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https://www.readbyqxmd.com/read/28441404/angiopoietin-like-4-and-minimal-change-disease
#1
Gabriel Cara-Fuentes, Alfons Segarra, Cecilia Silva-Sanchez, Heiman Wang, Miguel A Lanaspa, Richard J Johnson, Eduardo H Garin
BACKGROUND: Minimal Change Disease (MCD) is the most common type of nephrotic syndrome in children. Angiopoietin-like-4 (Angplt4) has been proposed as mediator of proteinuria in MCD. The aim of this study was to evaluate the role of Angptl4 as a biomarker in MCD. METHODS: Patients with biopsy-proven primary MCD, focal segmental glomerulosclerosis, membranous nephropathy (60, 52 and 52 respectively) and 18 control subjects had urinary and serum Angptl4 measured by Elisa...
2017: PloS One
https://www.readbyqxmd.com/read/28434525/quiz-page-may-2017-ckd-and-nephrotic-syndrome-after-allogeneic-hematopoietic-cell-transplantation
#2
Jonathan J Hogan, Matthew D Palmer, Alison W Loren, Benjamin L Laskin
No abstract text is available yet for this article.
May 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28434126/relapse-of-nephrotic-syndrome-during-post-rituximab-peripheral-blood-b-lymphocyte-depletion
#3
Mai Sato, Koichi Kamei, Masao Ogura, Kenji Ishikura, Shuichi Ito
BACKGROUND: Rituximab is effective against complicated childhood steroid-dependent nephrotic syndrome (SDNS). Peripheral blood B-lymphocyte (B-cell) depletion is strongly correlated with persistent remission, relapse rarely occurring during B-cell depletion; however, we have encountered several such patients. METHODS: We retrospectively analyzed the characteristics and clinical course of 82 patients with SDNS treated with rituximab from January 2007 to December 2012 in our institution...
April 22, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28428331/a-personalized-model-of-coq2-nephropathy-rescued-by-the-wild-type-coq2-allele-or-dietary-coenzyme-q10-supplementation
#4
Jun-Yi Zhu, Yulong Fu, Adam Richman, Zhanzheng Zhao, Patricio E Ray, Zhe Han
Clinical studies have identified patients with nephrotic syndrome caused by mutations in genes involved in the biosynthesis of coenzyme Q10 (CoQ10), a lipid component of the mitochondrial electron transport chain and an important antioxidant. However, the cellular mechanisms through which these mutations induce podocyte injury remain obscure. Here, we exploited the striking similarities between Drosophila nephrocytes and human podocytes to develop a Drosophila model of these renal diseases, and performed a systematic in vivo analysis assessing the role of CoQ10 pathway genes in renal function...
April 20, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28427453/the-italian-society-for-pediatric-nephrology-sinepe-consensus-document-on-the-management-of-nephrotic-syndrome-in-children-part-i-diagnosis-and-treatment-of-the-first-episode-and-the-first-relapse
#5
REVIEW
Andrea Pasini, Elisa Benetti, Giovanni Conti, Luciana Ghio, Marta Lepore, Laura Massella, Daniela Molino, Licia Peruzzi, Francesco Emma, Carmelo Fede, Antonella Trivelli, Silvio Maringhini, Marco Materassi, Giovanni Messina, Giovanni Montini, Luisa Murer, Carmine Pecoraro, Marco Pennesi
This consensus document is aimed at providing an updated, multidisciplinary overview on the diagnosis and treatment of pediatric nephrotic syndrome (NS) at first presentation. It is the first consensus document of its kind to be produced by all the pediatric nephrology centres in Italy, in line with what is already present in other countries such as France, Germany and the USA. It is based on the current knowledge surrounding the symptomatic and steroid treatment of NS, with a view to providing the basis for a separate consensus document on the treatment of relapses...
