An Open Label, Multicenter Study to Evaluate the Pharmacokinetics, Efficacy and Safety of ASCENIV™ (IGIV) in Pediatric Subjects With Primary Immunodeficiency Diseases (PIDD)

Inclusion Criteria:

1. Subject and/or legal guardian must be able to understand the study procedures, haveagreed to participate in the study and have voluntarily signed an IEC/IRB approvedwritten informed consent. The consent form or a specific assent form, where required,will be signed and dated by minors.
2. Have confirmed and documented clinical diagnosis of primary immunodeficiency diseaseincluding but not limited to: common variable immunodeficiency, X-linked and autosomalforms of agammaglobulinemia, hyper-IgM syndrome, or antibody deficiencies.
3. Be male or female, and ≥ 2 years and < 12 years at the time of informed consent bysubject or legal guardian.
4. Have been receiving IGIV at a dose that has not been changed by > 25% of the mean doseon a mg/kg basis for at least 3 months prior to study entry.
5. Have two trough levels of IgG in the last year (screening level may be used), andmaintained a trough serum IgG level > 500 mg/dL on the previous 2 assessments prior toreceiving Asceniv™. (The trough level must be at least 300 mg/dL above pre-treatmentserum IgG levels; with exception for cases of X-linked agammaglobulinemia where nopre-treatment value is available. Documentation will need to include dose, treatmentinterval and trade name of the IGIV products used for the three doses prior to thefirst Asceniv™ infusion in this study.
6. For female subjects, be of non-childbearing potential or have a negative pregnancytest prior to study start and be deemed not at risk of becoming pregnant by adherenceto a reliable contraceptive method for the duration of the study. Females ofnon-childbearing potential are defined as prepubertal girls.

Exclusion Criteria:

1. Have a known hypersensitivity to immunoglobulin or any excipient in Asceniv™.
2. Have a history of any severe anaphylactic or anaphylactoid reaction to blood or anyblood-derived product.
3. Have a specific Immunoglobulin A (IgA) deficiency (IgA ≤ 5 mg/dL and normal IgG andIgM), history of allergic reaction to products containing IgA or has demonstrableantibodies to IgA.
4. Have uncompensated, hemodynamically significant, congenital or other heart disease.Including but not limited to acute coronary syndromes and chronic stable angina.
5. Have a medical condition that is known to cause secondary immune deficiency, such aschronic lymphocytic leukemia, lymphoma, multiple myeloma, or HIV infection.
6. Have a significant T-cell or granulocyte deficiency in number or function (chronic orrecurrent absolute neutrophil count <1000 x 109/L).
7. Have significant renal impairment (defined as an estimated Glomerular Filtration Rate ≤ 50 mL/min/1.73m2); or have a history of acute renal failure.
8. Have abnormal liver function, defined as ALT or AST ≥ 2.5 x ULN.
9. Have any chronic lung disease (uncontrolled or chronic, severe asthma, etc.)
10. Have an infusion port, catheter, or other foreign body present (excluding PE tubes).Long-standing, infection-free ports may be permitted at the discretion of the MedicalMonitor.
11. Be planned or scheduled to undergo surgery during the course of study participation.
12. Have ongoing failure to thrive per PI assessment.
13. Be receiving chronic anti-coagulation therapy.
14. Have a history of DVT, thrombotic or thrombo-embolic event, or are at increased riskfor thrombotic event due to presence of, but not limited to, atrial fibrillation,disease or injury requiring prolonged immobilization, or other risk factor(s)including significant proteinuria or protein losing enteropathy.
15. Current daily use of the following medications:

• corticosteroids (> 0.15 mg/kg/day of prednisone equivalent) Note: Intermittentcorticosteroid use during the study is allowable, if medically necessary andapproved by the ADMA Medical Director: i.e. 1 mg/kg twice a day for ten days to amaximum of 40 mg per dose
• immunomodulatory drugs (e.g. TNF (inhibitors -Enbrel, Humira, etc.) Xolair andDupixent administration permitted.
• immunosuppressive drugs (excluding topical pimecrolimus (Elidel) and tacrolimus (Protopic))

16. Administration of a hyperimmune or specialty high titer Immunoglobulin product (e.g.Cytogam, VZIG, HBIG, etc.) within 30 days of screening, or expectation that ahyperimmune Immunoglobulin product will be given during the course of the study.
17. Have uncontrollable arterial hypertension.
18. Have anemia at screening (hemoglobin <10 g/dL).
19. Have a history of hemolysis while undergoing treatment with IGIV therapy.
20. Be morbidly obese as indicated by a Body Mass Index (BMI) ≥40.
21. Have an active viral or bacterial infection or symptoms/signs consistent with such aninfection, within the two weeks prior to the Screening Visit. Subjects may bereceiving antibiotics as long as signs/symptoms of infection have been absent for twoweeks prior to the initial infusion of investigational product (IP).
22. Have received any blood product (other than Immunoglobulin G) within 3 months prior toscreening.
23. Have received any RSV specific products, including palivizumab (Synagis®) within 3months prior to screening.
24. Have abused alcohol, opiates, psychotropic agents, or other chemicals or drugs withinthe past 12 months.
25. Have an acute or chronic medical condition that, in the opinion of the investigator,may interfere with the conduct of the study.
26. Have any condition judged by the study physician to preclude participation in thestudy, including any psychological disorder, which might hinder compliance.
27. Have any laboratory assessment result that, in the opinion of the investigator,warrants exclusion from participation in the study.
28. Are currently pregnant or nursing.
29. Have acute hepatitis A, acute or chronic Hepatitis B or C, or HIV infection.
30. Have received investigational product within 3 weeks of the anticipated first infusionof Asceniv™.