Table 1.

Serum immunoglobulin and IgG subclass levels in ICOS deficiency




Age at testing, y

IgG, g/L

IgG1, mg/dL

IgG2, mg/dL

IgG3, mg/dL

IgG4, mg/dL

IgA, g/L

IgM, g/L

IgE, IU/mL
Family A          
    Patient 1   29   < 1.9*  ND   ND   ND   ND   < 0.3*  < 0.2*  < 28  
    Patient 2   21   4.4*  ND   ND   ND   ND   0.4*  < 0.2*  < 17.5  
Family B          
    Patient 3   35   2.49*  118*  71.7*  21.2*  7*  0.46*  0.26*  < 17.5  
    Patient 4   25   1.05*  78.2*  10.4*  38.7   < 6*  < 0.06*  0.31*  < 17.5  
Family C          
    Patient 5   46   1.01*  < 2.8*  66.9*  44.9   < 0.72*  < 0.06*  0.38*  < 17.5  
    Patient 6   42   0.57*  ND   ND   ND   ND   < 0.06*  1.8   < 19.1  
Family D          
    Patient 7   15   2.55*  181*  36*  38   0*  < 0.25*  0.26*  < 30  
    Patient 8   4   2.13*  118*  10*  11*  0*  0.58*  0.57   ND  
    Patient 9   3   0.54*  50*  20*  4*  0*  0.2*  0.43   < 17.5  
Normal range
 

 
7.0-16
 
360-940
 
170-550
 
30-120
 
6-120
 
0.7-4
 
0.4-2.3
 
< 100
 



Age at testing, y

IgG, g/L

IgG1, mg/dL

IgG2, mg/dL

IgG3, mg/dL

IgG4, mg/dL

IgA, g/L

IgM, g/L

IgE, IU/mL
Family A          
    Patient 1   29   < 1.9*  ND   ND   ND   ND   < 0.3*  < 0.2*  < 28  
    Patient 2   21   4.4*  ND   ND   ND   ND   0.4*  < 0.2*  < 17.5  
Family B          
    Patient 3   35   2.49*  118*  71.7*  21.2*  7*  0.46*  0.26*  < 17.5  
    Patient 4   25   1.05*  78.2*  10.4*  38.7   < 6*  < 0.06*  0.31*  < 17.5  
Family C          
    Patient 5   46   1.01*  < 2.8*  66.9*  44.9   < 0.72*  < 0.06*  0.38*  < 17.5  
    Patient 6   42   0.57*  ND   ND   ND   ND   < 0.06*  1.8   < 19.1  
Family D          
    Patient 7   15   2.55*  181*  36*  38   0*  < 0.25*  0.26*  < 30  
    Patient 8   4   2.13*  118*  10*  11*  0*  0.58*  0.57   ND  
    Patient 9   3   0.54*  50*  20*  4*  0*  0.2*  0.43   < 17.5  
Normal range
 

 
7.0-16
 
360-940
 
170-550
 
30-120
 
6-120
 
0.7-4
 
0.4-2.3
 
< 100
 

ND indicates not determined; —, not applicable.

*

Pathologic value.

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