April 21, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28425160/causes-of-nephrotic-syndrome-and-nephrotic-range-proteinuria-are-different-in-adult-chinese-patients-a-single-centre-study-over-33%C3%A2-years
#6
Jack Kit-Chung Ng, Terry King-Wing Ma, Fernand Mac-Moune Lai, Kai Ming Chow, Bonnie Ching-Ha Kwan, Chi Bon Leung, Philip Kam-To Li, Cheuk Chun Szeto
BACKGROUND: The reported causes of nephrotic syndrome (NS) varied between different countries. Less is known about the causes of nephrotic-range proteinuria (NPU). METHODS: In this single-centre, retrospective study of adult patients who underwent renal biopsy between 1983-2015 in a tertiary referral hospital in Hong Kong, we aimed to evaluate the underlying causes of NS and NPU. We also determined the distribution of histopathological diagnoses with regard to the age subgroups and time periods RESULTS: Among 7,456 patients who underwent renal biopsy, 982 and 838 patients had NS and NPU, respectively...
April 20, 2017: Nephrology
https://www.readbyqxmd.com/read/28424276/thrombin-induced-podocyte-injury-is-protease-activated-receptor-dependent
#7
Ruchika Sharma, Amanda P Waller, Shipra Agrawal, Katelyn J Wolfgang, Hiep Luu, Khurrum Shahzad, Berend Isermann, William E Smoyer, Marvin T Nieman, Bryce A Kerlin
Nephrotic syndrome is characterized by massive proteinuria and injury of specialized glomerular epithelial cells called podocytes. Studies have shown that, whereas low-concentration thrombin may be cytoprotective, higher thrombin concentrations may contribute to podocyte injury. We and others have demonstrated that ex vivo plasma thrombin generation is enhanced during nephrosis, suggesting that thrombin may contribute to nephrotic progression. Moreover, nonspecific thrombin inhibition has been shown to decrease proteinuria in nephrotic animal models...
April 19, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28420111/plantago-asiatica-l-ameliorates-puromycin-aminonucleoside-induced-nephrotic-syndrome-by-suppressing-inflammation-and-apoptosis
#8
Min Chul Kho, Ji Hun Park, Byung Hyuk Han, Rui Tan, Jung Joo Yoon, Hye Yoom Kim, You Mee Ahn, Yun Jung Lee, Dae Gill Kang, Ho Sub Lee
OBJECTIVE: Nephrotic syndrome, a kidney disease with a variety of causes, is mainly characterized by heavy proteinuria, hypoproteinemia, and ascites. This study was designed to evaluate the underlying mechanism of action of Plantago asiatica L. (PAL) in treating nephrotic syndrome induced by puromycin aminonucleoside. METHODS: PAL has been used in Asia as a traditional medicine and dietary health supplement. Sprague-Dawley (SD) rats were intravenously injected with puromycin aminonucleoside (75 mg/kg/day), then treated with either Losartan (30 mg/kg/day) or PAL (200 mg/kg/day) by oral gavage for seven days...
April 14, 2017: Nutrients
https://www.readbyqxmd.com/read/28420061/metabolic-profiling-reveals-new-serum-biomarkers-of-lupus-nephritis
#9
J Li, X-W Xie, H Zhou, B Wang, M-J Zhang, F-Y Tang
Metabolomics has been applied to explore altered metabolite profiles in disease and identify unique metabolic signatures specific to certain pathologies. The aim of the current study is to characterize the metabolic profile of patients diagnosed with lupus nephritis (LN) and explore new insights into underlying disease processes. A metabolomic approach using ultra-high-performance liquid chromatography coupled with high-resolution mass spectrometry (UPLC-HRMS) was developed in serum samples from 32 LN patients, 30 idiopathic nephrotic syndrome (INS) patients and 28 healthy controls (HCs)...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28416778/steroid-pulse-therapy-for-de-novo-minimal-change-disease-during-pregnancy
#10
Hiroshi Sato, Yuna Asami, Reona Shiro, Masato Aoki, Miki Yasuda, Saeko Imai, Rie Sakai, Kenji Oida, Kanako Kawaharamura, Hiroko Yano, Nao Taguchi, Takako Suzuki, Masaya Hirose
BACKGROUND Nephrotic syndrome occurs very rarely, in only about 0.01%-0.02% of all pregnancies, and de novo minimal change disease during pregnancy is especially rare. Nephrotic syndrome and, especially, minimal change disease are highly responsive to steroids, and preterm labor may be avoidable if the maternal condition is improved with steroid therapy. Therefore, prompt diagnosis and proper management are critical to maternal and fetal outcome when severe proteinuria occurs during pregnancy. CASE REPORT A 30-year-old pregnant Japanese woman presented with systemic edema, oliguria, and severe proteinuria and hypoalbuminemia at 25 weeks of gestation, although she was normotensive...
