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, 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.

Objective To investigate the impact of esketamine hydrochloride in combination with ultrasound-guided transverse thoracic muscle plane block on stress response and inflammatory levels in patients undergoing cardiac valve replacement under general anesthesia.Methods A total of 120 patients who underwent elective extra-corporeal circulation-supported median open heart valve replacement were selected and randomly assigned into four groups using the random number table method:general anesthesia alone(Group G),general anesthesia with intrave-nous administration of esketamine(Group E),general anesthesia with transverse thoracic plane block(Group T),and esketamine combined with transverse thoracic muscle plane block(Group ET);each group consisted of 30 cases.Patients in group E and group ET received a continuous infusion of esketamine hydrochloride injection at a rate of 0.2 mg/kg-1?h-1 until the completion of the surgical procedure,while patients in group G and group T received an equivalent volume of saline solution until the completion of the surgical procedure.After the induction of general anesthesia,patients in group T and group ET underwent ultrasound-guided bilateral transverse thoracic muscle plane block,while patients in group G and group E did not receive any specific intervention.All four groups received identical protocols for anesthesia induction and maintenance,with self-controlled intravenous analgesic pumps administered to all patients postoperatively.The following time points were recorded:1 day prior to surgery(T0),pre-induction of anesthesia(T1),1 minute post-tracheal intubation(T2),1 minute post-median sternotomy(T3),1 minute prior to initiation of cardiopulmonary circulation(T4),1 minute after cessation of cardiopulmonary circula-tion(T5),1 minute after completion of surgery(T6),1 day post-surgery(T7),2 days post-surgery(T8),and 3 days post-surgery(T9).Mean Arterial Pressure(MAP)and Heart Rate(HR)were continuously monitored from T1 to T6.The levels of blood glucose and lactate were measured and recorded at T1,T4 to T6.The levels of White Blood Cells(WBC)and C-Reactive Protein(CRP)were assessed at T0,as well as at T7 to T9.The occurrence of postoperative adverse reactions was documented in all four groups.Results(1)Comparison of hemodynamics among the four groups:Compared with group G,there was a significant decrease in MAP and HR at T3 in group T(P<0.05).At the T5 time point,MAP was lower in group ET compared to group E,while HR was higher in group ET compared to group T(P<0.05).(2)The lactate and blood glucose levels of the four patient groups after extracorporeal circulation transfer were higher than those at the T1 time point(P<0.05).Patients in group E had lower lactate values at the T5 time point and lower blood glucose values at the T6 time point compared to group G(P<0.05).Additionally,patients in group E exhibited lower lactate and blood glucose values at both the T5 and T6 time points compared to those in group T(P<0.05).(3)Compared to T0,the levels of white blood cells(WBC)and C-reactive protein(CRP)were increased in all four groups after surgery(P<0.05).At the T7 time point,the WBC levels in group E and group T were significantly lower than those in group G(P<0.05).Furthermore,compared to group E and group T,the level of WBC in group ET was significantly lower at T7,while the level of CRP was significantly lower at T8(P<0.05).(4)There were no significant differences observed in postoperative adverse reactions among the four groups(P>0.05).Conclusion Combining low-dose esketamine hydrochloride with transverse thoracic muscle plane block under general anesthesia during open heart valve replacement surgery can effectively stabilize the patient's hemodynamics,mitigate perioperative stress response and postoperative inflammation levels,thereby demonstrating significant clini-cal utility.