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Table 2 Survey of HHT patients regarding consultation for epistaxis with ENT specialists and ER physicians and relative timing of HHT diagnosis

From: Targeting under-diagnosis in hereditary hemorrhagic telangiectasia: a model approach for rare diseases?

Proportion of respondents (%)

940/2836 (33 %)

Mean age ± SD at time of survey

54.1 ± 14.1 years

Mean age ± SD at time of diagnosis

37.1 ± 15.5 years

Proportion of females (%)

630/940 (67 %)

Proportion of North Americans

738/940 (79 %)

Proportion of patients with recurrent epistaxis (%)

807/940 (86 %)

Proportion of patients with ENT consult prior to HHT diagnosis (%)

349/940 (37 %)

Proportion of patients with ER consult prior to HHT diagnosis (%)

154/940 (16 %)

Mean age ± SD at first ENT consult

27.8 ± 16.8 years

Mean age ± SD at first ER consult

30.1 ± 17.4 years

Mean interval of time ± SD from initial ENT consult to HHT diagnosis

13.9 ± 12.2 years

Mean interval of time ± SD from initial ER consult to HHT diagnosis

11.3 ± 12.6 years