{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,6]],"date-time":"2025-10-06T05:58:14Z","timestamp":1759730294561},"reference-count":47,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[1989,12,1]],"date-time":"1989-12-01T00:00:00Z","timestamp":628473600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Shriners Hospitals for Crippled Children","award":["#15958"],"award-info":[{"award-number":["#15958"]}]},{"name":"General Clinical Research Center Branch, Division of Research Facilities and Resources, National Institutes of Health","award":["RR-00036"],"award-info":[{"award-number":["RR-00036"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1989,12,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>IBIDS is a syndrome characterized by ichthyosis, brittle hair, impaired intelligence, decreased fertility, and short stature, but unassociated with skeletal lesions. This condition is considered a form of trichothiodys-trophy because hair from several cases has been found to have a low sulfur content. We describe a 9-year and 10-month-old white boy whose clinical features resemble the IBIDS syndrome (ichthyosis, brittle hair, cataracts, and short stature), but who also has marked axial osteosclerosis and peripheral osteopenia. No abnormalities of mineral homeostasis were noted. Histopathologic assessment of nondecalcified bone specimens excluded osteopetrosis, but suggested slow skeletal remodeling. When subjected to polarized light microscopy, his hair exhibited the band-like pattern of birefringence described in trichothiodystrophy. Literature review disclosed 8 patients, 2 of whom had been diagnosed as trichothiodystrophy, with like clinical features including osteosclerosis. These skeletal abnormalities together with clinical features of the IBIDS\/trichiodystrophy syndrome, we believe, reflect the prototype of a disorder that seems best described as central osteosclerosis with ectodermal dysplasia.<\/jats:p>","DOI":"10.1002\/jbmr.5650040611","type":"journal-article","created":{"date-parts":[[2010,2,3]],"date-time":"2010-02-03T18:05:58Z","timestamp":1265220358000},"page":"863-875","source":"Crossref","is-referenced-by-count":15,"title":["Central osteosclerosis with ectodermal dysplasia: Clinical, laboratory, radiologic, and histopathologic characterization with review of the literature"],"prefix":"10.1093","volume":"4","author":[{"given":"Roberto","family":"Civitelli","sequence":"first","affiliation":[{"name":"Division of Endocrinology and Metabolism, The Jewish Hospital of St. Louis, St. Louis, MO 63110"},{"name":"Metabolic Research Unit, Shriners Hospital for Crippled Children, St. Louis, MO 63131"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William H.","family":"McAlister","sequence":"additional","affiliation":[{"name":"Mallinckrodt Institute of Radiology, St. Louis Children's Hospital, St. Louis, MO"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Steven L.","family":"Teitelbaum","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, The Jewish Hospital of St. Louis, St. Louis, MO 63110 and Washington University Medical Center, St. Louis, MO 63110"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael P.","family":"Dr. Whyte","sequence":"additional","affiliation":[{"name":"Division of Endocrinology and Metabolism, The Jewish Hospital of St. Louis, St. Louis, MO 63110"},{"name":"Metabolic Research Unit, Shriners Hospital for Crippled Children, St. Louis, MO 63131"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[1989,12,1]]},"reference":[{"key":"2024012712255231800_bib1","doi-asserted-by":"crossref","DOI":"10.1007\/978-1-4471-1292-1","volume-title":"Sclerosing Bone Dysplasias","author":"Beighton","year":"1980"},{"key":"2024012712255231800_bib2","first-page":"2972","volume-title":"Diagnosis of bone and joint disorders","author":"Resnick","year":"1981"},{"key":"2024012712255231800_bib3","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/BF00349088","article-title":"Dense bone-Too much bone: Radiological considerations and differential diagnosis. 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