Endocrinology and Metabolism

Case Report

A Case of Isolated ACTH Deficiency.: Sang Jin Kim, Kyung Mook Choi, Sei Hyun Baik, Dong Seop Choi; J Korean Endocr Soc. 1995;10(4):445-450. Published online November 7, 2019

Abstract PDF: solated ACTH deficiency is a very uncommon cause of hypoadrenocorticism, with less than 200 cases reported in the literatures. The clinical presentation can be similar to that of primary adrenal insufficiency, but there is a greater tendency for hypoglycemia and absence of hyperpigmentation. The diagnosis is established by demonstrating hypocortisolism with undetectable serum levels of ACTH, normal adrenal responsiveness to prolonged ACTH infusion, and an absent ACTH response to insulin-induced hypoglycemia. Other endocrine function is normal.We experienced a case of isolated ACTH deficiency in 34 years old female who was admitted due to fever and drowsy mentality.So we present this case with a review of literatures.

Original Article

Clinical and Hormonal Analysis of Five New Cases and Eight Previously Reported Cases of Isolated ACTCH Deficiency in Korea.: Eun Young Oh, Byoung Joon Kim, Yun Jae Chung, Dong Joon Kim, Jong Ryul Hahm, Jae Hoon Chung, Yong Ki Min, Myung Shik Lee, Moon Kyu Lee, Kwang Won Kim; J Korean Endocr Soc. 1999;14(3):568-577. Published online January 1, 2001

Abstract PDF: Isolated ACTH deficiency is an uncommon disorder, which is defined by low cortisol production with low or normal plasma ACTH levels and no other pituitary abnormalities. We report five new cases of this disorder, and summarize the clinical and hormonal features of 8 previously reported cases in Korea plus 5 new cases. 1) The clinical manifestations of isolated ACTH deficiency are variable, non-specific and similar to those seen in adrenocortical insufficiency of any cause, the age of patients ranged from 21 to 66 years old with an average age of 46 years, and the male to female ratio was 10:3. 2) Hyponatremia and hypoglycemia were commmon laboratory findings, so the presence of unexplained hyponatremia or hypoglycemia should always warrant consideration of the diagnosis of isolated ACTH deficiency. 3) 3 of 13 patients accompanied by empty sella suggesting selective destruction of pituitary ACTH producing cells. 4) ACTH response to exogenous CRH or vasopressin was not elicited in all tested cases, suggesting pituitary disorders. 5) Most patients showed dramatic response with oral predinisone. In conclusion, when there are unexplained general weakness, fatigue, weight loss, nausea, vomiting, hypoglycemia, or hyponatremia, isolated ACTH deficiency should be excluded. Immunologic and pathologic studies, and hormonal evolution with glucocorticoid treatment are needed to understand the pathogenesis of isolated ACTH deficiency.

Case Reports

A Case of Isolated ACTH Deficiency with Rapid Deterioration.: Sung Kwan Hong, Eun Joo Lee, Ki Soo Kim, Chul Ryong Lee, Hyung Joo Park, Hun Ho Song, Young Soo Lee; J Korean Endocr Soc. 1999;14(2):396-400. Published online January 1, 2001

Abstract PDF: Isolated ACTH deficiency is a rare disorder, and usually characterized by its chronic course. The 59 year-old woman patient who had been healthy until 2 months ago, admitted because of abdomial pain, general weakness and loss of weight about 12kg for 2 months. She looked a little pallor but color of skin was not remarkable. Blood pressure, serum electrolyte, and glucose on admission were within normal range. Serum calcium was elevated with the value of 12.6mg/dL, which was normalized after hydration. Cortisol response to RI induced hypoglycemia did not show any response. Anterior pituitary hormone except ACTH showed normal response during combined pituitary hormone stimulation test. These clinical and laboratory finding reveals that isolated ACTH deficiency was developed in a short term period. There were no abnormalities in sellar MRI except pineal cyst. Her complaints were disappeared dramatically after hydroccetisone replacement at 4th. hospital day. Here we report a case of isolated ACTH deficiency, which was rapidly developed, with hypercalcemia, abdominal pain, and loss of weight about 12kg for 2 months.

A Case of Isolated ACTH Deficiency Manifestated by Atrial Fibrillation and Hypoglycemia.: Won Seob Kim, Ki Se Lee, Sun Kyung Song, In Suk Seo, Soo Jin Choi, Dong Yoon Kim; J Korean Endocr Soc. 1998;13(4):646-651. Published online January 1, 2001

Abstract PDF: Isolated ACTH deficiency is a rare cause of secondary adrenocortical insufficiency caused by the defect of synthesis or release of ACTH in pituitary gland. The clinical presentation can be simiilar to that of primary adrenal insufficiency, but there is a greater tendency for hypoglycemia and absence of hyperpigmentation. The patient, 80 year-old female, was admitted in chief complaint of deteriorated mental states. On admission, serum sodium was 127mEq/L, potassium 4.4mEq/L, blood glucose 27mg/dL and on routine E.C.G. was atrial fibrillation was revealed. The basal morning serum cortisol level was 9.97 pg/dL it dosent respond to insulin-induced hypoglycemia enoughly, but other pituitary functions were intact in pituitary cocktail stimulation test. In CRH stimulation test, there was no remarkable response in serum ACTH and cortisol level. Brain MRI failed to reveal any anatomic abnormalities of the sellar or suprasellar area consistent with the defect of pituitary ACTH secretion. This case was a isolated ACTH deficiency, So, we conclude that associated with atrail fibrillation and hypoglycemia.

A Case of Isolated ACTH Deficiency Accompanied by hyperprolactinemia.: Young Seung Kim, Gwang Il Kim, Dae Su Kim, Jeon Ok An, Sang Jeong Yoon, Hee Cheol Jang, Kang Seo Park; J Korean Endocr Soc. 1997;12(3):462-467. Published online January 1, 2001

Abstract PDF: Isolated ACTH deficiency is a uncommon disorder causing secondary adrenocortical insufficiency. Less than 200 cases have been reported in the world. The major clinical manifestations are hypoglycemia, weight loss, hypotension, anemia, weakness, nausea, inability to excrete water load, and hyponatremia. A 56-year-old male was admitted because of weakness, fatigue, nausea and vomiting. He was pallor and not associated with hyperpigmentation. The basal plasma ACTH and cortisol levels were 8.30 pg/ml and 0.6 ug/dl. The serum cortisol did not response to rapid ACTH stimulation test. On combined pituitary stimulation test, the cortisol did not response to insulin-induced hypoglycemia. Other anterior pituitary hormones showed normal responses except elevated prolactin level. Six months after glucocorticoid replacement therapy, the elevated basal prolactin level returned to normal. Brain MRI did not show any anatomic abnormalities of the sellar and suprasella area. We report a case of isolated ACTH deficiency accompanied by hyperprolactineia, which respond to glucocorticoid replacement therapy.

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