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Table 1 Demographic data, HHT manifestations, and gene mutations

From: Predictors of mortality in patients with hereditary hemorrhagic telangiectasia

Characteristics Overall Alive Dead
Count 1286 1227 59
Age at enrollment, mean ± SD 46.0 ± 19.7 45.3 ± 19.5 60.8 ± 18.0
Age category  
 < 20 171 (13%) 168 (14%) 3 (5%)
 20–39 263 (20%) 258 (21%) 5 (8%)
 40–59 511 (40%) 500 (41%) 11 (19%)
 > 60 341 (27%) 301 (25%) 40 (68%)
Female 749 (58%) 718 (59%) 31 (53%)
Smoker (past or current) 428/1231 (35%) 395/1178 (34%) 33/53 (62%)
Brain VM 278 (22%) 268 (22%) 10 (17%)
Pulmonary AVM 615/1235 (50%) 585/1178 (50%) 30/57 (53%)
Symptomatic liver VM 217/1217 (18%) 196/1162 (17%) 21/55 (38%)
GI bleeding 216/1233 (18%) 189/1177 (16%) 27/56 (48%)
Anemia 591/1234 (48%) 550/1181 (47%) 41/53 (77%)
Genotype  
 ACVRL1 357/886 (40%) 343/854 (40%) 14/32 (44%)
 ENG 496/886 (56%) 482/854 (56%) 14/32 (44%)
 SMAD4 33/886 (4%) 29 (3%) 4/32 (13%)
  1. Values are n (%) or n/total (%), unless otherwise specified