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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.