1.Effect of esketamine combined with transversus thoracis plane block on stress response and inflammation level in patients undergoing cardiac valve replacement
Xiaoyu KANG ; Siming SONG ; Yulin ZHONG ; Liuyu LU ; Xiaotong QIN ; Yonghao WANG ; Yang LU ; Zheng GONG
The Journal of Practical Medicine 2024;40(21):3082-3089
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.
2.A Case of Older Patient with Right Vocal Cord Paralysis Caused by Herpes Simplex Virus Infection Using Comprehensive Intervention by a Geriatric Interdisciplinary Team
Xiu GAO ; Kanghao ZHOU ; Ning ZHANG ; Yunlong LI ; Lin KANG ; Xiaohong LIU ; Xiaoyi ZHAO
Medical Journal of Peking Union Medical College Hospital 2024;15(5):1146-1151
This article presents a case report of right vocal cord paralysis resulting from herpes simplex virus infection in an older adult. The patient initially presented with fever, blisters on the lips and right cheek, followed by the gradual onset of hoarseness and difficulty in swallowing. Laryngoscopy revealed fixation of the right vocal cord while the left vocal cord exhibited normal movement. A high level of herpes simplex virus type 1 IgM antibody was detected during the disease progression. Treatment involving mid-dose glucocorticoid and methylcobalamin neurotrophic therapy, swallowing and vocal rehabilitation training, as well as enteral nutrition support, led to alleviation of hoarseness and improved ability to drink water in small sips. Follow-up laryngoscopy indicated partial restoration of movement in the right vocal cord. The article not only outlines the diagnosis and treatment of this case but also reviews relevant literature to broaden clinicians' knowledge of viral-induced vocal cord paralysis in the elderly. It also emphasizes the importance of a geriatric interdisciplinary team in managing complex diseases in older patients.
3.Construction of recombinant CVI988 vector vaccine integrating IBDV-VP2 gene
Lele GONG ; Xinxiang HUANG ; Yunzhe KANG ; Lele WANG ; Xiangqi QIU ; Yuanyuan ZHANG ; Meijie GAO ; Wenhui ZHU ; Yulin ZHANG ; Guoqing ZHUANG ; Aijun SUN
Chinese Journal of Veterinary Science 2024;44(9):1865-1871
The emergence of high virulent mutant strains of infectious bursal disease virus(IBDV)becomes a serious threat to the poultry industry.However,the live attenuated IBDV vaccine can potentially revert to a virulent strain.Therefore,it is a necessary to develop safe and effective IB-DV-associated vaccines.The construction of a recombinant Marek's disease(MD)vaccine strain,CVI988,expressing the IBDV VP2 protein,can protect against disease induced by both IBDV and Marek's disease virus(MDV).Here,the IBDV-VP2 gene was integrated into the UL55 locus of CVI988 by bacterial artificial chromosome(BAC)technique,resulting in the recombinant virus CVI988 BAC-VP2.The recombinant virus was characterized by PCR,IFA and subsequently the bi-ological properties of the recombinant virus were investigated.The results showed that the recom-binant virus CVI988 BAC-VP2 was successfully rescued.The VP2 protein stably expressed in chick-en embryo fibroblasts(CEF).The growth kinetics and plague size assays showed that there was comparable replication ability between recombinant virus and parental virus.This study provides the basis for the development of a low-cost vaccine against both IBDV and MDV infections.
4.Characteristics of kidney diseases in children aged 0-3 years old
Xiaoe ZHANG ; Lei SUN ; Ping WANG ; Sheng HAO ; Yulin KANG ; Guanghua ZHU ; Wenyan HUANG ; Ying WU
Chinese Journal of Nephrology 2023;39(7):499-505
Objective:To investigate the characteristics of renal disease spectrum in children aged 0-3 years old, and to evaluate the clinical value of renal biopsy in children aged 0-3 years old with renal diseases.Methods:It was a retrospective analysis study. The children aged 0-3 years old with kidney diseases receiving renal biopsy and having complete clinical data in Shanghai Children's Hospital from January 1, 2009 to December 31, 2020 were enrolled. The clinical and pathological data of the children were collected. The spectrum of renal diseases, clinical phenotype, renal pathology, and the relationship between renal pathology/genotype and clinical phenotype were analyzed.Results:A total of 117 children aged 0-3 years old with kidney diseases were enrolled in the study, accounting for 6.5% (117/1 790) of all children (0-18 years old) with renal biopsies during the same period. There were 77 males and 40 females. The age was (2.20±0.51) years old (5-35 months). All cases of renal biopsies in children aged 0-3 years old were successful without serious complications. Nephrotic syndrome was the common clinical phenotype of kidney diseases in children aged 0-3 years old (59.0%, 69/117), followed by hematuria and proteinuria (29.1%, 34/117). Primary glomerular disease (69.2%, 81/117) was the major clinical type of renal diseases, followed by hereditary kidney diseases (29.1%, 34/117), in which Alport syndrome was the main hereditary kidney disease (79.4%, 27/34). Renal pathological types of children aged 0-3 years old were mainly distributed in minimal change disease (30.8%, 36/117), followed by glomerular minor lesion (26.5%, 31/117), mesangial proliferative glomerulonephritis (15.4%, 18/117), and focal segmental glomerulosclerosis (10.3%, 12/117). Among 40 children aged 0-3 years old with hematuria with/without proteinuria, 25 cases were diagnosed as Alport syndrome by abnormal immunofluorescence of type IV collagen in renal tissues. Among the 28 children with kidney diseases who underwent genetic testing, 23 cases had gene mutations, mainly in COL4A5 gene (60.9%, 14/23), among which 4 children had gene mutations in 8 children with refractory nephrotic syndrome. Among the children aged 0-3 years old with clinical manifestations of hematuria, the proportion of gross hematuria in children diagnosed with Alport syndrome (59.3%, 16/27) was significantly higher than that in children without Alport syndrome (20.0%, 3/15, χ2=5.999, P=0.014). Conclusions:Primary glomerular disease is the principal type of kidney diseases in children aged 0-3 years old, followed by hereditary kidney disease. Attention should be paid to children aged 0-3 years old with gross hematuria. Renal biopsy in children aged 0-3 years old is safe and reliable, and it is an essential means for the diagnosis of renal diseases. Renal biopsy combined with gene testing can better understand the etiology of kidney diseases and guide treatment in children aged 0-3 years old.
