21,963 results match your criteria Nephrotic Syndrome


The quest for optimal control of relapses in children with nephrotic syndrome.

Kidney Int 2019 Mar;95(3):717

Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, the Netherlands.

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http://dx.doi.org/10.1016/j.kint.2019.01.002DOI Listing

Hodgkin's lymphoma, nephrotic syndrome, and echinococcosis cysts: an unusual association and literature review.

Pediatr Hematol Oncol 2019 Feb 20:1-6. Epub 2019 Feb 20.

a Pediatrics Department A, Soroka University Medical Center, and Faculty of Health Sciences , Ben-Gurion University of the Negev , Beer Sheva , Israel.

We present a 5-year-old female with minimal change nephrotic syndrome (MCNS). Within several months, she became steroid-dependent with progression of edema and ascites. Imaging studies revealed abnormal solid mass and liver cysts and she was diagnosed with both abdominal Hodgkin's lymphoma (cHD) and large hepatic cystic echinococcosis (CE). Read More

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http://dx.doi.org/10.1080/08880018.2019.1571541DOI Listing
February 2019

Oestrogen inhibits PTPRO to prevent the apoptosis of renal podocytes.

Exp Ther Med 2019 Mar 14;17(3):2373-2380. Epub 2019 Jan 14.

Department of Medical Ultrasound Center, The Northwest Women's and Children's Hospital, Xi'an, Shaanxi 710061, P.R. China.

Podocytes are a major component of the glomerular filtration membrane, and their apoptosis is involved in a variety of nephrotic syndromes. In the current study, the effects and molecular mechanisms of oestrogen on the proliferation and apoptosis of podocytes were investigated to elucidate the role of oestrogen in the pathogenesis of childhood nephrotic syndrome. The cell proliferation of mouse renal podocytes (MPC-5) and human primary renal podocytes was promoted by 17β-oestradiol (E2) in what appear to be a time-dependent manner. Read More

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http://dx.doi.org/10.3892/etm.2019.7167DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6364249PMC

Kimura's disease associated with IgA nephropathy: A case report.

Exp Ther Med 2019 Mar 17;17(3):1837-1840. Epub 2018 Dec 17.

Department of Nephrology, Second Affiliated Hospital of Dalian Medical University, Dalian, Liaoning 116000, P.R. China.

Kimura's disease (KD) is a rare chronic inflammatory disorder of unknown etiology that manifests as painless subcutaneous lesions in the head and neck. It primarily affects young Asian males and is characterized by peripheral eosinophilia and elevated levels of serum Immunoglobulin (Ig)E. Histologically, a variety of renal lesions have been observed in patients with KD, although IgA nephropathy is rarely reported. Read More

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http://dx.doi.org/10.3892/etm.2018.7103DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6364220PMC

Clinical significance of autoantibodies in the assessment and treatment of idiopathic membranous nephropathy.

Exp Ther Med 2019 Mar 18;17(3):1825-1830. Epub 2018 Dec 18.

Department of Nephrology, Qianfoshan Hospital, Shandong University, Jinan, Shandong 250014, P.R. China.

The present study aimed to explore the correlation between the dynamic serum levels of phospholipase A2 receptor (PLA2R), aldose reductase (AR) and superoxide dismutase 2(SOD2) antibodies with disease activity and treatment response in patients with idiopathic membranous nephropathy (IMN). The present study included 56 patients with IMN who were diagnosed through a renal biopsy and presenting with nephrotic syndrome. The patients were divided into two treatment groups: One treated with cyclophosphamide (CTX) and one with tacrolimus (FK506). Read More

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http://dx.doi.org/10.3892/etm.2018.7108DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6364219PMC

Group I metabotropic glutamate receptor activation induces TRPC6-dependent calcium influx and RhoA activation in cultured human kidney podocytes.

Biochem Biophys Res Commun 2019 Feb 16. Epub 2019 Feb 16.

Department of Nephrology, The Second Affiliated Hospital of Harbin Medical University, Harbin, China; Institute of Nephrology, Harbin Medical University, Harbin, China. Electronic address:

Researches have shown that mice lacking the metabotropic glutamate receptor 1 (mGluR) showed albuminuria, remodeling of F-actin, with loss of stress fibers. Selective group I mGluRs agonist (S)-3,5-dihydroxyphenylglycine (DHPG) attenuated albuminuria in several rodent models of nephrotic syndrome. However, the molecular mechanism is obscure. Read More

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http://dx.doi.org/10.1016/j.bbrc.2019.02.062DOI Listing
February 2019

[Clinical features of nephrotic syndrome accompanied by eosinophilia in children].

