22,090 results match your criteria Nephrotic Syndrome


Most common histopathological patterns of the Minas Gerais Association of the Centers of Nephrology.

Rev Assoc Med Bras (1992) 2019 Mar 11;65(3):441-445. Epub 2019 Apr 11.

MD, Nephrologist, MSc in Science from Unifesp, São Paulo/ São João de Deus Hospital - Brasil.

Introduction: We analyzed the distribution and frequency of glomerular diseases in patients biopsied between 1992 and 2016 in centers that make up the AMICEN (Minas Gerais Association of Nephrology Centers).

Methods: We analyzed the biopsy reports of patients from 9 AMICEN nephrology centers. We took note of their age, gender, ultrasound use, post-biopsy resting time, whether the kidney was native or a graft, number of glomeruli and indication for the biopsy. Read More

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http://dx.doi.org/10.1590/1806-9282.65.3.441DOI Listing

Effect of LDL apheresis for nephrotic idiopathic membranous nephropathy as initial induction therapy.

Ther Apher Dial 2019 Apr 17. Epub 2019 Apr 17.

Department of Nephrology, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Aichi, Japan.

Low-density lipoprotein apheresis (LDL-A) has been used for nephrotic syndrome (NS) by focal segmental glomerulosclerosis in Japan. Idiopathic membranous nephropathy (iMN) can also cause treatment-resistant NS. Therefore we investigated the effect of LDL-A during initial induction for it. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/1744-9987.12
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http://dx.doi.org/10.1111/1744-9987.12811DOI Listing
April 2019
1 Read

Nephrotic Syndrome in South African Children: Changing Perspectives in the New Millennium.

Kidney Int Rep 2019 Apr 12;4(4):522-534. Epub 2019 Feb 12.

Department of Paediatrics and Child Health, College of Health Sciences, Nelson R Mandela School of Medicine, University of KwaZulu-Natal, Durban, South Africa.

The epidemiological landscape of nephrotic syndrome (NS) in South Africa has changed drastically in the New Millennium. Although the pattern of disease in the 3 main non-Black racial groups (White, Indian, and Mixed race) mirror that seen in Western countries, Black African children show a pattern of disease that is at variance with these 3 racial groups. The incidence of infectious diseases, particularly hepatitis B virus associated nephropathy has sharply declined to being almost extinct in Black children in the New Millennium whereas HIV-related nephropathy surfaced. Read More

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http://dx.doi.org/10.1016/j.ekir.2019.01.019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6451079PMC

Examining Uncertainty in Illness in Parents and Children With Chronic Kidney Disease and Systemic Lupus Erythematosus: A Mediational Model of Internalizing Symptoms and Health-Related Quality of Life.

J Clin Psychol Med Settings 2019 Apr 15. Epub 2019 Apr 15.

Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania and the Children's Hospital of Philadelphia, 34th Street and Civic Center Blvd, Philadelphia, PA, 19104, USA.

To examine if parent illness uncertainty is indirectly associated with child depression, anxiety, and HRQOL in the CKD/SLE population. Parent-child dyads (N = 31) from outpatient rheumatology and nephrology clinics included children (ages 9-18) diagnosed with CKD (Stage 1, 2, or nephrotic syndrome) or SLE. Parents completed demographic and uncertainty measures and children completed uncertainty, depression, anxiety, and HRQOL measures. Read More

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http://dx.doi.org/10.1007/s10880-019-09617-3DOI Listing

Renoprotective Effect of Against Proteinuria and Apoptosis Induced by Adriamycin in Rat.

J Pharmacopuncture 2019 Mar 31;22(1):35-40. Epub 2019 Mar 31.

Department of Physiology, faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

Objective: Adriamycin (ADR) is an important anti-cancer drug which can cause renal toxicity. Given the known anti-inflammatory and antioxidant effects of ), the aim of this study was to determine the effects of hydroalcoholic extract of on ADR- induced nephropathy in rats.

Methods: Fifty male Wistar albino rats were randomly divided into 5 groups including: control, ADR (5 mg/kg), ADR + (600 and 1200 mg/kg) and (1200 mg/kg). Read More

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http://koreascience.or.kr/journal/view.jsp?kj=DHOCBS&py=
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http://dx.doi.org/10.3831/KPI.2019.22.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6461299PMC
March 2019
1 Read

Re: Bakkum et al. "Effects of psychological stress on proteinuria in childhood steroid-sensitive nephrotic syndrome".

J Psychosom Res 2019 Apr 9. Epub 2019 Apr 9.

Health Psychology Section, Psychology Department, Institute of Psychiatry, Psychology and Neuroscience, King's College London, UK. Electronic address:

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http://dx.doi.org/10.1016/j.jpsychores.2019.04.005DOI Listing

Membranous Nephropathy Post-Transplantation: An Update of the Pathophysiology and Management.

