TABLE 1.

Metabolic and immunological parameters before and after PEG-ADA treatment

Parameter	Controls	Patient at the following times (wk)
		0	13-18	23-28
Metabolic parameters
ADA activity (nmol/h/mg)	40.1a	1.07	101	1.3
% dAXP	0	12.3	0	15.8
Lymphocyte subsets (no. of cells/μl [%])
T (CD4)	1,000-4,600 (31-54)b	150 (19)	512 (32)	192 (24)
T (CD8)	400-2,100 (12-28)	464 (58)	720 (45)	448 (56)
B (CD20)	600-2,700 (15-39)	16 (2)	144 (9)	10 (1.2)
NK (CD56+/CD3−)	200-1,200 (3-17)	55 (7)	192 (12)	40 (5)
T-cell subsets (% of total CD4 or CD8 counts)
CD4/CD45RA		16	50	17
CD4/CD45RO		100	69	100
CD4/CD28	<100c	100	100	88
CD8/CD28	50-68	10	20	18
CD4/CD95	7-20	100	100	100
CD8/CD95	12-16	100	82	100
Apoptosis (%)
Annexin V CD4	8d	59	32	59
Annexin V CD8	11	82	83	96
Cytokine concn (pg/ml)
IL-2	<3a	26	3	24
IL-4	<3	81	6	51
IL-5	<3	46	10	57
IL-10	<10	168	38	284
IFN-γ	<10	1,430	493	1,959
TNF-α	<10	210	17	221
IL-2 concn secretion upon stimulation (pg/ml)
PMA-ionomycine	5,000a	140	2824	27
PHA	190	2	20	20
Lymphocyte proliferation (cpm)
PHA (medium)	>90,000a (>500)	10,000 (1,000)	69,000 (400)	2,300 (100)
Anti-CD3	>50,000	18,000	53,000	300

^aFor the measurements of metabolic parameters, plasma cytokines, cytokines secretion, and lymphocyte proliferation, we used healthy adults as a daily control.

^bValues for healthy controls were obtained from reference 10; 70 samples from a group of 9- to 15-month-old healthy children were analyzed.

^cValues for healthy controls were obtained from reference 21; samples from 20 healthy children (age, 5.3 ± 2.7 years) were analyzed.

^dValues for healthy controls were obtained from reference 20; samples from 10 healthy children (age, 3.5 ± 2.9 years) were investigated.