Analysis of 130 cases of pediatric primary nephrotic syndrome without routine hormone therapy immediately after diagnosis / 中华肾脏病杂志

ABSTRACT

Objective:To investigate the causes and outcomes in the children who did not immediately receive glucocorticoids therapy after initial diagnosis of primary nephrotic syndrome (PNS).Methods:The clinical data of PNS patients not immediately receiving glucocorticoids therapy after initial diagnosis at the Department of Nephrology, the Children's Hospital, Zhejiang University School of Medicine from January 1, 2005 to December 31, 2014 were retrospectively analyzed.Results:A total of 1 431 cases were initially diagnosed with PNS, including 1 061 males and 370 females. Among them, 130 cases did not receive conventional glucocorticoids treatment immediately, accounting for 9.1%. Of whom, 75 cases were found showing spontaneous remission after symptomatic treatment; 23 cases were directly treated with adrenocorticotropic hormone (ACTH), one case with mycophenolate mofetil (MMF), and 31 cases not given glucocorticoids or immunosuppressants because of parental refusal. Among 75 cases with spontaneous remission, 16 cases were found in sustained remission; 39 cases were treated with glucocorticoids and 6 cases with ACTH at relapse; 14 cases were lost. Among 29 cases using ACTH, 7 cases were found in sustained remission. Among the 31 cases who refused glucocorticoids or immunosuppressants therapy, one died. The case treated with MMF, later were given with halved hormone because of no-effective response.Conclusions:Spontaneous remission is found in a small proportion of PNS patients at first-onset, but most subsequently relapse. Hormone therapy should be routinely given unless remission has occurred before application. Some children's parents refuse hormone therapy, and need further communication. Some cases initially treated with ACTH are found in sustained remission, which should be further observed and studied to clear the efficacy and safety of ACTH.