study to evaluate safety and pharmacokinetics of bivigam® in primary immune deficiency subjects aged 2 to 16

Conditions: Humoral Immune Response

Summary: this study is part of the bivigam® post marketing requirement (pmr). it is being conducted in subjects aged 2-16 with primary immune deficiency disorders associated with defects in humoral immunity to generate additional data on these populations, and more specifically safety and pharmacokinetic (pk) assessments.


Eligibility criteria:
inclusion criteria: * written informed consent/assent * male or female between 2 and 16 years, inclusive, at time of signing informed consent/assent * have a confirmed and documented clinical diagnosis of primary immune deficiency disorder, including hypogammaglobulinemia or agammaglobulinemia. * have received igiv therapy which was maintained at a steady dose (± 25% of the mean dose) for at least 3 months prior to study entry, and have maintained a trough igg level at least 500mg/dl prior to receiving bivigam®. * subjects and/or parents/legal guardians must be able to understand and adhere to the study visit schedule and all other protocol requirements. exclusion criteria: * known intolerance to immunoglobulins or comparable substances (e.g. vaccination reaction). * known intolerance to proteins of human origin or known allergic reactions to components of the study product(s). * any previous randomization/participation in this clinical study must be discussed with and approved by the medical director (or designee). * inability or lacking motivation to participate in the study. * medical condition, laboratory finding, or physical exam finding (specify, e.g., vital signs outside of specific range that precludes participation. per lab results at the screening visit through baseline. * confirmed screening visit laboratory results ˃2.5 x uln as defined for pediatric populations for any of the following: alt (alanine aminotransferase/sgpt), ast (aspartate aminotransferase/sgot), ldh (lactate dehydrogenase), bun (blood urea nitrogen), serum creatinine * has selective iga deficiency or demonstrated antibodies to iga. * history of thrombotic complications of igiv therapy or history of (deep vein thrombosis)dvt. * current use of daily corticosteroids (\>10 mg of prednisone equivalent/day),immunosuppressants or immunomodulators are not allowed unless approved in advance by the medical monitor. intermittent use of corticosteroids during the study is allowed if medically necessary. * positive diagnosis of hepatitis b or hepatitis c. * positive human immunodeficiency virus (hiv) test. * subject has had a serious bacterial infection (sbi) within the last 3 months. * subject has an active infection and is receiving antibiotic therapy for the treatment of this infection at the time of screening. note: if the subject is deemed a screen failure due to a nonserious active infection requiring antibiotic therapy, the subject may be rescreened 3 or 4 weeks (depending on drug administration schedule) after the initial screening. * subject has a history of thrombotic events (including deep vein thrombosis, myocardial infarction, cerebrovascular accident and pulmonary embolism) within 6 months before 1st igiv dose or has preexisting risk factors for thrombotic events. * acquired medical condition known to cause secondary immune deficiency such as chronic lymphacitic leukemia, lymphoma or multiple lymphoma. * subjects with protein-losing enteropathies, hypoalbuminaemia. * females taking oral contraceptives. * pregnancy or unreliable contraceptive measures or lactation period (females of childbearing potential (female capable of becoming pregnant) only. males capable of reproduction must agree to a double barrier method of contraception during their study participation.