22,929 results match your criteria Nephrotic Syndrome


Soluble RARRES1 induces podocyte apoptosis to promote glomerular disease progression.

J Clin Invest 2020 Jul 7. Epub 2020 Jul 7.

Division of Nephrology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, United States of America.

Utilizing the Nephrotic Syndrome Study Network Consortium and other publicly available transcriptomic datasets, we identified Retinoic acid receptor responder protein 1 (RARRES1) as a gene whose expression positively correlated with renal function decline in human glomerular disease. The glomerular expression of RARRES1, which is largely restricted to podocytes, increased in focal segmental glomerulosclerosis (FSGS) and diabetic kidney disease (DKD). Tumor necrosis factor-α (TNF-α) was a potent inducer of RARRES1 expression in cultured podocytes, and transcriptomic analysis showed the enrichment of cell death pathway genes with RARRES1 overexpression. Read More

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http://dx.doi.org/10.1172/JCI140155DOI Listing

Systemic Amyloidosis Recognition, Prognosis, and Therapy: A Systematic Review.

JAMA 2020 Jul;324(1):79-89

Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.

Importance: Many patients with systemic amyloidosis are underdiagnosed. Overall, 25% of patients with immunoglobulin light chain (AL) amyloidosis die within 6 months of diagnosis and 25% of patients with amyloid transthyretin (ATTR) amyloidosis die within 24 months of diagnosis. Effective therapy exists but is ineffective if end-organ damage is severe. Read More

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http://dx.doi.org/10.1001/jama.2020.5493DOI Listing

variants are associated with multiple congenital anomalies including ciliopathy phenotypes.

J Med Genet 2020 Jul 6. Epub 2020 Jul 6.

Pediatric Genomics Discovery Program, Department of Pediatrics and Genetics, Yale University School of Medicine, New Haven, Connecticut, USA

Background: Cilia are dynamic cellular extensions that generate and sense signals to orchestrate proper development and tissue homeostasis. They rely on the underlying polarisation of cells to participate in signalling. Cilia dysfunction is a well-known cause of several diseases that affect multiple organ systems including the kidneys, brain, heart, respiratory tract, skeleton and retina. Read More

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http://dx.doi.org/10.1136/jmedgenet-2019-106805DOI Listing

Anakinra in the treatment of protracted paradoxical inflammatory reactions in HIV-associated tuberculosis in the United Kingdom: a report of two cases.

Int J STD AIDS 2020 Jul;31(8):808-812

Department of Rheumatology, Sheffield Teaching Hospitals NHS Foundation Trust and Sheffield Children's Hospital NHS Foundation Trust, Sheffield, UK.

Paradoxical reactions, including immune reconstitution inflammatory syndrome (IRIS), are common in patients co-infected with human immunodeficiency virus (HIV) and tuberculosis (TB). Paradoxical reactions may confer substantial morbidity and mortality, especially in cases of central nervous system (CNS) TB, or through protracted usage of corticosteroids. No high-quality evidence is available to guide management in this scenario. Read More

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http://dx.doi.org/10.1177/0956462420915394DOI Listing

Remission of Proteinuria May Protect against Progression to Chronic Kidney Disease in Pediatric-Onset IgA Nephropathy.

J Clin Med 2020 Jun 30;9(7). Epub 2020 Jun 30.

Seoul National University College of Medicine and Seoul National University Children's Hospital, Seoul 03080, Korea.

Immunoglobulin A nephropathy (IgAN) is one of the most common primary glomerulopathies diagnosed in children and adolescents. This study aimed to evaluate the clinical features in and outcomes of pediatric IgAN over the last 30 years. Patients who were diagnosed before age of 18 at 20 centers in Korea were evaluated retrospectively. Read More

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http://dx.doi.org/10.3390/jcm9072058DOI Listing

Monoclonal Gammopathies of Renal Significance: Renal Biopsy and Beyond.

Cancers (Basel) 2020 Jun 30;12(7). Epub 2020 Jun 30.

Divisions of Nephrology, General Pathology, and Pathology, Department of Clinical and Molecular Medicine, "Sapienza" University of Rome, 00189 Rome, Italy.

Monoclonal Gammopathies of Renal Significance (MGRS) are a rather heterogeneous group of renal disorders caused by a circulating monoclonal (MC) immunoglobulin (Ig) component, often in the absence of multiple myeloma (MM) or another clinically relevant lymphoproliferative disorder. Nevertheless, substantial kidney damage could occur, despite the "benign" features of the bone-marrow biopsy. One example is renal amyloidosis, often linked to a small clone of plasma cells, without the invasive features of MM. Read More

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http://dx.doi.org/10.3390/cancers12071741DOI Listing

A single-center analysis of primary nephrotic syndrome with acute pancreatitis in children.

