{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,7]],"date-time":"2026-02-07T22:48:50Z","timestamp":1770504530250,"version":"3.49.0"},"reference-count":22,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2004,10,7]],"date-time":"2004-10-07T00:00:00Z","timestamp":1097107200000},"content-version":"vor","delay-in-days":7677,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[1983,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>This study reports a case of type VII mucopolysaccharidosis (\u03b2\u2010glucuronidase deficiency) presenting as lethal hydrops fetalis. Skin fibroblast cultures established postmortem revealed deficient \u03b2\u2010glucuronidase activity. Mucopolysaccharides were stored in various cells of the brain, heart, kidney, liver, and spleen. The stages of maturation of the bones, kidneys, and brain were discrepant, the brain being the least mature organ. A delay in central nervous system maturation may account for psychomotor retardation in some patients with this enzyme deficiency.<\/jats:p>","DOI":"10.1002\/ana.410140415","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T15:24:00Z","timestamp":1104593040000},"page":"486-490","source":"Crossref","is-referenced-by-count":41,"title":["Postmortem observations on \u03b2\u2010glucuronidase deficiency presenting as hydrops fetalis"],"prefix":"10.1002","volume":"14","author":[{"given":"Dina","family":"Irani","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Han\u2010Seob","family":"Kim","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Huda","family":"El\u2010Hibri","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert V.","family":"Dutton","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arthur","family":"Beaudet","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dawna","family":"Armstrong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,10,7]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(82)80523-4"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(80)80734-7"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(75)80968-1"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4471-3304-9"},{"key":"e_1_2_1_6_2","first-page":"251","article-title":"Different clinical and biochemical phenoctpes associated with \u03b2\u2010glucuronidase deficiency","volume":"10","author":"Danes BS","year":"1974","journal-title":"Birth Defects"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-7091-3338-5"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00282212"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(73)80441-X"},{"key":"e_1_2_1_10_2","first-page":"413","article-title":"Unusually mild course of \u03b2\u2010glucuronidase deficiency in two brothers (mucopolysaccharidosis VII)","volume":"33","author":"Gitzelmann R","year":"1978","journal-title":"Helv Paediatr Acta"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.128.6.961"},{"key":"e_1_2_1_12_2","first-page":"29","article-title":"Mucopolysaccharidose type VII par deficit en \u03b2\u2010glucuronidase: \u00e9tude \u010fune famille","volume":"27","author":"Guibaud P","year":"1979","journal-title":"J Genet Hum"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.18.3.237"},{"key":"e_1_2_1_14_2","first-page":"43A","article-title":"Congenital sialidosis: biochemical studies; 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