| A Case of Female Pseudohermaphroditism With Congenital Adrenal Hyperplasia. |
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Hyo Jung Kim, Hye Kyung Lee, Hyang Sook Kim, Soon Jai Lee, Sung Woo Shin |
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Department of Pediatrics, Han-Il Hospital, Seoul，Korea |
| 선천성 부신증식증을 동반한 여성가성반음양 1 례 |
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김효정, 이혜경, 김향숙, 이순재, 신성우 |
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한일병원 소아과 |
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| Abstract |
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We experienced a case of female pseudohermaphroditism with congenital adrenal hyperplasia.
The patient, a 10 day old female infant, was presented characteristic findings such as ambiguous sex and dehydration. The vaginogram revealed normal internal genital organs. The chromosome analysis was
46, XX. The electrolyte study showed hyponatremia and hyperkalemia. The 24 hours urinary
17-ketosteroid and 17-OHCS were 2.5 mg and 0.9 mg respectively, prior to treatment.
Fluid therapy, hydrocortisone and salt were tried with improvement.
The patient was considered to have 21-hydroxylation defect, “Salt-loser” from the clinical evidence.
A brief review of related literatures was made.
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| Key Words:
Congenital adrenal hyperplasia, Ambiguous external genitalia, Female pseudohermaphroditism.
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