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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