April 18, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28413416/immunohistochemical-glomerular-expression-of-phospholipase-a2-receptor-in-primary-and-secondary-membranous-nephropathy-a-retrospective-study-in-an-indian-cohort-with-clinicopathological-correlations
#11
Sanjeet Roy, Anila Korula, Gopal Basu, Shibu Jacob, Santosh Varughese, Veeraswamy Tamilarasi
BACKGROUND: Limited published literature exists on the utility and standardization of anti-phospholipase A2 receptor (anti-PLA2R) immunohistochemistry (IHC) for the diagnosis of primary membranous nephropathy (MN). The study aimed to validate anti-PLA2R IHC for the diagnosis of primary MN and clinicopathological correlations in an Indian cohort. METHODS: Subjects included patients with primary and secondary MN diagnosed between January 2012 and August 2014 with an adequate renal biopsy and at least 1 year of clinical follow-up...
January 2017: Nephron Extra
https://www.readbyqxmd.com/read/28408801/effects-of-%C3%AE-d-mannuronic-acid-as-a-novel-non-steroidal-anti-inflammatory-medication-within-immunosuppressive-properties-on-il17-ror%C3%AE-t-il4-and-gata3-gene-expressions-in-rheumatoid-arthritis-patients
#12
Anis Barati, Ahmad Reza Jamshidi, Hossein Ahmadi, Zahra Aghazadeh, Abbas Mirshafiey
Rheumatoid arthritis (RA) is the most common form of chronic inflammatory arthritis characterized by pain, swelling and destruction of joints, with a resultant disability. Disease-modifying anti-rheumatic drugs (DMARDs) and biological drugs can interfere with the disease process. In this study, the effect of β-d-mannuronic acid (M2000) as a novel non-steroidal anti-inflammatory drug (NSAID) with immunosuppressive and anti-inflammatory effects together with antioxidant effects was evaluated on IL17, RORγt, IL4 and GATA3 gene expression in 12 RA patients...
2017: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/28405891/renalase-in-children-with-glomerular-kidney-diseases
#13
Piotr Skrzypczyk, Joanna Przychodzień, Małgorzata Mizerska-Wasiak, Elżbieta Kuźma-Mroczkowska, Magdalena Okarska-Napierała, Elżbieta Górska, Anna Stelmaszczyk-Emmel, Urszula Demkow, Małgorzata Pańczyk-Tomaszewska
Studies suggest that renalase, a renal catecholamine-inactivating enzyme, plays a major role in the pathogenesis of kidney and cardiovascular diseases in adults. This study seeks to determine the role of renalase in children with glomerular kidney diseases. We evaluated the serum renalase, arterial stiffness, intima-media thickness, blood pressure, and clinical and biochemical parameters in 78 children (11.9 ± 4.6 years of age) with glomerulopathies such as idiopathic nephrotic syndrome (40 cases), IgA nephropathy (12 cases), Henoch-Schönlein nephropathy (12 cases), and other glomerulopathies (14 cases)...
April 13, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28405841/focal-segmental-glomerulosclerosis-and-medullary-nephrocalcinosis-in-children-with-adck4-mutations
#14
Eujin Park, Hee Gyung Kang, Young Hun Choi, Kyoung Bun Lee, Kyung Chul Moon, Hyeon Joo Jeong, Michio Nagata, Hae Il Cheong
BACKGROUND: Mutations in the AarF domain containing kinase 4 gene (ADCK4), one of the novel genes causing steroid-resistant nephrotic syndrome (SRNS), usually manifest as isolated adolescent-onset focal segmental glomerulosclerosis (FSGS). ADCK4 interacts with components of the coenzyme Q10 (CoQ10) biosynthesis pathway. METHODS: The incidence and phenotypes of patients with ADCK4 mutations were investigated in a cohort of Korean pediatric patients with SRNS. RESULTS: Among the 53 patients enrolled in the study the incidence of ADCK4-associated FSGS was 7...
