ADAGEN®

"Adagen® (pegademase bovine) Injection is indicated for enzyme replacement therapy for adenosine deaminase (ADA) deficiency in patients with severe combined immunodeficiency disease (SCID) who are not suitable candidates for – or who have failed – bone marrow transplantation. Adagen® (pegademase bovine) Injection is recommended for use in infants from birth or in children of any age at the time of diagnosis. Adagen® (pegademase bovine) Injection is not intended as a replacement for HLA identical bone marrow transplant therapy. Adagen® (pegademase bovine) Injection is also not intended to replace continued close medical supervision and the initiation of appropriate diagnostic tests and therapy (e.g., antibiotics, nutrition, oxygen, gammaglobulin) as indicated for intercurrent illnesses"

ADA-deficient SCID is considered the most difficult form of SCID to treat.41 For patients whose immunodeficiency persists in spite of long-term treatment with Adagen®, decreased diversity of B lymphocytes, accelerated apoptosis of peripheral lymphocytes, and reduced thymic output have been suggested as mechanisms for the incomplete immune recovery.41 Evaluating the degree of thymic reserve at the time of Adagen® initiation has been proposed as a means of determining those patients who will benefit most from enzyme replacement