Zhongguo Dang Dai Er Ke Za Zhi 2019 Feb;21(2):165-167

Department of Nephrology, Tianjin Children's Hospital, Tianjin 300134, China.

Objective: To study the clinical features of nephrotic syndrome (NS) accompanied by eosinophilia in children.

Methods: A retrospective analysis was performed for the clinical manifestations, laboratory findings and treatment outcomes of 18 cases of eosinophilia (15 children, 3 of whom also had eosinophilia at the second recurrence) in children with NS.

Results: Of the 18 cases, 16 (89%) had mild eosinophilia, 1 (6%) had moderate eosinophilia, and 1 (6%) had severe eosinophilia. Read More

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February 2019
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A Novel Homozygous Truncating Mutation in Gene in a Chinese Uyghur Patient With Severe Phenotype Pierson Syndrome.

Front Med (Lausanne) 2019 4;6:12. Epub 2019 Feb 4.

Pediatric, First Affiliated Hospital of Xinjiang Medical University, Urumqi, China.

Pierson syndrome (OMIM 609049) is a rare autosomal recessive disorder characterized by congenital nephrotic syndrome and complex ocular abnormalities. Severe renal symptoms had be associated with truncating mutations. Few Chinese patients from diverse ethnic background had been evaluated and reported with this syndrome. Read More

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http://dx.doi.org/10.3389/fmed.2019.00012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6369156PMC
February 2019

Urinary proteome signature of Renal Cysts and Diabetes syndrome in children.

Sci Rep 2019 Feb 18;9(1):2225. Epub 2019 Feb 18.

Mosaiques Diagnostics GmbH, Hannover, Germany.

Renal Cysts and Diabetes Syndrome (RCAD) is an autosomal dominant disorder caused by mutations in the HNF1B gene encoding for the transcriptional factor hepatocyte nuclear factor-1B. RCAD is characterized as a multi-organ disease, with a broad spectrum of symptoms including kidney abnormalities (renal cysts, renal hypodysplasia, single kidney, horseshoe kidneys, hydronephrosis), early-onset diabetes mellitus, abnormal liver function, pancreatic hypoplasia and genital tract malformations. In the present study, using capillary electrophoresis coupled to mass spectrometry (CE-MS), we investigated the urinary proteome of a pediatric cohort of RCAD patients and different controls to identify peptide biomarkers and obtain further insights into the pathophysiology of this disorder. Read More

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http://dx.doi.org/10.1038/s41598-019-38713-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6379363PMC
February 2019

Repository Corticotropin Versus Glucocorticoid for Nephrotic Syndrome: When Will We See the Evidence?

Am J Kidney Dis 2019 Feb 11. Epub 2019 Feb 11.

VA Portland Health Care System, Research Service, Portland, OR; Division of Nephrology and Hypertension, Department of Medicine, Oregon Health & Science University, Portland, OR. Electronic address:

Despite little evidence supporting its superiority to glucocorticoid therapy, use and expenditures for repository corticotropin (rACTH) injection (H.P. Acthar Gel; Mallinckrodt) have increased dramatically in the last 5 years, particularly among a small number of nephrologists, rheumatologists, and neurologists. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S02726386193000
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http://dx.doi.org/10.1053/j.ajkd.2018.12.025DOI Listing
February 2019
4 Reads

Genetics of Childhood Steroid Sensitive Nephrotic Syndrome: An Update.

Front Pediatr 2019 29;7. Epub 2019 Jan 29.

Division of Nephrology, Departments of Pediatrics, Duke University Medical Center, Durham, NC, United States.

Advances in genome science in the last 20 years have led to the discovery of over 50 single gene causes and genetic risk loci for steroid resistant nephrotic syndrome (SRNS). Despite these advances, the genetic architecture of childhood steroid sensitive nephrotic syndrome (SSNS) remains poorly understood due in large part to the varying clinical course of SSNS over time. Recent exome and genome wide association studies from well-defined cohorts of children with SSNS identified variants in multiple MHC class II molecules such as HLA-DQA1 and HLA-DQB1 as risk factors for SSNS, thus stressing the central role of adaptive immunity in the pathogenesis of SSNS. Read More

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http://dx.doi.org/10.3389/fped.2019.00008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6361778PMC
January 2019
1 Read

miR-939-5p decreases the enrichment of RNA polymerase II in the promoter region of CD2AP involved in nephrotic syndrome.

J Cell Biochem 2019 Feb 12. Epub 2019 Feb 12.

Department of Pediatrics, the First Affiliated Hospital, Nanjing Medical University, Nanjing, Jiangsu Province, China.

