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nephrotic syndrome review

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https://www.readbyqxmd.com/read/28722531/patterns-of-renal-disease-in-south-korea-a-20-year-review-of-a-single-center-renal-biopsy-database
#1
Ho Sik Shin, Dae Hyeon Cho, Soo Kyoung Kang, Hyun Jeong Kim, Soo Young Kim, Joung Wook Yang, Gyong Hoon Kang, Ye Na Kim, Yeonsoon Jung, Bong-Kwon Cheon, Hark Rim
BACKGROUND: Several registries and centers have reported the results of renal biopsies from different parts of the world. As there are few data regarding the epidemiology of glomerulonephritis (GN) in South Korea, we conducted this study on renal biopsy findings during the last 20 years from a single center. METHODS: Data for 818 patients who underwent renal biopsy at our center between 1992 and 2011 were collected retrospectively. All kidney specimens were examined with light microscopy (LM) and immunofluorescent microscopy (IF)...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/28717938/rituximab-in-steroid-sensitive-nephrotic-syndrome-lessons-from-clinical-trials
#2
REVIEW
Kazumoto Iijima, Mayumi Sako, Koichi Kamei, Kandai Nozu
Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. A total of 80-90% of patients with childhood idiopathic nephrotic syndrome achieve remission with steroid therapy [steroid-sensitive nephrotic syndrome (SSNS)]. However, approximately 50% of children with SSNS develop frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS). Children with FRNS or SDNS are usually treated with immunosuppressive agents, but 10-20% of children receiving immunosuppressive agents still show frequent relapses or steroid dependence during or after treatment, defined as complicated FRNS or SDNS...
July 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28668147/acute-myocardial-infarction-in-a-young-girl-with-nephrotic-syndrome-a-case-report-and-literature-review
#3
Yan Zhao, Wenhua Su, Shiqi Liu, Qian Huo, Hong Zhang
Acute myocardial infarction is not a very rare complication of nephrotic syndrome. The pathogenesis of ischemic heart disease among patients with nephrosis is commonly thrombosis, whereas atherosclerosis is rare, especially in young individuals. In this case report, we present a 15-year-old girl with nephrotic syndrome who had acute non-ST-elevation myocardial infarction secondary to atherosclerosis of the 3 coronary arteries.
July 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28661269/clinical-characteristics-and-risk-factors-of-severe-infections-in-hospitalized-adult-patients-with-primary-nephrotic-syndrome
#4
Jie Li, Qiankun Zhang, Bofeng Su
Objective Infection is a common condition in patients with nephrotic syndrome. The objective of the present study is to investigate the clinical characteristics and risk factors of infections in adult patients with primary nephrotic syndrome (PNS). Methods Medical charts of 138 consecutive patients with PNS and infections who were admitted to hospital from April 2013 to April 2016 were systematically reviewed. Results Patients were divided into three groups according to the degree of infections: mild infection group (n = 45), moderate infection group (n = 60), and severe infection group (n = 33)...
January 1, 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28660550/clinical-and-economic-evaluation-of-repository-corticotropin-injection-a-narrative-literature-review-of-treatment-efficacy-and-healthcare-resource-utilization-for-seven-key-indications
#5
REVIEW
Michael Philbin, John Niewoehner, George J Wan
INTRODUCTION: Repository corticotropin injection (RCI; H.P. Acthar(®) Gel; Mallinckrodt Pharmaceuticals Inc., Hampton, NJ) is a highly purified, prolonged-release porcine preparation of adrenocorticotropic hormone (ACTH) analogue that is FDA-approved for treatment of 19 autoimmune and inflammatory disorders. The diverse physiological actions of RCI at the melanocortin receptors (MCRs) affect processes involved in inflammation, pigmentation, steroidogenesis, and immunomodulation. Although RCI has been approved to treat inflammatory and autoimmune diseases for more than 60 years, recent progress in understanding both MCRs and the effects of RCI in modulating immune responses has led to increased interest in RCI as a therapeutic choice...