Transplantation 2019 Apr 10. Epub 2019 Apr 10.

Renal Division, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Membranous nephropathy (MN) is a common cause of nephrotic syndrome after transplantation and is associated with an increased risk of allograft loss. MN may occur either as a recurrent or as a de novo disease. As in native kidneys, the pathophysiology of the MN recurrence is in most cases associated with anti-phospholipid A2 receptor antibodies (antiPLA2R). Read More

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http://dx.doi.org/10.1097/TP.0000000000002758DOI Listing
April 2019
1 Read

Quality Improvement Initiative to Reduce Admissions for Nephrotic Syndrome Relapse in Pediatric Patients.

Front Pediatr 2019 29;7:112. Epub 2019 Mar 29.

Division of Nephrology, Children's National Health System, Washington, DC, United States.

Childhood nephrotic syndrome is frequently seen in pediatric nephrology practice and often requires patient hospitalization for management. Numerous complications of this disease can be managed in an outpatient setting if brought to the attention of the medical team in a timely manner. Outpatient management will reduce healthcare cost and improve patient safety. Read More

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http://dx.doi.org/10.3389/fped.2019.00112DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6449647PMC
March 2019
1 Read

C1q Nephropathy in a Patient of Neurofibromatosis Type 1: A Rare Case Report.

Indian J Nephrol 2019 Mar-Apr;29(2):125-127

Department of Nephrology and Transplantation Medicine, G.R. Doshi and K.M. Mehta Institute of Kidney Diseases and Research Centre - Dr. H.L. Trivedi Institute of Transplantation Sciences, Ahmedabad, Gujarat, India.

C1q nephropathy is a rare glomerular disease defined by the presence of characteristic mesangial dominant or codominant C1q deposition on immunofluorescence microscopy. Neurofibromatosis type 1 (NF-1) is an autosomal dominant syndrome caused by a mutation of a gene located on chromosomal segment 17q11.2. Read More

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http://www.indianjnephrol.org/preprintarticle.asp?id=252208
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http://dx.doi.org/10.4103/ijn.IJN_353_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6440327PMC
April 2019
1 Read

Loss of Subpodocytic Space Predicts Poor Response to Tacrolimus in Steroid-Resistant Calcineurin Inhibitor-Naïve Adult-Onset Primary Focal Segmental Glomerulosclerosis.

Indian J Nephrol 2019 Mar-Apr;29(2):90-94

Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Focal segmental glomerulosclerosis (FSGS) is the most common cause of adult-onset nephrotic syndrome, but its pathophysiology is poorly understood. The question as to why only a subset of patients responds to treatment in unanswered. In the past few years, change of podocytic phenotype from stationary type in health to migratory type in disease has been described, of which loss of subpodocytic space is a surrogate marker. Read More

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http://dx.doi.org/10.4103/ijn.IJN_422_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6440328PMC

Geniposide alleviates lipopolysaccharide-caused apoptosis of murine kidney podocytes by activating Ras/Raf/MEK/ERK-mediated cell autophagy.

Artif Cells Nanomed Biotechnol 2019 Dec;47(1):1524-1532

a Department of Nephrology , Jining No.1 People's Hospital , Jining , China.

Proteinuria is one of the most important clinical features of nephrotic syndrome (NS). Injury of podocyte has been proved to contribute to the occurrence of proteinuria. This study explored the effects of geniposide (GEN) on lipopolysaccharide (LPS)-caused murine kidney podocyte MPC5 apoptosis and autophagy. Read More

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http://dx.doi.org/10.1080/21691401.2019.1601630DOI Listing
December 2019

[Coronary involvement and nephrotic syndrome in systemic lupus: A case report].

Rev Med Interne 2019 Apr 10. Epub 2019 Apr 10.

Service de médecine interne 2, Centre national de référence maladies systémiques rares, lupus et syndrome des anticorps antiphospholipides, hôpital de la Pitié-Salpêtrière, Sorbonne université, Assistance publique-Hôpitaux de Paris, 47-83, boulevard de l'Hôpital, 75013 Paris, France. Electronic address:

Introduction: Heart failure during systemic lupus erythematosus has various causes.

Case Report: A 29-year-old female presented with a systemic lupus flare and a nephrotic syndrome, followed by cardiogenic shock requiring extra-corporeal membranous oxygenation. Ventricular dysfunction was related to massive myocardial infarction due to an anterior interventricular artery thrombosis and an underlying atheroma. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S02488663193009
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http://dx.doi.org/10.1016/j.revmed.2019.03.001DOI Listing
April 2019
2 Reads

APOL1 risk genotype in Europe: Data in patients with focal segmental glomerulosclerosis and after renal transplantation.

