{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,8]],"date-time":"2026-01-08T03:39:08Z","timestamp":1767843548635,"version":"3.49.0"},"reference-count":9,"publisher":"Walter de Gruyter GmbH","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,1,26]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec id=\"j_jpem-2017-0235_s_999_w2aab3b7c21b1b6b1aab1c15b1Aa\">\n                  <jats:title>Background:<\/jats:title>\n                  <jats:p>Increased adrenal androgen hormones in congenital adrenal hyperplasia (CAH) can rarely cause giant ovarian cysts in the neonatal period. Although the exact mechanism of the development of ovarian cysts is unknown, it is thought that increased androgen levels stimulate folicle development by increasing follicle stimulating hormone (FSH) levels.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2017-0235_s_998_w2aab3b7c21b1b6b1aab1c15b2Aa\">\n                  <jats:title>Case presentation:<\/jats:title>\n                  <jats:p>A 16-day-old newborn with ambiguous genitalia was presented to our clinic. Laboratory test results were as follows: sodium: 126 mEq\/L, potassium: 5.4 mEq\/L, renin: 132 pg\/mL, adrenocorticotropic hormone (ACTH): 207 pg\/mL, cortisole: 7.8 \u03bcg\/dL, basal 17OH progesterone: 21 ng\/mL, androstenedione: 5.1 ng\/mL, testosterone: 1188 ng\/dL and dehydroepiandrosterone sulfate (DHEAS)&gt;1500 \u03bcg\/dL. Karyotype analysis resulted in <jats:italic>46,XX<\/jats:italic>. A homozygous mutation of <jats:italic>R356W<\/jats:italic> was detected in the <jats:italic>CYP21A2<\/jats:italic> gene. The classical severe form of salt wasting 21 hydroxylase deficiency was diagnosed and treatment was started with hydrocortisone and fludrocortisone. Good metabolic control was ensured by monthly visits but the baby presented with vaginal bleeding as soiling at 4 months. The cystic lesion which extended to the epigastric area from the pelvis in the midline abdomen, had a size of 90\u00d780\u00d760 mm and medially, thin ovarian parenchyma was detected in ultrasonography.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2017-0235_s_997_w2aab3b7c21b1b6b1aab1c15b3Aa\">\n                  <jats:title>Conclusions:<\/jats:title>\n                  <jats:p>The findings in our patient suggest that a decline in adrenal androgens after glucocorticoid treatment resulted in an increase in gonadotropin levels and the giant cyst is developed by activation of gonadotropin cascade and increased gonadotropin receptors, instead of androgens.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1515\/jpem-2017-0235","type":"journal-article","created":{"date-parts":[[2017,12,18]],"date-time":"2017-12-18T22:16:32Z","timestamp":1513635392000},"page":"229-233","source":"Crossref","is-referenced-by-count":4,"title":["Vaginal bleeding and a giant ovarian cyst in an infant with 21-hydroxylase deficiency"],"prefix":"10.1515","volume":"31","author":[{"given":"Nursel Murato\u011flu","family":"\u015eahin","sequence":"first","affiliation":[{"name":"Clinic of Pediatric Endocrinology, Dr. Sami Ulus Obstetrics and Gynecology and Pediatrics Training and Research Hospital , Ankara , Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elvan","family":"Bayramo\u011flu","sequence":"additional","affiliation":[{"name":"Dr. Sami Ulus Obstetrics and Gynecology and Pediatrics Training and Research Hospital, Pediatric Endocrinology Clinic , Alt\u0131nda\u011f\/Ankara, 06020 , Turkey , Phone: +90 03123056513, Fax: +90 03123170353"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Semra","family":"\u00c7etinkaya","sequence":"additional","affiliation":[{"name":"Clinic of Pediatric Endocrinology, Dr. Sami Ulus Obstetrics and Gynecology and Pediatrics Training and Research Hospital , Ankara , Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"\u015eenay \u015eava\u015f","family":"Erdeve","sequence":"additional","affiliation":[{"name":"Clinic of Pediatric Endocrinology, Dr. Sami Ulus Obstetrics and Gynecology and Pediatrics Training and Research Hospital , Ankara , Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ay\u015fe","family":"Karaman","sequence":"additional","affiliation":[{"name":"Clinic of Pediatric Surgery, Dr. Sami Ulus Obstetrics and Gynecology and Pediatrics Training and Research Hospital , Ankara , Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Melek Pala","family":"Akdo\u011fan","sequence":"additional","affiliation":[{"name":"Clinic of Radiology, Dr. Sami Ulus Obstetrics and Gynecology and Pediatrics Training and Research Hospital , Ankara , Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zehra","family":"Aycan","sequence":"additional","affiliation":[{"name":"Clinic of Pediatric Endocrinology, Dr. Sami Ulus Obstetrics and Gynecology and Pediatrics Training and Research Hospital , Ankara , Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"374","published-online":{"date-parts":[[2017,12,18]]},"reference":[{"key":"2021021317335690610_j_jpem-2017-0235_ref_001_w2aab3b7c21b1b6b1ab2b1b1Aa","doi-asserted-by":"crossref","unstructured":"New MI, Wilson RC. Steroid disorder in children: congenital adrenal hyperplasia and apparent mineralocorticoid excess. Proc Natl Acad Sci USA 1999;96:12790\u20137.","DOI":"10.1073\/pnas.96.22.12790"},{"key":"2021021317335690610_j_jpem-2017-0235_ref_002_w2aab3b7c21b1b6b1ab2b1b2Aa","unstructured":"Bhangoo A, Buyuk E, Oktay K, Ten S. Phenotypic features of 46, XX females with StAR protein mutations. Pediatr Endocrinol Rev 2007;5:633\u201341."},{"key":"2021021317335690610_j_jpem-2017-0235_ref_003_w2aab3b7c21b1b6b1ab2b1b3Aa","doi-asserted-by":"crossref","unstructured":"Topaloglu AK, Vade A, Zeller WP. Congenital adrenal hyperplasia and bilateral ovarian cysts in a neonate. Clin Pediatr (Phila) 1997;36:719\u201320.","DOI":"10.1177\/000992289703601210"},{"key":"2021021317335690610_j_jpem-2017-0235_ref_004_w2aab3b7c21b1b6b1ab2b1b4Aa","doi-asserted-by":"crossref","unstructured":"Uli N, Chin, D, David R, Geneiser N, Roche K, et al. Menstrual bleeding in a female infant with congenital adrenal hyperplasia: Altered maturation of the hypothalamic-pituitary-ovarian axis. J Clin Endocrinol Metab 1997;82:3298\u2013302.","DOI":"10.1210\/jcem.82.10.4277"},{"key":"2021021317335690610_j_jpem-2017-0235_ref_005_w2aab3b7c21b1b6b1ab2b1b5Aa","doi-asserted-by":"crossref","unstructured":"Maesaka H, Suwa S, Tachibana K, Katsumata N. Prolonged activation of hypothalamo-pituitary-ovarian axis during early infancy in female patients with salt-losing 21-hydroxylase deficiency. Pediatr Res 1985;19:1258\u201362.","DOI":"10.1203\/00006450-198512000-00007"},{"key":"2021021317335690610_j_jpem-2017-0235_ref_006_w2aab3b7c21b1b6b1ab2b1b6Aa","doi-asserted-by":"crossref","unstructured":"Anderson E, Lee MT, Lee GY. Cystogenesis of the ovarian antral follicle of the rat: ultrastructural changes and hormonal profile following the administration of dehydroepiandrosterone. 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