Clinical analysis of patients with adult isolated ACTH deficiency / 医学研究生学报

ABSTRACT

Objective Adult isolated ACTH deficiency ( IAD) is an uncommon and underestimated disorder with high rate of misdiagnosis.This paper aimed to analyze and summarize the clinical features of 6 patients with IAD to have deeper understanding of the disease. Methods Retrospective analysis was made on the data of 6 IAD cases enrolled in Nanjing Hospital of Nanjing Medical University from 2007 to 2015.Statistical analysis was made on the clinical manifestations, therapeutic schemes, laboratory and imaging results, along with outpatient follow-up(ranging from 0.5-10 years), in order to analyze clinical efficacy and prognosis result. Results Of 6 IAD patients, 4 patients showed main symptoms of long-term fatigue, anorexia, weight loss;5 patients were with differ-ent degrees of hypoglycemia (fasting blood glucose2.70 [1.2-3.0]mmol/L), among which 3 had hypoglycemic coma (fasting blood glucose <1.8mmol/L), 1 had chronic hypoglycemia and 1 had asymptomatic hypoglycemia( asymptomatic hypoglycemia by continu-ous glucose monitor system).4 of them had refractory hyponatremia (Serum Na+117 [110-120]mmol/L).ACTH values of all the patients were less than 0.01ng/L.After glucocorticoid replacement therapy, all the patients were followed up for six months after treat-ment and cortisol rhythm, plasma cortisol, urine cortisol, fasting blood glucose, serum sodium of the patients were in normal range, but still at a low level of ACTH. Conclusion The clinical manifestation of IAD is characterized as hypoglycemia, hyponatremia, low ACTH with other normal pituitary hormones.Long-term glucocor-ticosteroid therapy has favorable prognosis for patients with adult isola-ted ACTH deficiency.