24,589 results match your criteria Nephrotic Syndrome


Fanconi syndrome in an elderly patient with membranous nephropathy during treatment with the immunosuppressant mizoribine.

CEN Case Rep 2022 Jun 24. Epub 2022 Jun 24.

Department of Nephrology, Faculty of Medical Sciences, University of Fukui, 23-3 Shimoaizuki, Matsuoka, Eiheiji-cho, Yoshida-gun, Fukui, 910-1193, Japan.

We report on an 80-year-old man diagnosed with Fanconi syndrome induced by mizoribine after 4 weeks of administration to treat membranous nephropathy. Mizoribine is an oral immunosuppressant that inhibits inosine monophosphate dehydrogenase and is widely used in Japan for the treatment of autoimmune diseases and nephrotic syndrome, as well as after renal transplantation. Acquired Fanconi syndrome is often caused by drugs (antibacterial, antiviral, anticancer, and anticonvulsant drugs) and is sometimes caused by autoimmune diseases, monoclonal light chain-associated diseases, or heavy metal poisoning. Read More

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The association between hypogammaglobulinemia severity and infection risk in rituximab-treated patients with childhood-onset idiopathic nephrotic syndrome.

Pediatr Nephrol 2022 Jun 24. Epub 2022 Jun 24.

Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-ku, Tokyo, 157-853, Japan.

Background: Hypogammaglobulinemia is a major adverse effect from rituximab. However, the association between rituximab-induced hypogammaglobulinemia and infection frequency is unknown.

Methods: Patients who received rituximab for complicated nephrotic syndrome between February 2006 and October 2020 were enrolled in this retrospective observational study. Read More

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A rare cause of nephrotic syndrome-sphingosine-1-phosphate lyase (SGPL1) deficiency: 6 cases and a review of the literature.

Pediatr Nephrol 2022 Jun 24. Epub 2022 Jun 24.

Department of Pediatric Nephrology, Faculty of Medicine, Hacettepe University, Sihhiye, 06100, Ankara, Turkey.

Background: Recently, recessive mutations in SGPL1 (sphingosine-1-phosphate lyase), which encodes the final enzyme of sphingolipid metabolism, have been reported to cause steroid-resistant nephrotic syndrome, adrenal insufficiency, and many other organ/system involvements. We aimed to determine the clinical and genetic characteristics, and outcomes in patients with SGPL1 mutations.

Methods: The study included 6 patients with bi-allelic SGPL1 mutation. Read More

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Biomarkers in Primary Focal Segmental Glomerulosclerosis in Optimal Diagnostic-Therapeutic Strategy.

J Clin Med 2022 Jun 8;11(12). Epub 2022 Jun 8.

Department of Nephrology and Transplantation Medicine, Wroclaw Medical University, 50-556 Wroclaw, Poland.

Focal segmental glomerulosclerosis (FSGS) involves podocyte injury. In patients with nephrotic syndrome, progression to end-stage renal disease often occurs over the course of 5 to 10 years. The diagnosis is based on a renal biopsy. Read More

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Membranous Nephropathy Secondary to Graves' Disease: A Case Report.

Front Immunol 2022 6;13:824124. Epub 2022 Jun 6.

Pathology Division, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.

Membranous nephropathy (MN) is a form of kidney disease that is idiopathic in 70%-80% of cases. Glomerular involvement in autoimmune thyroiditis can occur in 10%-30% of patients, and MN manifests in association with Hashimoto thyroiditis in up to 20% of the cases with glomerular involvement. Reports of MN associated with Graves' disease (GD) are extremely rare in the current literature. Read More

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Nephrotic Syndrome Complications - New and Old. Part 1.

Maedica (Bucur) 2022 Mar;17(1):153-168

"Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.

Nephrotic syndrome is a rare condition with an incidence of 2-7 cases/100.000 children per year and three new cases/100.000 adults per year. Read More

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Economic Evaluation of Using Daily Prednisolone versus Placebo at the Time of an Upper Respiratory Tract Infection for the Management of Children with Steroid-Sensitive Nephrotic Syndrome: A Model-Based Analysis.

Pharmacoecon Open 2022 Jun 22. Epub 2022 Jun 22.

Department of Paediatric Nephrology, Nottingham Children's Hospital, Nottingham, UK.

