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    Clinical Study

  • Chen Qiuxin, Yang Zhenhua, Pan Xiaoting, Ma Xiaobo, Chen Xiaonong
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    Objective To investigate the clinical characteristics and vaccination of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in maintenance hemodialysis (MHD) patients. Methods It was a cross-sectional survey. Through wechat questionnaire star mode, the status of SARS-CoV-2 infection, clinical manifestation after infection and vaccination of MHD patients in Ruijin Hospital Affiliated to Shanghai Jiaotong University School of Medicine between December 8, 2022 and January 12, 2023 were retrospectively collected during the period that SARS-CoV-2 infection was managed with measures against class B infectious diseases. The patients were grouped according to whether they were infected with SARS-CoV-2 and whether they were complicated with pneumonia after infection during the study period. Logistic regression analysis was used to analyze the risk factors of SARS-CoV-2 infection combined with pneumonia or persistent symptoms after SARS-CoV-2 turning negative. Results A total of 241 questionnaires were issued, and 223 valid questionnaires were returned, of which 175 patients (78.5%) were infected with SARS-CoV-2 during the study period. The time of negative conversion of antigen or nucleic acid after SARS-CoV-2 infection was 10 (7,14) days. Asymptomatic infections accounted for 10.3% (18/175). Symptomatic infections accounted for 89.7% (157/175), and the top five most frequent symptoms were cough (70.1%, 110/157), fever (64.3%, 101/157), pharyngeal pain (35.0%, 55/157), muscle pain (28.7%, 45/157), and nasal obstruction (21.0%, 33/157). Pneumonia was reported in 54.3% (95/175) of infected patients. There were no statistically significant differences in age, gender distribution, proportion of diabetes mellitus, and proportion of history of tumors between SARS-CoV-2-infected and uninfected patients (all P>0.05). Compared with the non-pneumonia group, the proportions of dyspnea (12.6% vs. 1.3%, χ2=8.181, P=0.004) and chest tightness (23.2% vs. 10.0%, χ2=5.293, P=0.021) in the pneumonia group were both higher. There were no significant differences in age, gender distribution, time of negative SARS-CoV-2 transition, number of symptoms and SARS-CoV-2 vaccination rate,and proportions of diabetes mellitus and tumors between the pneumonia group and the non-pneumonia group (all P>0.05). There were 48.6% (85/175) infected patients having long-lasting clinical symptoms after SARS-CoV-2 turning negative. Logistic regression analysis results showed that diarrhea was an independent influencing factor of pneumonia in MHD patients infected with SARS-CoV-2 [OR (95% CI)=6.323 (1.061-37.682), P=0.043]. The number of symptoms at onset [OR (95% CI)=1.571 (1.318-1.873), P<0.001] and history of tumors [OR (95% CI)=0.206 (0.054-0.777), P=0.020] were independent influencing factors of long-lasting clinical symptoms. The SARS-CoV-2 vaccination rate was 7.2% (16/223) in MHD patients due to safety concerns (81.2%, 164/202). Conclusions MHD patients are SARS-CoV-2-susceptible population. The clinical symptoms after infection are diverse, heterogeneous, and the proportion of complicated pneumonia is high. Diarrhea is an independent influencing factor of pneumonia in SARS-CoV-2-infected MHD patients. Nearly half of patients experience long-lasting clinical symptoms after SARS-CoV-2 turning negative. The low rate of vaccination in the MHD population necessitates increased awareness about the safety and efficacy of SARS-CoV-2 vaccine.