Nephrol Ther 2019 Apr;15 Suppl 1:S85-S89

Inserm U1163, 24, boulevard du Montparnasse, 75015 Paris, France; Institut Imagine, 24, boulevard du Montparnasse, 75015 Paris, France; Université Paris Descartes, 24, boulevard du Montparnasse, 75015 Paris, France; Genetic Department, hôpital Necker-Enfants-malades, AP-HP, 149, rue de Sèvres, 75015 Paris, France.

Apolipoprotein L1 (APOL1) risk variants are strongly associated with sporadic focal segmental glomerulosclerosis in populations with African ancestry. We determined the frequency of G1/G2 variants in patients with steroid-resistant nephrotic syndrome/focal segmental glomerulosclerosis with African or French West Indies origin in France and its relationships with other steroid-resistant nephrotic syndrome genes. In a cohort of 152 patients (139 families), the APOL1 risk variants were genotyped: the two risk allele (high risk) genotype was found in 43. Read More

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http://dx.doi.org/10.1016/j.nephro.2019.02.005DOI Listing

[Treatment of AL amyloidosis].

Nephrol Ther 2019 Apr;15 Suppl 1:S115-S121

Service de néphrologie et transplantation, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers, France; Centre de référence « Amylose AL et autres maladies par dépôt d'immunoglobulines monoclonales », 2, rue de la Milétrie, 86021 Poitiers, France.

AL amyloidosis is caused by the conversion of monoclonal immunoglobulin light chains into amyloid fibrillar aggregates that deposit in tissue and lead to organ dysfunction. Diagnosis is histological and relies primarily on non-invasive biopsies, showing Congo red-positive amorphous deposits containing immunoglobulin light chains, most commonly of lambda isotype. The clinical presentation is extremely polymorphous, due to the large number of organs that can be affected by the disease. Read More

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http://dx.doi.org/10.1016/j.nephro.2019.03.002DOI Listing

Association of low birth weight and prematurity with clinical outcomes of childhood nephrotic syndrome: a prospective cohort study.

Pediatr Nephrol 2019 Apr 11. Epub 2019 Apr 11.

Child Health Evaluative Sciences, Research Institute, Hospital for Sick Children, 686 Bay St, Toronto, ON, M5G 0A4, Canada.

Background: Low birth weight (LBW)/prematurity have been proposed as risk factors for the development of kidney disease in adulthood. Whether there is an association between LBW/prematurity and poor renal outcomes in childhood onset nephrotic syndrome remains unknown.

Methods: Children with nephrotic syndrome diagnosed between 1 and 18 years of age were followed prospectively from 1996 to 2016 at The Hospital for Sick Children (N = 377). Read More

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http://link.springer.com/10.1007/s00467-019-04255-1
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http://dx.doi.org/10.1007/s00467-019-04255-1DOI Listing
April 2019
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Membranous nephropathy due to chronic mercury poisoning from traditional Indian medicines: report of five cases.

Clin Kidney J 2019 Apr 3;12(2):239-244. Epub 2018 Jun 3.

Department of Nephrology, Apollo Hospitals, Chennai, Tamil Nadu, India.

Mercury contained in traditional medicines can cause chronic poisoning, which can cause membranous nephropathy (MN). We report five cases of nephrotic syndrome caused by MN with evidence of chronic mercury poisoning due to consumption of traditional Indian medicines such as Siddha and Ayurveda, which to our knowledge are the first such reports. All patients were seronegative for antibodies against phospholipase A2 receptor (PLA2R). Read More

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http://dx.doi.org/10.1093/ckj/sfy031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6452196PMC

Continuous B-cell depletion in frequently relapsing, steroid-dependent and steroid-resistant nephrotic syndrome.

Clin Kidney J 2019 Apr 24;12(2):224-231. Epub 2018 Jul 24.

Vasculitis and Glomerulonephritis Center, Division of Nephrology, Massachusetts General Hospital, Boston, MA, USA.

Background: Patients with frequently relapsing (FR), steroid-dependent (SD) and steroid-resistant (SR) nephrotic syndrome are a therapeutic challenge with limited treatment options. Here, we retrospectively analyze the efficacy and safety of rituximab-induced continuous B-cell depletion in these populations.

Methods: Patients were included if they were at least 18 years of age and had FR, SD or SR minimal change disease (MCD) or primary focal segmental glomerulosclerosis (FSGS) and were treated with a strategy of continuous B-cell depletion. Read More

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http://dx.doi.org/10.1093/ckj/sfy067DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6452201PMC
April 2019
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Novel homozygous OSGEP gene pathogenic variants in two unrelated patients with Galloway-Mowat syndrome: case report and review of the literature.