The expression changes of CD2-associated protein (CD2AP) can lead to kidney diseases with proteinuria, including nephrotic syndrome (NS). A recent study reported that miRNAs may be important transcriptional regulators. In this study, we found increased expression of miR-939-5p and decreased expression of CD2AP in the peripheral blood of patients with NS. Read More

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http://dx.doi.org/10.1002/jcb.28413DOI Listing
February 2019
1 Read
3.263 Impact Factor

Comparison of measurements of anti-PLA2R antibodies in Japanese patients with membranous nephropathy using in-house and commercial ELISA.

Clin Exp Nephrol 2019 Feb 13. Epub 2019 Feb 13.

Department of Life Science, Akita University Graduate School of Engineering Science, Akita City, Japan.

Background: The prevalence of antibodies against M-type anti-phospholipase A2 receptor (PLA2R) was reported to be ~ 70-80% in early studies on idiopathic membranous nephropathy (iMN) cohorts from Western countries, China, and Korea, and ~ 50% in recent studies on two Japanese iMN cohorts.

Methods: We developed an in-house enzyme-linked immunosorbent assay (ELISA) for the detection of anti-PLA2R antibodies, and examined sera from 217 patients with iMN, 22 patients with secondary MN (sMN), and 50 healthy individuals. All patients and healthy individuals were Japanese. Read More

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http://dx.doi.org/10.1007/s10157-019-01712-xDOI Listing
February 2019
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Use of Intravenous Immunoglobulin Therapy at Unconventional Doses in Refractory Fulminant Systemic Lupus Erythematosus.

Eur J Case Rep Intern Med 2018 27;5(9):000934. Epub 2018 Sep 27.

Rare Diseases and Periodic Fevers Research Centre, Department of Internal Medicine, Agostino Gemelli University Hospital Foundation, Catholic University of the Sacred Heart, Rome, Italy.

The use of human intravenous immunoglobulins (IVIg) in systemic lupus erythematosus (SLE) currently relies on evidence from small case series and is mainly regarded as an off-label strategy in cases that are refractory to conventional therapies or poorly controlled with high doses of corticosteroids. Standard dosage regimens typically entail the administration of a total amount of 2 g/kg of IVIg divided into five consecutive days in order to minimize the risk of severe adverse events. We herein describe the case of a 28-year-old woman with a known history of antiphospholipid syndrome (APS) who was admitted to our hospital following fulminant onset of SLE in spite of ongoing immunosuppressive therapy. Read More

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http://dx.doi.org/10.12890/2018_000934DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346821PMC
September 2018
1 Read

Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture.

Eur J Case Rep Intern Med 2018 27;5(12):0001010. Epub 2018 Dec 27.

Serviço de Medicina 1, ULSAM, Viana do Castelo, Portugal.

Light-chain deposition disease is a rare paraproteinaemia characterized by deposition of monoclonal light-chain immunoglobulins with a non-amyloid structure, most frequently affecting the kidney. The authors present the case of a 58-year-old man admitted due to haemorrhagic shock caused by spontaneous splenic rupture. Investigation showed coagulopathy, homogeneous hepatomegaly with elevated cholestasis enzymes but normal bilirubinaemia, and a nephrotic syndrome with monoclonal kappa light chains. Read More

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http://dx.doi.org/10.12890/2018_0001010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346975PMC
December 2018
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A comparison of cyclophosphamide versus tacrolimus in terms of treatment effect for idiopathic membranous nephropathy: A meta-analysis.

Nefrologia 2019 Feb 9. Epub 2019 Feb 9.

Department of Nephrology, Beijing Chao-Yang Hospital, Beijing, China. Electronic address:

Background And Aims: Idiopathic membranous nephropathy (IMN), which is considered one of the most common causes of nephrotic syndrome in adult patients, is frequently managed with immunosuppressive agents. Both tacrolimus (TAC) and cyclophosphamide (CTX) are recommended as immunosuppressive agents in the management of IMN. However, profound effects and moderate evidence on the two drugs remains poorly defined at this period. Read More

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http://dx.doi.org/10.1016/j.nefro.2018.10.008DOI Listing
February 2019
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Collapsing glomerulopathy in a child with LCHAD deficiency: a rare association.

CEN Case Rep 2019 Feb 12. Epub 2019 Feb 12.

Department of Paediatrics, Sheikh Khalifa Medical City, P O Box 51900, Abu Dhabi, United Arab Emirates.

Metabolic disorders, although rare, can involve multiple organ systems and have a varied presentation. Renal involvement has been reported in several metabolic disorders in the pediatric age group. We report a rare metabolic disorder, long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, in a child who developed steroid-resistant nephrotic syndrome at the age of 5 years. Read More

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http://dx.doi.org/10.1007/s13730-019-00387-3DOI Listing
February 2019
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Antiphospholipase 2 receptor antibody levels to predict complete spontaneous remission in primary membranous nephropathy.