  • Yang Guang, Ge Yifei, Huang Yaoyu, Lou Jizhuang, Jiang Chunming, Lu Guoyuan, Chen Fengling, Shen Jiansong, Chen Xiaolan, Dai Houyong, Liu Changhua, Yang Min, Li Xiurong, Sun Zhuxing, Wang Liang, Liu Bin, Zheng Donghui, Xu Yong, Chen Maojie, Wang Ling, Zhang Yilai, Zhang Xu, He Jianqiang, Zhang Liyuan, Wan Huiting, Guo Honglei, Yang Jiahui, Xu Wei, Xing Changying, Mao Huijuan
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    Objective To investigate the epidemiology of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in patients with maintenance hemodialysis (MHD) in Jiangsu province during SARS-CoV-2 pandemic in China from December 7, 2022 to January 27, 2023, and to analyze the influencing factors of all-cause death. Methods It was a multi-center cross-sectional investigation. Structured questionnaire was used to collect patient information by medical staff of each hemodialysis center (room) as investigators. Part of the demography data and laboratory examination data came from the Jiangsu Province Hemodialysis Data Information System. MHD patients from hemodialysis centers (rooms) at all levels of medical institutions and independent hemodialysis institutions in Jiangsu province during the outbreak of SARS-CoV-2 infection were included, and the clinical characteristics and all-cause mortality of confirmed and suspected cases of SARS-CoV-2 infection were analyzed. Results Questionnaire surveys and data analysis on 57 278 patients in 407 hemodialysis centers (rooms) were completed, accounting for 90.41% of the total number of MHD patients (63 357 cases) in Jiangsu province during the same period. There were 24 038 cases (41.97%) of SARS-CoV-2 infection and 14 805 cases (25.85%) of suspected infection, which were widely distributed in all dialysis centers in Jiangsu province. After clinical classification of 38 843 confirmed and suspected SARS-CoV-2 infection cases, 3 662 cases were severe and critical cases, accounting for 9.43% of the infected and suspected cases. Among the patients who had completed the questionnaires, there were 1 812 all-cause deaths, with an all-cause mortality rate of 3.16%. Multivariate logistic regression analysis showed that elderly (taking ≤50 years as a reference, 51-59 years: OR=1.583, 95% CI 1.279-1.933, P=0.001; 60-69 years: OR=3.972, 95% CI 3.271-4.858, P<0.001; 70-79 years: OR=7.236, 95% CI 5.917-8.698, P<0.001; ≥80 years: OR=11.738, 95% CI 9.459-14.663, P<0.001), male (OR=1.371, 95% CI 1.229-1.529, P<0.001), and co-infection with hepatitis B virus (HBV) (positive serum HBV surface antigen, OR=0.629, 95% CI 0.484-0.817, P<0.001) were independent influencing factors for all cause mortality. Receiver-operating characteristic curve analysis showed that the area under the curve for male, age and current HBV infection prediction of all-cause death was 0.529 (P<0.001), 0.724 (P<0.001) and 0.514 (P=0.042), respectively, and the cut-off value for age prediction of all-cause death was 65.5 years old. Compared with patients without HBV infection, MHD patients with HBV infection significantly reduced the proportion of severe and critically ill patients, all-cause hospitalizations and all cause deaths when infected with SARS-CoV-2 (4.99% vs. 6.41%, χ2=6.136, P=0.013; 8.90% vs. 11.44%, χ2=11.662, P<0.001; 2.01% vs. 3.37%, χ2=10.713, P=0.001, respectively). Conclusion The MHD patients in Jiangsu province are susceptible to SARS-CoV-2. Elderly age and male gender are independent risk factors for death in MHD patients during the epidemic, while the HBV infection may be a protective factor for death of MHD patients infected with SARS-CoV-2.

  • Wang Li, Li Jingjing, Xu Jinghan, Bai Ying, Sun Yue, Duan Huikun, Sun Gege, Zhao Ganye, Kong Xiangdong
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    Objective To detect and analyze the gene variation types of 64 unrelated pedigrees affected with autosomal dominant polycystic kidney disease (ADPKD), and explore the detection efficiency of multiple gene analysis techniques and variation characteristics. Methods It was a cross-sectional study. The clinical data of 64 pedigrees with ADPKD from Nephrology Department or Genetic and Prenatal Diagnosis Center of the First Affiliated Hospital of Zhengzhou University from December 2017 to August 2020 were retrospectively analyzed. The blood samples of probands and other family members were collected. Genetic analysis was carried out by next generation sequencing, and suspected mutations were verified by multiplex ligation-dependent probe amplification, or long-range PCR combined with Sanger sequencing. Prenatal diagnosis for high-risk fetuses was performed by fetal villi or amniotic fluid cells after genotyping without maternal genomic DNA contamination. Results Among detected 64 pedigrees, 57 pedigrees (89.06%) had genetic variants in PKD1/PKD2. A total of 49 pathogenic/likely pathogenic variants in PKD1/PKD2 were identified in 51 pedigrees (79.69%), including 14 nonsense variants (28.57%), 14 frameshift variants (28.57%), 11 missense variants (22.45%), 5 splicing variants (10.20%) and 5 deletion variants (10.20%). Of these variants, 87.76% (43/49) were in PKD1 and 12.24% (6/49) were in PKD2. Totally, 14 novel variants in PKD1/PKD2 were identified, including 7 frameshift variants, 3 splicing variants, 2 nonsense variants, 1 deletion variant and 1 missense variant, of which 11 variants were in PKD1 and 3 variants were in PKD2. Twenty high-risk fetuses from 17 pedigrees received prenatal diagnosis, in whom 6 fetuses had PKD1 variation, and other 14 fetuses had no PKD1/PKD2-genetic variation. Conclusions The combination of next-generation sequencing, multiplex ligation-dependent probe amplification, and long-range PCR combined with Sanger sequencing can be helpful for rapid, efficient and accurate genetic diagnosis of ADPKD pedigrees. Point mutations are the most common types in PKD1/PKD2. Fourteen novel variants in PKD1/PKD2 extend its pathogenic variant spectrum and can provide basis for genetic counseling and prenatal diagnosis of ADPKD pedigrees.

