본문 바로가기
KERD
논문KCI 등재

부신피질자극호르몬 단독결핍이 동반된 빈안장증후군 1예

Isolated adrenocorticotropic hormone deficiency associated with empty sella syndrome

박병현|최준호|조정구
대한내과학회 (The Korean Association Of Internal Medicine)|2008.11|대한내과학회지 (The Korean Journal of Medicine)|제75권 제5호|pp. 597-601

0

조회

0

다운로드

1

인용

0

참고문헌

키워드

부신피질자극호르몬 단독결핍; 빈안장증후군Empty sella syndrome; Isolated ACTH deficiency
주제분류내과학

초록

Isolated adrenocorticotropic hormone (ACTH) deficiency is an uncommon disorder for which the pathogenetic mechanism has not yet been identified. It has been reported that isolated ACTH deficiency (ICD) may be accompanied by deficiencies in other pituitary hormones; impaired growth hormone (GH) secretion was noted in 20 to 30% of ICD patients. Here, we describe a female patient with isolated ACTH deficiency accompanied by empty sella syndrome presenting as hypoglycemia, which was confirmed via various endocrine tests and magnetic resonance imaging (MRI) of the sella turcica. The patient’s symptoms improved rapidly with prednisolone therapy and, during follow-up, her previously impaired GH response to provocative stimuli and high TSH levels were corrected by glucocorticoid replacement alone. However, treatment failed to normalize plasma IGF-1 levels, suggesting that physiological cortisol levels are necessary for a normal plasma GH response to provocative stimuli. (Korean J Med 75:597-601, 2008)

Abstract

Isolated adrenocorticotropic hormone (ACTH) deficiency is an uncommon disorder for which the pathogenetic mechanism has not yet been identified. It has been reported that isolated ACTH deficiency (ICD) may be accompanied by deficiencies in other pituitary hormones; impaired growth hormone (GH) secretion was noted in 20 to 30% of ICD patients. Here, we describe a female patient with isolated ACTH deficiency accompanied by empty sella syndrome presenting as hypoglycemia, which was confirmed via various endocrine tests and magnetic resonance imaging (MRI) of the sella turcica. The patient’s symptoms improved rapidly with prednisolone therapy and, during follow-up, her previously impaired GH response to provocative stimuli and high TSH levels were corrected by glucocorticoid replacement alone. However, treatment failed to normalize plasma IGF-1 levels, suggesting that physiological cortisol levels are necessary for a normal plasma GH response to provocative stimuli. (Korean J Med 75:597-601, 2008)

참고문헌 (17)