Clin Kidney J 2019 Feb 9;12(1):36-41. Epub 2018 Mar 9.

Servicio de Nefrología y Trasplante Renal, Hospital Clínic, Departamento de Medicina, Universidad de Barcelona, Institut d' Invesigacions biomèdiques Agust Pi i Sunyer (IDIBAPS), Barcelona, Spain.

Background: M-type phospholipase A2 receptor (APLA2R) is considered the major antigen involved in the pathogenesis of adult primary membranous nephropathy (MN), which is the leading cause of non-diabetic nephrotic syndrome. Antibodies to this antigen have been proved to be an excellent biomarker of disease activity in primary MN. In fact, preliminary data suggest that the higher the antibody level the more proteinuria, and that a decrease in antibody level precedes the remission of proteinuria, but more solid evidence is needed. Read More

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http://dx.doi.org/10.1093/ckj/sfy005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6366127PMC
February 2019
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Anti-phospholipase A2 receptor antibody and spontaneous remission in membranous nephropathy.

Clin Kidney J 2019 Feb 3;12(1):33-35. Epub 2018 Oct 3.

Nephrology Department, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.

Membranous nephropathy (MN) is one of the most common causes of nephrotic syndrome in native kidney biopsies from adults. In 2009, antibodies to the M-type receptor of phospholipase A2 (anti-PLA2R) were identified in idiopathic MN patients, both within the kidney and in the circulation. The clinical course of idiopathic MN is variable and ranges from spontaneous remission to end-stage renal disease. Read More

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https://academic.oup.com/ckj/article/12/1/33/5114409
Publisher Site
http://dx.doi.org/10.1093/ckj/sfy079DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6366130PMC
February 2019
2 Reads

Biallelic Variants in the Nuclear Pore Complex Protein NUP93 Are Associated with Non-progressive Congenital Ataxia.

Cerebellum 2019 Feb 11. Epub 2019 Feb 11.

Institute of Biochemistry and Molecular Cell Biology, Medical School, RWTH Aachen University, 52074, Aachen, Germany.

Nuclear pore complexes (NPCs) are the gateways of the nuclear envelope mediating transport between cytoplasm and nucleus. They form huge complexes of 125 MDa in vertebrates and consist of about 30 different nucleoporins present in multiple copies in each complex. Here, we describe pathogenic variants in the nucleoporin 93 (NUP93) associated with an autosomal recessive form of congenital ataxia. Read More

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http://dx.doi.org/10.1007/s12311-019-1010-5DOI Listing
February 2019
1 Read
2.864 Impact Factor

Predictors of Mortality in Pulmonary Haemorrhage during SLE: A Single Centre Study Over Eleven Years.

Open Access Maced J Med Sci 2019 Jan 14;7(1):92-96. Epub 2019 Jan 14.

Department of Nephrology, King Abdul Aziz Specialist Hospital, Taif, Saudi Arabia.

Background: Pulmonary haemorrhage (PH) is a serious complication during Systemic Lupus Erythematosus (SLE).

Aim: The aim was to present data on 12 patients of SLE with classic symptoms and signs of PH admitted throughout eleven years.

Methods: This retrospective study was carried out at King Abdul Aziz Specialist hospital in Taif-a tertiary care hospital in the western region of Saudi Arabia. Read More

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http://dx.doi.org/10.3889/oamjms.2019.038DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6352490PMC
January 2019
1 Read

A longitudinal study on the effects of psychological stress on proteinuria in childhood steroid-sensitive nephrotic syndrome.

J Psychosom Res 2019 Jan 19. Epub 2019 Jan 19.

Department of Pediatric Nephrology, Emma Children's Hospital, AMC, Amsterdam, The Netherlands.

Objective: Steroid-sensitive nephrotic syndrome (SSNS) in children is often complicated by one or more relapses, as manifested by the appearance of proteinuria. Besides health-related triggers, psychological stress might be related to relapse. This longitudinal study examined the link between perceived stress, emotional valence (feeling happy vs. Read More

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http://dx.doi.org/10.1016/j.jpsychores.2019.01.011DOI Listing
January 2019
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The first identified heterozygous nonsense mutations in podocalyxin offer new perspectives on the biology of podocytopathies.

Clin Sci (Lond) 2019 Feb 8;133(3):443-447. Epub 2019 Feb 8.