  • Yang Sanju, Lin Na, Liu Yunguang, Lu Zhuangnian, Yang Lijuan, Liang Liting, Wei Aibo, Huang Yueyang, Huang Yunfeng
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    Objective To investigate the relationship between single nucleotide polymorphic (SNP) loci of PLCE1 gene and primary nephrotic syndrome (PNS) and its response to glucocorticoid therapy in Guangxi Zhuang children. Methods It was a retrospective cohort study. One hundred and fifty-five Guangxi Zhuang children with PNS in the Affiliated Hospital of Youjiang Ethnic Medical College from October 2020 to May 2022, and 100 healthy Zhuang children during the same period as controls were included. Four SNP loci including rs17109674, rs10786156, rs3740360 and rs2274224 of PLCE1 gene were selected and high-throughput sequencing was used to analyze the genotypes. Logistic regression analysis model was used to analyze the correlation between each SNP locus and onset of PNS and steroid-resistant nephrotic syndrome. The SHEsis online software was used to analyze the link disequilibrium of each SNP locus, and construct the haploid type. Results (1) Logistic regression analysis results showed that AC+CC genotype (AA as reference, OR=0.449, 95% CI 0.256-0.786, P=0.005), AC genotype (AA as reference, OR=0.354, 95% CI 0.188-0.667, P=0.001) and C allele gene (A as reference, OR=0.615, 95% CI 0.390-0.971, P=0.037) of rs3740360 were correlated with the risk of PNS in children. The genotypes and allele genes of rs17109674, rs10786156, rs3740360 and rs2274224 were not associated with the risk of steroid-resistant nephrotic syndrome in children (all P>0.05). (2) Strong linkage disequilibrium existed between rs10786156 and rs2274224 (D'=0.702, r2=0.484). rs17109674 and rs10786156 (D'=0.128, r2=0.007), rs17109674 and rs3740360 (D'=0.142, r2=0.007), rs17109674 and rs2274224 (D'=0.045, r2=0.001), rs10786156 and rs3740360 (D'=0.255, r2=0.023), and rs3740360 and rs2274224 (D'=0.281, r2=0.028) all had weak linkage disequilibrium. (3) The haploid AGCG (OR=0.282, 95% CI 0.079-1.008, P=0.038), GGCC (OR=0.327, 95% CI 0.111-0.967, P=0.034) and GGAG (OR=4.616, 95% CI 1.179-18.069, P=0.016) were all correlated with the risk of PNS in children. Conclusions AC genotype, AC+CC genotype, and C allele gene of rs3740360 SNP locus may reduce the risk of PNS in Guangxi Zhuang children. Haploid AGCG and GGCC may be associated with decreased incidence of PNS, while GGAG may be associated with increased incidence of PNS in Guangxi Zhuang children. The genotypes and alleles of 4 SNP loci are not associated with the risk of steroid-resistant nephrotic syndrome.

  • Pan Bin, Chen Siyu, Lei Xin, Chen Jianghua, Zhang Ping
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    Objective To investigate the graft survival rate after total parathyroidectomy (TPTX) plus forearm muscle auto-transplantation in patients with secondary hyperparathyroidism (SHPT) and evaluate the effect of graft survival status on calcium and phosphorus metabolism. Methods It was a retrospective cohort study. The end-stage renal disease patients who were diagnosed with SHPT and underwent TPTX plus forearm muscle auto-transplantation from November 2015 to December 2018 at the First Affiliated Hospital of Zhejiang University School of Medicine were enrolled. The clinical data including serum calcium, phosphorus, intact parathyroid hormone and alkaline phosphatase preoperative and postoperative 1 week, 1 month and 6 months, and cumulative requirements of calcium carbonate and calcitriol in postoperative 1 month and 6 months were collected. The graft survival rate was summarized and the differences of serum calcium, phosphorus, and supplementation dosage of calcium and calcitriol after surgery between the graft survival group and the graft non-survival group were compared. Results A total of 191 patients were included in the study, with 95 males (49.7%), and 172 patients of age <60 years old. There were 154 grafts surviving with a graft survival rate of 80.6%. There were no significant differences in the levels of serum calcium, phosphorus, alkaline phosphatase at 1 week, 1 month and 6 months after surgery, and cumulative dosage of calcium carbonate at 1 month and 6 months after surgery between the two groups (all P>0.05). The dose of calcitriol in the graft non-survival group was significantly higher than that in the graft survival group within 1 month after surgery [41.50 (30.00, 45.00) μg vs. 32.75 (25.50, 40.50) μg, Z=-2.307, P=0.021]. However, there was no significant difference in cumulative calcitriol supplementation between the two groups within 6 months after surgery (P>0.05). Conclusions The graft survival rate after TPTX plus forearm muscle auto-transplantation is high in SHPT patients. Within 6 months after surgery, there is no significant difference in serum calcium, phosphorus and cumulative supplemental doses of calcium and calcitriol between the graft survival and non-survival groups.

