{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"omics_type":["Unknown"],"volume":["43(5)"],"submitter":["Dorsey MJ"],"funding":["Leadiant Biosciences, Inc."],"pubmed_abstract":["<h4>Purpose</h4>Metabolic detoxification with enzyme replacement therapy (ERT) promotes immune recovery in patients with adenosine deaminase (ADA)-deficient severe combined immunodeficiency (ADA-SCID). Elapegademase is a PEGylated recombinant bovine ADA ERT developed to replace the now-discontinued bovine-derived pegademase. This study was a 1-way crossover from pegademase to elapegademase in 7 patients with ADA-SCID to assess efficacy and safety outcomes for elapegademase.<h4>Methods</h4>After once-weekly pegademase dosage was adjusted to achieve therapeutic metabolic detoxification and trough ADA activity, patients transitioned to a bioequivalent dose of elapegademase. Maintenance of metabolic detoxification and adequate ADA activity were evaluated periodically.<h4>Results</h4>One patien"],"journal":["Journal of clinical immunology"],"pagination":["951-964"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC10276086"],"repository":["biostudies-literature"],"pubmed_title":["PEGylated Recombinant Adenosine Deaminase Maintains Detoxification and Lymphocyte Counts in Patients with ADA-SCID."],"pmcid":["PMC10276086"],"pubmed_authors":["Fausnight T","Wiley JM","Haddad E","Dorsey MJ","Lehman H","Rubinstein A"],"additional_accession":[]},"is_claimable":false,"name":"PEGylated Recombinant Adenosine Deaminase Maintains Detoxification and Lymphocyte Counts in Patients with ADA-SCID.","description":"<h4>Purpose</h4>Metabolic detoxification with enzyme replacement therapy (ERT) promotes immune recovery in patients with adenosine deaminase (ADA)-deficient severe combined immunodeficiency (ADA-SCID). Elapegademase is a PEGylated recombinant bovine ADA ERT developed to replace the now-discontinued bovine-derived pegademase. This study was a 1-way crossover from pegademase to elapegademase in 7 patients with ADA-SCID to assess efficacy and safety outcomes for elapegademase.<h4>Methods</h4>After once-weekly pegademase dosage was adjusted to achieve therapeutic metabolic detoxification and trough ADA activity, patients transitioned to a bioequivalent dose of elapegademase. Maintenance of metabolic detoxification and adequate ADA activity were evaluated periodically.<h4>Results</h4>One patien","dates":{"release":"2023-01-01T00:00:00Z","publication":"2023 Jul","modification":"2025-04-22T02:05:57.301Z","creation":"2025-04-05T20:13:42.109Z"},"accession":"S-EPMC10276086","cross_references":{"pubmed":["36840835"],"doi":["10.1007/s10875-022-01426-y"]}}