{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T03:20:20Z","timestamp":1785468020070,"version":"3.56.0"},"reference-count":17,"publisher":"Oxford University Press (OUP)","issue":"2","content-domain":{"domain":["eje.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2016,8]]},"abstract":"<jats:sec>\n<jats:title>Objective<\/jats:title>\n<jats:p>Congenital lipoid adrenal hyperplasia (lipoid CAH) results in impairment of adrenal and gonadal steroidogenesis caused by <jats:italic>STAR<\/jats:italic> mutations. Our previous study revealed upregulation of genes associated with inflammatory or immune response and macrophage infiltration in the adrenal cortex of <jats:italic>Star<\/jats:italic>-knockout mice. This study aimed at investigating macrophage infiltration in the gonads from human patients with lipoid CAH.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Design<\/jats:title>\n<jats:p>This study includes seven patients with lipoid CAH who underwent gonadectomy: two XX women (age, 22 and 40 years) and five XY boys (1 year). Two women with ovarian cysts (32 and 40 years) and six boys with autopsy or tumor (1 year) were examined as controls. Immunohistochemical analysis of their gonads was performed to determine steroidogenic cells by NR5A1 or CYP17A1 and macrophages by IBA1 or CD68.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>An increased number of macrophages infiltrated into the ovaries of lipoid CAH and consisted of two subpopulations: one scattered within and around a layer of theca cells of maturing follicles and the other massively aggregated in the stroma. Abundant cytoplasmic lipid droplets were observed not only in the theca cells but also in the stromal macrophages. There was no significant difference in the number of macrophages in the testicular interstitium between lipoid CAH (95% confidence interval (95% CI: 19.3\u201347.7 per 0.2mm<jats:sup>2<\/jats:sup>) and controls (95% CI: 13.3\u201325.8 per 0.2mm<jats:sup>2<\/jats:sup>) (<jats:italic>P<\/jats:italic>=0.10).<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>These results demonstrate that macrophages infiltrate the ovaries of lipoid CAH, where the theca cells and the stromal macrophages have abundant cytoplasmic lipid droplets.<\/jats:p>\n<\/jats:sec>","DOI":"10.1530\/eje-16-0194","type":"journal-article","created":{"date-parts":[[2016,5,18]],"date-time":"2016-05-18T01:59:32Z","timestamp":1463536772000},"page":"127-132","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-1","source":"Crossref","is-referenced-by-count":11,"title":["Gonadal macrophage infiltration in congenital lipoid adrenal hyperplasia"],"prefix":"10.1093","volume":"175","author":[{"given":"Tomohiro","family":"Ishii","sequence":"first","affiliation":[{"name":"1Department of PediatricsKeio University School of Medicine, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryuji","family":"Fukuzawa","sequence":"additional","affiliation":[{"name":"2Department of Pathology and Laboratory MedicineTokyo Metropolitan Children\u2019s Medical Center, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takeshi","family":"Sato","sequence":"additional","affiliation":[{"name":"1Department of PediatricsKeio University School of Medicine, Tokyo, Japan"},{"name":"4Department of Endocrinology and MetabolismKanagawa Children\u2019s Medical Center, Yokohama, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koji","family":"Muroya","sequence":"additional","affiliation":[{"name":"4Department of Endocrinology and MetabolismKanagawa Children\u2019s Medical Center, Yokohama, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masanori","family":"Adachi","sequence":"additional","affiliation":[{"name":"4Department of Endocrinology and MetabolismKanagawa Children\u2019s Medical Center, Yokohama, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenji","family":"Ihara","sequence":"additional","affiliation":[{"name":"5Department of PediatricsGraduate School of Medical Sciences, Kyushu University, Fukuoka, Japan, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junko","family":"Igaki","sequence":"additional","affiliation":[{"name":"3Department