Table 1.

Demographics, Characteristics, and Diagnosis for 88 PIDD Infants and Children at the Start of Hizentra®

Patient characteristic	34 Patients, Aged 0 to <2 Years, No. (%)	54 Patients, Aged 2 to 5 Years, No. (%)	88 Patients Total, No. (%)
Gender, n (%)
Male	23 (26)	30 (34)	53 (60)
Female	11 (13)	24 (27)	35 (40)
Race, n (%)
White	29 (33)	41 (46)	70 (80)
Black or African American	4 (5)	4 (5)	8 (9)
Asian	1 (1)	3 (3)	4 (4)
Other	0 (0)	6 (7)	6 (7)
Age and Growth Parameters
Age, months, mean (range)	11 (2–23)	44 (25–59)	34 (2–59)
Height, cm, mean (range)	70.0 (50.6–91)	94.5 (76.3–116.5)	84.3 (50.6–116.5)
Weight, kg, mean (range)	8.7 (4.1–15.5)	15.0 (9.3–28.4)	12.5 (4.1–28.4)
Diagnosis, n (%)
T-cell and combined immunodeficiencies
Severe combined immunodeficiency	6 (6.8)	8 (9)	14 (16)
Hyperimmunoglobulin M syndrome	0 (0)	1 (1)	1 (1)
22q11 deletion	0 (0)	5 (5.7)	5 (5.7)
Ataxia-telangiectasia	0 (0)	2 (2)	2 (2)
Wiscott-Aldrich syndrome	1 (1)	0 (0)	1 (1)
Combined immunodeficiency, unspecified	3 (3.4)	0 (0)	3 (3.4)
Total	10 (11.4)	16 (18.2)	26 (29.6)
Predominantly antibody deficiencies
Common variable immunodeficiency	0 (0)	5* (6)	5* (6)
X-linked agammaglobulinemia	7 (8)	4 (4.5)	11 (12.5)
Immunoglobulin G subclass deficiency	0 (0)	2 (2)	2 (2)
Autosomal recessive agammaglobulinemia	0 (0)	1 (1)	1 (1)
Total	7 (8)	12 (14)	19 (22)
Disorders of innate immunity
NF-κB essential modulator (NEMO)	2 (2)	1 (1)	3 (3)
Total	2 (2)	1 (1)	3 (3)
Other
Hypogammaglobulinemia with specific antibody deficiency	1 (1)	7 (8)	8 (9)
Isolated Hypogammaglobulinemia with repeated infections	11 (12.5)	14 (16)	25 (28.4)
Transient hypogammaglobulinemia of infancy	2 (2)	2 (2)	4 (4.5)
Hyperimmunoglobulin E syndrome (STAT3)	0 (0)	2 (2)	2 (2)
Netherton syndrome	1 (1)	0 (0)	1 (1)
Total	15 (17)	25 (28)	40 (45)