Table 1

Baseline characteristics of patients

Characteristic
Sex, n (%)  
    Female 7 (58) 
    Male 5 (42) 
Age, y  
    Mean 35.0 
    SD 14.3 
Weight, kg  
    Mean 70.2 
    SD 13.2 
Platelet count by cell counter, ×109/L  
    Mean 16.5 
    SD 10.5 
Platelet count by microscopy, ×109/L  
    Mean 31.2 
    SD 14.8 
Mean platelet volume, fL  
    Mean 15.1 
    SD 4.3 
Mean platelet diameter,* μm  
    Mean 5.1 
    SD 1.1 
WHO bleeding scale, n (%)  
    Grade 0 2 (17) 
    Grade 1 4 (33) 
    Grade 2 6 (50) 
Serum thrombopoietin level,pg/mL  
    Median 43.7 
    IQR 7.3-78.2 
Mean time since previous splenectomy, n (%)  
    Yes 4 (33) [26.2 y] 
    No 8 (67) 
Proteinuric nephropathy,n (%)  
    Yes 3 (25) 
    No 9 (75) 
Sensorineural hearing loss,§n (%)  
    Yes 9 (75) 
    No 3 (25) 
Cataract, n (%)  
    Yes 1 (8) 
    No 11 (92) 
Position of MYH9 mutation, n (%)  
    Motor domain 4 (33) 
    Tail domain 8 (67) 
Characteristic
Sex, n (%)  
    Female 7 (58) 
    Male 5 (42) 
Age, y  
    Mean 35.0 
    SD 14.3 
Weight, kg  
    Mean 70.2 
    SD 13.2 
Platelet count by cell counter, ×109/L  
    Mean 16.5 
    SD 10.5 
Platelet count by microscopy, ×109/L  
    Mean 31.2 
    SD 14.8 
Mean platelet volume, fL  
    Mean 15.1 
    SD 4.3 
Mean platelet diameter,* μm  
    Mean 5.1 
    SD 1.1 
WHO bleeding scale, n (%)  
    Grade 0 2 (17) 
    Grade 1 4 (33) 
    Grade 2 6 (50) 
Serum thrombopoietin level,pg/mL  
    Median 43.7 
    IQR 7.3-78.2 
Mean time since previous splenectomy, n (%)  
    Yes 4 (33) [26.2 y] 
    No 8 (67) 
Proteinuric nephropathy,n (%)  
    Yes 3 (25) 
    No 9 (75) 
Sensorineural hearing loss,§n (%)  
    Yes 9 (75) 
    No 3 (25) 
Cataract, n (%)  
    Yes 1 (8) 
    No 11 (92) 
Position of MYH9 mutation, n (%)  
    Motor domain 4 (33) 
    Tail domain 8 (67) 

SD indicates standard deviation; and IQR, interquartile range.

*

Normal value calculated in 40 healthy subjects was 2.6 μm (SD 0.32).

Value determined in 40 healthy controls was 14.6 pg/mL (IQR 7.0-23.2).

Defined by 24 hours of proteinuria of 0.5 g or more, in at least 2 consecutive examinations, in the absence of any other possible cause of proteinuria.

§

As determined by audiometric examination.

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