Medicine (Baltimore) 2020 Jul;99(27):e21056

Department of Nephrology and Rheumatology, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China.

Primary nephrotic syndrome (PNS) is one of the most common primary glomerular diseases in children. Patients complicated nephrotic syndrome with pancreatic lesions are rarely reported, and the clinical manifestations in children are atypical. This study has observed the incidence, clinical types, and prognosis of acute pancreatitis (AP) in children with primary nephrotic syndrome, and analyzed its related factors, early diagnosis, and treatment. Read More

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http://dx.doi.org/10.1097/MD.0000000000021056DOI Listing

Hypokalemia After Rituximab Administration in Steroid-Dependent Nephrotic Syndrome: A Case Report.

Front Pharmacol 2020 17;11:915. Epub 2020 Jun 17.

Nephrology and Dialysis Unit, Department of Pediatrics, Meyer Children's University Hospital, Florence, Italy.

The monoclonal antibody rituximab is a commonly used steroid sparing agent for steroid-dependent idiopathic nephrotic syndrome of childhood. With this brief report, we describe the first case of symptomatic hypokalemia after intravenous rituximab administration in a young woman. The sudden onset of dizziness and palpitation prompted acute life-threatening hypokalemia recognition by blood gas analysis and electrocardiography. Read More

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http://dx.doi.org/10.3389/fphar.2020.00915DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7311762PMC

Transcription factor MafB in podocytes protects against the development of focal segmental glomerulosclerosis.

Kidney Int 2020 Apr 28. Epub 2020 Apr 28.

Department of Anatomy and Embryology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan; Laboratory Animal Resource Center (LARC), Faculty of Medicine, University of Tsukuba, Ibaraki, Japan; International Institute for Integrative Sleep Medicine (WPI-IIIS), University of Tsukuba, Ibaraki, Japan; Life Science Center of Tsukuba Advanced Research Alliance (TARA), University of Tsukuba, Ibaraki, Japan. Electronic address:

Focal segmental glomerulosclerosis (FSGS) is a common cause of steroid-resistant nephrotic syndrome. Spontaneous remission of FSGS is rare and steroid-resistant FSGS frequently progresses to renal failure. Many inheritable forms of FSGS have been described, caused by mutations in proteins that are important for podocyte function. Read More

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http://dx.doi.org/10.1016/j.kint.2020.02.038DOI Listing

Rapid progression to end-stage renal disease in a child with IgA-dominant infection-related glomerulonephritis associated with parvovirus B19.

CEN Case Rep 2020 Jul 3. Epub 2020 Jul 3.

Department of Pediatric Nephrology, School of Medicine, Tokyo Women's Medical University, 8-1, Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.

Parvovirus B19 (PVB19) has been known to cause acute glomerulonephritis and nephrotic syndrome with various renal histologic patterns, such as endocapillary glomerulonephritis and collapsing glomerulopathy. Remission is achieved spontaneously or by treatment with steroid and/or immunosuppressants in most patients, except those with sickle cell anemia or two APOL1 risk alleles. In this study, we report the case of a previously healthy 5-year-old boy with infection-related glomerulonephritis (IRGN) associated with PVB19 that progressed to end-stage renal disease (ESRD). Read More

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http://dx.doi.org/10.1007/s13730-020-00501-wDOI Listing

Value of electron microscopy in the pathological diagnosis of native kidney biopsies in children.

Pediatr Nephrol 2020 Jul 3. Epub 2020 Jul 3.

Laboratory of Electron Microscopy, Pathological Center, Peking University First Hospital, No. 8. Xishiku Street, Xi Cheng District, Beijing, 100034, China.

Background: Pediatric native kidney diseases are common worldwide. The pathological diagnosis of kidney lesions is crucial for clinical treatment and prognosis. The aim of the current study was therefore to evaluate the value of electron microscopy (EM) to the final diagnosis of native kidney biopsies in children. Read More

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http://dx.doi.org/10.1007/s00467-020-04681-6DOI Listing

Spectrum of biopsy proven renal diseases in Central China: a 10-year retrospective study based on 34,630 cases.

Sci Rep 2020 Jul 3;10(1):10994. Epub 2020 Jul 3.

Department of Nephrology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, People's Republic of China.

Chronic kidney diseases have become a major issue worldwide. The spectrum of biopsy proven renal diseases differs between locations and changes over time. It is therefore essential to describe the local epidemiological trends and the prevalence of renal biopsy in various regions to shine new light on the pathogenesis of various renal diseases and provide a basis for further hypothesis-driven research. Read More

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http://dx.doi.org/10.1038/s41598-020-67910-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7335090PMC

Structural features and oligomeric nature of human podocin domain.

Biochem Biophys Rep 2020 Sep 25;23:100774. Epub 2020 Jun 25.

Department of Biochemistry, School of Life Sciences, University of Hyderabad, Hyderabad, 500046, India.

