Congenital nephrotic syndrome,the common cause of end stage renal disease in chidren,is a rare kidney disorder.With the advanced molecular biology,much progress have been made in its etiology,diagnosis and treatment.This paper will mainly focus on its classification,diagnosis and therapy.

Objective To analyze the efficacy of kaolin intake amount as an index for motion sickness (MS)induced by different motion patterns stimulating the vestibular receptors of rats.Methods Rats were randomly divided into 5 groups.Three groups were subjected to one of the following stimulations,respectively-linear acceleration along either the interaural axis(IA)or body axis (AP),and double rotation(DR)stimulation.Other 2 groups were used as control.Kaolin intake was recorded for consecutive 6 d,3 d before and 3 d after stimulation,and the data were statistically analyzed.Results It was found that:1)following IA,AP and DR stimulations,25%,17% and 58% of the rats in each group increased mean kaolin intake by 1 g in the 3 d phase post-stimulation compared with that in the same duration of pre-stimulation,respectively;2)in contrast to some prewous reports,the present observation showed that high Ievel of kaolin intake post-stimulation may persist for more than one day.Conclusion All 3 tvpes of stimulation methods can serve as ways of specifically stimulating vestibular end-organs to induce kaolin intake increase,and double rotation iS the most effective.

Objective To explore the relationship between the expression of transient receptor potential cation channel subfamily C member 6 (TRPC6) and podocyte injury in children with primary nephrotic syndrome (PNS) and its clinical significance. Methods The renal tissue of 18 children with PNS was obtained. The pathological changes of kidney were observed by routine section staining and light microscopy. The structural changes of podocyte were observed by electron microscope. The mRNA and protein expressions of TRPC6 in tissues were determined by qPCR and immunohistochemistry, respectively. Further the correlation of TRPC6 mRNA with serum levels of albumin (Alb), creatinine (Cr), triacylglycerol (TG), cholesterol (Tch), complement C3 and 24 h urinary protein quantitation and estimated glomerular filtration rate (eGFR) were analyzed respectively. Results The expression of TRPC6 protein in renal tissue of children with PNS was higher than that in the control group, and the difference was statistically different (P<0.05). The relative expression of TRPC6 mRNA in renal tissue of children with PNS was positively related to the expression of TRPC6 protein (r=0.508, P<0.05), but there was no correlation of expression of TRPC6 mRNA with serum levels of Alb, Cr, TG, Tch, C3, eGFR and 24h urinary protein quantitation (P>0.05). Conclusion The pathological types of PNS were mainly podocyte lesions, and the expression of TRPC6 protein was increased in podocytes. TRPC6 detection may be helpful in the diagnosis of podocyte lesions.

Aim To make a research of rats with focal cerebral ischemia/reperfusion injury on pathological changes in brain and the changes of AQP4 and related proteins, in order to explore the relationship between AQP4 and brain edema. Methods Adult male SD rats, weighting 250~300 g, were randomly divided in-to Sham group and cerebral ischemia/reperfusion ( I/R) injury model group. The I/R model group was di-vided into the I/R-6 h, 12 h, 24 h, 48 h-four time point groups. The animal model of the right MCA is-chemia/reperfusion was established by suture method in mature SD rats. The nerve symptom score was con-ducted in the corresponding time points. Then, the permeability of brain tissue was detected by EB stai-ning;TTC staining was conducted to observe the cere-bral infarction volume;the dry wet weight method was used to detect the changes of brain water content; im-munohistochemical( IHC) , WB and RT-PCR were ap-plied to detect the expression of AQP4 , and the related factors at different time points of the model rats after is-chemia-reperfusion around infarcts. Results Com-pared with the Sham group, then ever function score of the rats in I/R model groups were much higher. With the increase of the reperfusion time, the cerebral in-farction volume, brain tissue permeability and the brain water content were also increased. IHC results showed that AQP4 expression gradually rose with widen distribution. WB and RT-PCR results verified the in-creasing level of AQP4 expression. The detection of the related proteins expression showed apparent changes. The expression of MMP-9 was increased, while the Oc-cludin and JAM-1 expression showed a decreasing trend. The I/R-48 h model group showed the most ob-vious differences in the expression of the related pro-teins and mRNA ( P <0. 01 vs Sham, respectively ) . Conclusion Accompanied with the aggravating cere-bral injury after cerebral ischemia/reperfusion, the ex-pression of AQP4 and MMP-9 level were activated, while the degradation of TJPs, Occludin and JAM-1, was increased. These factors are combined to make the formation of brain edema. This study makes a further research on the formation mechanism of the early stage for cerebral edema on I/R model and offers a potential for intervention in the filed of looking for a reliable drug therapy on cerebral edema.

