Table 7.

Self-reported impairment of the joints in patients with moderate hemophilia




1972

1978

1985

1992

2001
Aged 0-16 y      
    Median joint score* (range)   0 (0-10)   0 (0-6)   0 (0-14)   0 (0-8)   0 (0-7)  
    Severe joint impairment, proportion of patients (%)  5/42 (12)   2/41 (5)   1/59 (2)   —   1/46 (2)  
Aged 17-25 y      
    Median joint score* (range)   2 (0-9)   1 (0-7)   1 (0-8)   1 (1-5)   1 (0-10)  
    Severe joint impairment, proportion of patients (%)   1/14 (7)   —   1/22 (5)   —   1/23 (4)  
Aged 25-40 y      
    Median joint score* (range)   5 (0-27)   7 (0-17)   3 (0-20)   3 (0-12)   4 (0-16)  
    Severe joint impairment, proportion of patients (%)   2/18 (11)   6/24 (25)   10/58 (17)   8/45 (18)   6/35 (17)  
Older than 40 y of age      
    Median joint score* (range)   4 (0-10)   3 (0-15)   5 (0-24)   6 (0-24)   5 (0-44)  
    Severe joint impairment, proportion of patients (%)   3/9 (33)   3/15 (20)   6/36 (17)   16/53 (30)   23/72 (32)  
Overall      
    Median joint score* (range)   1 (0-27)   1 (0-17)   1 (0-24)   2 (0-24)   2 (0-44)  
    Severe joint impairment, proportion of patients (%)
 
11/83 (13)
 
11/95 (10)
 
18/157 (10)
 
24/173 (14)
 
31/176
 



1972

1978

1985

1992

2001
Aged 0-16 y      
    Median joint score* (range)   0 (0-10)   0 (0-6)   0 (0-14)   0 (0-8)   0 (0-7)  
    Severe joint impairment, proportion of patients (%)  5/42 (12)   2/41 (5)   1/59 (2)   —   1/46 (2)  
Aged 17-25 y      
    Median joint score* (range)   2 (0-9)   1 (0-7)   1 (0-8)   1 (1-5)   1 (0-10)  
    Severe joint impairment, proportion of patients (%)   1/14 (7)   —   1/22 (5)   —   1/23 (4)  
Aged 25-40 y      
    Median joint score* (range)   5 (0-27)   7 (0-17)   3 (0-20)   3 (0-12)   4 (0-16)  
    Severe joint impairment, proportion of patients (%)   2/18 (11)   6/24 (25)   10/58 (17)   8/45 (18)   6/35 (17)  
Older than 40 y of age      
    Median joint score* (range)   4 (0-10)   3 (0-15)   5 (0-24)   6 (0-24)   5 (0-44)  
    Severe joint impairment, proportion of patients (%)   3/9 (33)   3/15 (20)   6/36 (17)   16/53 (30)   23/72 (32)  
Overall      
    Median joint score* (range)   1 (0-27)   1 (0-17)   1 (0-24)   2 (0-24)   2 (0-44)  
    Severe joint impairment, proportion of patients (%)
 
11/83 (13)
 
11/95 (10)
 
18/157 (10)
 
24/173 (14)
 
31/176
 

Median joint score over 16 joints (min = 0, max = 48) and severe joint impairment in the left and right ankle, elbow and knee joints.

— indicates not present.

*

The median joint score was calculated as the median over the sum of the scores of 16 joints, which have been scored as follows: 0 indicates no impairment; 1, some impairment; 2, some impairment with daily problems; and 3, severe impairment with total loss of function.

Severe impairment with total loss of function reported in one or more of the six main joints.

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