[Multicenter survey of diagnostic and therapeutic status in Chinese childhood patients with steroid-sensitive, relaping/steroid-dependent nephrotic syndrome].

OBJECTIVE: Nephrotic syndrome (NS) was recognized as a clinical syndrome caused by massive proteinuria, leading to hypoalbuminemia and edema. Primary nephrotic syndrome, accounted for about 90% of NS, seems to be one of common urologic-kidney diseases in childhood. The diagnosis and treatment of NS had so far failed to be unified nationwide. The purpose of this research was to survey the diagnosis and treatment status of steroid-sensitive, relaping/steroid-dependent nephrotic syndrome (SSNS, RNS/SDNS) in Chinese children and to evaluate the applicability and promotion of the evidence-based guideline on diagnosis and treatment of SSNS, RNS/SDNS (for trial implementation) conducted by The Subspecialty Group of Nephrology, the Society of Pediatrics, Chinese Medical Association in 2009.

METHOD: Questionnaires concerning children with SSNS, RNS/SDNS were designed by The Subspecialty Group of Nephrology, Society of Pediatrics, Chinese Medical Association and distributed to the doctors of 37 centers of 27 cities, 4 municipalities and 2 autonomous regions. A nationwide survey was conducted and data of hospitalized children (younger than 18 years of age) with SSNS, RNS/SDNS in 2008 and 2011 were analyzed retrospectively.

RESULT: Children diagnosed as primary nephrotic syndrome (PNS) accounted for 20.0% (7 444/37 134) of the hospitalized cases with urologic-kidney diseases in the corresponding time period; 3 725 cases were enrolled into this study. The male to female ratio was 3.2: 1 and the median age was 4.1 years (Q = 4.2 years); 3 186 (85.5%) cases were diagnosed as simple NS and 539 (14.5%) as nephritic NS; 2 825 (75.8%) PNS cases were the first episode and 1 050 (28.2%) cases were the relapse; 221 (5.9%) cases were diagnosed as SDNS and 749 (20.1%) as frequently relapse NS (FRNS) . At NS diagnosis, the proteinuria was 3+-4+ (3 260 cases, 87.5%) , median quantitive proteinuria for 24 hours was 112.0 mg/kg (Q = 109.3 mg/kg) , mean plasma albumin was (17.9 ± 6.4) g/L and mean cholesterol was (10.0 ± 3.1) mmol/L. Oral prednisone 2 mg/(kg·d) was the main treatment of the first episode cases (92.8%, 2 622/2 825). The median time of proteinuria remission was 9 (6) days; 96.1% (2 714/2 825) cases reached complete remission in 4 weeks and 3.6% (101/2 825) in 4-8 weeks. The median time for using full dose corticosteroid (GC) was 31 days and total time for using GC was 10 months. Of 1 050 cases, 402 (38.3%) RNS and 33.6% (252/749) FR/SD cases were treated with full dose GC again. After different treatment regimens, 77.5% (814/1 050) RNS and 75.4% (565/749) FR/SDNS cases reached remission. The median time of proteinuria remission was 8 days. 53.7% (402/749) FR/SDNS were treated with immunosuppressant and the first choice was CTX 211 cases (52.5%) , then CsA 108 cases (26.9%), MMF 70 cases (17.4%) and FK506 40 cases(10.0%). The cases treated with ACEI accounted for 24.0% (894 cases),ARB and levamisole accounted for 0.8% (28 cases) and 2.8% (106 cases), respectively.

CONCLUSION: In recent years, the proportion of children with PNS in the hospitalized childhood patients with urologic-kidney diseases was stable. The most common cases were the first episode, mainly in pre-school boys. The evidence-based guideline on diagnosis of SSNS, RNS/ SDNS (for trial implementation) conducted by The Subspecialty Group of Nephrology, Society of Pediatrics, Chinese Medical Association in 2009 was suitable for Chinese children with PNS, and remained to be further popularized. The guideline recommendation on treatment had a satisfactory recent curative outcome, and the long-term outcome needs further research.