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nephrotic syndrome steroid

Rossana Malatesta-Muncher, Karen W Eldin, Laurence H Beck, Mini Michael
BACKGROUND: Idiopathic membranous nephropathy is an uncommon cause of nephrotic syndrome in children and can present treatment challenges. The current treatment options of steroids and cyclophosphamide, cyclosporine, or mycophenolate require prolonged treatment durations and the associated side effects may result in nonadherence in children, especially in adolescents. CASE-DIAGNOSIS: We report two adolescent patients with idiopathic membranous nephropathy with nephrotic range proteinuria and elevated anti-phospholipase A2 receptor levels who did not achieve remission with steroids and were later treated with rituximab...
March 15, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Daniela A Braun, Jillian K Warejko, Shazia Ashraf, Weizhen Tan, Ankana Daga, Ronen Schneider, Tobias Hermle, Tilman Jobst-Schwan, Eugen Widmeier, Amar J Majmundar, Makiko Nakayama, David Schapiro, Jia Rao, Johanna Magdalena Schmidt, Charlotte A Hoogstraten, Hannah Hugo, Sevcan A Bakkaloglu, Jameela A Kari, Sherif El Desoky, Ghaleb Daouk, Shrikant Mane, Richard P Lifton, Shirlee Shril, Friedhelm Hildebrandt
Background: Nephrotic syndrome (NS), a chronic kidney disease, is characterized by significant loss of protein in the urine causing hypoalbuminemia and edema. In general, ∼15% of childhood-onset cases do not respond to steroid therapy and are classified as steroid-resistant NS (SRNS). In ∼30% of cases with SRNS, a causative mutation can be detected in one of 44 monogenic SRNS genes. The gene LAMA5 encodes laminin-α5, an essential component of the glomerular basement membrane. Mice with a hypomorphic mutation in the orthologous gene Lama5 develop proteinuria and hematuria...
March 9, 2018: Nephrology, Dialysis, Transplantation
Fei Liu, Jian-Hua Mao
BACKGROUND: Calcineurin inhibitors (CNIs) are commonly given to transplant recipients of kidneys and other solid organs and to patients with immune disorders, such as steroid-resistant nephrotic syndrome, steroid-dependent nephrotic syndrome, and frequent relapse nephrotic syndrome. Although CNIs remain the most effective available immunosuppressant agent, there is clinical concern regarding possible long-term nephrotoxicity. This concern is especially significant in children who have a longer life expectancy and greater growth rate...
March 12, 2018: World Journal of Pediatrics: WJP
N Prasad, R Manjunath, D Rangaswamy, A Jaiswal, V Agarwal, D Bhadauria, A Kaul, R Sharma, A Gupta
Calcineurin inhibitors (CNIs) are the preferred drugs for treatment of childhood steroid-resistant nephrotic syndrome (SRNS) who are also resistant to cyclophosphamide (CYC). Although few studies have shown a benefit of one over the other, efficacy and safety of either CNIs (tacrolimus [TAC] or cyclosporine [CSA]) in this special population remained to be assessed in long-term studies. Forty-five children with SRNS who were also resistant to CYC (CYC-SRNS) from January 2006 to June 2011, were included in the study...
January 2018: Indian Journal of Nephrology
Gabriel Cara-Fuentes, Miguel A Lanaspa, Gabriela E Garcia, Mindy Banks, Eduardo H Garin, Richard J Johnson
Minimal Change Disease (MCD) is the most common type of nephrotic syndrome in children. The etiology has remained unknown, although it is commonly thought to be due to an unknown circulating factor that triggers podocyte dysfunction. To date, several changes in podocytes have been reported in MCD, of which one is the expression of CD80, also known as B7.1, which is a costimulatory molecule that is normally expressed on antigen -presenting cells. Some studies suggest that subjects with steroid-sensitive MCD may express CD80 in their podocytes during relapse and that this expression is associated with high urinary levels of CD80...