5.Clinical characteristics and one-year follow-up outcomes of 6 children with primary nephrotic syndrome infected with SARS-CoV-2 during the Omicron variant epidemic
Rufeng DAI ; Qian SHEN ; Lei YIN ; Yulin KANG ; Yufeng LI ; Jing CHEN ; Mei ZENG ; Hong XU
Chinese Journal of Nephrology 2023;39(10):729-737
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.
6.An Easily Compatible Eye-tracking System for Freely-moving Small Animals.
Kang HUANG ; Qin YANG ; Yaning HAN ; Yulin ZHANG ; Zhiyi WANG ; Liping WANG ; Pengfei WEI
Neuroscience Bulletin 2022;38(6):661-676
Measuring eye movement is a fundamental approach in cognitive science as it provides a variety of insightful parameters that reflect brain states such as visual attention and emotions. Combining eye-tracking with multimodal neural recordings or manipulation techniques is beneficial for understanding the neural substrates of cognitive function. Many commercially-available and custom-built systems have been widely applied to awake, head-fixed small animals. However, the existing eye-tracking systems used in freely-moving animals are still limited in terms of their compatibility with other devices and of the algorithm used to detect eye movements. Here, we report a novel system that integrates a general-purpose, easily compatible eye-tracking hardware with a robust eye feature-detection algorithm. With ultra-light hardware and a detachable design, the system allows for more implants to be added to the animal's exposed head and has a precise synchronization module to coordinate with other neural implants. Moreover, we systematically compared the performance of existing commonly-used pupil-detection approaches, and demonstrated that the proposed adaptive pupil feature-detection algorithm allows the analysis of more complex and dynamic eye-tracking data in free-moving animals. Synchronized eye-tracking and electroencephalogram recordings, as well as algorithm validation under five noise conditions, suggested that our system is flexibly adaptable and can be combined with a wide range of neural manipulation and recording technologies.
7.Risk factors for venous thrombosis in elderly patients with severe trauma
Qiang RUI ; Jiasheng SHEN ; Qi YANG ; Jie XU ; Yangbo KANG ; Baojun YU ; Yulin LI ; Yong'an XU
Chinese Journal of Geriatrics 2022;41(10):1183-1186
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.
8.Progress of multifunctional protein p62 in kidney disease
Shu YUN ; Rong YANG ; Yulin KANG
International Journal of Pediatrics 2022;49(9):592-596
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.