Podocytes are crucial cells of the glomerular filtration unit and plays a vital role at the interface of the blood-urine barrier. Podocyte slit-diaphragm is a modified tight junction that facilitates size and charge-dependent permselectivity. Several proteins including podocin, nephrin, CD2AP, and TRPC6 form a macromolecular assembly and constitute the slit-diaphragm. Read More

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http://dx.doi.org/10.1016/j.bbrep.2020.100774DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322680PMC
September 2020

[Cryptococcal Encephalitis Complicating Anti-N-methyl-D-aspartate Receptor Encephalitis in an Immunosuppressed Patient].

Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2020 Jun;42(3):417-420

Department of Geriatric Neurology,Xiangya Hospital,Central South University,Changsha 410008,China.

Cryptococcal encephalitis is a fatal central nervous system infectious disease,whereas anti-N-methyl-D-aspartate(NMDA)receptor encephalitis(NMDARE)is an autoimmune syndrome associated with psychological symptoms,behavioural abnormalities,seizures,and dyskinesias.Despite their distinct pathologies and pathogenic mechanisms,both of them can lead to cognitive dysfunction and abnormal behaviors,although anti-NMDARE can also have mood and mental disorders as its core manifestations.A patient with nephrotic syndrome accompanied by both cryptococcal encephalitis and anti-NMDARE was treated in our center,which for the first confirmed that these two conditions could coexist in one patient. Read More

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http://dx.doi.org/10.3881/j.issn.1000-503X.11472DOI Listing

Obesity is associated with severe clinical course in children with Henoch-Schonlein purpura.

Pediatr Nephrol 2020 Jun 29. Epub 2020 Jun 29.

Faculty of Medicine, Department of Pediatric Rheumatology, Dokuz Eylul University, Izmir, Turkey.

Background: We aimed to evaluate the role of obesity on the clinical course and response to treatment in patients with Henoch-Schonlein purpura (HSP).

Methods: Data charts of children with HSP followed in a tertiary hospital between 2000 and 2018 were reviewed retrospectively. Persistent purpura was defined as skin involvement persisting for ≥ 30 days. Read More

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http://dx.doi.org/10.1007/s00467-020-04672-7DOI Listing

Hydralazine-Induced Isolated Lupus Nephritis.

Ochsner J 2020 ;20(2):187-192

Clinic Operations, Ochsner Clinic Foundation, Baton Rouge, LA.

Hydralazine has been known to cause multiple side effects, both localized and systemic. The literature includes case reports of systemic vasculitis caused by hydralazine. A 79-year-old male with stage 3 chronic kidney disease attributable to hypertension and type 2 diabetes was started on hydralazine to control his hypertension. Read More

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http://dx.doi.org/10.31486/toj.18.0128DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7310176PMC
January 2020

Recommended physical activity and all cause and cause specific mortality in US adults: prospective cohort study.

BMJ 2020 07 1;370:m2031. Epub 2020 Jul 1.

Department of Epidemiology, School of Public Health, Cheeloo College of Medicine, Shandong University, Jinan250012, China

Objective: To determine the association between recommended physical activity according to the 2018 physical activity guidelines for Americans and all cause and cause specific mortality using a nationally representative sample of US adults.

Design: Population based cohort study.

Setting: National Health Interview Survey (1997-2014) with linkage to the National Death Index records to 31 December 2015. Read More

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http://dx.doi.org/10.1136/bmj.m2031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7328465PMC

Low-Density Lipoprotein Apheresis in Patients with Acute Kidney Injury Due to Minimal Change Disease Requiring Acute Renal Replacement Therapy.

Int J Nephrol Renovasc Dis 2020 18;13:157-162. Epub 2020 Jun 18.

Department of Nephrology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan.

Low-density lipoprotein apheresis (LDL-A) has been developed as a therapy for familial hypercholesterolemia, but LDL-A has also been used as a general treatment for drug-resistant nephrotic syndrome (NS) due to focal segmental glomerulosclerosis (FSGS). The patients with NS due to minimal change disease (MCD) are often difficult to control effective circulating plasma volume, causes acute kidney injury (AKI), and when diuretics are not effective and the respiratory condition of patients worsens, patients require acute renal replacement therapy (ARRT). The effectiveness of LDL-A is not only reduction of serum low-density lipoprotein but also various other benefits. Read More

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http://dx.doi.org/10.2147/IJNRD.S248610DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7308121PMC

Successful Treatment of Hepatitis C Virus Infection Using Direct-Acting Antiviral Agents (DAAs) in Adolescents with Kidney Transplantation: A Case Series.

Int J Nephrol Renovasc Dis 2020 8;13:139-146. Epub 2020 Jun 8.

Department of Child Health, Faculty of Medicine, Universitas Indonesia - Cipto Mangunkusumo Hospital, Jakarta, Indonesia.