  • Short Original Article

  • Liu Bingyan, Cai Huacong, Zhou Zijuan, Wang Ying, Hu Yan, Chen Zhichun, Wang Haiyun, Chen Limeng
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    Renal replacement therapy and perioperative management have difficulties in hemophilia patients with end-stage renal disease. The paper summarized the diagnosis and treatment experience of six hemophilia patients complicated with end-stage renal disease from January 1, 2000 to March 31, 2023 in Peking Union Medical College Hospital. Among 6 patients treated with peritoneal dialysis, 3 were treated with hemodialysis or continuous venous-venous hemodialysis. Altogether 11 dialysis access procedures were conducted successfully, and no serious bleeding or thrombotic events. In further conjunction with literature review, the paper summarized the key points of dialysis access appliance relevant to such patients, to provide reference for renal replacement treatment paths.

  • Case Report

  • Chang Xiaoyan, Ge Shuwang
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    Parathyroid carcinoma (PC) is a rare endocrine malignancy. We report a case of lung metastases of PC in a maintenance hemodialysis patient with renal allograft loss and review the relevant literature. The patient developed hyperparathyroid hormone and hypercalcemia 4 years after hemodialysis treatment. CT examination showed that a round-shaped, soft tissue density shadow without typical enhancement was located in infero-posterior left lobe of thyroid gland. Dual-phase 99mTc-MIBI parathyroid imaging showed abnormal 99mTc-MIBI uptake behind the left lobe of the thyroid gland on both early and delayed imaging. The patient underwent subtotal parathyroidectomy under general anesthesia. Histopathological diagnosis was parathyroid adenoma and parathyroid hyperplasia. The serum parathyroid hormone decreased significantly, but remained above normal, and the serum calcium returned to normal range after the surgery. However, the parathyroid hormone level gradually increased several months after the surgery. 99mTc-MIBI parathyroid imaging showed multiple low-density nodules with increased uptake of 99mTc-MIBI in superior lobe of both lungs one year after the surgery. Ultimately, pulmonary metastasis of PC was confirmed by pathological examination.

  • Liu Ye, Sun Fuyun
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    Fusarium is a kind of conditional pathogenic fungus. There are few reports of peritonitis caused by Fusarium at home and abroad. This paper reports a case of Fusarium verticillioides peritonitis complicated with bacteremia and reviews relevant literature. The patient was admitted to the hospital due to fever and abdominal pain. After peritonitis was diagnosed, the initial treatment was vancomycin plus ceftazidime kept in the abdominal cavity every night for 3 days, but the effect was not good. The antibiotic was upgraded to vancomycin plus meropenem for 2 days, but still, there was no effect. Subsequently, Fusarium was found after ascites fluid culture, and the treatment regimen was changed to voriconazole antifungal therapy and hemodialysis. However, the patient developed a dialysis catheter-related infection. After the infection was cured, the fever was still repeated, and the infection indexes did not improve. Further, the pathogenic microorganism of whole blood infection was detected by high-throughput gene detection, and it was clinically confirmed as Fusarium verticillioides. At last, the patient recovered and was discharged from the hospital after treatment with voriconazole combined with amphotericin B. Fusarium is highly pathogenic, and it is hoped to attract more attention of clinicians.