9.Current status of prevention and treatment of esophagogastric variceal bleeding in cirrhotic portal hypertension patients in Ningxia region: a multicenter study
Ronghua DING ; Yanmei HU ; Xiaoguo LI ; Chao SHI ; Jigang RUAN ; Jianping HU ; Jie XUAN ; Yang LI ; Ping WANG ; Yuzhen QI ; Fang PENG ; Hailong QI ; Wei YANG ; Qian SHEN ; Shuiping KU ; Ruichun SHI ; Xuejuan WEI ; Yanping ZHANG ; Yulin DING ; Peifang ZHANG ; Zhanbin HOU ; Xiaojuan ZHANG ; Yuanlan TIAN ; Guizhen WANG ; Ping ZHANG ; Yanxia QI ; Tianneng WANG ; Ying LI ; Ning KANG ; Dan XU ; Ruiling HE ; Chuan LIU ; Shengjuan HU ; Yang BO ; Xiaolong QI
Chinese Journal of Digestive Surgery 2021;20(10):1078-1084
Objective:To investigate the current status of prevention and treatment of esophagogastric variceal bleeding (EVB) in cirrhotic portal hypertension patients in Ningxia region.Methods:The retrospective and descriptive study was conducted. The clinical data of 820 cirrhotic portal hypertension patients who were admitted to 21 medical centers in Niangxia region from January 2018 to December 2020 were collected, including 85 cases in Ningxia Hui Autonomous Region People′s Hospital, 73 cases in the Fifth People′s Hospital of Ningxia Hui Autonomous Region, 59 cases in the Wuzhong People′s Hospital, 52 cases in the Qingtongxia People′s Hospital, 50 cases in the Guyuan People′s Hospital, 47 cases in the Yuanzhou District People′s Hospital of Guyuan City, 47 cases in the Yinchuan Second People′s Hospital, 40 cases in the General Hospital of Ningxia Medical University, 40 cases in the Tongxin People′s Hospital, 35 cases in the Yinchuan First People′s Hospital, 34 cases in the Third People′s Hospital of Ningxia Hui Autonomous Region, 32 cases in the Zhongwei People′s Hospital, 30 cases in the Lingwu People′s Hospital, 30 cases in the Wuzhong New District Hospital, 30 cases in the Yanchi People′s Hospital, 29 cases in the Ningxia Hui Autonomous Region Academy of Traditional Chinese Medicine, 28 cases in the Shizuishan Second People′s Hospital, 25 cases in the Shizuishan First People′s Hospital, 21 cases in the Haiyuan People′s Hospital, 20 cases in the Pengyang People′s Hospital, 13 cases in the Longde People′s Hospital. There were 538 males and 282 females, aged (56±13)years. Observation indicators: (1) clinical charac-teristics of cirrhotic portal hypertension patients; (2) overall prevention and treatment of EVB in cirrhotic portal hypertension patients; (3) prevention and treatment of EVB in cirrhotic portal hypertension patients from different grade hospitals. Measurement data with normal distribution were represented as Mean± SD. Count data were described as absolute numbers, and comparison between groups was analyzed using the chi-square test. Results:(1) Clinical characteristics of cirrhotic portal hypertension patients: of 820 cirrhotic portal hypertension patients, 271 cases were in compensated stage and 549 cases were in decompensated stage. Of the 271 cases in compensated stage, there were 183 maels and 88 females, aged (53±12)years. There were 185 Han people, 85 Hui people and 1 case of other ethic group. The etiological data of liver cirrhosis showed 211 cases of viral hepatitis B, 4 cases of alcoholic liver disease, 8 cases of viral hepatitis C, and 48 cases of other etiology. There were 235 cases of Child-Pugh grade A and 36 cases lack of data. Of the 549 cases in decompensated stage, there were 355 males and 194 females, aged (57±14) years. There were 373 Han people, 174 Hui people and 2 cases of other ethic group. The etiological data of liver cirrhosis showed 392 cases of viral hepatitis B, 33 cases of alcoholic liver disease, 10 cases of viral hepatitis C, and 114 cases of other etiology. There were 80 cases of Child-Pugh grade A, 289 cases of grade B, 170 cases of grade C and 10 cases lack of data. (2) Overall prevention and treatment of EVB in cirrhotic portal hypertension patients: of 271 patients in compensated stage, 38 cases received non-selective β-blocker (NSBB) therapy, 16 cases received endoscopic treatment, 6 cases received interventional therapy. Of 549 patients in decompensated stage, 68 cases received NSBB therapy, 46 cases received endoscopic treatment, 28 cases received interventional therapy. (3) Prevention and treatment of EVB in cirrhotic portal hypertension patients from different grade hospitals: of 271 patients in compensated stage, 181 cases came from tertiary hospitals, of which 28 cases received NSBB therapy, 15 cases received endoscopic treatment, 6 cases received interventional therapy. Ninety cases came from secondary hospitals, of which 10 cases received NSBB therapy, 1 cases received endoscopic treatment. There was no significant difference in NSBB for prevention of EVB between tertiary and secondary hospitals ( χ2=0.947, P>0.05), while there was a significant difference in endoscopic treatment for prevention of EVB between tertiary and secondary hospitals ( χ2=5.572, P<0.05). Of 549 patients in decompensated stage, 309 cases came from tertiary hospitals, of which 22 cases received NSBB therapy, 29 cases received endoscopic treatment, 22 cases received interventional therapy. Two hundreds and fourty cases came from secondary hospitals, of which 46 cases received NSBB therapy, 17 cases received endoscopic treatment, 6 cases received interven-tional therapy. There were significant differences in NSBB and interventional therapy for prevention of EVB between tertiary and secondary hospitals ( χ2=18.065, 5.956, P<0.05). Conclusions:The proportion of receiving EUB prevention in cirrhotic portal hypertension in Ningxia is relatively low. For patients with compensated liver cirrhosis, the proportion of NSBB therapy and endoscopic treatment in the secondary hospitals was lower than that in tertiary hospitals. For patients with decompensated liver cirrhosis, the proportion of interventional treatment in secondary hospitals is lower than that of tertiary hospitals, but the proportion of NSBB in secondary hospitals taking is higher than that of tertiary hospitals.
10.Four cases of nephrotic syndrome with TRPC6 gene variations and literature review
Liwen SUN ; Lei SUN ; Ping WANG ; Yulin KANG ; Ying WU ; Guanghua ZHU ; Wenyan HUANG
Chinese Journal of Pediatrics 2021;59(3):223-227
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.

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