BMC Nephrol 2019 Apr 11;20(1):126. Epub 2019 Apr 11.

Molecular Biology Laboratory, Fundació Puigvert, Instituto de Investigaciones Biomédicas Sant Pau (IIB-Sant Pau), Universitat Autònoma de Barcelona, REDinREN, Instituto de Investigación Carlos III, Cartagena 340-350, 08025, Barcelona, Catalonia, Spain.

Background: Galloway-Mowat syndrome (GAMOS) is a rare autosomal recessive disorder characterized by early-onset nephrotic syndrome and microcephaly with brain anomalies. WDR73 pathogenic variants were described as the first genetic cause of GAMOS and, very recently, four novel causative genes, OSGEP, LAGE3, TP53RK, and TPRKB, have been identified.

Case Presentation: We present the clinical and genetic characteristics of two unrelated infants with clinical suspicion of GAMOS who were born from consanguineous parents. Read More

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https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882
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http://dx.doi.org/10.1186/s12882-019-1317-yDOI Listing
April 2019
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Evaluation of glucocorticoid treatment on different pathological types of primary nephrotic syndrome.

J Biol Regul Homeost Agents 2019 Mar-Apr;33(2):427-432

Nephrology Department, The Second Affiliated Hospital of Qiqihar Medical University, Qiqihar City, China.

This study aimed to assess the distribution of pathotypes in primary nephrotic syndrome (PNS) and their relationship with glucocorticoid treatment efficacy. The study included 120 patients who were treated in the nephrology, internal medicine and pediatrics wards of The Second Affiliated Hospital of Qiqihar Medical University between March 2014 and October 2017 and who underwent renal biopsy to confirm PNS. The patients with PNS were divided into a child group (40 cases, aged 0~17 years) and an adult group (80 cases, aged over 18 years). Read More

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Ofatumumab in post-transplantation recurrence of focal segmental glomerulosclerosis in a child.

Pediatr Transplant 2019 Apr 11:e13413. Epub 2019 Apr 11.

Children's Hospital at Montefiore, Bronx, New York.

FSGS is a potentially devastating form of nephrotic syndrome. Treatment of SRNS can be difficult, especially post-transplantation. The current therapy of post-transplant SRNS includes plasmapheresis, ACE-I, CNI, and monoclonal antibodies (rituximab). Read More

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http://dx.doi.org/10.1111/petr.13413DOI Listing
April 2019
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Simplified Algorithm for Evaluation of Proteinuria in Clinical Practice: How should A Clinician Approach?

Int J Prev Med 2019 5;10:35. Epub 2019 Mar 5.

Department of Pediatrics, Section of Nephrology, Rush University Medical Center, Chicago, Illinois, USA.

Background: Proteinuria is a common laboratory finding among children and adolescents. It can be identified as either a transient or a persistent finding and can represent a benign condition or a serious disease.

Methods: Pertinent medical literature for asymptomatic proteinuria in children and adolescents published in English was searched between January 1980 and May 2017 using PubMed, MEDLINE, EMBASE, and Google Scholar research databases. Read More

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http://dx.doi.org/10.4103/ijpvm.IJPVM_557_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6425769PMC
March 2019
1 Read

Open-Label Clinical Trials of Oral Pulse Dexamethasone for Adults with Idiopathic Nephrotic Syndrome.

Am J Nephrol 2019 Apr 9;49(5):377-385. Epub 2019 Apr 9.

Kidney Disease Section, National Institute of Diabetes and Digestive and Kidney Disease, Bethesda, Maryland, USA,

Background: In adults with primary focal segmental glomerulosclerosis (FSGS), daily prednisone may induce complete remissions (CR) and partial remissions (PR), but relapses are frequent and adverse events are common.

Methods: We carried out 2 open-label, uncontrolled trials to explore the efficacy and tolerability of pulse oral dexamethasone as an alternative to daily prednisone. We enrolled adult patients with proteinuria > 3. Read More

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http://dx.doi.org/10.1159/000497064DOI Listing
April 2019
1 Read

Detailed clinical manifestations at onset and prognosis of neonatal-onset Denys-Drash syndrome and congenital nephrotic syndrome of the Finnish type.

Clin Exp Nephrol 2019 Apr 8. Epub 2019 Apr 8.

Division of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan.

Background: Neonatal-onset Denys-Drash syndrome (NODDS) is a distinctive clinical entity and has a poor renal and life outcome. Early diagnosis of NODDS is important for managing disorders of sexual development and determining assigned gender. Although patients with NODDS and congenital nephrotic syndrome of the Finnish type (CNF) present with nephrotic syndrome in neonatal life or infancy, the clinical course of NODDS and factors distinguishing these diseases at onset is unknown. Read More

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http://dx.doi.org/10.1007/s10157-019-01732-7DOI Listing
April 2019
1 Read

Glomerulonephritis and nephrotic syndrome in a child with DiGeorge syndrome: Answers.

