ABSTRACT
Objective To analyze the pathologic constitution,repeated renal biopsy,treatment,prognosis and focal segmental glomerulosclerosis (FSGS) risk factors of children with steroid-resistant nephrotic syndrome (SRNS).Methods A retrospective analysis was made of 172 SRNS cases of renal biopsy in the Pediatric Nephrology Center,the First Affiliated Hospital of Sun Yat-Sen University from September 1,2006 to August 31,2016.Results The main pathological types of 172 children with SRNS were FSGS in 72 cases (41.9％),minimal change disease (MCD) in 52 cases (30.2％),and mesangial proliferative glomerulonephritis (MsPGN) in 31 cases (18.0％).There were 11 cases (6.4％) with repeated renal biopsy,5 cases of 6 children with MCD changed to FSGS;3 cases of FSGS whose repeated renal biopsy were still FSGS,but the subtype had changed;2 cases of MsPGN changed to FSGS in repeated renal biopsy.Compared to non-FSGS,the age of onset of FSGS was smaller [3.0(1.7,6.0) years old vs.5.8 (3.4,8.9) years old],the plasma albumin of FSGS was lower [18.0 (14.0,22.9) g/L vs.20.0 (15.1,29.1) g/L],the 24 hours urine protein level was higher [136.0(76.0,200.0) mg/(kg · d) vs.93.0(55.3,150.0) mg/(kg · d)],and the differences were all significant(all P ＜ 0.05).Logistic regression analysis showed that the smaller the age(P =0.007),the higher the 24-hour urine protein(P =0.028),the greater the risk of FSGS.The receiver operating characteristic (ROC) curve analysis showed that the optimal critical value of 24 hour urine protein was 131 mg/(kg · d).The effective rate of Cycloposphamide (CTX) treatment in MCD children (10/12 cases) was higher than that of FSGS (1/5 cases) and MsPGN (1/2 cases),and the differences were statistically significant (all P ＜0.05).There was no significant difference in the curative effect of Tacrolimas (TAC) and Ciclosporin A (CsA) in children with FSGS,MCD and MsPGN (all P ＞ 0.05).In 62 cases of FSGS,25 cases (56.4％) were effective,and 37 cases (84.1％) were effective in 44 cases of MCD,15 cases (60.0％) were effective in 25 cases of MsPGN,and the difference of prognosis between different pathological types was statistically significant (P ＜ 0.05).Conclusions The most common pathological types of children with SRNS are FSGS,MCD,and MsPGN,but the pathological types can be converted to each other.The smaller the age is,the higher the 24-hour urine protein level is,and the greater the risk of FSGS of the pathological type.When the quantity of 24-hours urine protein was more than 131 mg/ (kg · d),it should be alert to the possibility of pathological type of FSGS.In children with MCD,the effective rate of CTX is higher than that of children with FSGS and MsPGN.The prognosis of FSGS is the worst but the prognosis of MCD is better.