of Endocrinology and MetabolismTokyo Metropolitan Children\u2019s Medical Center, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukihiro","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"3Department of Endocrinology and MetabolismTokyo Metropolitan Children\u2019s Medical Center, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seiji","family":"Sato","sequence":"additional","affiliation":[{"name":"6Department of PediatricsSaitama Municipal Hospital, Saitama, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshikatsu","family":"Mitsui","sequence":"additional","affiliation":[{"name":"1Department of PediatricsKeio University School of Medicine, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomonobu","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"1Department of PediatricsKeio University School of Medicine, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","reference":[{"key":"2016071318292324000_175.2.127.1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.physiol.63.1.193"},{"key":"2016071318292324000_175.2.127.2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI200216771"},{"key":"2016071318292324000_175.2.127.3","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199612193352503"},{"key":"2016071318292324000_175.2.127.4","doi-asserted-by":"publisher","DOI":"10.1210\/jc.82.5.1511"},{"key":"2016071318292324000_175.2.127.5","doi-asserted-by":"crossref","first-page":"1265","DOI":"10.1172\/JCI119284","article-title":"Spontaneous puberty in 46,XX subjects with congenital lipoid adrenal hyperplasia. Ovarian steroidogenesis is spared to some extent despite inactivating mutations in the steroidogenic acute regulatory protein (StAR) gene","volume":"99","author":"Fujieda","year":"1997","journal-title":"Journal of Clinical Investigation"},{"key":"2016071318292324000_175.2.127.6","doi-asserted-by":"publisher","DOI":"10.1210\/me.14.9.1462"},{"key":"2016071318292324000_175.2.127.7","doi-asserted-by":"publisher","DOI":"10.1210\/me.2001-0320"},{"key":"2016071318292324000_175.2.127.8","doi-asserted-by":"publisher","DOI":"10.1210\/me.2007-0493"},{"key":"2016071318292324000_175.2.127.9","doi-asserted-by":"publisher","DOI":"10.1210\/en.2011-1627"},{"key":"2016071318292324000_175.2.127.10","doi-asserted-by":"publisher","DOI":"10.1507\/endocrj.K08E-102"},{"key":"2016071318292324000_175.2.127.11","doi-asserted-by":"publisher","DOI":"10.1189\/jlb.0303114"},{"key":"2016071318292324000_175.2.127.12","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1159\/000185446","article-title":"Testicular histopathology in congenital lipoid adrenal hyperplasia: a light and electron microscopic study","volume":"47","author":"Aya","year":"1997","journal-title":"Hormone Research"},{"key":"2016071318292324000_175.2.127.13","doi-asserted-by":"publisher","DOI":"10.1189\/jlb.0210072"},{"key":"2016071318292324000_175.2.127.14","doi-asserted-by":"publisher","DOI":"10.1210\/endo-116-6-2682"},{"key":"2016071318292324000_175.2.127.15","doi-asserted-by":"publisher","DOI":"10.1095\/biolreprod64.3.790"},{"key":"2016071318292324000_175.2.127.16","doi-asserted-by":"publisher","DOI":"10.1677\/jme.0.0320507"},{"key":"2016071318292324000_175.2.127.17","doi-asserted-by":"publisher","DOI":"10.1038\/nrcardio.2009.55"}],"container-title":["European Journal of Endocrinology"],"original-title":[],"link":[{"URL":"https:\/\/eje.bioscientifica.com\/view\/journals\/eje\/175\/2\/127.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/eje.bioscientifica.com\/view\/journals\/eje\/175\/2\/127.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,2,14]],"date-time":"2023-02-14T16:34:06Z","timestamp":1676392446000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ejendo\/article\/175\/2\/127\/6655035"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,8]]},"references-count":17,"journal-issue":{"issue":"2"},"URL":"https:\/\/doi.org\/10.1530\/eje-16-0194","relation":{},"ISSN":["0804-4643","1479-683X"],"issn-type":[{"value":"0804-4643","type":"print"},{"value":"1479-683X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,8]]}}}