Pediatr Nephrol 2019 Apr 8. Epub 2019 Apr 8.

Wilf Children's Hospital, Pediatric Department, Shaare Zedek Medical Center, Shmuel Bait Street 12, 9103102, Jerusalem, Israel.

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http://dx.doi.org/10.1007/s00467-019-04243-5DOI Listing
April 2019
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Glomerulonephritis and nephrotic syndrome in a child with DiGeorge syndrome: Questions.

Pediatr Nephrol 2019 Apr 8. Epub 2019 Apr 8.

Wilf Children's Hospital, Pediatric Department, Shaare Zedek Medical Center, Shmuel Bait Street 12, 9103102, Jerusalem, Israel.

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http://dx.doi.org/10.1007/s00467-019-04242-6DOI Listing
April 2019
1 Read

Diagnostic role of renal biopsy in PLAR1-antibody-positive patients with nephrotic syndrome.

Mod Pathol 2019 Apr 8. Epub 2019 Apr 8.

III. Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Renal biopsy is the gold standard for diagnosis of membranous nephropathy. Circulating PLAR1 antibody found in 75% of patients with membranous nephropathy is very specific for the diagnosis of this disease. Therefore, the question arises whether PLAR1-antibody-positive patients still need a diagnostic renal biopsy. Read More

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http://dx.doi.org/10.1038/s41379-019-0267-zDOI Listing
April 2019
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Late-Onset Neutropenia after Rituximab Treatment for Adult-Onset Nephrotic Syndrome.

Case Rep Nephrol 2019 5;2019:3580941. Epub 2019 Mar 5.

Department of Nephrology, Tokyo Women's Medical University, Tokyo, Japan.

A 41-year-old woman developed nephrotic syndrome at the age of 32 and was diagnosed with minimal change nephrotic syndrome based on a renal biopsy. Although remission was achieved with administration of prednisolone (PSL) and cyclosporine, the nephrotic syndrome recurred. She was also started on rituximab (RTX). Read More

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http://dx.doi.org/10.1155/2019/3580941DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6425356PMC
March 2019
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Genetics of Nephrotic Syndrome Presenting in Childhood: Core Curriculum 2019.

Am J Kidney Dis 2019 Apr 4. Epub 2019 Apr 4.

Department of Pediatrics-Nephrology, 4 Center for Computational Medicine and Bioinformatics, University of Michigan School of Medicine, Ann Arbor, MI. Electronic address:

Nephrotic syndrome (NS) is one of the most challenging conditions to manage and treat, partly because we lack a specific molecular understanding of its pathogenesis and progression. This limits our ability to provide targeted therapy or precise prognostications. Fortunately, genomic discovery in NS and its translation to genomic-informed medicine is allowing us to improve our understanding of the molecular anatomy of NS and our ability to care for patients with NS. Read More

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http://dx.doi.org/10.1053/j.ajkd.2019.01.033DOI Listing
April 2019
3 Reads

Methylprednisolone or cyclosporine a in the treatment of Henoch-Schönlein nephritis: a nationwide study.

Pediatr Nephrol 2019 Apr 6. Epub 2019 Apr 6.

Department of Pediatric Nephrology and Transplantation, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Stenbäckinkatu 9, 00290, Helsinki, Finland.

Background: Optimal treatment of Henoch-Schönlein purpura nephritis (HSN) remains unclear. We evaluated outcome of pediatric HSN patients treated initially with either methylprednisolone (MP) or cyclosporine A (CyA) in Finland between 1996 and 2011.

Methods: Outcome of 62 HSN patients was evaluated by screening urine and blood samples (n = 51) or by collecting clinical parameters from medical charts until last follow-up visit (n = 11). Read More

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http://dx.doi.org/10.1007/s00467-019-04238-2DOI Listing
April 2019
2 Reads

Protein-losing gastropathy associated with cytomegalovirus in two sisters - Case reports and review of the literature.

Arch Pediatr 2019 Apr 3. Epub 2019 Apr 3.

Pediatric department, Centre Hospitalier Intercommunal de Créteil, 40, avenue de Verdun, 94000 Créteil, France; Centre de recherche clinique, Centre Hospitalier Intercommunal de Créteil, 40, avenue de Verdun, 94000 Créteil, France.

Ménétrier's disease is a protein-losing gastropathy that is uncommon in childhood. Its symptoms are unspecific, with abdominal pain, vomiting, and edema. Blood tests show hypoproteinemia and hypoalbuminemia, and upper digestive endoscopy reveals giant gastric folds. Read More

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http://dx.doi.org/10.1016/j.arcped.2019.03.005DOI Listing
April 2019
1 Read

TRPC channels: Regulation, dysregulation and contributions to chronic kidney disease.

