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Table 1 Clinical characteristics of 23 patients with HHT and acute VTE

From: Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry

  Pulmonary embolism Deep vein thrombosis Odds ratio (95% CI) /
P value
Patients, N 14 9  
 Clinical characteristics,
  Male gender 6 (43%) 7 (78%) 0.21 (0.03–1.43)
  Mean age (years±SD) 60 ± 16 62 ± 19 P = 0.708
  Mean body weight (kg ± SD) 87 ± 28 72 ± 9.7 P = 0.145
 Data on HHT,a
  Spontaneous, recurrent epistaxis (n = 18) 12 (86%) 6 (67%)  
  Epistaxis severity score (n = 9 and 7) 3.5 (0. 9-8.4; ±2.1) 5.4 (2. 7-8.1;±2.3) P = 0.097
   ESS > 4 3 (21%) 4 (44%) P = 0.615
   ESS > 7 1 (7%) 3 (33%) P = 0.262
  Genetic study (n = 11)
   ENG mutations 2 (14%) 3 (33%)  
   ACVRL1 mutations 1 (7%) 1 (11%)  
   Negative 1 (7%) 0  
  Family history (n = 16) 10 (71%) 6 (67%)  
 Risk factors for VTE,
  Recent surgery 1 (7%) 1 (11%) 0.62 (0.03–11.28)
  Recent immobilization ≥4 days 4 (29%) 2 (22%) 1.40 (0. 20-9.87)
  Cancer 1 (7%) 0
  Prior VTE 1 (7%) 1 (11%) 0.62 (0.03–11.28)
  None of the above 6 (43%) 6 (67%) 0.38 (0.07–2. 15)
 Underlying conditions,
  Prior ischemic stroke 3 (21%) 0
  Atrial fibrillation 3 (21%) 0
  Gastroduodenal ulcer 1 (7%) 0
  Angiodisplasia in the GI tract 3 (21%) 0
  Recent (< 30 days) major bleeding 0 3 (33%)
 Laboratory data,
  Anemia 6 (43%) 5 (56%) 0.60 (0. 11-3.25)
  CrCl levels < 30 mL/min 0 0
  CrCl levels 30–60 mL/min 2 (14%) 3 (33%) 0.33 (0.04–2.56)
 sPESI,
  ≥ 1 points 7 (50%)
  1. Abbreviations: SD standard deviation, HHT hemorrhagic hereditary telangiectasia, ENG endoglin gene, ACVRL1 activin A receptor type II-like 1 gene, VTE venous thromboembolism, GI gastrointestinal, sPESI simplified version of the Pulmonary Embolism Severity Index, CrCl creatinine clearance levels; 95%CI, 95% confidence intervals
  2. a Retrospective information added, not included in the RIETE Registry