June 28, 2017: Advances in Therapy
https://www.readbyqxmd.com/read/28637442/membranous-nephropathy-a-retrospective-observational-study-of-membranous-nephropathy-in-north-east-and-central-london
#6
Sanjana Gupta, John Connolly, Ruth J Pepper, Stephen B Walsh, Magdi M Yaqoob, Robert Kleta, Neil Ashman
BACKGROUND: Membranous nephropathy (MN) is the leading cause of nephrotic syndrome in adults. MN is a clinically heterogeneous disease and it is difficult to accurately predict outcomes (including end stage renal failure) at presentation and whom to treat with potentially toxic therapies. We aimed to identify factors predicting outcome in MN in our cohort from two large tertiary London units by undertaking a retrospective data analysis of 148 biopsy-proven MN patients from North East and Central London between 1995 and 2015...
June 21, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28615961/the-role-of-novel-biomarkers-in-childhood-idiopathic-nephrotic-syndrome-a-narrative-review-of-published-evidence
#7
REVIEW
Samuel N Uwaezuoke
Two histological subtypes of idiopathic nephrotic syndrome are commonly recognized in children, namely minimal change nephropathy and focal segmental glomerulosclerosis. Children with minimal change nephropathy (the majority of whom are steroid-sensitive) and focal segmental glomerulosclerosis (the majority of whom are steroid-resistant) require early identification in order to ensure appropriate therapeutic intervention and better outcome. Although renal biopsy and histology remain the ideal diagnostic steps to identify these histological subtypes, reports indicate that serum and urinary biomarkers are now being utilized in the investigation of childhood idiopathic nephrotic syndrome...
2017: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/28560690/extrarenal-determinants-of-kidney-filter-function
#8
REVIEW
Eunsil Hahm, Vasil Peev, Jochen Reiser
The kidney is an organ involved in cross talk with many human organs. The link between the immune system and the kidney has been studied in some detail, although data precisely elucidating their interaction are sparse, in particular with regard to the function of the kidney filter apparatus. Current research suggests that an understanding of the impairment of this cross talk between the bone marrow, as a fundament of the immune system and the kidney will provide meaningful insights into the pathophysiological mechanisms of impaired kidney filter function...
July 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/28553650/recent-treatment-advances-and-new-trials-in-adult-nephrotic-syndrome
#9
REVIEW
Eva Königshausen, Lorenz Sellin
The etiology of nephrotic syndrome is complex and ranges from primary glomerulonephritis to secondary forms. Patients with nephrotic syndrome often need immunosuppressive treatment with its side effects and may progress to end stage renal disease. This review focuses on recent advances in the treatment of primary causes of nephrotic syndrome (idiopathic membranous nephropathy (iMN), minimal change disease (MCD), and focal segmental glomerulosclerosis (FSGS)) since the publication of the KDIGO guidelines in 2012...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28550082/primary-membranous-nephropathy
#10
William G Couser
Membranous nephropathy (MN) is a unique glomerular lesion that is the most common cause of idiopathic nephrotic syndrome in nondiabetic white adults. About 80% of cases are renal limited (primary MN, PMN) and 20% are associated with other systemic diseases or exposures (secondary MN). This review focuses only on PMN. Most cases of PMN have circulating IgG4 autoantibody to the podocyte membrane antigen PLA2R (70%), biopsy evidence PLA2R staining indicating recent immunologic disease activity despite negative serum antibody levels (15%), or serum anti-THSD7A (3%-5%)...
June 7, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28546764/optimal-management-of-primary-focal-segmental-glomerulosclerosis-in-adults
#11
REVIEW
Séverine Beaudreuil, Hans Kristian Lorenzo, Michele Elias, Erika Nnang Obada, Bernard Charpentier, Antoine Durrbach
Focal segmental glomerulosclerosis (FSGS) is a frequent glomerular kidney disease that is revealed by proteinuria or even nephrotic syndrome. A diagnosis can be established from a kidney biopsy that shows focal and segmental glomerulosclerosis. This histopathological lesion may be caused by a primary podocyte injury (idiopathic FSGS) but is also associated with other pathologies (secondary FSGS). The first-line treatment for idiopathic FSGS with nephrotic syndrome is a prolonged course of corticosteroids. However, steroid resistance or steroid dependence is frequent, and despite intensified immunosuppressive treatment, FSGS can lead to end-stage renal failure...