Biochim Biophys Acta Mol Basis Dis 2019 Apr 3. Epub 2019 Apr 3.

Department of Biology and Biochemistry, University of Houston, Houston, TX, USA.

Mutations in the gene encoding canonical transient receptor potential-6 (TRPC6) channels result in severe nephrotic syndromes that typically lead to end-stage renal disease. Many but not all of these mutations result in a gain in the function of the resulting channel protein. Since those observations were first made, substantial work has supported the hypothesis that TRPC6 channels can also contribute to progression of acquired (non-genetic) glomerular diseases, including primary and secondary FSGS, glomerulosclerosis during autoimmune glomerulonephritis, and possibly in type-1 diabetes. Read More

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http://dx.doi.org/10.1016/j.bbadis.2019.04.001DOI Listing
April 2019
2 Reads

Management of Adult Minimal Change Disease.

Clin J Am Soc Nephrol 2019 Apr 5. Epub 2019 Apr 5.

Division of Nephrology, Department of Medicine, Rush University Medical Center, Chicago, Illinois.

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http://cjasn.asnjournals.org/lookup/doi/10.2215/CJN.01920219
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http://dx.doi.org/10.2215/CJN.01920219DOI Listing
April 2019
9 Reads

Severe hypertriglyceridaemia and pancreatitis in a patient with lipoprotein lipase deficiency based on mutations in lipoprotein lipase (LPL) and apolipoprotein A5 (APOA5) genes.

BMJ Case Rep 2019 Apr 3;12(4). Epub 2019 Apr 3.

Universitair Medisch Centrum Utrecht, Utrecht, The Netherlands.

A 44-year-old woman was admitted with pancreatitis caused by hypertriglyceridaemia (fasting triglycerides 28 mmol/L). She used oral contraceptives and ezetimibe 10 mg. She was overweight (body mass index 29. Read More

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http://dx.doi.org/10.1136/bcr-2018-228199DOI Listing

Lipomatosis of spinal epidural space, peritoneum, and renal sinus: a rare complication of long-term steroid therapy in a child with nephrotic syndrome.

Childs Nerv Syst 2019 Apr 2. Epub 2019 Apr 2.

Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.

Excessive visceral adipose tissue proliferation, resulting in diffuse lipomatosis, is a rare complication of long-term steroid therapy. A 10-year-old boy presented with severe radicular back pain with limitation of lower limb movements. He was diagnosed with steroid-resistant nephrotic syndrome and was on unregulated steroid therapy. Read More

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http://dx.doi.org/10.1007/s00381-019-04141-zDOI Listing
April 2019
3 Reads
1.163 Impact Factor

[CME: Nephrotic Syndrome in Adults: Presentation, Diagnosis, Therapy].

Praxis (Bern 1994) 2019 ;108(5):347-355

1 Klinik für Innere Medizin, Stadtspital Triemli Zürich.

CME: Nephrotic Syndrome in Adults: Presentation, Diagnosis, Therapy Abstract. The nephrotic syndrome is defined by renal protein loss with hypalbuminaemia and edema. Hyperlipoproteinemia and thrombophilia are not diagnostic criteria, but are frequently associated conditions. Read More

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http://dx.doi.org/10.1024/1661-8157/a003223DOI Listing
January 2019
1 Read

Kidney Lipidomics by Mass Spectrometry Imaging: A Focus on the Glomerulus.

Int J Mol Sci 2019 Apr 1;20(7). Epub 2019 Apr 1.

Institut Mondor de Recherche Biomédicale, INSERM, U955 EQ21, 8, rue du Général Sarrail, 94010 Créteil, France.

Lipid disorders have been associated with glomerulopathies, a distinct type of renal pathologies, such as nephrotic syndrome. Global analyses targeting kidney lipids in this pathophysiologic context have been extensively performed, but most often regardless of the architectural and functional complexity of the kidney. The new developments in mass spectrometry imaging technologies have opened a promising field in localized lipidomic studies focused on this organ. Read More

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http://dx.doi.org/10.3390/ijms20071623DOI Listing
April 2019
2 Reads

Cyclosporin population pharmacokinetics in pediatric refractory nephrotic syndrome based on real-world studies: Effects of body weight and spirolactone administration.

Exp Ther Med 2019 Apr 27;17(4):3015-3020. Epub 2019 Feb 27.

Department of Pharmacy, Children's Hospital of Fudan University, Shanghai 201102, P.R. China.