  • Zhang Lina, Liu Juhong, Li Jia, Zhang Beibei, Geng Jian, Wu Shunan
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    The patient was a young woman with a history of syphilis, gross hematuria onset, chronic course, exacerbation in the second and third trimesters. The clinical manifestations were gross hematuria, nephrotic syndrome, rapidly progressive glomerulonephritis requiring dialysis, decreased complement C3 and C4, and monoclonal IgG-kappa deposition in blood and urine immunofixation electrophoresis. Membranous proliferative glomerulonephritis with crescent formation under renal pathological light microscopy, only single IgG3 deposition in the immunofluorescent IgG subtype, and restrictive expression of light chain kappa, were diagnosed as proliferative glomerulonephritis with monoclonal IgG (IgG3-kappa) deposits (PGNMID). The was the first case with a history of syphilis that worsened during pregnancy. After treatment with bortezomib, cyclophosphamide, and dexamethasone, the patient was finally released from dialysis, renal function recovered, and proteinuria improved.

  • Zhang Lingxia, Wang Jingjing, Jin Yanyan, Liu Fei, Fu Haidong, Chen Bingrong, Mao Jianhua
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    Systemic lupus erythematosus (SLE) is a serious autoimmune disease that affects multiple systems and organs throughout the body. Thrombotic microangiopathy (TMA) is a rare clinical syndrome that can lead to multiple organ dysfunction and even threaten life. Clinically, SLE patients with TMA are relatively rare, especially in children, but the mortality of SLE patients with TMA is significantly increased. The article reports 2 cases of successful remission of SLE complicated with TMA after treatment with eculizumab, and discusses the diagnosis, clinical manifestations and treatment of SLE complicated with TMA in children, so as to provide clinical basis for the diagnosis and treatment of SLE complicated with TMA in children.

  • Experience Exchange

  • Kailai Lau, Hu Shenling, Zhou Peiru, Huang Jiewei, Zhou Ge, Yu Huixia, Hu Bo
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    This study conducted a bibliometric analysis of the literature related to the field of hemodialysis arteriovenous fistula from the Web of Science database. The current and future research hotspots and development trends in this field were visualized by sorting and interpreting the research data and drawing a visualization chart. This study suggests that there are certain academic barriers between China and developed countries, and international multicenter clinical trials should be conducted in the future with early identification of arteriovenous fistula dysfunction and emerging diagnostic tools as the academic development direction.

  • Review

  • Xu Qiuyu, Chen Gang, Li Xuemei
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    Erythroferrone is a newly discovered and important factor regulating iron homeostasis and mainly produced by erythroblasts. Erythroferrone responds to the increase of erythropoietin and regulates plasma iron level, along with the absorption and utilization of iron via hepcidin, which plays an important role in the pathophysiology of iron metabolism-related diseases. Erythropoietin deficiency and iron metabolism disturbance are prominent features of renal anemia complicated with chronic kidney disease. Accordingly, erythroferrone is corresponding to the pathogenesis, treatment and prognosis of renal anemia. The in-depth study contributes to the further understanding of the mechanism related to iron metabolism disorder, and erythroferrone is also expected to be used as a valuable biomarker in the detection of renal anemia and the evaluation of therapeutic response. The article systematically reviews the physiological function of erythroferrone and its research progress in iron metabolism and renal anemia.

  • Wang Yanru, Zhou Xiaoshuang, Li Rongshan
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    In recent years, artificial intelligence has received extensive attention in the field of kidney pathology, such as identifying kidney tissue structure and evaluating the degree of lesions. Renal pathology is the gold standard for the diagnosis of renal diseases, and histochemistry staining is the prerequisite for the assessment of renal lesions. Renal biopsy is usually evaluated by various staining methods, including hematoxylin-eosin staining, periodic acid-Schiff reaction, Masson's trichrome staining and immunol staining, among that, different staining methods focus on different structures. The paper reviewed the application and progress of artificial intelligence in renal pathology, especially in different staining methods.

  • Guide Interpretation

  • Yang Xiao, Tian Na, Lu Wanhong, Chen Jin, Ye Zhiming, Dong Jie, Kai-ming Chow, Li Philip Kam-tao
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    Peritoneal dialysis (PD) catheter-related infections are important risk factors for catheter loss and peritonitis. 2023 International Society for Peritoneal Dialysis (ISPD) catheter-related infection recommendations have revised and clarified definitions and classifications of exit site infection and tunnel infection, such as cause-specific catheter-related infection, culture-negative catheter-related infection, refractory catheter-related infection, and infection- related catheter removal. A new target for the exit site infection rate should not exceed 0.40 episodes per year at risk. The recommendation about topical antibiotic cream or ointment to catheter exit site has been downgraded. New recommendations for exit site infection include clarified suggestion of exit site dressing cover and revised topical antibacterial agents as well as antibiotics treatment duration. In addition to catheter removal and reinsertion, new salvage options for catheter are suggested. The paper outlines the updated main content of the guide.