  1. De Marinis L, "Primary empty sella", J Clin Endocrinol Metab, 90, pp.5471~5477 (2005)
  2. Gallardo E, "The empty sella: results of treatment in 76 successive cases and high frequency of endocrine and neurological disturbances", Clin Endocrinol, 37, pp.529~533 (1992)
  3. Hashimoto K, "Hyperresponsiveness of TSH and prolactin and impaired responsiveness of GH in Japanese patients with isolated ACTH deficiency", Nippon Naibunpi Gakkai Zasshi, 68, pp.1096~1111 (1992)
  4. Yamamoto T, "Isolated corticotropin deficiency in adults: report of 10 cases and review of literature", Arch Intern Med, 152, pp.1705~1712 (1992)
  5. Oh EY, "Clinical and hormonal analysis of five new cases and eight previously reported cases of isolated ACTH deficiency in Korea", J Korean Soc Endocrinol, 14, pp.568~577 (1999)
  6. Busch W, "Morphology of sella turcica and its relation to the pituitary gland", Virchows Arch, 320, pp.437~458 (1951)
  7. Bjerre P, "The empty sella: a reappraisal of etiology and pathogenesis", Acta Neurol Scand Supp, 130, pp.1~25 (1990)
  8. Brown JD, "Headache and the primary empty sella syndrome", Arch Otolaryngol Head Neck Surg, 112, pp.883~885 (1986)
  9. Kim ES, "Primary empty sella syndrome", J Korean Soc Endocrinol, 12, pp.386~392 (1997)
  10. Stacpole PW, "Primary empty sella, hyperprolactinemia, and isolated ACTH deficiency after postpartum hemorrhage", Am J Med, 74, pp.905~908 (1983)
  11. Woolf PD, "Hypopituitarism secondary to hypothalamic deficiency", Ann Intern Med, 78, pp.88~90 (1973)
  12. Stephens WP, "Isolated adrenocorticotrophin deficiency and empty sella associated with hypothyroidism", Clin Endocrinol, 22, pp.771~776 (1985)
  13. Stacpole PW, "Isolated ACTH deficiency: a heterogenous disorder: critical review and report of four new cases", Medicine, 61, pp.13~24 (1982)
  14. Murakami T, "Thyroid dysfunction in isolated adrenocorticotropic hormone deficiency", Endocr J, 40, pp.473~478 (1993)
  15. Hatazoe T, "Isolated adrenocorticotropic hormone deficiency associated with growth hormone deficiency and empty sella", Intern Med, 34, pp.688~691 (1995)
  16. Manser TJ, "Pseudo-Addison’ disease: isolated corticotropin deficiency associated with hyporeninemic hypoaldosteronism", Arch Intern Med, 146, pp.996~997 (1986)
  17. Kubo S, "Endonasal endoscpic transsphenoidal chiasmapexy with silicone plates for empty sella syndrome", Neurol Med Chir, 45, pp.428~432 (2005)

관련 논문AI추천

정상 혹은 감소된 혈장 ACTH를 보이면서 IPSS에 의해 진단된 쿠싱증후군 2예김용기, 김인주, 김지홍, 김학진, 남대근, 문재현, 문태건, 손석만, 안진광, 조용구, 황지윤대한내과학회 · 대한내과학회지2002 · 제62권 제5호뇌하수체줄기 형성 부전으로 발생한 시상하부성 뇌하수체 기능저하증 1예김병준, 박근용, 엄유진, 윤혜진, 이승주, 임동미, 조아름대한내과학회 · 대한내과학회지2013 · 제85권 제4호Delayed diagnosis of pituitary stalk interruption syndrome with severe recurrent hyponatremia caused by adrenal insufficiency고철우, 장경미대한소아내분비학회 · Annals of Pediatric Endocrinology & Metabolism2017 · 제22권 제3호범뇌하수체저하증, 요붕증을 보인 원발성 육아종성 뇌하수체염 1예김지은, 김지현, 목지영, 오현진, 이정민, 장상아, 조성배대한내과학회 · 대한내과학회지2015 · 제88권 제5호비기능성 뇌하수체 종양에 연관된 항이뇨호르몬 분비이상 증후군 1예구본정, 권기현, 김설영, 김윤정, 이인석, 이준철, 정사라대한내과학회 · 대한내과학회지2007 · 제72권 제3호외상성 뇌하수체 기능 저하증과 동반된 호산구성 위장관염 1예김영철, 양진모, 이강문, 이보인, 이석주, 장우임, 정우철, 정인식, 최규용, 홍창균대한소화기내시경학회 · Clinical Endoscopy2007 · 제34권 제1호

이 간행물의 다른 논문 (9건)

위장관 외 질환에서 Helicobacter pylori 감염의 치료최연호pp. 508-514가성 장폐쇄김재환, 양성연pp. 531-532우측 대장 게실염과 좌측 대장 게실염의 임상양상 비교이완석, 김상엽, 정민규, 김성국, 최용환, 전성우, 권영오, 조창민, 탁원영, 이현철, 이주영, 김대진pp. 533-538원발성 간내 종괴가 없는 간외담관 간세포암 1예맹치훈, 임성직, 이정일, 주광로, 박철기, 오원택, 김경엽pp. 559-563거대 우심실 종양혈전을 동반한 간내 육종양 담관암 1예이재경, 김인경, 김진원, 김혜령, 황진혁, 홍경섭pp. 569-573
원문 보기