The Biomedical Research Centre, University of British Columbia, Vancouver, Canada

In the last two decades, our understanding of the genetic underpinnings of inherited podocytopathies has advanced immensely. By sequencing the genomes of a large pool of families affected by focal segmental glomerulosclerosis (FSGS), researchers have identified a common theme: familial podocytopathies are frequently caused by genes selectively expressed in podocytes. Podocalyxin is a podocyte-specific surface sialomucin that has long been known to play important roles in podocyte morphogenesis and function. Read More

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http://dx.doi.org/10.1042/CS20181067DOI Listing
February 2019
1 Read

Treatment with 2,4-Dihydroxybenzoic Acid Prevents FSGS Progression and Renal Fibrosis in Podocyte-Specific Knockout Mice.

J Am Soc Nephrol 2019 Feb 8. Epub 2019 Feb 8.

Division of Nephrology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts;

Background: Although studies have identified >55 genes as causing steroid-resistant nephrotic syndrome (SRNS) and localized its pathogenesis to glomerular podocytes, the disease mechanisms of SRNS remain largely enigmatic. We recently reported that individuals with mutations in COQ6, a coenzyme Q (also called CoQ, CoQ, or ubiquinone) biosynthesis pathway enzyme, develop SRNS with sensorineural deafness, and demonstrated the beneficial effect of CoQ for maintenace of kidney function.

Methods: To study function in podocytes, we generated a podocyte-specific knockout mouse ( ) model and a transient siRNA-based knockdown in a human podocyte cell line. Read More

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http://dx.doi.org/10.1681/ASN.2018060625DOI Listing
February 2019
2 Reads

Interstitial pneumonia induced by cyclophosphamide: A case report and review of the literature.

Respir Med Case Rep 2019 26;26:212-214. Epub 2019 Jan 26.

Department of Respiration and Critical Care Medicine, Guizhou Provincial People's Hospital, Guiyang, Guizhou Province, China.

Introduction: Recently, interstitial lung disease significantly increases and it is difficult to treat. Cyclophosphamide(CP) is one drug administrated in interstitial lung disease, which can also cause pulmonary fibrosis and lung function lesion. This article present a case which exacerbated interstitial pneumonia after treatment by CP, aiming to enhance the understanding of the side effects of CP and standardize usage of the CP. Read More

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http://dx.doi.org/10.1016/j.rmcr.2019.01.014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6354618PMC
January 2019
1 Read

Prevalence of Asthma and Allergies and Risk of Relapse in Childhood Nephrotic Syndrome: Insight into Nephrotic Syndrome Cohort.

J Pediatr 2019 Feb 4. Epub 2019 Feb 4.

Child Health and Evaluative Sciences, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada; University of Toronto, Toronto, Ontario, Canada; Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada; University Health Network, Toronto, Ontario, Canada. Electronic address:

Objective: To determine the lifetime prevalence of allergies in childhood nephrotic syndrome, the seasonality of presentation and relapses, and the impact of allergies on subsequent relapses.

Study Design: In a longitudinal cohort of children with nephrotic syndrome (ages 1-18 years), assessment for allergic diseases was conducted using the validated and modified version of the International Study of Asthma and Allergies in Childhood questionnaire at enrollment. Outcomes included frequently relapsing nephrotic syndrome, relapse rates, and the relapse-free duration after initial steroid therapy. Read More

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http://dx.doi.org/10.1016/j.jpeds.2018.12.048DOI Listing
February 2019
1 Read

Clinical course & management of childhood nephrotic syndrome in Germany: a large epidemiological ESPED study.

BMC Nephrol 2019 Feb 7;20(1):45. Epub 2019 Feb 7.

Department of Radiology, Pediatric Radiology, University Hospital of Bonn, Bonn, Germany.

Background: Nephrotic syndrome (NS) is one of the most frequent occurring chronic kidney diseases in childhood, despite its rarely occurrence in the general population. Detailed information about clinical data of NS (e.g. Read More

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http://dx.doi.org/10.1186/s12882-019-1233-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367765PMC
February 2019
5 Reads

Nephrotic syndrome co-existing with type 1 diabetes in a 12-year-old boy: Case report and literature review.

SAGE Open Med Case Rep 2019 31;7:2050313X19827734. Epub 2019 Jan 31.

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

Occurrence of early nephrotic syndrome in type 1 diabetes mellitus patients is extremely rare. Herein, we report the case of a 12-year-old boy who presented to our pediatric nephrology clinic with generalized edema. He had been diagnosed with type 1 diabetes mellitus at age 9 and had been treated with regular insulin. Read More

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http://dx.doi.org/10.1177/2050313X19827734DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6357289PMC
January 2019
1 Read

Retinal and choroidal thickness in paediatric patients with hypoalbuminaemia caused by nephrotic syndrome.

BMC Ophthalmol 2019 Feb 6;19(1):44. Epub 2019 Feb 6.

Department of Ophthalmology, Peking University Frist Hospital, No. 8 Xishiku Street, Xicheng District, Beijing, 100034, China.