ABSTRACT
Objective To analyze the podocyte gene mutation in children with steroid -resistant nephrotic syndrome (SRNS),and to explore the clinical manifestations and prognosis of children with gene mutation,so as to pro-vide a theoretical basis for the diagnosis and treatment of SRNS gene mutation in children. Methods Twenty-four pa-tients with SRNS diagnosis and ages less than 14 years old were selected from the Pediatric Nephrology Center of First Affiliated Hospital of Sun Yat-Sen University during August 31,2014 to September 1,2016. The gene detection was performed through PCR amplification and second DNA general sequencing,in which the target genes were detected in 23 cases with nephrotic panel,and 1 case was sequenced with the exon gene. Results There were 14 cases of male and 10 cases of female in 24 cases of genetic testing. The median age of onset was 4. 7 years old. There were 9 cases of sim-ple type,15 cases of nephritis type. And all the cases were primary steroid-resistant. Within the 20 cases of renal biop-sy,there were 5 cases of minimal change disease (MCD),11 cases of focal segmental glomerulosclerosis(FSGS),and 4 cases of mesangial proliferative glomerulonephritis (MsPGN). In the 24 cases,there were 8 cases of gene mutation. Their age was (3. 97 ± 3. 61)years old. The ratio of male and female was 1. 67:1. 00. The main clinical classification was nephritis type (6/8 cases). The major genes were NPHS2(3 cases),NPHS1(2 cases),INF2(2 cases),MYO1E(1 case). And FSGS was the main pathological type (4 cases). Most of them were no remission or end stage renal disease (ESRD)(6/8 cases),including 2 cases of renal transplantation. The 24 hour urine protein level in the gene mutation group was significantly higher than that in the non-mutation group [195. 4 (166. 0,262. 4)mg/(kg·d)vs. 85. 4 (74. 5,101. 3 ) mg/(kg·d )],and the difference was statistically significant (Z ＝ -3. 674,P ＜ 0. 001 ). Conclusion The main mutation genes of children with SRNS were NPHS2,NPHS1 and so on. FSGS was the main pathological type. Most of them were no remission or ESRD. The higher of the 24 hour urine protein level,the more pos-sibility of genetic mutation.
ABSTRACT
Objective To investigate the clinical, pathological features and risk factors of hyperuricemia in children with IgA nephropathy (IgAN). Methods A retrospective study of 269 primary IgAN children diagnosed between January 1, 2006 to December 31, 2017 at the Children Kidney Disease Center, the First Affiliated Hospital of Sun Yat-sen University, was performed in the hyperuricemia group (uric acid>350 μmol/L) and the normal uric acid group. The clinical and pathological characteristics were analyzed, and the risk factors of hyperuricemia were analyzed by using multivariate logistic regression analysis. Results There were 185 males and 84 females in the 269 IgAN children with age of (9.2 ± 3.1) years old, among whom there were 70 patients (26.0％) accompanied by hyperuricemia. Clinical indicators such as hypertension, urea nitrogen, serum creatinine, blood lipids, urinary protein in hyperuricemia group were higher than those in normal uric acid group (all P<0.05), while estimated glomerular filtration rate, serum total protein and albumin were less (all P<0.05). There were 58 patients (23.0％) and 12 patients (70.5％) associated with hyperuricemia among IgAN children with CKD 1-2 and CKD 3-5. The proportion of hyperuricemia in CKD stage 3-5 IgAN children was statistically higher than that in normal uric acid group (P<0.01). The hyperuricemia group had a higher proportion of Lee IV and V grade, and a lower proportion of the Lee III grade than the normal uric acid group (all P<0.05). According to the Oxford pathological classification score, there was no significant difference in total scores of renal lesions, glomerular score, and tubulointerstitial score between the two groups (all P>0.05). According to the Katafuchi semi-quantitative score, there was no significant difference in the total scores of renal lesions, glomeruli, and tubulointerstitial scores (all P>0.05), while the hyperuricemia group had higher renal vascular scores than the normal uric acid group (P<0.01). Multivariate logistic regression analysis showed that hypertension (OR=12.596, 95％CI 1.778-89.243, P=0.011), higher total cholesterol (OR=1.192, 95％CI 1.064-1.336, P=0.002), higher urea nitrogen (OR=1.273, 95％CI 1.104-1.468, P=0.001), proteinuria 3+(OR=1.875, 95％CI 1.309-2.684, P=0.001), proteinuria 4+(OR=1.627, 95％CI 1.241-2.134, P<0.001) and CKD stage 3 (OR=3.355, 95％CI 1.376-8.181, P=0.008) were the risk factors of hyperuricemia in children with IgAN. Conclusions Twenty-six percent IgAN children patients are accompanied by hyperuricemia, and their clinical parameters and pathological changes are more severe than those in normal uric acid group. Hypertension, higher total cholesterol, higher urea nitrogen, proteinuria 3+/4+and CKD stage 3 are the risk factors of hyperuricemia in children with IgAN.