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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2015.1
- 수록면
- 409 - 414 (6page)
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Congenital adrenal hyperplasia (CAH) with 17α-hydroxylase/17,20-lyase deficiency is usually characterized by primary amenorrhea, characterized by hypertension and primary amenorrhea, sexual infantilism in women, and pseudohermaphroditism in men. hypertension, and sexual infantilism in women and pseudohermaphroditism in men. In rare cases, a huge adrenal gland tumor can present as a clinical manifestation in untreated CAH. Adrenal cortical adenoma is an even more rare phenotype in CAH with 17α-hydroxylase/17,20-lyase deficiency. A 36-year-old female presented with hypertension and abdominal pain caused by a huge adrenal mass. Due to mass size and symptoms, left adrenalectomy was performed. After adrenalectomy, blood pressure remained high. Based on hormonal and genetic evaluation, the patient was diagnosed as CAH with 17α-hydroxylase/17,20-lyase deficiency. The possibility of a tumorous change in the adrenal gland due to untreated CAH should be considered. It is important that untreated CAH not be misdiagnosed as primary adrenal tumor as these conditions require different treatments. Adequate suppression of adrenocorticotropic hormone (ACTH) in CAH is also important to treat and to prevent the tumorous changes in the adrenal gland. Herein, we report a case of untreated CAH with 17α-hydroxylase/17,20-lyase deficiency presenting with large adrenal cortical adenoma and discuss the progression of adrenal gland hyperplasia due to inappropriate suppression of ACTH secretion.
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참고문헌 (20)
참고문헌 신청
Speiser PW / 2003 / Congenital adrenal hyperplasia / wN Engl J Med 349:776~788
Bao X / 2011 / Prevalence of common mutations in the CYP17A1 gene in Chinese Han population / Clin Chim Acta 412:1240~1243
van Seters AP / 1981 / Adrenocortical tumour in untreated congenital adrenocortical hyperplasia associated with inadequate ACTH suppressibility / Clin Endocrinol (Oxf) 14:325~334
Falke TH / 1986 / Computed tomography in untreated adults with virilizing congenital adrenal cortical hyperplasia / Clin Radiol 37:155~160
Ravichandran R / 1996 / Congenital adrenal hyperplasia presenting as massive adrenal incidentalomas in the sixth decade of life: report of two patients with 21-hydroxylase deficiency / J Clin Endocrinol Metab 81:1776~1779
Bao X / 2011 / Prevalence of common mutations in the CYP17A1 gene in Chinese Han population / Clin Chim Acta 412:1240~1243
van Seters AP / 1981 / Adrenocortical tumour in untreated congenital adrenocortical hyperplasia associated with inadequate ACTH suppressibility / Clin Endocrinol (Oxf) 14:325~334
Falke TH / 1986 / Computed tomography in untreated adults with virilizing congenital adrenal cortical hyperplasia / Clin Radiol 37:155~160
Ravichandran R / 1996 / Congenital adrenal hyperplasia presenting as massive adrenal incidentalomas in the sixth decade of life: report of two patients with 21-hydroxylase deficiency / J Clin Endocrinol Metab 81:1776~1779
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