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Biochemical pathway of purine metabolism. AMP = adenosine monophosphate, APRT = adenine phosphoribosyltransferase, GMP = guanosine monophosphate, HGPRT = hypoxanthine-guanine phosphoribosyltransferase, IMP = inosine monophosphate, NP = nucleoside phosphorylase, PPriboseP = 5-phosphorylribose-1-pyrophosphate.

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Table 1. Immunologic Studies and Findings in Adenosine Deaminase Deficiency

Study	Infantile Onset	Late Onset	Adult Onset
Lymphopenia	Markedly decreased	Decreased	Decreased
CD3⁺ cells	Absent or trace	Markedly reduced	Markedly reduced
CD4/CD8 ratio	Too few to test	< 1	< 1
Phytohemagglutinin response	Absent	Reduced	Reduced
Antigen response	Absent	Trace	Trace
Mixed lymphocyte culture response	Reduced	...	...
Ig response	Absent	Low to absent	Normal (low IgG2)
IgE	Low	Elevated	Elevated
Antibody response	Absent	Absent to low	Low to polysaccharides antigens
Eosinophilia	Rare	Common	Common
Infections	Predominantly viral, fungal, opportunistic, bacterial	Bacterial sinopulmonary	Bacterial sinopulmonary, varicella-zoster, herpes simplex, candidal

Table 2. Intravenous Immunoglobulin ^{[39, 40, 41]}

Brand (Manufacturer)	Manufacturing Process	pH	Additives*	Parenteral Form and Final Concentration	IgA Content (mcg/mL)
Carimune NF (CSL Behring)	Kistler-Nitschmann fractionation; pH 4, nanofiltration	6.4-6.8	6% solution: 10% sucrose < 20 mg NaCl/g protein	Lyophilized powder 3%, 6%, 9%, 12%	Trace
Flebogamma (Grifols USA)	Cohn-Oncley fractionation, polyethyline glycol (PEG) precipitation, ion-exchange chromatography, pasteurization	5.1-6	Sucrose-free, contains 5% D-sorbitol	Liquid 5%	< 50
Gamunex (Talecris Biotherapeutics)	Cohn-Oncley fractionation, caprylate-chromatography purification, cloth and depth filtration, low pH incubation	4-4.5	Contains no sugar, contains glycine	Liquid 10%	46
Iveegam EN (Baxter Bioscience)	Cohn-Oncley fraction II/III; ultrafiltration; pasteurization	6.4-7.2	5% solution: 5% glucose, 0.3% NaCl	Lyophilized powder 5%	< 10
Gammagard S/D, Polygam S/D (Baxter Bioscience for the American Red Cross)	Cohn-Oncley cold ethanol fractionation, cation and anion exchange chromatography, solvent detergent treated, nanofiltration, low pH incubation	6.4-7.2	5% solution: 0.3% albumin, 2.25% glycine, 2% glucose	Lyophylized powder 5%, 10%	< 1.6 (5% solution)
Gammagard Liquid 10% (Baxter Bioscience)	Cohn-Oncley cold ethanol fractionation, cation and anion exchange chromatography, solvent detergent treated, nanofiltration, low pH incubation	4.6-5.1	0.25M glycine	Ready-for-use Liquid 10%	37
Octagam (Octapharma USA)	Cohn-Oncley fraction II/III; ultrafiltration; low pH incubation; S/D treatment pasteurization	5.1-6	10% maltose	Liquid 5%	200
Panglobulin (Swiss Red Cross for the American Red Cross)	Kistler-Nitschmann fractionation; pH 4, trace pepsin, nanofiltration	6.6	Per gram of IgG: 1.67 g sucrose, < 20 mg NaCl	Lyophilized powder 3%, 6%, 9%, 12%	720
Privigen Liquid 10% (CSL Behring)	Cold ethanol fractionation, octanoic acid fractionation, and anion exchange chromatography; pH 4 incubation and depth filtration	4.6-5	L-proline (~250 mmol/L) as stabilizer; trace sodium; does not contain carbohydrate stabilizers	Ready-for use liquid 10%	< 25
*IVIG products containing sucrose are more often associated with renal dysfunction, acute renal failure, and osmotic nephrosis, particularly with preexisting risk factors (eg, history of renal insufficiency, diabetes mellitus, age >65 y, dehydration, sepsis, paraproteinemia, nephrotoxic drugs).

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