Introduction: Hepatitis C virus (HCV) infection is common among end-stage renal disease patients undergoing hemodialysis. The standard treatment for HCV infection has been interferon-ribavirin combination prior to renal transplantation. However, compared to direct-acting antiviral agents (DAAs), the risk of graft rejection is higher with interferon therapy. Read More

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http://dx.doi.org/10.2147/IJNRD.S248632DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7292374PMC

Genetic Study in Korean Pediatric Patients with Steroid-Resistant Nephrotic Syndrome or Focal Segmental Glomerulosclerosis.

J Clin Med 2020 Jun 26;9(6). Epub 2020 Jun 26.

Department of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Korea.

Steroid-resistant nephrotic syndrome (SRNS) is one of the major causes of end-stage renal disease (ESRD) in childhood and is mostly associated with focal segmental glomerulosclerosis (FSGS). More than 50 monogenic causes of SRNS or FSGS have been identified. Recently, the mutation detection rate in pediatric patients with SRNS has been reported to be approximately 30%. Read More

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http://dx.doi.org/10.3390/jcm9062013DOI Listing

Relationship between Modified Body Mass Index and Prognosis of Renal Amyloid a Amyloidosis.

Sisli Etfal Hastan Tip Bul 2018 4;52(2):103-108. Epub 2018 Jun 4.

Department of Nephrology, Mehmet Akif Inan Training and Research Hospital, Sanliurfa, Turkey.

Objectives: Overhydration occurs in nephrotic syndrome related to kidney involvement of amyloid A (AA) amyloidosis, which can cause an overestimation of body mass index (BMI). Modified BMI (mBMI, albumin×BMI) may be a better marker of nutritional status; therefore, we investigated the relationship between mBMI and the prognosis of patients with renal AA amyloidosis.

Methods: We retrospectively reviewed the data of patients with biopsy-proven renal AA amyloidosis who were followed up between January 2001 and May 2013. Read More

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http://dx.doi.org/10.14744/SEMB.2017.89410DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7315068PMC

Individualizing Treatment of Steroid-Resistant Nephrotic Syndrome: Registries to the Fore.

Clin J Am Soc Nephrol 2020 Jun 29. Epub 2020 Jun 29.

Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

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http://dx.doi.org/10.2215/CJN.08080520DOI Listing
June 2020
4.613 Impact Factor

Nephrotic syndrome due to preeclampsia before 20 weeks of gestation: a case report.

BMC Nephrol 2020 Jun 29;21(1):240. Epub 2020 Jun 29.

Division of Nephrology and Hypertension, Department of Internal Medicine, St. Marianna University School of Medicine, 216-8511, 2-16-1, Sugao, Miyamae-ku, Kawasaki, Kanagawa, Japan.

Background: Preeclampsia (PE) refers to the development of hypertension and new-onset proteinuria or progressive organ damage (especially kidney) in a previously normotensive pregnant women after 20 weeks of gestation. Thus, new-onset nephrotic syndrome due to PE before 20 weeks of gestation seems to be rare, making its diagnosis difficult in this time period.

Case Presentation: A 28-year-old woman presented with a new-onset nephrotic syndrome at 16 weeks of gestation. Read More

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http://dx.doi.org/10.1186/s12882-020-01876-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322849PMC

Pericardectomy after pericarditis constrictiva led to onset of transplant kidney function after 98 days of anuric kidney graft: a case report.

BMC Nephrol 2020 Jun 29;21(1):241. Epub 2020 Jun 29.

Department of Nephrology and Hypertension, University Erlangen-Nürnberg, Ulmenweg 18, 91054, Erlangen, Germany.

Background: Constrictive pericarditis is easily overlooked and can lead to severe problems in hemodynamics and end-organ perfusion, in our patient leading to 98 days of anuria after living kidney transplantation. This was completely reversible after pericardectomy.

Case Presentation: A 43-year-old female caucasian patient received a living kidney donation from her mother. Read More

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http://dx.doi.org/10.1186/s12882-020-01899-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7324971PMC

Dysbiosis of gut microbiota in adult idiopathic membranous nephropathy with nephrotic syndrome.

Microb Pathog 2020 Jun 26:104359. Epub 2020 Jun 26.

Department of Nephrology, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, Guangdong, China.

Background: Gut bacterial microbiota is altered in patients with chronic kidney disease (CKD) and those on dialysis. However, it is not yet clear what bacterial composition changes occur in patients with idiopathic nephrotic syndrome (INS). We present in this report the changes in gut bacterial microbiota in INS patients with membranous nephropathy. Read More

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http://dx.doi.org/10.1016/j.micpath.2020.104359DOI Listing

The clinicopathologic spectrum of segmental membranous glomerulopathy.

