{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T09:34:24Z","timestamp":1777887264484,"version":"3.51.4"},"reference-count":0,"publisher":"S. Karger AG","license":[{"start":{"date-parts":[[2009,4,15]],"date-time":"2009-04-15T00:00:00Z","timestamp":1239753600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"},{"start":{"date-parts":[[2009,4,15]],"date-time":"2009-04-15T00:00:00Z","timestamp":1239753600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Gerontology"],"abstract":"<jats:p>The Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disease characterized by a triad of oculocutaneous albinism, accumulation of auto-fluorescent ceroid-like pigments within macrophages, and bleeding tendency due to a storage pool disease of the platelets. We report an autopsy case (39y, male), who died of pulmonary fibrosis and hemorrhage, with analysis of auto-fluorescent ceroid-like pigments (CLP). Pigmented macrophages were seen in almost all organs, especially marked in bone marrow, spleen, liver, colon, lymph nodes and kidneys. Ultrastructually, CLP was intracytoplasmic electron-dense and -lucent congeries. Histochemical characteristics and auto-fluorescence of CLP showed similarities to ceroid. Substance which revealed brilliant lemon yellow autofluorescent was extracted from homogenized tissue of spleen with chloroform\/methanol (2:1), then separated by thin-layer chromatography. The fluorescent substance had an excitation maximum at 360nm and a fluorescence maximum at 440nm, which is the same characteristic as described fluorescent lipid peroxidation products in vitro.<\/jats:p>","DOI":"10.1159\/000213751","type":"journal-article","created":{"date-parts":[[2009,4,20]],"date-time":"2009-04-20T11:36:39Z","timestamp":1240227399000},"page":"297-304","source":"Crossref","is-referenced-by-count":11,"title":["HERMANSKY-PUDLAK SYNDROME; A CASE REPORT WITH ANALYSIS OF AUTO-FLUORESCENT CEROID-LIKE PIGMENTS"],"prefix":"10.1159","author":[{"given":"C.","family":"Ohbayashi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N.","family":"Kanomata","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Y.","family":"Imai","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H.","family":"Ito","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H.","family":"Shimasaki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"127","published-online":{"date-parts":[[2009,4,15]]},"container-title":["Gerontology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/213751","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/karger.com\/article-pdf\/doi\/10.1159\/000213751","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T04:06:39Z","timestamp":1777608399000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000213751"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,4,15]]},"references-count":0,"URL":"https:\/\/doi.org\/10.1159\/000213751","archive":["Portico"],"relation":{},"ISSN":["0304-324X","1423-0003"],"issn-type":[{"value":"0304-324X","type":"print"},{"value":"1423-0003","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,4,15]]}}}