Objective To explore the clinical characteristics and influencing factors of primary nephrotic syndrome (PNS) combined with hypercoagulability in children. Methods The clinical data of 57 children with primary PNS were analyzed retrospectively. The clinical features and treatment were compared among high coagulation state group, non high coagulation state group and control group (20 children). At the same time, the differences between the simple nephrotic syndrome group (SNS) and nephritic syndrome group (NNS) in hypercoagulable state were analyzed. In addition, the correlation analysis was performed. Results Among 57 patients, there were 50 patients in high coagulation state group and 7 in non high coagulation state group. There was no significant difference in gender, age and clinical manifestations between two groups (P>0.05). The platelet (PLT) count, platelet aggregation (PCT), albumin (Alb), fibrinogen (Fib), D-dimer (D2) were significantly higher than those in the control group, and there were statistically significant differences (P all<0.01). There were significant differences in the levels of PLT, Fib, D2 and complement C4 between hypercoagulable state group and non hypercoagulable state group (P all<0.05). There were significant differences in HCT, TC, LDL, PT and complement C3 levels between SNS group (n = 32) and NNS group (n =18) in 50 patients with high coagulation state (P<0.05). There was positive correlation between HCT and complement C3 (r=0.30, P<0.05), while there was no correlation between PLT and other indices (P>0.05). All of the 57 patients were improved and has no thrombosis after the treatment. Conclusion Children with primary PNS were usually associated with different degrees of hypercoagulable state, and PLT, Fib, D2 could be used as reference indices for the severity of hypercoagulable state, and the activation of complement system might be related to the occurrence and development of hypercoagulable state.

Objective To provide a reference for early detection and diagnosis of nutcracker syndrome(NCS) by analyzing the clinical features of children with NCS in different age groups and different gender groups.Methods Data of 112 children with simple NCS diagnosed at the Department of Nephrology of Chongqing Children's Hospital from January 2008 to January 2018 were analyzed retrospectively.Follow-up was conducted.Results In children with NCS,71 girls accounted for 63.4％,mainly in preschool age(82.4％,28 cases);41 boys accounted for 36.6％,mainly in school age(48.5％,8 cases) and adolescence(42.2％,7 cases).Fifty-one point two percent (21/41 cases) of boys presented with both hematuria and proteinuria after activities,60.6％ (43/59 cases) of girls presented with isolated hematuria after activities,while 46.7％ (21/45 cases) children in adolescence were most likely to appear lumbar and abdominal pain after activities,and the incidence of boys(63.2％,12 cases) was higher than girls (34.6％,9 cases),and the differences above were all statistically significant (x2 =6.939,P ＜ 0.05).The 24-hour urinary protein level in adolescent group was significantly higher than that in 2 groups of younger children,and the male children[0.09 (0.02-0.21) g/d] in this group were significantly higher than that in female children[0.06 (0.01-0.21) g/d] (x2 =6.48,P ＜ 0.05).The detection rate of CT angiography (CTA) (95.7 ％,67/70 cases) was significantly higher than that of color Doppler ultrasound (82.4％,75/91 cases),and the difference was statistically significant (x2 =6.721,P ＜ 0.05).Children with NCS in adolescence had smaller aortomesenteric angles (AMA) and larger ratios of the internal diameter of left renal vein(LRV) 's dilation part (a) to the stenosis part(b) (a/b) than those in preschool age and school age,and the difference was statistically significant (F =4.797,P ＜ 0.05).By follow-up of 96 cases for 3 months-7 years,there were 51 cases(58.0％) whose urine was back to normal in 88 cases who had reexamination of urine,and among the 54 patients who underwent color doppler ultrasound,25 cases(46.3％) showed relief of LRV compression.Conclusions There are more girls with NCS than boys,and girls are mainly in preschool age and always present with isolated hematuria,while boys are mainly in school age and adolescence and always presented with both hematuria and proteinuria.Children in adolescence were most likely to appear lumbar and abdominal pain,and boys have a higher incidence rate than girls.Children in school age and adolescence have the relatively higher quantitation level of proteinuria,and the smaller AMA and the larger a/b ratio,the more serious compression of LRV,and it's especially obvious in adolescence.