Background: Childhood steroid-sensitive nephrotic syndrome is a frequently relapsing disease with significant short- and long-term complications, leading to high healthcare costs and reduced quality of life for patients. The majority of relapses are triggered by upper respiratory tract infections (URTIs) and evidence shows that daily low-dose prednisolone at the time of infection may reduce the risk of relapse.

Objective: The aim of this study was to assess the cost effectiveness of a 6-day course of low-dose prednisolone at the start of a URTI when compared with placebo. Read More

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A Case of Chronic Graft-versus-host Disease-associated Membranous Nephropathy Following Bone Marrow Transplantation, Successfully Treated with Rituximab.

Intern Med 2022 Jun 21. Epub 2022 Jun 21.

Department of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Japan.

A 67-year-old woman who had undergone bone marrow transplantation 2 years previously for acute myeloid leukemia (AML) developed complications of chronic graft-versus-host disease (cGVHD). She thereafter also developed nephrotic syndrome, and membranous nephropathy (MN) was diagnosed by a renal biopsy. Although the causative antigens of the MN were not detected, immunofluorescence staining showed codominant deposition of immunoglobulins G2 and G3, a finding indicating secondary MN, thereby suggesting an association between MN and cGVHD. Read More

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Predicting Future Outcomes from Kidney Biopsies with MCD/FSGS Lesions: Opportunities and Limitations.

J Am Soc Nephrol 2022 Jun 21. Epub 2022 Jun 21.

Institute of Pathology, Department of Nephrology, RWTH University Hospital Aachen, Aachen, Germany

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Focal segmental glomerulosclerosis and proteinuria associated with Myo1E mutations: novel variants and histological phenotype analysis.

Pediatr Nephrol 2022 Jun 20. Epub 2022 Jun 20.

Department of Pathology, Atrium Health Wake Forest Baptist Medical Center, Winston-Salem, NC, USA.

Background: Pathogenic mutations in the non-muscle single-headed myosin, myosin 1E (Myo1e), are a rare cause of pediatric focal segmental glomerulosclerosis (FSGS). These mutations are biallelic, to date only reported as homozygous variants in consanguineous families. Myo1e regulates the actin cytoskeleton dynamics and cell adhesion, which are especially important for podocyte functions. Read More

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Network Pharmacology and In Vivo Experimental Validation to Uncover the Renoprotective Mechanisms of Fangji Huangqi Decoction on Nephrotic Syndrome.

Evid Based Complement Alternat Med 2022 8;2022:4223729. Epub 2022 Jun 8.

Department of Nephrology, Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University (Hangzhou Hospital of Traditional Chinese Medicine), Hangzhou 310007, China.

Background: Fangji Huangqi decoction (FHD) is a traditional Chinese medicine formula that has the potential efficacy for nephrotic syndrome (NS) treatment. This study aims to explore the effects and underlying mechanisms of FHD against NS via network pharmacology and in vivo experiments.

Methods: The bioactive compounds and targets of FHD were retrieved from the TCMSP database. Read More

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A critical role of the podocyte cytoskeleton in the pathogenesis of glomerular proteinuria and autoimmune podocytopathies.

Acta Physiol (Oxf) 2022 Jun 18:e13850. Epub 2022 Jun 18.

National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China.

Selective glomerular filtration relies on the membrane separating the glomerular arterioles from the Bowman space. As a major component of the glomerular filtration barrier, podocytes form foot processes by the actin cytoskeleton, which dynamically adjusts in response to environmental changes to maintain filtration barrier integrity. The slit diaphragms bridge the filtration slits between neighboring foot processes and act as signaling hubs interacting with the actin cytoskeleton. Read More

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Inferior Vena Cava Collapsibility Index: A Precise, Noninvasive Tool for Evaluation of Edema in Children with Nephrotic Syndrome.

Indian J Pediatr 2022 Jun 17. Epub 2022 Jun 17.

Pediatric Nephrology Unit, Pediatrics Department, Mansoura University Children's Hospital, Faculty of Medicine, Mansoura University, Mansoura, 35516, Egypt.

This study aimed to evaluate available volume status assessment tools in nephrotic syndrome (NS). Sixty children with INS were subdivided into hypovolemic and nonhypovolemic groups based on fractional excretion of sodium (FeNa%); all were studied for inferior vena cava collapsibility index (IVCCI), plasma atrial natriuretic peptide (ANP), and body composition monitor (BCM). Forty-four patients had nonhypovolemic and 16 had hypovolemic states. Read More

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Nephrotic Syndrome and Renin Angiotensin System: pathophysiological role and therapeutic potential.

