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Carefully monitoring the cardiovascular status, we administered monthly single plasma volume exchanges for a 3 to 8 mo period. The results indicate a substantial gain of enzyme activity, persisting with a t<jats:sub>50%<\/jats:sub> = 19 \u00b1 5 hr. The maximal level and persistence of increased enzyme activity did not change after repeated exchanges, suggesting that immune responses were not elicited. Despite this, no demonstrable clinical benefit was apparent when the study group was compared with an age\u2010matched control group of Hunter syndrome patients.<\/jats:p>","DOI":"10.1002\/ajmg.1320130314","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T13:22:16Z","timestamp":1118496136000},"page":"309-318","source":"Crossref","is-referenced-by-count":8,"title":["Administration of iduronate sulfatase by plasma exchange to patients with the hunter syndrome: A clinical study"],"prefix":"10.1002","volume":"13","author":[{"suffix":"III","given":"Frank R.","family":"Brown","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Clara W.","family":"Hall","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elizabeth F.","family":"Neufeld","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Louis L.","family":"Munoz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hayden","family":"Braine","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stanley","family":"Andrzejewski","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Edwaldo E.","family":"Camargo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shirley A.","family":"Mark","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James M.","family":"Richard","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hugo W.","family":"Moser","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J\u00fcrgen","family":"Herrmann","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,6,2]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.70.7.2134"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1973.tb08067.x"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(73)80166-0"},{"key":"e_1_2_1_5_1","first-page":"519","article-title":"Enzyme replacement by plasma exchange in Hunter syndrome","volume":"14","author":"Brown FR","year":"1980","journal-title":"Pediatr Res"},{"key":"e_1_2_1_6_1","unstructured":"CarpD BrownFRIII Growth in the Hunter syndrome.Am J Dis Child in press."},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.1973.04160040045009"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/257609a0"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI109267"},{"key":"e_1_2_1_10_1","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1542\/peds.50.5.688","article-title":"Effects of fresh plasma or whole blood transfusions on patients with various types of mucopolysaccharidosis","volume":"50","author":"Dekaban AS","year":"1972","journal-title":"Pediatrics"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.68.2.303"},{"key":"e_1_2_1_12_1","doi-asserted-by":"crossref","first-page":"693","DOI":"10.1542\/peds.50.5.693","article-title":"Inefficacy of fresh frozen plasma therapy of mucopolysaccharidosis II","volume":"50","author":"Erickson RP","year":"1972","journal-title":"Pediatrics"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/0076-6879(78)50048-7"},{"issue":"12","key":"e_1_2_1_14_1","first-page":"212","article-title":"Treatment of mucopolysaccharidoses","volume":"10","author":"Hussels IE","year":"1975","journal-title":"Birth Defects"},{"key":"e_1_2_1_15_1","first-page":"1002","article-title":"Widespread abnormalities of radiocolloid distribution in patients with mucopolysaccharidoses","volume":"16","author":"Klingensmith WC","year":"1975","journal-title":"J Nucl Med"},{"key":"e_1_2_1_16_1","first-page":"227","volume-title":"Hereditary Retinal and Choroidal Diseases","author":"Krill AE","year":"1971"},{"key":"e_1_2_1_17_1","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1203\/00006450-197608000-00007","article-title":"Iduronate sulfatase activity in serum, lymphocytes, and fibroblasts, simplified diagnosis of the Hunter syndrome","volume":"10","author":"Liebaers I","year":"1976","journal-title":"Pediatr Res"},{"key":"e_1_2_1_18_1","series-title":"Publication no. 2015","volume-title":"Minimal Brain Dysfunction in Children","author":"Mark HJ","year":"1969"},{"key":"e_1_2_1_19_1","first-page":"1282","volume-title":"The Metabolic Basis of Inherited Disease","author":"McKusick VA","year":"1978"},{"key":"e_1_2_1_20_1","unstructured":"McKusickVA NeufeldEF.The mucopolysaccharide storage diseases. In Stanbury JB Wyngaarden JB Fredrickson DF Goldstein JL Brown MS (eds): \u201cThe Metabolic Basis of Inherited Disease.\u201d 5th Ed in press."},{"key":"e_1_2_1_21_1","first-page":"525","article-title":"Hunter syndrome. Enzyme replacement by repeated plasmaphoreses","volume":"14","author":"Murphy JV","year":"1980","journal-title":"Pediatr Res"},{"key":"e_1_2_1_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4684-1006-8_6"},{"key":"e_1_2_1_23_1","doi-asserted-by":"publisher","DOI":"10.1620\/tjem.114.385"},{"key":"e_1_2_1_24_1","doi-asserted-by":"publisher","DOI":"10.1901\/jeab.1974.22-261"},{"key":"e_1_2_1_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0001-2998(80)80032-8"},{"key":"e_1_2_1_26_1","first-page":"19","article-title":"Complications of intensive plasma exchange","volume":"2","author":"Sutton DMC","year":"1981","journal-title":"Plasma Therapy and Transfusion Technology"},{"key":"e_1_2_1_27_1","unstructured":"WastesonA NeufeldEFIduronate sulfatase of human plasma. Methods Enzymol in press."},{"key":"e_1_2_1_28_1","first-page":"294","article-title":"Lung uptake of colloid during liver\u2010spleen scanning: a normal finding in children","volume":"15","author":"Witner PF","year":"1976","journal-title":"Nucl Med (Stuttg)"},{"key":"e_1_2_1_29_1","first-page":"802","article-title":"A therapeutic trial of fresh plasma infusions over a period of 22 mo in two sibs with Hunter Syndrome","volume":"11","author":"Yatziv S","year":"1975","journal-title":"Isr J Med Sci"}],"container-title":["American Journal of Medical Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajmg.1320130314","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.1320130314","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,13]],"date-time":"2023-11-13T00:04:31Z","timestamp":1699833871000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajmg.1320130314"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1982,11]]},"references-count":28,"journal-issue":{"issue":"3","published-print":{"date-parts":[[1982,11]]}},"alternative-id":["10.1002\/ajmg.1320130314"],"URL":"https:\/\/doi.org\/10.1002\/ajmg.1320130314","archive":["Portico"],"relation":{},"ISSN":["0148-7299","1096-8628"],"issn-type":[{"value":"0148-7299","type":"print"},{"value":"1096-8628","type":"electronic"}],"subject":[],"published":{"date-parts":[[1982,11]]}}}