<HashMap><database>biostudies-literature</database><scores/><additional><omics_type>Unknown</omics_type><volume>43(5)</volume><submitter>Dorsey MJ</submitter><funding>Leadiant Biosciences, Inc.</funding><pubmed_abstract>&lt;h4>Purpose&lt;/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.&lt;h4>Methods&lt;/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.&lt;h4>Results&lt;/h4>One patien</pubmed_abstract><journal>Journal of clinical immunology</journal><pagination>951-964</pagination><full_dataset_link>https://www.ebi.ac.uk/biostudies/studies/S-EPMC10276086</full_dataset_link><repository>biostudies-literature</repository><pubmed_title>PEGylated Recombinant Adenosine Deaminase Maintains Detoxification and Lymphocyte Counts in Patients with ADA-SCID.</pubmed_title><pmcid>PMC10276086</pmcid><pubmed_authors>Fausnight T</pubmed_authors><pubmed_authors>Wiley JM</pubmed_authors><pubmed_authors>Haddad E</pubmed_authors><pubmed_authors>Dorsey MJ</pubmed_authors><pubmed_authors>Lehman H</pubmed_authors><pubmed_authors>Rubinstein A</pubmed_authors></additional><is_claimable>false</is_claimable><name>PEGylated Recombinant Adenosine Deaminase Maintains Detoxification and Lymphocyte Counts in Patients with ADA-SCID.</name><description>&lt;h4>Purpose&lt;/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.&lt;h4>Methods&lt;/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.&lt;h4>Results&lt;/h4>One patien</description><dates><release>2023-01-01T00:00:00Z</release><publication>2023 Jul</publication><modification>2025-04-22T02:05:57.301Z</modification><creation>2025-04-05T20:13:42.109Z</creation></dates><accession>S-EPMC10276086</accession><cross_references><pubmed>36840835</pubmed><doi>10.1007/s10875-022-01426-y</doi></cross_references></HashMap>