ISSN: 1300-0292
İndekslendiği Dizinler: SCIENCE CITATION INDEX EXPANDED
CINAHL, Index Copernicus,
Chemical Abstracts (CA),
Excerpta Medica / EMBASE
Dil: Türkçe, İngilizce
İçerik: Orijinal Araştırma, Derleme, Editöre Mektup, Olgu Sunumu, Tıp Eğitimi, Tıbbi Kitap İncelemeleri
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A Case With True Hermaphroditism And Congenital Adrenal Hyperplasia
Berrin DEMİRBAŞ*, Birol KORUKLUOĞLU**, Cavit ÇULHA*, Rüştü SERTER***, Gül GÜRSOY***,
Yalçın ARAL***, Ahmet KUŞDEMİR****, Ali Ulvi ÖNGÖREN**
* Dr., S.B Ankara Eğitim ve Araştırma Hastanesi Endokrinoloji ve Metabolizma Hastalıkları Kliniği,
** Op.Dr., S.B Ankara Eğitim ve Araştırma Hastanesi 2. Genel Cerrahi Kliniği,
*** Doç.Dr., S.B Ankara Eğitim ve Araştırma Hastanesi Endokrinoloji ve Metabolizma Hastalıkları Kliniği,
**** Op.Dr., S.B Ankara Eğitim ve Araştırma Hastanesi 5. Genel Cerrahi Kliniği, ANKARA
A 15 year old male patient presented with the complaints of short stature, headache and hypertensive attacks. Upon physical examination a mass was palpated under the umbilicus in addition to microphallus, penil hyposphadias and no testes in the scrotal sac. 17-OH progesterone (17-OH P), ACTH, free testosterone, dehydroepiandrosterone sulfate (DHEA-S), progesterone and 24 hours urinary VMA levels were all found to be high. Plasma cortisol level was found to be low. Using visualisation methods, bilateral adrenal and intraabdominal (frontally to vena cava inferior), total 3 masses were visualised. The ACTH stimulation test results were compatible with congenital adrenal hyperplasia (CAH). With the preliminary diagnoses CAH and malign pheochromocytoma the patient went under surgery after preparation. Postoperative pathologic evaluation revealed nodular cortical hyperplasia of both adrenal gland + hypertrophic myometrium, mature and immature ovarian and testes tissues. Karyotype analysis was found to be 46 XX. Consequently, the patient was diagnosed with CAH (11-b hydroxilase deficiency) and true hermaphroditism.Keywords: Hermaphroditism, Congenital adrenal hyperplasiaTurkiye Klinikleri J Med Sci 2002, 22:301-304
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