Table 1.

Clinical and laboratory characteristics of the patients investigated at the molecular level

Total patients fully characterizedTotal patients no or only partially characterizedPEU patients fully characterizedEU patients no or only partially characterizedPIR patients fully characterizedIR patients no or only partially characterizedP
207 24  105 16  102  
Sex          
 Male 87 (42%) 8 (33%)  37 (35.2%) 6 (37.5%)  50 (49%) 2 (25%)  
 Female 120 (58%) 16 (67%)  68 (64.8%) 10 (62.5%)  52 (51%) 6 (75%)  
Age at study inclusion, y 28 (16-43) 38 (20-51) .08 40 (25-49) 42 (33-54) .40 22 (8-30) 21 (14-34) .39 
Bleeding score 15 (8-21) 11 (7-18) .30 18 (14-25) 15 (11-19) .07 10 (4-16) 8 (5-10) .42 
N* 157 20  69 12  88  
VWF:Ag* 0.5 (0.5-0.5) 2.2 (0.8-3.9) <.001 0.5 (0.5-1.3) 3.9 (0.8-4.6) .003 0.5 (0.5-0.5) 1.8 (1.1-2.3) <.001 
FVIII:C* 2.4 (1.9-3.2) 2.7 (1.8-8.4) .29 2.7 (2.2-3.7) 5.5 (2.5-16.9) .07 2.2 (1.7-2.6) 1.8 (1.6-2.3) .42 
Total patients fully characterizedTotal patients no or only partially characterizedPEU patients fully characterizedEU patients no or only partially characterizedPIR patients fully characterizedIR patients no or only partially characterizedP
207 24  105 16  102  
Sex          
 Male 87 (42%) 8 (33%)  37 (35.2%) 6 (37.5%)  50 (49%) 2 (25%)  
 Female 120 (58%) 16 (67%)  68 (64.8%) 10 (62.5%)  52 (51%) 6 (75%)  
Age at study inclusion, y 28 (16-43) 38 (20-51) .08 40 (25-49) 42 (33-54) .40 22 (8-30) 21 (14-34) .39 
Bleeding score 15 (8-21) 11 (7-18) .30 18 (14-25) 15 (11-19) .07 10 (4-16) 8 (5-10) .42 
N* 157 20  69 12  88  
VWF:Ag* 0.5 (0.5-0.5) 2.2 (0.8-3.9) <.001 0.5 (0.5-1.3) 3.9 (0.8-4.6) .003 0.5 (0.5-0.5) 1.8 (1.1-2.3) <.001 
FVIII:C* 2.4 (1.9-3.2) 2.7 (1.8-8.4) .29 2.7 (2.2-3.7) 5.5 (2.5-16.9) .07 2.2 (1.7-2.6) 1.8 (1.6-2.3) .42 

For quantitative variables, median and interquartile range are reported; descriptive variables are reported as numbers with percentages. Mann-Whitney U test was used to evaluate the group comparisons.

*

Includes only the patients without prophylaxis.

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