title: [Multicenter study on present status of diagnosis and treatment of steroid-resistant nephrotic syndrome in children].
abstract: OBJECTIVE
  To investigate the treatment of multi-center hospitalized children with steroid resistant nephrotic syndrome (SRNS) retrospectively.
  
  
  METHODS
  The Subspecialty Group of Nephrology, The Society of Pediatrics, Chinese Medical Association organized 35 hospitals to investigate children with primary SRNS from 2008 to 2011. By filling in the questionnaire, data including age, gender, clinical type, related examination, pathology and treatment in local hospitals were collected, and then statistical analysis was performed.
  
  
  RESULTS
  A total of 35 hospitals participated in this survey, 577 patients were enrolled, their age was from 8 months to 18 years. The male to female ratio was 2: 1. School-age children accounted for 33.3% (n = 194). Clinical types were mostly simple nephrotic syndrome, which accounted for 60.5% (n = 349) and nephritis nephrotic syndrome accounted for 39.5% ( n = 228). The incidences of different types of NS were significantly different between 2008 and 2011 (χ(2) = 10.751, P = 0.001). There were 306 cases of infants who received renal biopsy. Renal pathology showed that 40 of them had mesangial proliferative glomerulonephritis (MsPNG), 17 focal segmental glomerulo- sclerosis (FSGS), 9 membranous nephropathy (MN), 9 minimal change disease (MCD), 8 minor lesions, 3 endocapillary proliferative glomerulonephritis (EnPGN) , 2 membrano-proliferative glomerulonephritis (MPGN), 2 sclerotic glomerulonephritis and 2 podocyte lesions in 2008. Otherwise there were 53 MsPGN, 35 FSGS, 25 minor lesions, 23 MCD, 13 MN, 5 MPGN, 3 podocyte lesions, 2 EnPGN, 1 sclerotic glomerulonephritis. Since the evidence-based guidelines had been issued in 2011, 209 cases were treated with adequate-dose prednisone therapy > 4 weeks program. Eighty-seven children with nephrotic syndrome whose urinary protein could not be turned negative after 4 weeks' glucocorticoid treatment were treated with high-dose methylprednisolone for 1 course. Thirty-eight of these children got negative urinary protein after the pulse therapy. Tacrolimus and cyclosporine treatment of SRNS significantly increased compared with 2008 (19.5% vs. 9.1% and 29.0% vs. 22.1% respectively). The most commonly used immunosuppressant was cyclophosphamide pulse therapy (56.5% vs. 58.1%). The situation of SRNS children with immunosuppression in two years was not statistically significant (χ(2) = 10.027, P > 0.05).
  
  
  CONCLUSIONS
  This study presented the current status of the diagnosis and treatment of SRNS of children in China. For children with nephrotic syndrome whose urinary protein cannot be turned negative after 4 weeks' glucocorticoid treatment, the best choice is to provide them with high-dose methylprednisolone pulse treatment for 1 course. Currently the treatment of SRNS diversifies, in which hormones and immunosuppressants are the main choices, and cyclophosphamide pulse therapy is still the preferred choice. However, according to different renal pathological types and different indications, choice of immunosuppressants remains to be further studied and standardized.
authors.affiliation: Department of Pediatrics, Nanjing General Hospital of Nanjing Military Command, Nanjing 21002, China. Email:njxzk@126.com.
mesh_terms: D000293:Adolescent; D015415:Biomarkers / Q000097:blood / Q000652:urine; D002648:Child; D002675:Child, Preschool; D002681:China; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006728:Hormones / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D007223:Infant; D007668:Kidney / Q000473:pathology; D008297:Male; D009404:Nephrotic Syndrome / Q000151:congenital* / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use; D012189:Retrospective Studies
chemical_list: D015415:Biomarkers; D006728:Hormones; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone