Systemic light chain (AL) amyloidosis is a rare plasma cell disease that often causes dysfunction of important organs such as heart and kidney. In recent years, some progress has been made in the field of molecular mechanism of AL amyloidosis, which helps us further understand structural characteristics of amyloid fibrils in AL amyloidosis and pathological mechanism of heart and kidney damage; In addition, there has been some advances in diagnosis and treatment of AL amyloidosis, which is of great significance for improving clinical practice of AL amyloidosis. This article will review recent progress in pathogenesis, diagnosis and treatment of AL amyloidosis.

Chronic kidney disease (CKD) has become a public health challenge worldwide and is closely associated with the risk of cardiovascular and other adverse events. How to identify the occurrence and progression of CKD early, stratify the risk of the population, and give early prevention and treatment is the focus of clinical attention. Metabolomics, a non-invasive, high-throughput assay elucidates the pathophysiological mechanisms of diseases by quantitative or semi-quantitative analysis of the abundance of metabolites and the metabolic pathways involved. Studies indicate that the metabolome of CKD patients can show significant differences with deteriorating renal function, and a variety of metabolites are closely associated with the deterioration of renal function, the risk of cardiovascular events, and even the risk of all-cause mortality in patients. Metabolomics has been successfully applied to improve the assessment of GFR and explore novel biomarkers for the risk of CKD and its complications. Furthermore, the application of metabolomics expands the understanding of the underlying pathophysiologic mechanisms of various complications during CKD and strengthens the understanding of the interactions of gut microbiome and their metabolites with CKD. This article reviews the research progression and application of metabolomics in CKD to provide novel directions for clinical diagnosis and treatment.

Sepsis is one of the most common causes of acute kidney injury (AKI) in clnical. Sepsis-associated AKI (SA-AKI) is difficult to detect in the early stage, and has a high mortality rate and poor prognosis. Its pathogenesis is complicated, with multiple factors involved,and its clinical phenotypes,disease progression, and treatment response are all potentially heterogeneous. Currently, there is no definite and effective means of intervention. This review summarized the pathophysiology of SAAKI, in order to provide a reference for further research.

This article introduces 4 national standards for the infrastructure construction and operation management of the biobank, GB／T 39766-2021Management specification for human biobank, GB／T 39767-2021 Management specification for human biomaterial, GB／T40364-2021 Fundamental terminology for human biobank and GB／T 39768-2021 Classification and coding for human biomaterial.We systematically describe the standardized organizational structure, personnel management, environmental facilities, reagents and consumables, information systems, sample management, etc. Through the 4 standards, we can strengthen the constructional standard of biobank, promote the standardization of management and operation, so as to offer samples of high quality to down-stream scientific research.

 This  case  reports  a  34⁃year⁃old  male  kidney  transplant  recipient  who  developed  systemic  herpet⁃like virus onset with severe liver damage, pancreatic  function damage, and transplanted kidney  function impairment, and was eventually  confirmed  as  visceral  disseminated  herpes  zoster  infection  by  varicella  zoster  virus  IgM  positive.  He  was successfully treated with peniclovir,intravenous hormone,and human immunoglobulin.

Diabetic nephropathy (DN) is one of the common microvascular complications of diabetes and the main cause of end-stage kidney injury. DN is an inflammatory and immune injury disease caused by metabolic factors, which is related to metabolic disorders,hemodynamic abnormalities, inflammation and other factors. The cyclin nucleotide adenine nucleotide synthase-interferon gene stimulator (cGAS-STING) pathway is a component of innate immunity. Stimulator of interferon gene (STING) is a kind of endoplasmic reticulum activating protein. It experiences dsDNA sensing (cGAS), intracellular signal transduction (STING, TBK1), immune response activation (IRF3, NF-κB), and participates in innate immune response and inflammatory response. Cgas-sting pathway plays an important role in infection, inflammation and tumor development, but its role in DN remains unclear. This article systematically summarizes the research progress of cGAS signaling pathway in the pathogenesis of DN in recent years.

Alternative polyadenylation (APA), as a crucial post-transcriptional regulatory mechanism, has been reported to generate distinct transcripts with variable 3′UTR lengths by selecting different polyadenylation sites (polyA sites, PAS) in 3′UTR. The cis-regulatory elements in pre-mRNA, corresponding trans-acting factors, as well as various enzymes and protein factors in the nucleus are involved in the regulation of APA. Many cis-regulatory elements, such as microRNA (miRNA) or RNA-binding protein (RBP) binding sites, are embedded in the 3′UTR sequence, therefore, the presence or absence of these cis-acting elements conferred by 3′UTR-APA has far-reaching effects on the stability, translation rate, nuclear export, cellular localization of target mRNAs, as well as proteins localization. APA-associated genetic variants have been proposed to affect diverse physiological and pathological processes. Here, we will systematically elaborate the regulatory mechanism of APA and its research progress in diabetic nephropathy.

Ｏｂｊｅｃｔｉｖｅ：To compare the 9 glomerular filtration rate (GFR) evaluation formulas proposed by foreign scholars with the 2 new GFR evaluation formulas for Chinese children proposed by Tang based on the data of 751 Chinese children with chronic kidney disease (CKD), and select the appropriate GFR evaluation formula for Chinese children with CKD.
Ｍｅｔｈｏｄｏｌｏｇｙ：The inpatient data of children with CKD in the Department of Pediatric Renal Rheumatology and Immunology of Shengjing Hospital of China Medical University from January 1, 2011 to December 31, 2021 were analyzed retrospectively. Take the GFR measured by 99mTcDTPA renal dynamic imaging (iGFR) as the gold standard, and compare the deviation, precision and accuracy between iGFR and eGFR estimated by each formula; Compare the accuracy of each formula for GFR evaluation.
Ｒｅｓｕｌｔｓ：949 children with CKD (547 males, 58％) were included. Among male and female children, regardless of puberty, the accuracy of GFR evaluation formula 2 for Chinese children proposed by Tang is the highest. Among male children, formula 1 of CKD 1 is the most accurate, formula 2 of CKD 2~4 is the most accurate, and formula C-B of CKD 5 is the most accurate; Among female children, the accuracy of CKD 1 formula 1 is the highest, CKD 2~3 formula 2 is the highest, CKD 4 C-G formula and Filler formula are the highest, and CKD 5 CKD-EPI-cysC formula is the highest; Taking iGFR<60 mL／(min·1.73m2) as the diagnostic standard of CKD, formula 2 has the highest accuracy in diagnosing CKD.
Ｃｏｎｃｌｕｓｉｏｎ：The GFR evaluation formula 2 for Chinese children proposed by Tang is recommended as the first GFR evaluation formula for Chinese CKD children.

Ｏｂｊｅｃｔｉｖｅ：To investigate clinicopathological features and prognosis of patients with acute phosphate nephropathy (APN) caused by oral sodium phosphate salts.
Ｍｅｔｈｏｄｏｌｏｇｙ：We retrospectively analyzed patients diagnosed with APN by renal biopsy at the National Clinical Research Center of Kidney Diseases, Jinling Hospital from January 2010 to November 2022, and analyzed their clinical manifestations, pathological features, treatment and prognosis.
Ｒｅｓｕｌｔｓ：A total of 9 patients with APN, including 7 males, aged 6011±914 years at the time of renal biopsy, were included. The patients were all taking oral sodium phosphate for the need of colonoscopy, and the serum creatinine (SCr) levels were in the normal range, but with a median presence of 3 highrisk factors. At the time of diagnosis of acute kidney injury (AKI), SCr was 25724±6365 μmol／L; except for one case with elevated blood phosphorus, the rest of the patients had normal blood calcium and phosphorus. Urinalysis was free of erythrocytes, and only 2 cases showed a small amount of proteinuria. Six patients were treated with prednisone (15~30 mg／d). At a median followup of 14 months, renal function improved in 7 patients, but none of the SCr returned to the normal range. SCr at the last followup was 17592±4505 μmol／L and the mean eGFR was 3633±1283 ml／(min·173 m2).
Ｃｏｎｃｌｕｓｉｏｎ：APN due to oral sodium phosphate salts usually has an insidious onset, and renal biopsy can help clarify the diagnosis. Phosphate lavage should be used with caution in patients with highrisk factors, and changes in renal function should be monitored closely for early recognition and management.

During the past decade, rapidly expanding highthroughput single cell omics technologies facilitated a lot of considerable breakthroughs in the field of kidney research. Based on the huge amounts of data generated by single cell omics technology, researchers may discover new cell types,identify the dynamics of cell state and gene expression patterns, explore the crosstalking between different cell types and deeply understand the pathogenesis and progression of diseases. In this review, we summarized the types of current singlecell omics approach,analyzed the benefits and drawbacks of each important omics technology, and probed into the applications and findings of single cell omics in the field of renal pathophysiology. In the end, we also prospected the application of single cell omics in experimental study and targeted therapy of kidney diseases in the future.

Podocytes are derived from nephron progenitor cells， and form the outer layer of glomerular filtration barrier by forming foot processes and slit diaphragm. Podocytopathies are caused by injury or dysfunction of podocytes. Explicating the regulation mechanism of podocyte genesis is helpful to understand the pathogenesis of podocytopathies， as well as to develop targeted clinical pathways. One of the frontiers in the field of podocytopathy research is to construct the disease model using organoids or induced differentiation techniques. This review focuses on the progress in podocyte genesis studies and the regenerative medicine researches about podocytopathy.

Ｏｂｊｅｃｔｉｖｅ：To analyze clinical and genetic features of a patient with lupus nephritis due to DNASE1L3 mutation.
Ｍｅｔｈｏｄｏｌｏｇｙ：Whole exome sequencing was carried out to detect genetic variants of a patient with lupus nephritis and pulmonary haemorrhage. The clinical characteristics and pathogenic variants spectrum of patients with lupus nephritis due to DNASE1L3 deficiency were summarized.
Ｒｅｓｕｌｔｓ：A male patient with lupus nephritis, onset at the age of 23. He presented with massive proteinuria and microscopic hematuria. Kidney biopsy showed lupus nephritis type V+IV. Other manifestations include pulmonary haemorrhage, severe anemia, urticarialike rash and arthritis. Whole exome sequencing detected a novel pathogenic homozygous variant c.565_568del (p.D189Sfs*17) in DNASE1L3 gene. The literature review of 25 reported cases of DNASE1L3 deficiency causing lupus nephritis, combined with present case, a total of 9 pathogenic variants in DNASE1L3 gene were identified. 53.8% were male, 786％ had hematologic involvement, 71.4％ had joint involvement, 71.4％ had urticaria, 42.9％ had pulmonary involvement, 357％ had gastrointestinal involvement, 21.4％ had ocular involvement, 21.4％ had cardiac involvement, and 57.7％ had positive ANCA, 50％ had poor prognosis.
Ｃｏｎｃｌｕｓｉｏｎ：DNASE1L3 deficiency is more frequent in men with lupus nephritis. Patients with lupus nephritis due to DNASE1L3 deficiency are susceptible to extra-renal involvement of the haematological system, joints, skin, lung and digestive system,as well as positive ANCA. Patients with lupus nephritis with positive ANCA or pulmonary haemorrhage should be carefully questioned about family history. Timely genetic testing should also be performed to consider the possibility of DNASE1L3 deficiency.

Chronic kidney disease (CKD) is widely recognized as one of the leading causes of death worldwide. In order to reduce the incidence of CKD and slow its progression, effective diagnosis, monitoring, treatment, and even preventive measures are urgently needed. In recent years, several large clinical studies have confirmed that sodium glucose transporter 2 inhibitors (SGLT2i) have a renoprotective effect in CKD patients, and can significantly improve the long-term prognosis of CKD patients, but the exact mechanism is still unclear. Exploring the intrinsic mechanism could provide new target strategies for delaying CKD progression. Here, we intend to discuss the mechanism of renal injury in CKD mediated by the Warburg effect related to fructose metabolism and explore the effects of SGLT2i on the Warburg effect.

Ｏｂｊｅｃｔｉｖｅ：To investigate the role and possible mechanism of Piezo 1 in LPS-induced sepsis-associated acute kidney injury (SA-AKI).
Ｍｅｔｈｏｄｏｌｏｇｙ：Establish in vivo and in vitro models of SA-AKI in mice and human proximal renal tubular epithelial cells (HK-2 cells) by LPS, and immunofluorescence and Western blot were used to detect Piezo 1 expression. GsMTx4 was injected into SA-AKI mice, and the renal pathology and renal function were detected. Piezo 1 siRNA or Yoda 1 were used to intervene in LPS-induced HK-2 cells, then the expressions of renal tubular injury markers, inflammatory factors and apoptosis-related marker proteins were detected by Western blot and RT-qPCR, inflammatory factors were detected by ELISA and apoptosis rate was detected by flow cytometry. After treated with Piezo 1 siRNA, the intracellular Ca2+ concentration was detected with a Fluo-4 AM calcium ion fluorescent probe and the expressions of Calpain 2 and Integrin β1 were detected. Integrin β1 expression was detected after PD151746 intervention. After Yoda1 and PD151746 simultaneously intervened in LPS-induced cells, inflammatory factors were detected by ELISA and apoptosis rate was detected by flow cytometry.
Ｒｅｓｕｌｔｓ：Piezo 1 expression was upregulated in the kidneys of mice with SA-AKI and HK-2 cells induced by LPS. GsMTx4 ameliorated renal pathology and renal function damage in SA-AKI mice. Silencing Piezo 1 reduced LPS-induced HK-2 cell damage, inflammation, and apoptosis; however, the above damage was further exacerbated after Yoda 1 intervention. Mechanistically, Piezo 1 knockdown reduced LPS-induced intracellular Ca2+ and the expression of Calpain 2 and Integrin β1. PD151746 reduced the expression of integrin β1 and inhibited inflammation and apoptosis caused by Yoda 1.
Ｃｏｎｃｌｕｓｉｏｎ：Piezo 1 expression is upregulated in renal tubular epithelial cells with SA-AKI, possibly causes inflammation and apoptosis by activating the Ca2+/Calpain 2/Integrin β1 pathway, and which is involved in the progression of SA-AKI.

Ｏｂｊｅｃｔｉｖｅ：To explore the application of artificial intelligence-based analytic renal pathology system (ARPS) in patients with primary membranous nephropathy (PMN).
Ｍｅｔｈｏｄｏｌｏｇｙ：Patients with biopsy-proven PMN in our center from January 2018 to December 2019 were enrolled. Their clinical and pathological data were collected. ARPS was applied to identify glomeruli lesions including global glomerular sclerosis (GS), segmental glomerular sclerosis (SS), crescents (C), and none of the above (NOA), and then quantify intraglomerular features, including intrinsic glomerular cells (M: mesangial cells; E: endothelial cells; P: podocytes) and glomerular area. The performance of ARPS was evaluated. The relationships between ARPSbased intraglomerular features and prognosis were further analyzed.
Ｒｅｓｕｌｔｓ：A total of 123 patients (65.0％ males) were included. The average age was 47.1±14.0 years at biopsy. For the identification of glomerular lesions, ARPS achieved the best effect in NOA with 0.967 F1-score; followed by GS with 0.811 F1-score and SS with 0.545 F1-score. The F1-scores of ARPS on identifying intrinsic cells were greater than 0.950. The glomerular area was larger in no response (NR) patients than that in partial remission (PR) and complete remission (CR) patients; the number, density and ratio of podocytes were higher in CR patients than that in PR and NR patients. The remission rate of urinary protein was higher in patients with higher podocytes number or density than that in patients with lower podocytes number or density. Renal phospholipase A2 receptor (PLA2R) and podocyte number were independent predictors of urinary protein remission.
Ｃｏｎｃｌｕｓｉｏｎ：ARPS performed well in the identification of glomerular lesions and intrinsic cell types in PMN. The ARPSbased intraglomerular characteristics were significantly correlated with prognosis in PMN patients.

The conduct of all the biomedical research involving human subjects must pass ethical review. With the introduction of various administrative approach and legal documents, the scope of ethical review is gradually becoming clear. This article focuses on principles of ethical review,standard of approval,review material preparation and legal liability,etc, offering help for researchers to carry out clinical and basic research in accordance with the ethical requirements.

Ｏｂｊｅｃｔｉｖｅ：To investigate the relationship of renal fractional excretion of solutes or electrolytes (FEx) and glomerular filtration rate (GFR) in chronic kidney disease (CKD) patients, and compare FEx in CKD patients with in acute kidney injury (AKI).
Ｍｅｔｈｏｄｏｌｏｇｙ：From January 2020 to August 2022, patients diagnosed as CKD or AKI in National Clinical Research Center of Kidney Diseases were retrospectively screened of whom with full records of blood and urine biochemical tests. Estimated GFR (eGFR) and fractional excretion of sodium (FENa), potassium (FEK), chloride (FECl), phosphorus (FEP), calcium (FECa), urea (FEUN) and uric acid (FEUA) were calculated. The relationship between FEx and eGFR in CKD was analyzed. Comparisons of FEx in AKI and CKD patients were made after propensity score matching (PSM).
Ｒｅｓｕｌｔｓ：261 CKD patients and 53 AKI patients were included in the study. Most of FEx increases significantly with the progression of CKD stage. A close relationship of FEx (FENa、FEK、FECl、FEP and FEUA) and eGFR was found. The ratio of individual FEx and the mean value of FEx in CKD stage 1 patients was calculated as relative FEx (RFEx), and nonlinear curvefitting equations of eGFRRFEx were established in Na, K, Cl, P, and UA. Scatter plots of eGFRFEx in AKI patients show similar patterns as seen in CKD patients. With a much lower eGFR in AKI group, significant differences were found in all FEx between AKI and CKD group. After PSM focusing on sex, age and eGFR, a significant difference between AKI and CKD group was only seen in FEUN in patients with eGFR≥60 mL/(min·173m2), and FEUA in patients with eGFR<60 mL/(min·173m2). The optimal cutoff value to distinguish AKI from CKD in patients with eGFR≥60 mL/(min·173m2) is FEUN<1295％ (AUC 065, sensitivity 700％, specificity 571％); while in patients with eGFR<60 mL/(min·173m2), it is FEUA>1141％ (AUC 074, sensitivity 758％, specificity 735％).
Ｃｏｎｃｌｕｓｉｏｎ：eGFR has a distinct impact on FEx, with most of FEx increase progressively along with the decline of eGFR regardless of CKD or AKI. It has a diagnostic value for FEUN in patients with eGFR≥60 mL/(min·173m2) and for FEUA in patients with eGFR<60 mL/(min·173m2) to distinguish AKI from CKD.

A 19-year-old female patient was referred to our hospital with asymptomatic proteinuria, normal renal function, hyperuricemia. Extrarenal manifestations included short stature, diabetes mellitus, hearing loss, pre-excitation syndrome. Her mother was also short stature. The kidney biopsy revealed focal segmental glomerulosclerosis (FSGS), with dysmorphic abnormal mitochondria in the podocytes, parietal epithelial cells and renal tubular epithelial cells on electron microscopy. Gene testing revealed a m.3243A>G mutation in mitochondrial MT-TL1 gene, maternal inherited. The final diagnosis was mitochondrial disease associated with m.3243A>G mutation.

Mitochondria play a crucial role in maintaining the normal function of kidney resident cells. Hyperglycemia resulting from diabetes can disrupt mitochondrial homeostasis and increase oxidative stress, leading to damage in kidney cells and contributing to the development of diabetic nephropathy. Therefore, the development of small molecule drugs that target mitochondrial homeostasis imbalance could offer new treatment options for diabetic nephropathy. This paper will provide a comprehensive review of recent research advancements in drugs targeting mitochondrial homeostasis, mitochondrial biogenesis and mitochondrial antioxidants.

 Chronic kidney disease-mineral and bone  disorder  （CKD-MBD） refers  to  a  syndrome  of  mineral  and bone metabolism abnormalities caused by chronic kidney disease.  It can affect the  cardiovascular  system and bone  tissue， eventually leading to damage to multiple organs  and systems  throughout  the  body， making  it  a  significant  cause  of  patient mortality. Hyperphosphatemia is the initiating factor of CKD-MBD， making the  management  of  blood phosphorus  levels  in dialysis patients extremely important; however， the rate of achieving target blood phosphorus levels in these patients is not high. In recent years， a series of new phosphate-lowering drugs have been developed based on new mechanisms， including iron-based  phosphate   binders，   calcium-magnesium   composite   phosphate   binders，   and   niacinamide，   among   others. Additionally， there  are  some  novel  phosphate-lowering  drugs  undergoing  clinical  trials.  This  article  reviews  the  current status of  blood  phosphorus  management  in  dialysis  patients  and  the  effects  and  characteristics  of  these  new  phosphate- lowering drugs.