Different models of population pharmacokinetics (PPK) of cyclosporin have been established in various populations. However, the cyclosporin PPK model in patients with pediatric refractory nephrotic syndrome (PRNS) has yet to be constructed. The present study aimed to establish the cyclosporin PPK model in PRNS, and to identify factors that may account for any variability. Read More

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http://dx.doi.org/10.3892/etm.2019.7325DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6434233PMC

Clinical Pharmacology in Diuretic Use.

Authors:
David H Ellison

Clin J Am Soc Nephrol 2019 Apr 1. Epub 2019 Apr 1.

Departments of Medicine and

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http://dx.doi.org/10.2215/CJN.09630818DOI Listing
April 2019
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Regression of chronic kidney disease in a patient with AL amyloidosis: A case report
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Clin Nephrol 2019 May;91(5):317-320

Background: AL amyloidosis is a disease that causes significant end-organ damage via deposition of insoluble amyloid fibrils, which cause disruption of normal tissue architecture and function. The mainstay of current treatment employs various chemotherapy regimens, all of which aim to suppress the underlying plasma cell dyscrasia and reduce the production of amyloidogenic precursor proteins. Renal disease is found in between 50 and 80% of sufferers and is often both progressive and irreversible, ultimately leading to end-stage renal failure and death. Read More

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http://dx.doi.org/10.5414/CN109676DOI Listing
May 2019
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Membranous Nephropathy in a Patient with Human Immunodeficiency Virus Shortly After Initiation of HAART with Atripla.

Cureus 2019 Jan 21;11(1):e3932. Epub 2019 Jan 21.

Internal Medicine, Charleston Area Medical Center, Charleston, USA.

A human immunodeficiency virus (HIV) infection has long been associated with kidney disease. The pathogenesis of renal complications may be directly related to the presence of HIV viral particles or may occur secondary to an immune response against the virus. A number of HIV medications have been associated with the development of acute and chronic kidney disease. Read More

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http://dx.doi.org/10.7759/cureus.3932DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6430309PMC
January 2019
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C5 Convertase Blockade in Membranoproliferative Glomerulonephritis: A Single-Arm Clinical Trial.

Am J Kidney Dis 2019 Mar 28. Epub 2019 Mar 28.

Istituto di Ricerche Farmacologiche Mario Negri IRCCS; L. Sacco Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy. Electronic address:

Rationale & Objective: Primary membranoproliferative glomerulonephritis (MPGN) is a rare glomerulopathy characterized by complement dysregulation. MPGN progresses rapidly to kidney failure when it is associated with nephrotic syndrome. We assessed the effects of C5 convertase blockade in patients with MPGN and terminal complement activation. Read More

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http://dx.doi.org/10.1053/j.ajkd.2018.12.046DOI Listing

Effectiveness of cryofiltration and mizoribine combination with oral steroid therapy in a patient with membranoproliferative glomerulonephritis due to essential cryoglobulinemia.

CEN Case Rep 2019 Mar 29. Epub 2019 Mar 29.

Division of Nephrology, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume City, Fukuoka, 830-0011, Japan.

A 65-year-old male patient with nephrotic syndrome was admitted to our hospital due to worsening systemic edema and purpura on the limbs. He had an impaired renal function, low serum complement level, and elevated rheumatoid factor level. He was positive for cryoglobulin (monoclonal IgM-κ and polyclonal mixed-type IgG), and the results of his kidney biopsy showed a tissue profile of membranoproliferative glomerulonephritis (MPGN). Read More

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http://dx.doi.org/10.1007/s13730-019-00394-4DOI Listing
March 2019
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Combined Plasmatic and Tissue Approach to Membranous Nephropathy-Proposal of a Diagnostic Algorithm Including Immunogold Labelling: Changing the Paradigm of a Serum-based Approach.

Appl Immunohistochem Mol Morphol 2019 Mar 28. Epub 2019 Mar 28.

Department of Medicine and Surgery, Pathology, San Gerardo Hospital.

Membranous nephropathy represents the most frequent cause of nephrotic syndrome in the adult, leading to end-stage renal disease in one third of all the patients. In the last years, the discovery of circulating autoantibodies against phospholipase A2 receptor 1 (PLA2R) and thrombospondin type-1 containing 7A domain (THSD7A), shed light on the pathogenesis of idiopathic forms, being responsible for 70% and 3% of all the cases, respectively. These identifications allowed the development of serological and histologic tests to detect autoantibodies and relative targets for diagnostic and prognostic purposes. Read More

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http://dx.doi.org/10.1097/PAI.0000000000000753DOI Listing
March 2019
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Successful pregnancy with tripterygium glycoside-induced premature ovarian insufficiency: a case report.

J Int Med Res 2019 Mar 28:300060519837834. Epub 2019 Mar 28.

Key Laboratory for Reproductive Medicine and Embryology, The Reproductive Medicine Special Hospital of the First Hospital of Lanzhou University, Lanzhou, Gansu Province, China.