April 12, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28396739/nephrotic-syndrome-due-to-minimal-change-disease-secondary-to-spider-bite-clinico-pathological-case-of-a-non-described-complication-of-latrodectism
#15
Gonzalo P Méndez, Daniel Enos, José Luis Moreira, Fátima Alvaredo, David Oddó
The patient was an 18-year-old man who developed nephrotic syndrome after a 'wheat spider' bite (Latrodectus mactans). Due to this atypical manifestation of latrodectism, a renal biopsy was performed showing minimal change disease. The nephrotic syndrome subsided after 1 week without specific treatment. This self-limited evolution suggests that the mechanism of podocyte damage was temporary and potentially mediated by a secondary mechanism of hypersensitivity or direct effect of the α-latrotoxin. The patient did not show signs of relapse in subsequent checkup...
April 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28392951/three-novel-mutations-in-the-nphs1-gene-in-vietnamese-patients-with-congenital-nephrotic-syndrome
#16
Thi Kim Lien Nguyen, Van Dem Pham, Thu Huong Nguyen, Trung Kien Pham, Thi Quynh Huong Nguyen, Huy Hoang Nguyen
Congenital nephrotic syndrome, a rare and severe disease, is inherited as an autosomal recessive trait. The disease manifests shortly after birth and occurs predominantly in families of Finnish origin but has now been observed in all countries and races. Mutations in the NPHS1 gene, which encodes nephrin, are the main causes of congenital nephrotic syndrome in patients. In this study, we report the first mutational analysis of the NPHS1 gene in three unrelated children from three different Vietnamese families...
2017: Case Reports in Genetics
https://www.readbyqxmd.com/read/28392820/genetics-of-hereditary-nephrotic-syndrome-a-clinical-review
#17
REVIEW
Tae-Sun Ha
Advances in podocytology and genetic techniques have expanded our understanding of the pathogenesis of hereditary steroid-resistant nephrotic syndrome (SRNS). In the past 20 years, over 45 genetic mutations have been identified in patients with hereditary SRNS. Genetic mutations on structural and functional molecules in podocytes can lead to serious injury in the podocytes themselves and in adjacent structures, causing sclerotic lesions such as focal segmental glomerulosclerosis or diffuse mesangial sclerosis...
March 2017: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28391348/membranous-nephropathy-thinking-through-the-therapeutic-options
#18
Daniel Cattran, Paul Brenchley
Idiopathic membranous nephropathy (IMN) remains the most common cause of the nephrotic syndrome in adults and one of the leading identifiable causes of end-stage kidney disease. Prior to considering the best approach to treatment, three important components need to be considered. First, the natural history of the typical membranous patient today; second, the importance of identifying the causative factors; and third, the integration of the current data on the known autoantibody/antigen systems involved in IMN into the diagnosis and management of the patient...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28391342/learning-to-live-with-nephrotic-syndrome-experiences-of-adult-patients-and-parents-of-children-with-nephrotic-syndrome
#19
Heather Beanlands, Maria Maione, Caroline Poulton, Emily Herreshoff, Michelle A Hladunewich, Marilyn Hailperin, Mary Margaret Modes, Lawrence An, Julie Wright Nunes, Howard Trachtman, Patrick Nachman, Debbie S Gipson
Background: People living with nephrotic syndrome (NS) need to develop an in-depth understanding of their condition in order to participate in treatment decisions, develop self-management skills and integrate illness into daily life. However, the learning needs of adult patients and parents of children with NS are unknown. We therefore explored patient and parent perspectives on learning needs related to NS as part of a larger study to develop a shared learning tool for NS. Methods: Qualitative data were collected using semistructured focus groups and individual interviews with adult patients (n = 22) and parents of children with NS (n = 25)...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28391338/what-are-we-missing-in-the-clinical-trials-of-focal-segmental-glomerulosclerosis
#20
Ladan Zand, Richard J Glassock, An S De Vriese, Sanjeev Sethi, Fernando C Fervenza
Focal segmental glomerulosclerosis (FSGS) is a lesion and not a disease. This conundrum is the crux of controversies regarding interventions to alter its natural history. In the broadest sense, the lesion can be primary (idiopathic), or secondary to a process originating outside the kidneys or to a genetic mutation. The organ-based target is the podocyte, and the mechanisms responsible for the podocytopathy are numerous and diverse. Recurrence of primary FSGS in renal allografts provides the best evidence for the existence of a circulating factor or factors, the nature of which remains uncertain...
January 1, 2017: Nephrology, Dialysis, Transplantation
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