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Table 1 Comparison of immunophenotypes.

From: Cardiac conduction abnormalities and congenital immunodeficiency in a child with Kabuki syndrome: Case report

Study Patient Normal for Age
Phenotype Analysis
   Lymphocytes/mm3 1818 6000 ± 1500
   CD2, % 70 73 ± 8
   CD3, % 62 66 ± 13
   CD4, % 42 43 ± 12
   CD8, %, 19 25 ± 9
   CD45RA+CD3, % 79 64 – 93
   CD45RO+CD3, % 11  
   CD25+CD4, % 14 <3
   CD20, % 14 8 ± 3
   smIgM, % 11 4 – 16
   smIgM, % 10 3 – 15
   CD56, % 10 13 ± 7
Lymphoproliferative Responses
   PHA, cpm 164,082 100,530 – 657,376
%NR 65 >50
   Con A, cpm 10 53,173 – 502,758
%NR 0 >50
   PWM, cpm 121,168 40,305 – 337,597
%NR 96 >50
   MLC, cpm 118,219 43,801 – 328,175
SI 23.2 >3.0
Immunoglobulins
IgG, mg/dl 113 399–1068
IgA, mg/dl 11 15–95
IgM, mg/dl 97 49–202
IgE, IU/ml <2 3–29
anti-HiB, μg/ml <0.5 >1.0
anti-Diphtheria toxoid, IU/ml 1.61 >0.05
anti-Tetanus toxoid, IU/ml 0.2 >0.5
anti-Streptococcus, μg/ml
Serotype 4 4.6 >2.0
Serotype 6 2.2 >2.0
Serotype 9 0.3 >2.0
Serotype 14 1.0 >2.0
Serotype 18 12.2 >2.0
Serotype 19 0.9 >2.0
Serotype 23 0.3 >2.0
CH50, U/ml 64 31 – 64
  1. Immunological studies in our 10 month old child. PHA, phytohemagglutinin; Con A, concanavalin A; PWM, pokeweed mitogen; MLC, mixed lymphocyte culture to B-cell alloantigens; %NR, percent normal response; SI, stimulation index; HiB, Hemophilus influenzae type B.