Curr Mol Pharmacol 2022 Jun 16. Epub 2022 Jun 16.

Departamento de Pediatria, Unidade de Nefrologia Pediátrica, Faculdade de Medicina, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Minas Gerais, Brazil.

Idiopathic Nephrotic Syndrome (INS) is the most frequent etiology of glomerulopathy in pediatric patients and one of the most common causes of chronic kidney disease (CKD) and end-stage renal disease (ESRD) in this population. In this review, we aimed to summarize evidence on the pathophysiological role and therapeutic potential of the Renin Angiotensin System (RAS) molecules for the control of proteinuria and for delaying the onset of CKD in patients with INS. This is a narrative review in which the databases PubMed, Web of Science, and SciELO were searched for articles about INS and RAS. Read More

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A possible role of classical complement pathway activation in the pathogenesis of immunoglobulin G nephropathy: a case report.

CEN Case Rep 2022 Jun 16. Epub 2022 Jun 16.

Department of Nephrology, Nippon Medical School, 1-1-5, Sendagi, Bunkyo-ku, Tokyo, 113-8602, Japan.

Immunoglobulin G (IgG) nephropathy refers to a rare group of diseases characterized by deposits of IgG in the mesangial region. However, IgG nephropathy is controversial as a single disease entity, and its pathogenesis remains to be elucidated. In the present report, we discuss a case of IgG nephropathy in which we observed activation of the classical complement pathway. Read More

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Isolated steroid-resistant nephrotic syndrome in a Chinese child carrying a de novo mutation in WT1 gene:a case report and literature review.

BMC Pediatr 2022 Jun 16;22(1):349. Epub 2022 Jun 16.

Department of Pediatrics, Shunde Women and Children's Hospital of Guangdong Medical University (Maternity and Child Healthcare Hospital of Shunde Foshan), Foshan, Guangdong Province, China.

Background: Isolated steroid-resistant nephrotic syndrome (ISRNS) is caused by mutations in the Wilms' tumor-1 (WT1) gene, which encodes glomerular podocytes and podocyte slit diaphragm.We report a novel 8-year-old female patient with ISRNS carrying a de novo missense mutation in WT1 gene and presenting a new type of pathology, have never been reported.We also systematically review previous reports of ISRNS in Chinese children. Read More

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Sequential Stem Cell-Kidney Transplantation in Schimke Immuno-osseous Dysplasia.

N Engl J Med 2022 06;386(24):2295-2302

From the Division of Hematology, Oncology, Stem Cell Transplantation, and Regenerative Medicine (A.B., K.W., R.P., K.M.K., G.B., R.A., P.F.S., A.J.S., M.G.R.), the Center for Definitive and Curative Medicine (A.B., K.W., R.P., K.M.K., G.B., R.A., P.F.S., A.J.S., M.G.R.), and the Divisions of Nephrology (P.C.G., W.C.) and Allergy, Immunology, and Rheumatology (E.L., G.D., V.R., J.M.S., D.B.L.), Department of Pediatrics, and the Departments of Surgery (A.G., W.C.) and Pediatrics (W.C.), Stanford University School of Medicine, and Department of Pharmacy (S.G.G.), Stanford Children's Health - both in Stanford, CA; the Divisions of Pediatric Nephrology (S.F.-S.) and Pediatric Allergy and Immunology (T.P.A.), Department of Pediatrics, University of Alabama, Birmingham; the Division of Nephrology, Department of Pediatrics, Oregon Health Sciences University, Portland (A. A.-U.); the Terry Fox Laboratory, BC Cancer Agency, Vancouver, Canada (G.A.); and the Department of Clinical Pharmacy, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands (K.E.).

Lifelong immunosuppression is required for allograft survival after kidney transplantation but may not ultimately prevent allograft loss resulting from chronic rejection. We developed an approach that attempts to abrogate immune rejection and the need for post-transplantation immunosuppression in three patients with Schimke immuno-osseous dysplasia who had both T-cell immunodeficiency and renal failure. Each patient received sequential transplants of αβ T-cell-depleted and CD19 B-cell-depleted haploidentical hematopoietic stem cells and a kidney from the same donor. Read More

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Venous Thromboembolism in Kidney Diseases and Genetic Predisposition.

