{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T10:03:24Z","timestamp":1787997804934,"version":"build-2784847793"},"reference-count":13,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[1998,4,1]],"date-time":"1998-04-01T00:00:00Z","timestamp":891388800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1998,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>In addition to systemic manifestations with skeletal, pulmonary, renal, and haematological signs, lysinuric protein intolerance (LPI), a membrane transport defect of cationic amino acids, is often complicated by severe life\u2010threatening immunological manifestations. A 10\u2010year\u2010old boy with LPI who exhibited a severe systemic immunohaematological disease is described here. This patient showed cutaneous lesions similar to the subacute form of systemic lupus erythematosus, severe anaemia and dysproteinaemia, and a marked reduction of circulating T lymphocytes, mainly the CD4<jats:sup>+<\/jats:sup> cells. In vitro bone marrow cell culture studies showed that addition of patient's serum induced macrophage proliferation and inhibited erythroid progenitor cell growth. Treatment with high\u2010dose intravenous immune globulin resolved most of the clinical and laboratory abnormalities.<\/jats:p>","DOI":"10.1023\/a:1005383307100","type":"journal-article","created":{"date-parts":[[2002,12,21]],"date-time":"2002-12-21T21:48:34Z","timestamp":1040507314000},"page":"95-102","source":"Crossref","is-referenced-by-count":20,"title":["Intravenous immune globulin in lysinuric protein intolerance"],"prefix":"10.1002","volume":"21","author":[{"given":"C.","family":"Dionisi\u2010Vici","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"De Felice","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"El Hachem","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Bottero","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Rizzo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Paoloni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Goffredo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Sabetta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Caniglia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[1998,4]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/00006454-199409000-00012"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199201093260206"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799272"},{"key":"e_1_2_1_5_1","first-page":"558","volume-title":"Clinical Pediatric Dermatology","author":"Hurowitz S","year":"1993"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)81703-2"},{"key":"e_1_2_1_7_1","first-page":"117","article-title":"Pulmonary alveolar proteinosis: experience with eight pediatric cases and review","volume":"97","author":"Mahut B","year":"1996","journal-title":"Pediatrics"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm\/144.6.1330"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00444955"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799838"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.104.4.1176"},{"key":"e_1_2_1_12_1","first-page":"3603","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Simell O","year":"1995"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(95)00237-1"},{"key":"e_1_2_1_14_1","doi-asserted-by":"crossref","unstructured":"WallaertB(1990)Subclinical alveolitis.Lung(supplement):974\u2013983..","DOI":"10.1007\/BF02718235"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1023%2FA%3A1005383307100","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1023\/A%3A1005383307100","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T22:47:50Z","timestamp":1694818070000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1023\/A%3A1005383307100"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1998,4]]},"references-count":13,"journal-issue":{"issue":"2","published-print":{"date-parts":[[1998,4]]}},"alternative-id":["10.1023\/A:1005383307100"],"URL":"https:\/\/doi.org\/10.1023\/a:1005383307100","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1998,4]]}}}