Kidney Int 2020 Jun 26. Epub 2020 Jun 26.

Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York. Electronic address:

Membranous glomerulopathy (MGN) is characterized by global subepithelial immune deposits that stain most intensely by immunofluorescence for IgG. Here we describe the clinical and pathologic findings in a cohort of patients with MGN in which, by definition, only segmental immune deposits are present. This rare variant, termed segmental MGN (sMGN), is poorly characterized. Read More

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http://dx.doi.org/10.1016/j.kint.2020.06.014DOI Listing

Efficacy of low or standard rituximab-based protocols and comparison to Ponticelli's regimen in membranous nephropathy.

J Nephrol 2020 Jun 27. Epub 2020 Jun 27.

CMID-Nephrology and Dialysis Unit (ERK-Net Member), Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, University of Turin and San Giovanni Bosco Hospital, Piazza del Donatore di Sangue 3, 10054, Turin, Italy.

Background: Patients (pts) with primary Membranous nephropathy (MN) have an autoimmune disease caused by autoantibodies against podocyte antigens and 60-80% of them have antibodies directed against the M-type phospholipase A2 receptor (PLA2R). Immunosuppressive treatment is recommended in high-medium risk pts. Recently the use of rituximab (RTX), has emerged as an important therapeutic option in pts with primary MN. Read More

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http://dx.doi.org/10.1007/s40620-020-00781-6DOI Listing

Clinical characteristics of acute glomerulonephritis with presentation of nephrotic syndrome at onset in children.

Int Immunopharmacol 2020 Jun 25;86:106724. Epub 2020 Jun 25.

Department of Nephrology, Xiangya Hospital, Central South University, No. 87 of Xiangya Road, Changsha, Hunan 410008, China. Electronic address:

Background: Acute glomerulonephritis (AGN) is a common disease in children, which places a huge burden on developing countries. The prognosis of it may not always be good. However, the clinical characteristics of AGN with nephrotic syndrome (NS) at onset have not been fully clarified. Read More

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http://dx.doi.org/10.1016/j.intimp.2020.106724DOI Listing

Evaluation of the mechanism of Danggui-Shaoyao-San in regulating the metabolome of nephrotic syndrome based on urinary metabonomics and bioinformatics approaches.

J Ethnopharmacol 2020 Jun 24;261:113020. Epub 2020 Jun 24.

School of Pharmacy, Anhui University of Chinese Medicine, Hefei, 230012, PR China; Key Laboratory of Chinese Medicine Formula of Anhui Province, Hefei, 230012, PR China. Electronic address:

Ethnopharmacological Relevance: Danggui-Shaoyao-San (DSS), a well-known classic Traditional Chinese medicine (TCM) formula for enhancing Qi (vital energy and spirit), invigorating blood circulation and promoting diuresis, has been widely used in the treatment of nephrotic syndrome (NS). Previously, we have reported some protective effects of DSS against NS, but the in-depth mechanisms remain unclear.

Aim Of The Study: In this study, an ultra performance liquid chromatography coupled with quadrupole-time-of-flight mass spectrometry (UPLC-Q/TOF-MS)-based urinary metabonomics coupled with bioinformatics method was employed to evaluate the mechanisms of DSS in treating NS from the perspective of metabolism. Read More

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http://dx.doi.org/10.1016/j.jep.2020.113020DOI Listing

Efficacy of abatacept treatment for focal segmental glomerulosclerosis and minimal change disease: A systematic review of case reports, case series, and observational studies
.

Clin Nephrol 2020 Jun 26. Epub 2020 Jun 26.

Introduction: The efficacy of abatacept has been demonstrated mainly in case reports, case series, and observational studies with small sample size. With current evidence, it is premature to conclude that abatacept is an effective treatment for nephrotic syndrome.

Materials And Methods: We searched MEDLINE, SCOPUS, and Cochrane Library until December 2019 for studies including patients with focal segmental glomerulosclerosis (FSGS) or minimal change disease (MCD) treated with abatacept. Read More

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http://dx.doi.org/10.5414/CN110134DOI Listing

Generation of an induced pluripotent stem cell (iPSC) line (IMAGINi007) from a patient with steroid-resistant nephrotic syndrome carrying the homozygous p.R138Q mutation in the podocin-encoding NPHS2 gene.

Stem Cell Res 2020 07 18;46:101878. Epub 2020 Jun 18.

Université de Paris, Imagine Institute, Laboratory of Hereditary Kidney Diseases, INSERM UMR 1163, Paris, France. Electronic address:

Mutations in the NPHS2 gene, encoding podocin, are responsible for the majority of familial cases of steroid-resistant nephrotic syndrome (SRNS), a rare glomerulopathy that rapidly progresses to end-stage renal disease. We obtained peripheral blood mononuclear cells (PBMCs) from a patient carrying the homozygous c.413G>A substitution (p. Read More

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http://dx.doi.org/10.1016/j.scr.2020.101878DOI Listing

Rituximab in children with steroid sensitive nephrotic syndrome: in quest of the optimal regimen.