Background: A study was conducted to evaluate the choroidal thickness (CT) and retinal thickness (RT) in paediatric patients with hypoalbuminaemia caused by nephrotic syndrome (NS). We also studied the correlation between the subfoveal choroidal thickness (SFCT) and serum protein concentration.

Methods: This was a cross-sectional study. Read More

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http://dx.doi.org/10.1186/s12886-019-1050-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6364472PMC
February 2019
1 Read

Thrombospondin type-1 domain-containing 7A-associated membranous nephropathy after resection of rectal cancer: a case report.

BMC Nephrol 2019 Feb 6;20(1):43. Epub 2019 Feb 6.

Department of Nephrology, Okazaki City Hospital, 3-1 Goshoai Koryuzi-cho, Okazaki, Aichi, 444-8553, Japan.

Background: Thrombospondin type-1 domain-containing 7A (THSD7A) is a target antigen in idiopathic membranous nephropathy (MN). Patients with THSD7A-associated MN are known to have a high possibility of developing malignancy. However, there are few case reports on THSD7A-associated MN with malignancy, and details of its characteristics have not been clarified thoroughly. Read More

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http://dx.doi.org/10.1186/s12882-019-1236-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6366086PMC
February 2019
1 Read

Dynamic Thiol/Disulphide Homeostasis in Children with Nephrotic Syndrome.

Nephron 2019 Feb 6:1-9. Epub 2019 Feb 6.

Department of Pediatric Nephrology, Saglik Bilimleri University, Dr. Sami Ulus Maternity and Child Health and Diseases Training and Research Hospital, Ankara, Turkey.

Background: There is a growing body of evidence showing that there is an association between oxidative damage and kidney injury in patients with nephrotic syndrome (NS). Dynamic thiol/disulphide homeostasis (DTDH) is an important antioxidant system. The aim of this study was to determine if DTDH could be used as a biomarker of antioxidant status in pediatric NS patients using a novel automated method. Read More

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http://dx.doi.org/10.1159/000496619DOI Listing
February 2019
2 Reads

Direct oral anticoagulant successfully used to treat an adult nephrotic patient complicated with portal vein thrombosis.

CEN Case Rep 2019 Feb 5. Epub 2019 Feb 5.

Department of Nephrology, Institute of Biomedical Sciences, Tokushima University Graduate School, 3-18-15 Kuramoto-cho, Tokushima, 770-8503, Tokushima, Japan.

Thromboembolism is a major complication of nephrotic syndrome, with the renal vein being the most frequent site. However, the incidence of portal vein thrombosis (PVT) in patients with nephrotic syndrome is rare. We report a case of a relapsed steroid-dependent minimal change disease with incidental PVT. Read More

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http://dx.doi.org/10.1007/s13730-019-00381-9DOI Listing
February 2019
1 Read

Genetics of congenital and infantile nephrotic syndrome.

World J Pediatr 2019 Feb 5. Epub 2019 Feb 5.

King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia.

Background: Congenital and infantile nephrotic syndrome (CNS and INS) are rare inherited defects in glomerular filtration involving a variety of gene mutations. This study aimed to analyze all genetic mutations associated with congenital and infantile nephrotic syndrome treated at our institution. We also discussed our different approach secondary to culture and resources. Read More

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http://link.springer.com/10.1007/s12519-018-00224-0
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http://dx.doi.org/10.1007/s12519-018-00224-0DOI Listing
February 2019
3 Reads

The long-term outcome of childhood nephrotic syndrome in Germany: a cross-sectional study.

Clin Exp Nephrol 2019 Feb 5. Epub 2019 Feb 5.

Department of General Pediatrics, University Children's Hospital Bonn, Bonn, Germany.

Background: Long-term outcomes of children with nephrotic syndrome have not been well described in the literature.

Methods: Cross-sectional study data analysis of n = 43 patients with steroid-sensitive (SSNS) and n = 7 patients with steroid-resistant (SRNS) nephrotic syndrome were retrospectively collected; patients were clinically examined at a follow-up visit (FUV), on average 30 years after onset, there was the longest follow-up period to date.

Results: The mean age at FUV was 33. Read More

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http://dx.doi.org/10.1007/s10157-019-01696-8DOI Listing
February 2019
1 Read

Dosage Optimization Based on Population Pharmacokinetic Analysis of Tacrolimus in Chinese Patients with Nephrotic Syndrome.

Pharm Res 2019 Feb 4;36(3):45. Epub 2019 Feb 4.

Department of Pharmacy, Shengjing Hospital of China Medical University, Shenyang, 110004, China.