IgG4-related disease is a newly recognized systematic inflammatory and fibrotic disease which affects one or more organs.This disease mainly occurs in the middle-aged and old males.Currently only a few cases were reported in children.Renal involvement was referred to as IgG4-related kidney disease,including IgG4-related tubulointerstitiai nephrisis,membraneous nephropathy and renal pyelitis.The clinical features,laboratory tests,pathological findings and treatment progresses have been summarized in this review.

p62 is a ubiquitous multifunctional protein with six domains in the body.p62 is mainly involved in selective autophagic degradation of ubiquitinated substrates.Hence, it has become an important biomarker of monitoring autophagic flux.p62 is also involved in oxidative stress.Additionally, p62 participates in series of cellular biological processes including nutrient sensing, apoptosis and metabolic reprogramming.The expression of p62 is regulated by some transcriptive factors such as TFEB.The mutations of p62 gene are associated with neurological and other disorders.It has been shown that p62 is necessary for maintaining normal function of glomerular podocytes, massangial cells and renal tubular cells.Based on the diversity and importance of p62 protein functions, it may turn to be a candidate of therapeutic target in renal diseases in future.

Objective: To summarize the characteristics of cuffed-tunneled catheters insertion and investigate the values of cuffed-tunneled catheters in pediatric patients. Methods: Between March 2015 and July 2017, all the pediatric patients who received maintenance hemodialysis at least 3 consecutive months in our center were included. Sixteen cuffed-tunneled hemodialysis catheters were inserted in patients for long-term hemodialysis access. The clinical manifestations and complications were retrospectively reviewed. Results: Fifteen pediatric patients with end stage ranal disease (ESRD) were included in this study and they received 16 cuffed-tunneled catheters for long-term vascular access, including 10 males and 5 females; median age at start of catheter insertion was 11.5 (4.2-14.5) years. Body weight was (27.8±8.0)kg (16.0-39.4 kg) . The size and the length of the catheters were based on the height of patients as follows: 28 cm for (115.6±10.6) cm (102.0-130.0 cm) ,36 cm for (148.6±9.9)cm (140.0-167.0 cm) . Cuffed-tunneled catheters outcome: 10 cuffed-tunneled catheters were still functional at the end of the study; 5 catheters were removed after successful kidney transplantation. Catheter failure occurred in 1 out of 16 cuffed-tunneled catheters due to catheter-related infections. The median catheter survival time was 11.9 months (range 3.5-21.3 months). Complications of cuffed-tunneled catheters: Catheter placements operation was successful in 15 cases using ultrasound guidance. No serious complications were observed in any patients receiving catheter inserting operation. The overall rate of catheter-related infections and thrombosis/malposition was 6.3% and 18.7%, respectively. Conclusions Ultrasound guidance is suggested in pediatric patients during the catheters insertion. The size and the length of the catheters should be based on the height of patients. Cuffed-tunneled hemodialysis catheters could be effectively used for maintenance of hemodialysis vascular access for pediatric patients with ESRD.

Objective:To analyze the epidemiological characteristics and influencing factors of venous thrombosis in elderly patients with severe trauma.Methods:A retrospective study was conducted to collect and statistically analyze general information[sex, age, body mass index(BMI)], causes of trauma, injury severity score(ISS), Glasgow coma score(GCS), coagulation function[prothrombin time(PT), international normalized ratio(INR), D-dimer], B-type natriuretic peptide(BNP), liver function(alanine aminotransferase, aspartate aminotransferase), creatinine, Caprini score, surgical approach, immobilization mode, days of hospitalization, and treatment cost.Results:Totally 179 elderly patients with severe trauma were enrolled, including 130 men(72.6%), aged(67.6±6.4)years.The BMI, ISS and GCS scores of elderly patients with severe trauma were(22.9±3.4)kg/m 2, 28.4±10.5 and 10.2±4.6, respectively.The Caprini score was 11.7±4.0.Of these patients, 32(17.9%)had VTE events.Compared with the VTE negative group, the VTE positive group was older( t=-2.214, P=0.028), with a higher Caprini score( t=-2.684, P=0.008)and more lower limb fractures( P=0.008)and pelvic fractures( P=0.001). There were no significant differences in coagulation function, liver function, atrial natriuretic peptide levels, creatinine levels and surgical approaches between the VTE negative group and the VTE positive group(all P<0.05). No significant difference was found in the proportion of patients receiving surgical treatment between the two groups( P=0.563). In the VTE positive group, 18.8% had no fracture, 50.0% had one fracture, and 31.2% had two or more fractures, and the difference was statistically significant compared with the VTE negative group( P=0.029). However, VTE events had no significant effect on the average length of stay and hospitalization costs in elderly trauma patients(all P<0.05). Conclusions:For elderly patients with severe trauma, VTE is more likely to occur with increased age, a high Caprini score, multiple fracture sites and pelvic fracture.In addition, pelvic fracture is an independent risk factor for VTE in very old trauma patients.Attention should be paid to prevention and treatment to achieve steady improvement in the overall prognosis of trauma in these patients.