Pediatr Nephrol 2020 Jun 24. Epub 2020 Jun 24.

Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Trust, London, UK.

Rituximab has emerged as an effective and important therapy in children with complicated frequently relapsing and steroid-dependent nephrotic syndrome to induce long-term disease remission and avoid steroid toxicities. The optimal rituximab regimen is not totally well defined, and there are many varying practices worldwide. We will in this review describe how patient factors, rituximab dose, and use of maintenance immunosuppression affect treatment outcomes. Read More

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http://dx.doi.org/10.1007/s00467-020-04609-0DOI Listing

Pediatric bilateral ureteral stone successfully removed using single-use flexible ureteroscopy with a holmium: YAG laser.

Clin Case Rep 2020 Jun 23;8(6):1073-1075. Epub 2020 Mar 23.

Departments of Urology and Renal Transplantation Yokohama City University Medical Center Yokohama Japan.

A 12-year-old boy received steroid for his minimal change nephrotic syndrome for 10 years, and bilateral renal and ureteral stones and hydronephrosis were observed. Single-use flexible ureteroscopy is usable for pediatric lithotripsy with Ho: YAG laser. Read More

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http://dx.doi.org/10.1002/ccr3.2817DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7303861PMC

Idiopathic nephrotic syndrome in children: role of regulatory T cells and gut microbiota.

Pediatr Res 2020 Jun 22. Epub 2020 Jun 22.

Department of Pediatrics, Kansai Medical University, Osaka, Japan.

Background: We investigated whether an association exists between regulatory T cells (Tregs) during initial presentation in children with idiopathic nephrotic syndrome (INS) and later development of frequently relapsing INS.

Methods: Blood samples were obtained at onset and at remission from 25 patients (median age, 4.0 years) with INS; eight did not show relapse after initial response (non-relapsing [NR]), whereas 17 showed frequent relapses (frequently relapsing [FR]). Read More

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http://dx.doi.org/10.1038/s41390-020-1022-3DOI Listing

Asymptomatic sinusitis as an origin of infection-related glomerulonephritis manifesting steroid-resistant nephrotic syndrome: A case report.

Medicine (Baltimore) 2020 Jun;99(25):e20572

Department of Nephrology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Bunkyo.

Rationale: Infection is a major trigger or pathogenic origin in a substantial proportion of glomerulonephritis (GN) patients, typically manifesting infection-related GN (IRGN). Various microorganisms, infection sites, and clinical and histopathological features are involved in IRGN. Once an infectious origin is identified and successfully eradicated, nephrotic syndrome or kidney dysfunction is spontaneously resolved. Read More

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http://dx.doi.org/10.1097/MD.0000000000020572DOI Listing

Treatment of parvovirus B19 viremia to facilitate kidney transplantation in a patient with collapsing glomerulopathy.

Clin Nephrol Case Stud 2020 29;8:41-45. Epub 2020 May 29.

Department of Surgery, and.

Collapsing glomerulopathy (CG) is a severe form of glomerulopathy which results in nephrotic syndrome and often ensues in rapid progression to end-stage kidney disease (ESKD). Although most commonly a result of HIV infection, other conditions such as parvovirus B19 (PB19) infection have been associated with CG. We present a case of an 18-year-old male with CG associated with PB19 infection who was heterozygous for G1 and G2 genetic variants. Read More

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http://dx.doi.org/10.5414/CNCS110113DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7303543PMC

ACTH Treatment for Management of Nephrotic Syndrome: A Systematic Review and Reappraisal.

Int J Nephrol 2020 4;2020:2597079. Epub 2020 Jun 4.

Akron Nephrology Associates/Cleveland Clinic Akron General, Akron, OH, USA.

Background: In recent years, the use of adrenocorticotropic hormone (ACTH) therapy for treatment of proteinuria due to nephrotic syndrome (NS) has been heavily explored. ACTH therapy, which comes in the natural (H. P. Read More

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http://dx.doi.org/10.1155/2020/2597079DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7292987PMC

Nephrotic syndrome and pulmonary hypertension as complications of intravenous drug abuse.

APMIS 2020 Jun 21. Epub 2020 Jun 21.

Departments of Internal Medicine, Ålesund Hospital, Ålesund, Norway.

This case report discusses a patient with nephrotic syndrome, pulmonary hypertension and repeated episodes of infections. He had a history of intravenous drug abuse. Kidney biopsy revealed the rare finding of numerous foam cells, mainly in glomeruli. Read More

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http://dx.doi.org/10.1111/apm.13065DOI Listing

Better remission rates in elderly Japanese patients with primary membranous nephropathy in nationwide real-world practice: The Japan Nephrotic Syndrome Cohort Study (JNSCS).

