TABLE 3.

Distribution of PIDDs by serostatus

Diagnosis	R(x): N = 70	R(+): N = 10	R(−): N = 10
Defects with poor production of specific IgG
ZAP70 mutation	2		1§
CD40 ligand deficiency	4	1‡
IKBa deficiency	1
PIK3CD mutation	1
STAT3 GOF mutation	1
IPEX syndrome	1	1‡
XLP	3		1§
SCID	41
WAS	11
Cartilage Hair Hypoplasia	2		1§
CID (not known gene defect)		1‡
Defects with intact production of specific IgG
Chediak-Higashi syndrome			1
Dyskeratosis congenita	1†
C1q deficiency	1†
HLH	1†	5	3
CGD		1	1
Congenital neutropenia			1
IL-10R deficiency		1	1

Note: Distribution of PIDDs by serostatus: PIDDs has been divided into two none: defects in production of specific IgG and capable of production of specific IgG.

Abbreviations: CGD, chronic granulomatous diseasCID, combined immunodeficiency; HLH, hemophagocytic lymphohistiocytosis; IPEX, Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome; PIDDs, primar y immunodeficiency disorders; R(−), seronegative; R(+), seropositive; R(x), unknown CMV serostatus; SCID, Severe combined Immunodeficiency; WAS, Wiskott-Aldrich syndrome; XLP, X-linked lymphoproliferative syndrome.

^†CMV IgG + and no passive immunity.

^‡CMV IgG + while on IVIg.

^§Despite the underlying defect, these patients demonstrated specific antibodies to tetanus, diphtheria, and pneumococcal vaccines.