Clinical characteristics and long-term follow-up of patients with idiopathic isolated ACTH deficiency
10.3760/cma.j.cn311282-20230522-00233
- VernacularTitle:特发性孤立性ACTH缺乏症患者临床特点及长期随访研究
- Author:
Deyue JIANG
1
;
Shengjie WANG
;
Qinghua GUO
;
Zhaohui LYU
;
Jingtao DOU
;
Juming LU
;
Yiming MU
Author Information
1. 解放军总医院第一医学中心内分泌科，北京 100853
- Keywords:
Idiopathic isolated ACTH deficiency;
Clinical characteristics;
Follow-up
- From:
Chinese Journal of Endocrinology and Metabolism
2023;39(10):846-851
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To summarize the clinical characteristics, reveal evolutionary patterns, and enhance the understanding of idiopathic isolated adrenocorticotripin(ACTH)deficiency(IIAD)by conducting a clinical analysis and follow-up.Methods:The clinical data of 12 patients with IIAD in our center were analyzed retrospectively, and the patients were further followed up to summarize the clinical characteristics of these patients.Results:Among 12 patients with IIAD, the ratio of male to female was 3∶1. The onset showed a bimodal trend, with 2 cases occurring below the age of 18(at 1 year and 11 years respectively), and 10 cases occurring in adults, with an average onset age of 59.4 years old. In adults, the main symptoms were chronic fatigue, anorexia, and weight loss, while pediatric patients exhibited hypoglycemia and seizures. Hyponatremia(50%)was a common biochemical abnormality. ACTH, cortisol, and 24 h urinary free cortisol were significantly lower in all patients, and the functions of other pituitary gland axes were normal. All patients were normal except 2 patients with pituitary MRI showing vacuolar sella turcica. The most common accompanying disease was Hashimoto thyroiditis. After glucocorticoid replacement therapy, all patients showed symptom improvement. The replacement doses include prednisone acetate at 2.5-7.5 mg/d or hydrocortisone at 12-20 mg/d. All the 8 patients were still alive with ongoing ACTH deficiency, without any decline in other pituitary axis functions or occurrence of other diseases.Conclusion:IIAD exhibits a bimodal onset pattern with a higher prevalence in males. Symptoms persist in a chronic and stable manner without remission. Prognosis is favorable with physiological dose of glucocorticoid replacement therapy.