2017: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/28540057/rituximab-in-minimal-change-disease-mechanisms-of-action-and-hypotheses-for-future-studies
#12
REVIEW
Nima Madanchi, Martin Bitzan, Tomoko Takano
Treatment with rituximab, a monoclonal antibody against the B-lymphocyte surface protein CD20, leads to the depletion of B cells. Recently, rituximab was reported to effectively prevent relapses of glucocorticoid-dependent or frequently relapsing minimal change disease (MCD). MCD is thought to be T-cell mediated; how rituximab controls MCD is not understood. In this review, we summarize key clinical studies demonstrating the efficacy of rituximab in idiopathic nephrotic syndrome, mainly MCD. We then discuss immunological features of this disease and potential mechanisms of action of rituximab in its treatment based on what is known about the therapeutic action of rituximab in other immune-mediated disorders...
2017: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/28530079/-kidney-diseases-in-north-israel-according-to-kidney-biopsies-bnai-zion-medical-center-14-years-experience
#13
Shimon Storch, Nadav Willner, Aurora Toubi, Simona Croitoru, Vladimir Wolfson, Ibrahim Matar, Elinor Grushka, Majed Odeh, Efrat Wolfovits, Elad Schiff, Yitzhak Rosner, Elias Toubi, Aharon Kessel, Ofer Ben Izhak, Boaz Moskovitz, Ofer Nativ
INTRODUCTION: Little is known about the prevalence of kidney diseases according to renal biopsy in Israel. Since updated literature worldwide emphasizes changing etiologies of chronic kidney disease, it is crucial to research and define the epidemiology and pathology of kidney disease in Israel. Hereby, we introduce an original review of the prevalence of kidney diseases in our study population, which we believe reflects the prevalence of kidney diseases in the population of Israel. AIMS: To investigate the prevalence of kidney diseases diagnosed by renal biopsy, according to age, gender, race and clinical symptoms...
September 2016: Harefuah
https://www.readbyqxmd.com/read/28528869/temporary-removal-pure-membranous-lupus-nephritis-description-of-a-cohort-of-150-patients-and-review-of-the-literature
#14
Lucía Silva-Fernández, Teresa Otón, Anca Askanase, Patricia Carreira, Francisco Javier López-Longo, Alejandro Olivé, Íñigo Rúa-Figueroa, Javier Narváez, Esther Ruiz-Lucea, Mariano Andrés, Enrique Calvo, Francisco Toyos, Juan José Alegre-Sancho, Eva Tomero, Carlos Montilla, Antonio Zea, Esther Uriarte, Jaime Calvo-Alén, Carlos Marras, Víctor M Martínez-Taboada, María Ángeles Belmonte-López, José Rosas, Enrique Raya, Gema Bonilla, Mercedes Freire, José María Pego-Reigosa, Isabel Millán, Adwoa Hughes-Morley
The publisher regrets that this article has been temporarily removed. A replacement will appear as soon as possible in which the reason for the removal of the article will be specified, or the article will be reinstated. The full Elsevier Policy on Article Withdrawal can be found at: https://www.elsevier.com/about/our-business/policies/article-withdrawal.
May 18, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28515908/renal-manifestations-of-primary-mitochondrial-disorders
#15
Josef Finsterer, Fulvio Alexandre Scorza
The aim of the present review was to summarize and discuss previous findings concerning renal manifestations of primary mitochondrial disorders (MIDs). A literature review was performed using frequently used databases. The study identified that primary MIDs frequently present as mitochondrial multiorgan disorder syndrome (MIMODS) at onset or in the later course of the MID. Occasionally, the kidneys are affected in MIDs. Renal manifestations of MIDs include renal insufficiency, nephrolithiasis, nephrotic syndrome, renal cysts, renal tubular acidosis, Bartter-like syndrome, Fanconi syndrome, focal segmental glomerulosclerosis, tubulointerstitial nephritis, nephrocalcinosis, and benign or malign neoplasms...