Purpose: The objective of this study was to merge genetic and non-genetic factors of tacrolimus pharmacokinetics to establish a more stable population pharmacokinetic model for individualized dosage regimen in Chinese nephrotic syndrome patients.

Methods: Nephrotic syndrome patients (>16 years old) treated with tacrolimus were included in the study. The population pharmacokinetic approach was analyzed using NONMEM version 7. Read More

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http://dx.doi.org/10.1007/s11095-019-2579-6DOI Listing
February 2019
1 Read

Mutation of microphthalmia-associated transcription factor (mitf) in zebrafish sensitizes for glomerulopathy.

Biol Open 2019 Feb 4. Epub 2019 Feb 4.

Department of Nephrology, Hannover Medical School, Hannover, Germany

Different glomerular diseases that affect podocyte homeostasis can clinically present as nephrotic syndrome with massive proteinuria, hypoalbuminemia, hyperlipidemia and edema. Up to now no drugs that specifically target the actin cytoskeleton of podocytes are on the market and model systems for library screenings to develop anti-proteinuric drugs are of high interest. We developed a standardized proteinuria model in zebrafish using puromycin aminonucleoside (PAN) via treatment in the fish water to allow for further drug testing to develop anti-proteinuric drugs for the treatment of glomerular diseases. Read More

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http://dx.doi.org/10.1242/bio.040253DOI Listing
February 2019
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The spectrum of kidney biopsy findings in patients with morbid obesity.

Kidney Int 2019 Mar 31;95(3):647-654. Epub 2019 Jan 31.

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

Morbid obesity, defined as body mass index (BMI) ≥40 kg/m, affects approximately 8% of United States adults and is a recognized risk factor for chronic kidney disease (CKD). We present the first focused biopsy-based study exploring the range of kidney diseases in this population. Among 3263 native kidney biopsies interpreted at Columbia University in 2017, we identified 248 biopsies from morbidly obese patients. Read More

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http://dx.doi.org/10.1016/j.kint.2018.11.026DOI Listing
March 2019
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Mutational spectrum and novel candidate genes in Chinese children with sporadic steroid-resistant nephrotic syndrome.

Pediatr Res 2019 Feb 2. Epub 2019 Feb 2.

Molecular Immunology Laboratory, Capital Institute of Pediatrics, YaBao Road 2, 100020, Beijing, China.

Background: Approximately 10-20% of children with idiopathic nephrotic syndrome (NS) fail to respond to steroid therapy. NS is divided into steroid-sensitive NS (SSNS) and steroid-resistant NS (SRNS). Over 45 recessive and dominant genes have been found to be associated with SRNS and/or focal segmental glomerulosclerosis (FSGS). Read More

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http://dx.doi.org/10.1038/s41390-019-0321-zDOI Listing
February 2019
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Rituximab is more effective than tacrolimus in steroid-dependent nephrotic syndrome.

Arch Dis Child Educ Pract Ed 2019 Feb 1. Epub 2019 Feb 1.

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

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http://dx.doi.org/10.1136/archdischild-2018-316537DOI Listing
February 2019
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Genetic determinants of the development and course of membranous nephropathy.

Ter Arkh 2018 Jun;90(6):105-111

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Moscow, Russia.

Membranous nephropathy (MN) is one of the most common causes of nephrotic syndrome in adults and is classified as either primary (idiopatic) or secondary MN according to underlying etiology (the later result from some known disease such as systemic autoimmune diseases, infections, malignancies, drugs, etc). In recent years, phospholipase A2 receptor 1 (PLA2R) and thrombospondin type-1 domain-containing 7A (THSD7A) were identified as two major podocytic antigens involved in the pathogenesis of idiopatic MN (IMN). And the discovery of circulating antibodies specific for these target antigens has transformed the diagnostic workup and significally improved management of IMN. Read More

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http://dx.doi.org/10.26442/terarkh2018906105-111DOI Listing
June 2018
4 Reads

Membranoproliferative glomerulonephritis in Russian population.

Ter Arkh 2018 Dec;90(12):39-47

Research Institute of Nephrology, I.P. Pavlov First Saint Petersburg State Medical University of the Ministry of Health of the Russian Federation, Saint Petersburg, Russia.

Aim: Analysis of etiology, clinical and morphological manifestations, approaches to therapy and prognosis of membranoproliferative glomerulonephritis (MPGN).

Materials And Methods: Cases of MPGN were retrospectively identified in the period 2000-2017 with subsequent analysis of etiology, clinical data and morphology (including deposits of immunoglobulins (Ig) and C3 complement fractions). The achievement of complete and partial remissions (PR, CR), overall survival, progression (by composite endpoint: decrease in the estimated GFR (eGFR) ≥50% from the baseline or eGFR <15 ml/min/1. Read More

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http://dx.doi.org/10.26442/00403660.2018.12.000007DOI Listing
December 2018
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FGF-23 in untreated nephrotic syndrome and effects of the renin-angiotensin-aldosterone system.

