{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,10]],"date-time":"2025-09-10T22:00:24Z","timestamp":1757541624069},"reference-count":50,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2005,6,2]],"date-time":"2005-06-02T00:00:00Z","timestamp":1117670400000},"content-version":"vor","delay-in-days":6423,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Am. J. Med. Genet."],"published-print":{"date-parts":[[1987,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Our recent studies of the teratogenic mechanisms of phenytoin (DPH) and glucocorticoid in mice have indicated that DPH utilizes the anti\u2010inflammatory pathway of glucocorticoids in producing congenital defects, such as cleft palate. This pathway is influenced by H\u20102 and H\u20103 histocompatibility\u2010linked genes in the mouse, such that congenic strains have H\u20102 or H\u20103 alleles that confer susceptibility to DPH\u2010induced\u2010induced congenital defects, and susceptible H\u20102 congenic strains have high glucocorticoid receptor levels. However, other H\u20102 or H\u20103 alleles confer resistance to these defects in their otherwise genetically identical congenic partner strains, and \u201cresistant\u201d H\u20102 alleles are associated with low levels of these receptors.<\/jats:p><jats:p>To determine whether this animal work is applicable to the human, we have sought to investigate whether the level of glucocorticoid receptors in circulating lymphocytes of children with the fetal hydantion syndrome (FHS) is as it is in the animals.<\/jats:p><jats:p>We found that children with FHS had glucocorticoid receptor levels significantly elevated above those of unaffected children was also significantly levels significantly elevated above those of unaffected children with similar DPH exposure in control families. The receptor level of affected children was also significantly elevated above that of fathers of children with the FHS and of fathers and mothers of control children. These findings are consistent with those documented in the animal models and suggest that an elevated level of glucocorticoid receptors in lymphocytes may be a marker for susceptibility to the FHS syndrome.<\/jats:p>","DOI":"10.1002\/ajmg.1320280308","type":"journal-article","created":{"date-parts":[[2005,6,12]],"date-time":"2005-06-12T00:49:50Z","timestamp":1118537390000},"page":"607-618","source":"Crossref","is-referenced-by-count":10,"title":["Elevated glucocorticoid receptor levels in lymphocytes of children with the fetal hydantoin syndrome (FHS)"],"prefix":"10.1002","volume":"28","author":[{"given":"Allen S.","family":"Goldman","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Don C.","family":"Van 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