 No clinical data26 (12.1)
Symptoms at onset, n (%)
 Nosebleeds197 (91.6)
 Central Nervous system event7 (3.2)
 Cerebral abscess4 (1.9)
 Stroke3 (1.4)
 Cutaneous telangiectases1 (0.5)
 Dyspnea1 (0.5)
 Anemia1 (0.5)
 No clinical data8 (3.7)
Muco-cutaneous telangiectasia, n (%)167 (77.7)
Baseline ESS3.65 (2.5)
Gene mutation, n (%)
ENG36 (16.7)
ACVRL177 (35.8)
 No clinical data102 (47.2)
Visceral involvement, n (%)
 Pulmonary AVM48 (22.3)
 Cerebral AVM5 (2.3)
 Hepatic involvement58 (27)
 Gastrointestinal involvementa26 (12.1)
  1. HHT Hereditary hemorrhagic telangiectasia, SD Standard deviation, ESS Epistaxis Severity Score, AVM Arteriovenous malformation
  2. aGastrointestinal involvement detected by fibrogastroscopy and / or colonoscopy