Authors:
Michael Pazianas

Nephrology (Carlton) 2019 Feb;24(2):273

Department of Orthopaedics, Rheumatology and Musculoskeletal Diseases, Institute of Musculoskeletal Sciences, Oxford University, Oxford, UK.

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http://dx.doi.org/10.1111/nep.13266DOI Listing
February 2019
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Nephrotic Syndrome: Oedema Formation and Its Treatment With Diuretics.

Front Physiol 2018 15;9:1868. Epub 2019 Jan 15.

UCL Centre for Nephrology, University College London, London, United Kingdom.

Oedema is a defining element of the nephrotic syndrome. Its' management varies considerably between clinicians, with no national or international clinical guidelines, and hence variable outcomes. Oedema may have serious sequelae such as immobility, skin breakdown and local or systemic infection. Read More

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http://dx.doi.org/10.3389/fphys.2018.01868DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341062PMC
January 2019
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Acute Kidney Injury in Nephrotic Syndrome.

Authors:
Shina Menon

Front Pediatr 2018 14;6:428. Epub 2019 Jan 14.

Division of Nephrology, Department of Pediatrics, University of Washington School of Medicine, Seattle Children's Hospital, Seattle, WA, United States.

Nephrotic syndrome (NS) is one of the commonest kidney diseases seen in childhood and is characterized by a relapsing remitting course. Various complications have been reported in children with NS, including infections, thromboembolism, hypovolemia, and acute kidney injury (AKI). There is often a modest decrease in renal function in patients with active proteinuria due to decreased glomerular permeability that improves when they go into remission. Read More

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https://www.frontiersin.org/article/10.3389/fped.2018.00428/
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http://dx.doi.org/10.3389/fped.2018.00428DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340287PMC
January 2019
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Keeping nephrotic syndrome on the emergency department edema differential: A case report.

World J Emerg Med 2019 ;10(2):116-118

Department of Emergency Medicine, Dalhousie University Faculty of Medicine, Halifax, Nova Scotia, Canada.

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http://dx.doi.org/10.5847/wjem.j.1920-8642.2019.02.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340815PMC
January 2019
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A young Chinese man with nephrotic syndrome due to lipoprotein glomerulopathy.

J Clin Lipidol 2018 Dec 19. Epub 2018 Dec 19.

Department of Medicine, University of Hong Kong, Queen Mary Hospital, Hong Kong. Electronic address:

Lipoprotein glomerulopathy (LPG) is a rare autosomal dominant renal disease with incomplete penetrance, associated with specific protein-modifying mutations in the APOE gene. LPG is associated with poor renal prognosis, in which lipoprotein thrombi are seen in the glomerular capillaries. Dyslipidemia in LPG generally resembles type III hyperlipoproteinemia with elevated serum apolipoprotein E level. Read More

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http://dx.doi.org/10.1016/j.jacl.2018.12.004DOI Listing
December 2018
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Urine markers of renal tubular injury in idiopathic membranous nephropathy: A cross sectional study.

Clin Chim Acta 2019 Jan 23;492:7-11. Epub 2019 Jan 23.

Department of Laboratory Medicine, Shengjing Hospital of China Medical University, China. Electronic address:

Introduction: Idiopathic membranous nephropathy (IMN) is a primary glomerular disease and a major cause of adult nephrotic syndrome. Presently, little is known about the capabilities of the urine markers to reflect the severity of IMN. We aimed at establishing whether urinary N-acetyl-β-glucosaminidase (NAG), Retinol binding protein (RBP), Kidney injury molecule-1 (KIM-1) and Neutrophil gelatianse-associated lipocalin (NGAL) are related with renal parameters and the histological grades tubular injury. Read More

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http://dx.doi.org/10.1016/j.cca.2019.01.015DOI Listing
January 2019
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Primary membranous nephropathy: comprehensive review and historical perspective.

Postgrad Med J 2019 Jan 25. Epub 2019 Jan 25.

Division of Nephrology, Department of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.

Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in non-diabetic Caucasian adults over 40 years of age. It has an estimated incidence of 8-10 cases per 1 million. Fifty per cent of patients diagnosed with primary MN continue to have nephrotic syndrome and 30% of patients may progress to end-stage renal disease over 10 years. Read More

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http://pmj.bmj.com/lookup/doi/10.1136/postgradmedj-2018-1357
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http://dx.doi.org/10.1136/postgradmedj-2018-135729DOI Listing
January 2019
5 Reads