Efficacy of adrenocorticotropic hormone in treating primary nephrotic syndrome in children with dual resistance to glucocorticoids and calcineurin inhibitors / 中华肾脏病杂志

ABSTRACT

Objective:To investigate the efficacy and safety of adrenocorticotropic hormone (ACTH) in treating primary nephrotic syndrome in children with dual resistance to glucocorticoids and calcineurin inhibitors (CNIs).Methods:Clinical data of 6 children with primary nephrotic syndrome treated with ACTH in the Children's Hospital of Zhejiang University School of Medicine from January 1, 2015 to December 31, 2019 were retrospectively collected. All the enrolled patients were children with primary nephrotic syndrome with dual resistance to glucocorticoids and CNIs. All the 6 children were given 0.4-1.0 IU·kg -1·d -1 ACTH (total ≤25 IU)+5% glucose 500 ml intravenous infusion for 8 h during the hormone reduction process, with a course of treatment for 5 days, once a month, and continuous treatment for 3-6 months. Clinical data such as 24 h urinary protein quantification, serum albumin, serum cholesterol, estimated glomerular filtration rate (eGFR) level and glucocorticoid dosage were collected at equal time points at 6 months before treatment, at the beginning of treatment, at the end of treatment and at 6 months of follow-up after treatment of ACTH to evaluate the efficacy and adverse reactions. Results:The onset age of 6 children was (4.89±1.77) years, and the age of the first treatment with ACTH was (9.49±3.06) years. All the 6 children completed 3 to 6 months of ACTH treatment, with 2 cases of complete remission, 2 cases of partial remission and 2 cases of no remission. At the end of ACTH treatment, 24 h urinary protein was significantly decreased ( P=0.026), serum albumin level was significantly increased ( P=0.003), and glucocorticoid dosage was significantly decreased ( P<0.001) than before treatment. At 6 months after the end of ACTH treatment, there was no statistical significance in 24 h urinary protein, serum albumin and hormone dosage compared with the end of ACTH treatment (all P>0.05), and the blood cholesterol level continued to decrease ( P=0.039). There was no significant change in eGFR during observation period ( P>0.05). In the process of ACTH infusion, all the 6 children showed transient decrease in urine output, rash in 2 cases, and elevated blood glucose in 1 case, which could be spontaneously relieved after drug withdrawal. There were no serious cardiovascular events, renal impairment, infection and other adverse reactions. Conclusions:ACTH has a good effect on children with primary nephrotic syndrome who are dual resistant to glucocorticoids and CNIs. ACTH can reduce proteinuria, decrease the dosage of glucocorticoids, improve the clinical remission rate, and has good security.