Objective:To investigate the clinical characteristics and short-term follow-up outcomes of primary nephrotic syndrome (PNS) children infected with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) during the Omicron variant outbreak in Shanghai, and to provide a reliable reference for clinicians in the diagnosis and treatment.Methods:It was a case-control study. The clinical data of children with PNS (PNS group) who were diagnosed and followed-up up to 1 year in the nephrology department of four children's medical centers in Shanghai, and the children (control group) who had no underlying diseases and were infected with SARS-CoV-2 in Shanghai Jinshan Public Health Center, including the data when they were infected with SARS-CoV-2, were retrospectively analyzed.Results:(1) From March 30th to April 13th, 2022, 6 PNS children in Shanghai were infected with SARS-CoV-2, including 5 boys and 1 girl. The median age was 4.5 (2.0, 11.0) years old. And 30 children were matched by sex, age and disease type as control group, including 20 males and 10 females. The median age was 4.5 (2.0, 9.0) years. There were no significant differences between the PNS group and the control group in clinical symptoms (including fever duration), treatment regimens, vaccine doses and virus clearance time (all P>0.05). (2) The 6 children with PNS included 3 cases of steroid-sensitive type, 3 cases of steroid-resistant type, 2 cases of minimal change disease, 2 cases of focal segmental glomerulosclerosis and 2 cases with no renal biopsy. Before SARS-CoV-2 infection, their primary disease-PNS were stable, and urine protein was negative, four of them were under maintenance treatment with oral steroids or immunosuppressive drugs. At the time of SARS-CoV-2 infection, the symptoms of all of the 6 cases were mild, no severe, critical or fatal cases, and they were all cured and discharged from hospital through medical isolation observation or symptomatic treatment of infections. (3) Five cases of them still had discomfort symptoms such as cough, anorexia, and fatigue after being discharged from the hospital, which lasted for about 1 week. Within 1 year of follow-up, none of the children have suffered from "recurrent positive PCR results" or "secondary infection" of the SARS-CoV-2. (4) Among them, 4 cases of PNS relapsed after SARS-CoV-2 infection, timely addition of steroids was effective, their urine protein quickly turned negative, and there was no recurrence after 1 year of follow-up. (5) Before infection with SARS-CoV-2, the levels of immunoglobulin IgG were lower than the normal reference value in the 4 cases with PNS recurrence. Conclusions:During the Omicron variant outbreak in Shanghai, the infection of SARS-CoV-2 in children with PNS are resulted in high transmission among household contacts. Most of them have mild symptoms and good prognosis. PNS is prone to relapse after SARS-CoV-2 infection, and steroid therapy is effective and safe for these relapse. IgG may be a potential marker for the prognosis of PNS children infected with SARS-CoV-2.

Objective:To investigate the clinical characteristics, treatment and prognosis of TRPC6 variation induced children with steroid-resistant nephrotic syndrome (SRNS).Methods:Clinical data of four patients with nephrotic syndrome carrying TRPC6 variations, who were admitted to the Department of Nephrology and Rheumatology, Children′s Hospital of Shanghai from Jan. 2017 to Dec. 2019, was retrospectively analyzed. The literature search was conducted with "nephrotic syndrome" "child" and "TRPC6 variation" as keywords in China National Knowledge Infrastructure (CNKI), Wanfang, Weipu and Pubmed databases until August 2020.Results:One of the four cases was male, and the others were female. Onset age ranged from 4-year-1-month to 12-year-2-month. They presented severe proteinuria, hypoalbuminemia or edema as a first symptom. Four patients had anemia, and two patients had secondary hyperparathyroidism, and one patient had renal atrophy. Renal pathology showed that one case was immune complex associated with glomerulonephritis, and the rest were focal segmental glomerular sclerosis (FSGS). They had been initially treated with corticosteroids for more than four weeks, but they had inadequate responses. They were then treated with corticosteroids combined with immunosuppressants (for example, cyclophosphamide, a calcineurin inhibitor, or mycophenolate mofetil). However, the symptoms did not improve. Additionally, four children progressed to end-stage renal disease within 2 to 6 months.Their whole exon gene testing suggested that the variation types of TRPC6 gene were respectively c.2684G>T, c.523C>T, c.2678G>A, c.2683C>T, and all patients had de novo variations in TRPC6. One article in Chinese and 9 articles in English were found, which made up 27 patients. The data of 31 cases (including this group) were analyzed. There were 18 missense variations, one frameshift variation, one synonymous variation and one splicing variation. The onset age was from 4 months age to 14 years old. Among all patients, 18 cases had massive proteinuria and hypoproteinemia, 6 cases only showed proteinuria. The pathological type of 19 cases were FSGS, 2 cases were IgA nephropathy, 2 cases were minimal change disease, 1 case was collapse glomerulopathy, 1 case was C1q nephropathy, and 1 case was immune complex associated glomerulonephritis. Glucocorticoid therapy was ineffective in 18 cases, and calcineurin inhibitor was ineffective in 11 cases. The prognosis of the disease was poor. Renal failure occurred in 12 cases, and the time to end stage renal disease was from 4 months to 13.8 years.Conclusions:TRPC6 variation can cause SRNS at a young age. FSGS is the primary pathological type of SRNS causing by TRPC6 variation. Glucocorticoid and immunosuppressive therapy are mostly ineffective. The disease progressed rapidly and the prognosis is poor.