{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,5]],"date-time":"2026-05-05T09:55:00Z","timestamp":1777974900971,"version":"3.51.4"},"reference-count":28,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2007,7,7]],"date-time":"2007-07-07T00:00:00Z","timestamp":1183766400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2007,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Adenosine deaminase (ADA) deficiency is an inherited disorder which leads to elevated cellular levels of deoxyadenosine triphosphate (dATP) and systemic accumulation of its precursor, 2\u2010deoxyadenosine. These metabolites impair lymphocyte function, and inactivate S\u2010adenosylhomocysteine hydrolase (SAHH) respectively, leading to severe immunodeficiency. Enzyme replacement therapy with polyethylene glycol\u2010conjugated ADA is available, but its efficacy is reduced by anti\u2010ADA neutralising antibody formation. We report here carrier erythrocyte encapsulated native ADA therapy in an adult\u2010type ADA deficient patient. Encapsulated enzyme is protected from antigenic responses and therapeutic activities are sustained. ADA\u2010loaded autologous carrier erythrocytes were prepared using a hypo\u2010osmotic dialysis procedure. Over a 9\u2010yr period 225 treatment cycles were administered at 2\u20133\u2003weekly intervals. Therapeutic efficacy was determined by monitoring immunological and metabolic parameters. After 9\u2003yr of therapy, erythrocyte dATP concentration ranged between 24 and 44\u2003\u03bcmol\/L (diagnosis, 234) and SAHH activity between 1.69 and 2.29\u2003nmol\/h\/mg haemoglobin (diagnosis, 0.34). Erythrocyte ADA activities were above the reference range of 40\u2013100\u2003nmol\/h\/mg haemoglobin (0 at diagnosis). Initial increases in absolute lymphocyte counts were not sustained; however, despite subnormal circulating CD20<jats:sup>+<\/jats:sup> cell numbers, serum immunoglobulin levels were normal. The patient tolerated the treatment well. The frequency of respiratory problems was reduced and the decline in the forced expiratory volume in 1\u2003s and vital capacity reduced compared with the 4\u2003yr preceding carrier erythrocyte therapy. Carrier erythrocyte\u2010ADA therapy in an adult patient with ADA deficiency was shown to be metabolically and clinically effective.<\/jats:p>","DOI":"10.1111\/j.1600-0609.2007.00927.x","type":"journal-article","created":{"date-parts":[[2007,8,7]],"date-time":"2007-08-07T02:07:46Z","timestamp":1186452466000},"page":"338-348","source":"Crossref","is-referenced-by-count":43,"title":["A 9\u2010yr evaluation of carrier erythrocyte encapsulated adenosine deaminase (ADA) therapy in a patient with adult\u2010type ADA deficiency"],"prefix":"10.1111","volume":"79","author":[{"given":"Bridget E.","family":"Bax","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Murray D.","family":"Bain","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lynette D.","family":"Fairbanks","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A David B.","family":"Webster","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philip W.","family":"Ind","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael S.","family":"Hershfield","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ronald A.","family":"Chalmers","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2007,7,7]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0090-1229(95)90288-0"},{"key":"e_1_2_6_3_2","first-page":"480","volume-title":"Immunologic Disorders in Infants and Children, 5th ed","author":"Hershfield MS","year":"2004"},{"key":"e_1_2_6_4_2","doi-asserted-by":"crossref","first-page":"S35","DOI":"10.1203\/00006450-199333011-00008","article-title":"Overview of biochemical abnormalities 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