{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T21:16:13Z","timestamp":1760044573026},"reference-count":0,"publisher":"Portland Press Ltd.","issue":"2","content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1973,2,1]]},"abstract":"<jats:p>1. Labelled glycine and\/or \u03b4-aminolaevulinic acid (ALA) were administered to a child with congenital erythropoietic porphyria (G\u00fcnther's disease), to three normal children and to three patients with erythropoietic protoporphyria.<\/jats:p>\n               <jats:p>2. The utilization of [15N]ALA for the synthesis of faecal \u2018urobilin\u2019 in the congenital erythropoietic patient was normal.<\/jats:p>\n               <jats:p>3. This suggests there is no significant increase of hepatic bile-pigment formation in congenital erythropoietic porphyria.<\/jats:p>\n               <jats:p>4. The utilization of glycine for synthesis of faecal \u2018urobilin\u2019 and protoporphyrin in all three patients with erythropoietic protoporphyria was increased. There was a similarly high utilization of [4-14C]ALA administered either orally or intravenously to one of the patients.<\/jats:p>\n               <jats:p>5. The utilization of [4-14C]ALA was not affected by phlebotomy.<\/jats:p>\n               <jats:p>6. Utilizations of both ALA and glycine for free erythrocyte and plasma protoporphyrins were low.<\/jats:p>\n               <jats:p>7. This study provides further evidence that in erythropoietic protoporphyria there is a greatly increased hepatic contribution to the early labelled fraction of bile pigment and that in this disease the excessive protoporphyrin is formed mainly in the liver.<\/jats:p>","DOI":"10.1042\/cs0440135","type":"journal-article","created":{"date-parts":[[2015,8,7]],"date-time":"2015-08-07T10:26:08Z","timestamp":1438943168000},"page":"135-150","update-policy":"http:\/\/dx.doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Isotopic Studies of the Erythropoietic and Hepatic Components of Congenital Porphyria and \u2018Erythropoietic\u2019 Protoporphyria"],"prefix":"10.1042","volume":"44","author":[{"given":"D. C.","family":"Nicholson","sequence":"first","affiliation":[{"name":"Department of Chemical Pathology, King's College Hospital Medical School, London"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. L.","family":"Cowger","sequence":"additional","affiliation":[{"name":"Department of Chemical Pathology, King's College Hospital Medical School, London"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Kalivas","sequence":"additional","affiliation":[{"name":"Department of Chemical Pathology, King's College Hospital Medical School, London"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R. P. H.","family":"Thompson","sequence":"additional","affiliation":[{"name":"Department of Chemical Pathology, King's College Hospital Medical School, London"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C. H.","family":"Gray","sequence":"additional","affiliation":[{"name":"Department of Chemical Pathology, King's College Hospital Medical School, London"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"288","container-title":["Clinical Science"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/portlandpress.com\/clinsci\/article-pdf\/44\/2\/135\/815113\/cs0440135.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/portlandpress.com\/clinsci\/article-pdf\/44\/2\/135\/815113\/cs0440135.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,10,25]],"date-time":"2019-10-25T13:17:18Z","timestamp":1572009438000},"score":1,"resource":{"primary":{"URL":"https:\/\/portlandpress.com\/clinsci\/article\/44\/2\/135\/70370\/Isotopic-Studies-of-the-Erythropoietic-and-Hepatic"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1973,2,1]]},"references-count":0,"journal-issue":{"issue":"2","published-print":{"date-parts":[[1973,2,1]]}},"URL":"https:\/\/doi.org\/10.1042\/cs0440135","relation":{},"ISSN":["0301-0538"],"issn-type":[{"value":"0301-0538","type":"print"}],"subject":[],"published":{"date-parts":[[1973,2,1]]}}}