Clin Exp Nephrol 2020 Jun 19. Epub 2020 Jun 19.

Department of Nephrology, Osaka University Graduate School of Medicine, 2-2-D11 Yamadaoka, Suita, Osaka, 565-0871, Japan.

Background: The aim of the present study was to clarify the prevalence of immunosuppressive drug use and outcomes in elderly and non-elderly patients with primary membranous nephropathy (MN) in nationwide real-world practice in Japan.

Patients And Methods: Between 2009 and 2010, 374 patients with primary nephrotic syndrome were enrolled in the cohort study (The Japan Nephrotic Syndrome Cohort Study, JNSCS), including 126 adult patients with MN. Their clinical characteristics were compared with those of nephrotic patients with primary MN registered in a large nationwide registry (The Japan Renal Biopsy Registry, J-RBR). Read More

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http://dx.doi.org/10.1007/s10157-020-01913-9DOI Listing

Decreased Excretion of Urinary Exosomal Aquaporin-2 in a Puromycin Aminonucleoside-Induced Nephrotic Syndrome Model.

Int J Mol Sci 2020 Jun 16;21(12). Epub 2020 Jun 16.

Department of Veterinary Pharmacology, University of Miyazaki, Miyazaki 889-2192, Japan.

Urinary exosomes, small extracellular vesicles present in urine, are secreted from all types of renal epithelial cells. Aquaporin-2 (AQP2), a vasopressin-regulated water channel protein, is known to be selectively excreted into the urine through exosomes (UE-AQP2), and its renal expression is decreased in nephrotic syndrome. However, it is still unclear whether excretion of UE-AQP2 is altered in nephrotic syndrome. Read More

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http://dx.doi.org/10.3390/ijms21124288DOI Listing

Immunoglobulin light chain toxicity in a mouse model of monoclonal immunoglobulin light-chain deposition disease.

Blood 2020 Jun 19. Epub 2020 Jun 19.

Centre National de l'Amylose AL et Autres Maladies par Dépôt d'Immunoglobulines Monoclonales, Centre Hospitalier Universitaire de Limoges, France.

Light chain deposition disease (LCDD) is a rare disorder characterized by glomerular and peritubular amorphous deposits of a monoclonal immunoglobulin (Ig) light chain (LC), leading to nodular glomerulosclerosis and nephrotic syndrome. We developed a transgenic model using site-directed insertion of the variable domain of a pathogenic human LC gene into the mouse Ig kappa locus, ensuring its production by all plasma cells (PCs). High free LC levels were achieved after backcrossing with mice presenting increased PC differentiation and no Ig heavy chain (HC) production. Read More

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http://dx.doi.org/10.1182/blood.2020005980DOI Listing

Low-density lipoprotein apheresis for PLA2R-related membranous glomerulonephritis accompanied by IgG4-related tubulointerstitial nephritis.

CEN Case Rep 2020 Jun 16. Epub 2020 Jun 16.

Department of Medicine, Tokyo Women's Medical University Medical Center East, 2-1-10, Nishiogu, Arakawa, Tokyo, 116-8567, Japan.

IgG4-related disease preferentially involves the kidney by tubulointerstitial nephritis with IgG4-positive plasma cell filtration and/or membranous glomerulonephritis. We reported the case of a 68-year-old man with IgG4-related tubulointerstitial nephritis combined with antiphospholipase A2 receptor (PLA2R)-related membranous glomerulonephritis, in which distinguishing between idiopathic PLA2R-related and IgG4-related secondary membranous glomerulonephritis was difficult. We diagnosed him as having IgG4-related disease, based on a serum IgG4 level of 170 mg/dL and the presence of IgG4-related parotiditis. Read More

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http://dx.doi.org/10.1007/s13730-020-00494-6DOI Listing

Clinicopathological characteristics and outcome of patients with fibrillary glomerulonephritis: DNAJB9 is a valuable histologic marker.

J Nephrol 2020 Jun 18. Epub 2020 Jun 18.

National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, East 305 Zhongshan Road, Nanjing, 210002, China.

Background: Fibrillary glomerulonephritis (FGN) is a rare glomerular disease. DNAJB9 as a new reliable diagnostic marker for the diagnosis of FGN was discovered recently. To investigate the clinicopathological features and prognosis of DNAJB9-positive FGN, we report on a case series in a single center in China. Read More

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http://dx.doi.org/10.1007/s40620-020-00783-4DOI Listing
June 2020
1.996 Impact Factor

Unfavorable impact of anti-rituximab antibodies on clinical outcomes in children with complicated steroid-dependent nephrotic syndrome.

Pediatr Nephrol 2020 Jun 16. Epub 2020 Jun 16.