Kidney Dis (Basel) 2022 May 11;8(3):181-189. Epub 2022 Apr 11.

Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Background: Many renal diseases have been associated with profound clinical effects on thrombosis. To our knowledge, patients with nephrotic syndrome (NS) and chronic kidney disease (CKD) display an elevated risk of vein thrombosis, which is among the common causes of mortality in patients with renal diseases. In addition, venous thrombosis, as a complication, has also been reported in a variety of other renal diseases such as glomerulonephritis without the NS, hypertensive nephropathy, and polycystic kidney disease. Read More

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Clinicopathological Spectrum of Cryoglobulinemic Glomerulonephritis without Evidence of Autoimmunity Disorders: A Retrospective Study from a Single Institute of China.

Kidney Dis (Basel) 2022 May 25;8(3):253-263. Epub 2022 Mar 25.

Renal Division, Department of Medicine, Peking University First Hospital, Institute of Nephrology, Peking University, Renal Pathology Center, Institute of Nephrology, Peking University, Key Laboratory of Renal Disease, Ministry of Health of China, Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, China.

Background: Cryoglobulinemic glomerulonephritis (Cryo-GN), caused by circulating cryoglobulins, has varied etiology and clinical-pathologic manifestations. This study aimed to investigate the clinicopathological spectrum and outcome of patients with various Cryo-GN in China.

Methods: A retrospective review of 74 Chinese patients with biopsy-proven cryoglobulin-related renal lesions in Peking University First Hospital from 2010 to 2020 was performed. Read More

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Polyclonal Immunotactoid Glomerulopathy Associated with Monoclonal Gammopathy of IgM Type and Underlying Plasmacellular Disease: Successful Treatment with Rituximab Alone.

Case Rep Nephrol Dial 2022 Jan-Apr;12(1):63-72. Epub 2022 Apr 25.

Division of General Internal Medicine, Department of Internal Medicine D, Nephrology and Rheumatology, University Hospital Münster, Münster, Germany.

Immunotactoid glomerulopathy (ITG) occurs infrequently and is characterized by organized IgG containing deposits. It most usually manifests as a concomitant disease of a broad spectrum of oncologic entities. We here present an exceptional case of ITG without glomerular light chain restriction secondary to a IgM kappa type monoclonal gammopathy of undetermined significance. Read More

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Diagnosis of monoclonal immunotactoid glomerulopathy with positive λ chain by immunoelectron microscopy.

CEN Case Rep 2022 Jun 14. Epub 2022 Jun 14.

Division of Endocrinology, Metabolism and Nephrology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.

We report the case of a 73-year-old-man who developed immunotactoid glomerulopathy (ITG). ITG is a rare disease characterized by proliferative glomerulonephritis and capillary wall deposits with a 10-60 nm diameter microtubular substructure. In monoclonal ITG, immunofluorescence analysis typically exhibits IgG with light chain restriction. Read More

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Clinical relevance of glomerular C4d deposition in children with early IgA nephropathy or Henoch-Schönlein purpura nephropathy.

Pediatr Nephrol 2022 Jun 13. Epub 2022 Jun 13.

Department of Nephrology, Chinese PLA General Hospital, Chinese PLA Institute of Nephrology, State Key Laboratory of Kidney Diseases, National Clinical Research Center for Kidney Diseases, 28th Fuxing Road, Beijing, 100045, China.

Background: Both IgA nephropathy (IgAN) and Henoch-Schönlein purpura nephropathy (HSPN) are characterized by glomerular mesangial IgA deposition. Several large studies on adults have suggested that glomerular C4d deposition has prognostic value in IgAN. However, there are few relevant studies on the clinical value of C4d deposition in children with IgAN or HSPN. Read More

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Predictors of early remission of proteinuria in adult patients with minimal change disease: a retrospective cohort study.

Sci Rep 2022 Jun 13;12(1):9782. Epub 2022 Jun 13.

Department of Nephrology, Kasugai Municipal Hospital, 1-1-1 Takakicho, Kasugai, Aichi, 486-8510, Japan.