May 2017: Biomedical Reports
https://www.readbyqxmd.com/read/28515029/tolvaptan-utilization-in-children-with-chronic-hyponatremia-due-to-inappropriate-antidiuretic-hormone-secretion-siadh-three-case-reports-and-review-of-the-literature
#16
Gerdi Tuli, Daniele Tessaris, Luisa De Sanctis, Patrizia Matarazzo
Hyponatremia is the most common electrolyte disorder among hospitalized patients and it is sometimes considered as a poor outcome predictor. Its correction is thus indicated, even in asymptomatic patients. The conventional treatment is represented by fluid restriction in presence of euvolemia or hypervolemia; whereas loop diuretics are used in some hypervolemic conditions (cardiac heart failure, liver cirrhosis and nephrotic syndrome) and intravenous isotonic or hypertonic solution are administered in hypovolemic conditions...
May 17, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/28503551/a-case-of-fibrillary-glomerulonephritis-with-fibril-deposition-in-the-arteriolar-wall-and-a-family-history-of-renal-disease
#17
Kentaro Watanabe, Kentaro Nakai, Nozomi Hosokawa, Shuhei Watanabe, Keiji Kono, Shunsuke Goto, Hideki Fujii, Shigeo Hara, Shinichi Nishi
Herein, we report a case of fibrillary glomerulonephritis (FGN). FGN usually shows non-amyloidal fibrils in the mesangium and glomerular capillary walls on electron microscopy. Inherited cases of FGN have been reported in only 3 families, and the suspected genetic form was autosomal dominant. In the present case, the deposition of microfibrils in the arteriolar wall as well as the glomerulus is unique. Our patient's father died of nephrotic syndrome, and his elder brother had a biopsy-proven glomerulopathy...
January 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28480100/the-burden-and-outcome-of-pediatric-renal-admissions-at-the-federal-teaching-hospital-abakaliki-a-3-year-review-2011-2013
#18
V U Muoneke, A F Una, C B Eke, O U Anyanwu
BACKGROUND: Renal diseases are important causes of morbidity and mortality in children worldwide particularly in the resource-poor countries of sub-Saharan Africa. Adequate data on these diseases in children in our setting are limited as a result of late/nonpresentation. AIM: The aim of the study is to review the pattern and outcome of pediatric renal admissions at the Federal Teaching Hospital (FETH) Abakaliki over a 3-year period. SUBJECTS AND METHODS: This was a retrospective observational review of all childhood renal admissions in FETH, Abakaliki, Ebonyi state between 2011 and 2013...
July 2016: Annals of Medical and Health Sciences Research
https://www.readbyqxmd.com/read/28474581/presentations-and-management-of-different-causes-of-chylothorax-in-children-one-medical-center-s-experience
#19
Chien-Heng Lin, Wei-Ching Lin, Jeng-Sheng Chang
BACKGROUND: Chylothorax in children is a relatively rare cause of pleural effusion. However, it is usually a common complication of cardiothoracic operations like open-heart surgery. Other etiologies for chylothorax, such as trauma or malignancy, occur more common in adults and rare in children. To explore the etiologies of chylothorax in children, this study analyzed the pediatric patients that were admitted in to onea medical center. METHODS: We retrospectively reviewed the medical records of the pediatric patients that were admitted to this tertiary transfer center with a diagnosis of chylothorax during the period of 1995 to 2005...
March 2017: BioMedicine
https://www.readbyqxmd.com/read/28451893/immunology-of-idiopathic-nephrotic-syndrome
#20
REVIEW
Manuela Colucci, Giorgia Corpetti, Francesco Emma, Marina Vivarelli
The pathogenesis of idiopathic nephrotic syndrome (INS) is as yet unknown, but several lines of evidence indicate that the immune system may play a crucial pathogenic role in non-genetic INS. The most important of these are, first, the effectiveness of therapy based on immunosuppression and, second, a vast body of data derived both from experimental models and from patient studies that implicate T cells and more recently B cells as major players in INS pathogenesis. However, recent findings also suggest a direct role of podocytes as drivers of the disease process, and the interplay between the glomerulus and the immune system is still being elucidated...
April 27, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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