Premature ovarian insufficiency is characterized by reduced ovarian function in a young woman, resulting in an earlier menopause compared with women with normal ovarian function. It is a term that reflects the variable nature of the condition and is substantially less emotive than the formerly used 'premature ovarian failure', which suggested a single event in time. Tripterygium glycosides can damage ovarian function and cause infertility. Read More

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http://dx.doi.org/10.1177/0300060519837834DOI Listing
March 2019
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La ponction biopsie rénale: indications, complications et résultats.

Pan Afr Med J 2018 20;31:44. Epub 2018 Sep 20.

Centre Hospitalier Universitaire Mohammed VI, Oujda, Maroc.

Renal needle biopsy (RNB) is the gold standard in the diagnosis of kidney diseases. It is an invasive technique causing several complications, in particular hemorrhagic events. This study aims to evaluate our RNB practice, to update the current understanding of the technique for percutaneous renal biopsy, to assess complications and to determine the prevalence of kidney diseases diagnosed in our region. Read More

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http://dx.doi.org/10.11604/pamj.2018.31.44.15604DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6430949PMC
April 2019
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Disseminated Intravascular Coagulation-like Reaction after Rituximab Infusion in a Patient with Nephrotic Syndrome: A Case Report.

Intern Med 2019 Mar 28. Epub 2019 Mar 28.

Division of Nephrology, Department of Medicine, Showa University Fujigaoka Hospital, Japan.

Several case series have suggested that rituximab is efficacious in adult patients with minimal change disease. We herein report a case of disseminated intravascular coagulation-like reaction after rituximab infusion in a patient with nephrotic syndrome. A 58-year-old Japanese man with minimal change disease diagnosed 15 years earlier was started on rituximab to prevent relapse of nephrotic syndrome when he presented to our clinic with low albuminemia, massive proteinuria, and leg edema. Read More

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http://dx.doi.org/10.2169/internalmedicine.2236-18DOI Listing
March 2019
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Atypical haemolytic uraemic syndrome in the eculizumab era: presentation, response to treatment and evaluation of an eculizumab withdrawal strategy.

Br J Haematol 2019 Mar 27. Epub 2019 Mar 27.

Department of Haematology, UCLH, Cardiometabolic programme- NIHR UCLH/UCL BRC, London, UK.

The complement inhibitor, eculizumab, has revolutionised the management of atypical haemolytic uraemic syndrome (aHUS), although the optimum treatment duration is debated. Twenty-two cases of acute aHUS managed with eculizumab were retrospectively reviewed, including outcomes after eculizumab withdrawal. Although 41% had an associated complement genetic abnormality, mutation status did not affect severity of clinical presentation. Read More

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http://dx.doi.org/10.1111/bjh.15899DOI Listing
March 2019
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Response to "Predictors of rituximab-related neutropenia in Japanese children with steroid-dependent nephrotic syndrome".

Pediatr Nephrol 2019 Mar 26. Epub 2019 Mar 26.

Department of Pediatric Nephrology, Armand-Trousseau Hospital, APHP, 26, Avenue du Docteur Arnold Netter, 75012, Paris, France.

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http://dx.doi.org/10.1007/s00467-019-04239-1DOI Listing
March 2019
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[A Case of Nephrotic Syndrome Due to SOX plus Bmab Therapy for Liver Metastasis of Rectal Cancer].

Gan To Kagaku Ryoho 2019 Feb;46(2):288-290

Dept. of Surgery, Tokyo Shinagawa Hospital.

A 66-year-old man was postoperatively diagnosed with pT4a, pN2, cM1a(H2), cP0, fStage Ⅳ, RAS wild type rectal cancer. He underwent SOX plus Bmab chemotherapy 4 weeks later. After 9 courses of SOX plus Bmab, he was admitted to the hospital for leg edema and proteinuria(4+). Read More

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February 2019
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Genetic Testing Proves Crucial in Case of Ambiguous Genitalia and Renal Masses.

Urology 2019 Mar 23. Epub 2019 Mar 23.

Division of Urology, St. Louis Children's Hospital, Washington University School of Medicine, St. louis, MO. Electronic address:

The Denys-Drash syndrome consists of a triad of ambiguous genitalia, Wilm's tumor and nephrotic syndrome. We present a diagnostically challenging case of an XY patient with female appearance and Müllerian structures with a WT1 mutation resulting in gonadal dysgenesis, end-stage renal disease, and precursor changes to Wilm's tumor in both kidneys. Genetic testing proved critical in this case, helping to solidify a diagnosis and guiding our decision to proceed with bilateral nephrectomy and bilateral gonadectomy. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00904295193026
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http://dx.doi.org/10.1016/j.urology.2019.03.011DOI Listing
March 2019
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