Previous studies reported conflicting results regarding an association between serum albumin concentration and the cumulative incidence of remission of proteinuria in adult patients with minimal change disease (MCD). The present study aimed to clarify the clinical impact of serum albumin concentration and the cumulative incidence of remission and relapse of proteinuria in 108 adult patients with MCD at 40 hospitals in Japan, who were enrolled in a 5-year prospective cohort study of primary nephrotic syndrome, the Japan Nephrotic Syndrome Cohort Study (JNSCS). The association between serum albumin concentration before initiation of immunosuppressive treatment (IST) and the cumulative incidence of remission and relapse were assessed using multivariable-adjusted Cox proportional hazards models. Read More

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The role of ABCB1 gene polymorphisms in steroid-resistant nephrotic syndrome: Evidence from a meta-analysis of steroid-receiving patients.

J Gene Med 2022 Jun 13:e3436. Epub 2022 Jun 13.

Department of Pharmacy, Faculty of Science, Noakhali Science and Technology University, Noakhali, Bangladesh.

Previous studies in nephrotic syndrome have shown that three common variants in the ABCB1 gene, including rs1128503, rs2032582, and rs1045642, change the expression and activity of ABCB1, which may be responsible for the drug resistance. However, as a result of the inconclusive outcomes of these studies, we performed a meta-analysis to validate the association between ABCB1 polymorphisms and the susceptibility of steroid-resistant nephrotic syndrome (SRNS). The association was evaluated by calculating the odds ratio (OR) and 95% confidence interval. Read More

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A 16-month-old boy with nephrotic syndrome of apparent idiopathic origin: Answer.

Pediatr Nephrol 2022 Jun 13. Epub 2022 Jun 13.

Nephrology Unit, Hospital General de Niños Pedro de Elizalde, Montes de Oca 40, 1270, Buenos Aires, Argentina.

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A 16-month-old boy with nephrotic syndrome of apparent idiopathic origin: Question.

Pediatr Nephrol 2022 Jun 13. Epub 2022 Jun 13.

Nephrology Unit, Hospital General de Niños Pedro de Elizalde, Montes de Oca 40, 1270, Buenos Aires, Argentina.

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Chronic Urticaria in a Child With Nephrotic Syndrome: A Double Whammy!

Indian Pediatr 2022 Jun;59(6):505-506

Department of Dermatology, Venereology and Leprology, Sardar Patel Medical College, Bikaner, Rajasthan.

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Toxic Nephropathy Secondary to Chronic Mercury Poisoning: Clinical Characteristics and Outcomes.

Kidney Int Rep 2022 Jun 18;7(6):1189-1197. Epub 2022 Mar 18.

Department of Occupational Medicine and Clinical Toxicology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, People's Republic of China.

Introduction: Kidney disease secondary to mercury poisoning has not been well documented and is often misdiagnosed and mistreated.

Methods: We performed a retrospective analysis of patients diagnosed with having mercury poisoning over a 6-year period between July 2013 and June 2019. Demographics, clinical measures, renal pathologic examinations, treatments, and outcomes were compared between patients with kidney disease and those without kidney disease. Read More

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Recent Advances in Clinical Diagnosis and Pharmacotherapy Options of Membranous Nephropathy.

Front Pharmacol 2022 26;13:907108. Epub 2022 May 26.

Faculty of Life Science & Medicine, Northwest University, Xi'an, China.

Membranous nephropathy (MN) is the most common cause of nephrotic syndrome among adults, which is the leading glomerular disease that recurs after kidney transplantation. Treatment for MN remained controversial and challenging, partly owing to absence of sensitive and specific biomarkers and effective therapy for prediction and diagnosis of disease activity. MN starts with the formation and deposition of circulating immune complexes on the outer area in the glomerular basement membrane, leading to complement activation. Read More

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Pathophysiology and system biology of rat c-BSA induced immune complex glomerulonephritis and pathway comparison with human gene sequencing data.

Int Immunopharmacol 2022 Aug 9;109:108891. Epub 2022 Jun 9.

State Key Laboratory of Bioactive Substances and Functions of Natural Medicines, Institute of Materia Medica, Department of Pharmacology, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100050, PR China. Electronic address:

Immune-complex glomerulonephritis (ICGN) is a major cause of nephrotic syndrome in adults and children. Cationic BSA (c-BSA) intravenous injection could produce significant albuminuria within a short time, and is a suitable in vivo experimental animal model to investigate the pathophysiology of ICGN and for drug screening, but lack of thorough study to clarify its dynamic pathophysiological alteration so far, as well as detailed changes in mRNA and LncRNA levels. The purpose of this study is to investigate the dynamic alteration in renal function, lipid metabolism and histopathology during the progress of c-BSA induced ICGN. Read More

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