Division of Nephrology, Saitama Children's Medical Center, 1-2 Shintoshin, Chuo-ku, Saitama, 330-8777, Japan.

Background: Anti-rituximab antibodies (ARA) are associated not only with adverse events, such as infusion reactions (IR) and serum sickness, but also with rituximab efficacy. However, the clinical relevance of ARA in children with steroid-dependent nephrotic syndrome (SDNS) remains unknown.

Methods: We retrospectively reviewed clinical outcomes of 13 children with complicated SDNS receiving repeated single-dose rituximab treatments at 375 mg/m to assess whether ARA formation could impact toxicity and efficacy of additional rituximab. Read More

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http://dx.doi.org/10.1007/s00467-020-04629-wDOI Listing

24-year-old man • prednisone therapy for nephrotic syndrome • diffuse maculopapular rash • pruritis • Dx?

J Fam Pract 2020 Jun;69(5):E1-E2

Section of Allergy and Immunology, Beaumont Health, Royal Oak, MI, USA.

► Prednisone therapy to treat nephrotic syndrome ► Diffuse maculopapular rash ► Pruritis. Read More

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Pseudouridylation defect due to and mutations causes nephrotic syndrome with cataracts, hearing impairment, and enterocolitis.

Proc Natl Acad Sci U S A 2020 Jun 17;117(26):15137-15147. Epub 2020 Jun 17.

MTA-SE Lendület Nephrogenetic Laboratory, Semmelweis University, HU 1083 Budapest, Hungary;

RNA modifications play a fundamental role in cellular function. Pseudouridylation, the most abundant RNA modification, is catalyzed by the H/ACA small ribonucleoprotein (snoRNP) complex that shares four core proteins, dyskerin (DKC1), NOP10, NHP2, and GAR1. Mutations in , , or cause dyskeratosis congenita (DC), a disorder characterized by telomere attrition. Read More

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http://dx.doi.org/10.1073/pnas.2002328117DOI Listing
June 2020
9.809 Impact Factor

Common risk variants in NPHS1 and TNFSF15 are associated with childhood steroid-sensitive nephrotic syndrome.

Kidney Int 2020 Jun 14. Epub 2020 Jun 14.

Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan. Electronic address:

To understand the genetics of steroid-sensitive nephrotic syndrome (SSNS), we conducted a genome-wide association study in 987 childhood SSNS patients and 3,206 healthy controls with Japanese ancestry. Beyond known associations in the HLA-DR/DQ region, common variants in NPHS1-KIRREL2 (rs56117924, P=4.94E-20, odds ratio (OR) =1. Read More

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http://dx.doi.org/10.1016/j.kint.2020.05.029DOI Listing

Case Report on Paediatric Nephrotic Syndrome.

EJIFCC 2020 Jun 2;31(2):164-168. Epub 2020 Jun 2.

Department of Paediatrics, Malla Reddy Institute of Medical Sciences, Secunderabad, Telangana, India.

Nephrotic syndrome (NS) is a glomerular disorder typically characterized by gross proteinuria, hypoalbuminemia, hyperlipidemia, and peripheral oedema. We report the case of a 2-year-old male toddler weighing 15 kg with a 1-week history of swelling around the eyes and both legs, and generalized body swelling. She had a history of fever, cough and decreased urine output. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7294819PMC

Bilateral Pulmonary Embolism in a 12-Year-Old Girl with Steroid-Resistant Nephrotic Syndrome.

Children (Basel) 2020 Jun 15;7(6). Epub 2020 Jun 15.

Faculty of Medicine, King Abdulaziz University, Jeddah 21414, Saudi Arabia.

Nephrotic syndrome is the most common glomerular disease among children. Although most cases respond to steroid therapy, approximately 10-20% of patients exhibit resistance to conventional steroid therapy and are labeled as steroid-resistant. Such patients are at risk of complications, including infection, thrombosis, and chronic kidney disease. Read More

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http://dx.doi.org/10.3390/children7060062DOI Listing

COQ8B nephropathy: Early detection and optimal treatment.

Mol Genet Genomic Med 2020 Jun 16:e1360. Epub 2020 Jun 16.

Department of Nephrology, Children's Hospital of Fudan University, National Pediatric Medical Center of China, Shanghai, China.

Background: Mutations in COQ8B (*615567) as a defect of coenzyme Q10 (CoQ10) cause steroid resistant nephrotic syndrome (SRNS).

Methods: To define the clinical course and prognosis of COQ8B nephropathy, we retrospectively assessed the genotype and phenotype in patients with COQ8B mutations from Chinese Children Genetic Kidney Disease Database. We performed the comparing study of renal outcome following CoQ10 treatment and renal transplantation between early genetic detection and delayed genetic detection group. Read More

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http://dx.doi.org/10.1002/mgg3.1360DOI Listing