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

Journal of Clinical Investigation

Volumn 99, Issue 6, 1997, Pages 1265-1271

  Fujieda, Kenji ^a   Tajima, Toshihiro ^a   Nakae, Jun ^a   Sageshima, Shunichi ^b   Tachibana, Katsuhiko ^c   Suwa, Seizou ^c   Sugawara, Teruo ^d   Strauss III, Jerome F ^d  

^a HOKKAIDO UNIVERSITY   (Japan)

^b Obihiro Kohsei Hospital   (Japan)

^c KANAGAWA CHILDREN'S MEDICAL CENTER   (Japan)

^d HOSPITAL OF THE UNIVERSITY OF PENNSYLVANIA   (United States)

Author keywords

lipoid adrenal hyperplasia; ovary; spontaneous puberty; StAR; steroidogenesis

Indexed keywords

17 HYDROXYCORTICOSTEROID; 17 OXOSTEROID; ESTRADIOL; FLUDROCORTISONE; GLUCOCORTICOID; HYDROCORTISONE; REGULATOR PROTEIN; STEROIDOGENIC ACUTE REGULATORY PROTEIN; UNCLASSIFIED DRUG;

ADOLESCENT; ADULT; ARTICLE; BREAST DEVELOPMENT; CASE REPORT; CONGENITAL ADRENAL HYPERPLASIA; FAILURE TO THRIVE; FEMALE; FRAMESHIFT MUTATION; HUMAN; HYPERKALEMIA; HYPERPIGMENTATION; HYPONATREMIA; KARYOTYPE 46,XX; MENARCHE; NONSENSE MUTATION; OVARY POLYCYSTIC DISEASE; PRIORITY JOURNAL; PUBERTY; STEROIDOGENESIS;

EID: 0030901671     PISSN: 00219738     EISSN: None     Source Type: Journal    
DOI: 10.1172/JCI119284     Document Type: Article

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