| Isolated adrenocorticotropic hormone deficiency associated with empty sella syndrome |
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Jun-Ho Choi, Byoung-Hyun Park, Chung-Gu Jo |
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한림대학교 의과대학 춘천성심병원 내과, 산부인과1 |
| 증례: 부신피질자극호르몬 단독결핍이 동반된 빈안장증후군 1예 |
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최준호.박병현.조정구, Byoung-Hyun Park, Chung-Gu Jo |
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| Abstract |
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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) |
| Key Words:
Empty sella syndrome; Isolated ACTH deficiency |
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