March 1, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Angela Martinez-Perez, Silvia Roure Díez, Moncef Belhasen-Garcia, Diego Torrús-Tendero, Jose Luis Perez-Arellano, Teresa Cabezas, Cristina Soler, Marta Díaz-Menéndez, Miriam Navarro, Begoña Treviño, Fernando Salvador-Vélez
INTRODUCTION: Strongyloides stercoralis is a globally distributed nematode that causes diverse clinical symptoms in humans. Spain, once considered an endemic country, has experienced a recent increase in imported cases. The introduction of serology helps diagnosis and is currently replacing microbiological techniques in some settings, but its sensitivity is variable and can be low in immunocompromised patients. Diagnosis can only be confirmed by identification of larvae. Often, this "gold standard" can only be achieved in severe cases, such as disseminated S...
February 23, 2018: PLoS Neglected Tropical Diseases
Shojiro Watanabe, Tomomi Aizawa, Hiroyasu Tsukaguchi, Koji Tsugawa, Kazushi Tsuruga, Akemi Shono, Kandai Nozu, Kazumoto Iijima, Kensuke Joh, Hiroshi Tanaka
Recent advances in high-throughput sequencing for clinical genetic testing have revealed novel disease-causing genes, such as Crumbs homolog 2 (CRB2) for early-onset steroid-resistant nephrotic syndrome (SRNS). We report the long-term clinicopathologic observation of a Japanese female patient with SRNS caused by a newly identified compound heterozygous mutation of CRB2 (p.Arg628Cys and p.Gly839Trp located in the 10th and 11th epidermal growth factor-like domains, respectively). She was initially examined during a mass urinary mass screening for 3...
February 23, 2018: Nephrology
Beata Bieniaś, Przemysław Sikora
In chronic glomerulopathies, renal fibrosis (RF) results from extracellular matrix remodeling processes regulated by matrix metalloproteinases (MMP) and tissue inhibitors of metalloproteinases (TIMP). We assessed urinary (u-) and serum (s-) MMP-1, -2, -9, TIMP-1, -2 concentrations and MMP-1, -2, -9/TIMP-1, -2 ratios in children with nephrotic syndrome. Steroid-dependent and steroid-resistant nephrotic patients (SDNS-Ps and SRNS-Ps, respectively) were compared with respect to measured parameters. The correlations of measured parameters with magnitude of proteinuria and histopathological diagnosis were determined...
February 2018: Medicine (Baltimore)
Bedra Sharif, Moumita Barua
PURPOSE OF REVIEW: The widespread adoption of next-generation sequencing by research and clinical laboratories has begun to uncover the previously unknown genetic basis of many diseases. In nephrology, one of the best examples of this is seen in focal and segmental glomerulosclerosis (FSGS) and nephrotic syndrome. We review advances made in 2017 as a result of human and molecular genetic studies as it relates to FSGS and nephrotic syndrome. RECENT FINDINGS: There are more than 50 monogenic genes described in steroid-resistant nephrotic syndrome and FSGS, with seven reported in 2017...
February 19, 2018: Current Opinion in Nephrology and Hypertension
Masashi Nishida, Hidekazu Kawakatsu, Kenji Hamaoka
BACKGROUND: Recent studies indicated the role of urinary liver-type fatty acid-binding protein (uL-FABP) as a biomarker for kidney injury. However, uL-FABP excretion in patients with relapsing nephrotic syndrome and tubular dysfunction in the pediatric population had not been reported previously. METHODS: We examined uL-FABP level in children with steroid-sensitive nephrotic syndrome (SSNS), in those with tubular dysfunction, and in control subjects. RESULTS: uL-FABP was markedly increased in patients with relapsing SSNS (12...
February 20, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
Hiroshi Yamaguchi, Atsutoshi Shiratori, Taku Nakagawa, Kyoko Kanda, Shigeo Hara, Norishige Yoshikawa, Ryojiro Tanaka
The underlying histopathology is very important in determining patient management, as the histopathology usually has direct repercussions on the treatment response and clinical course. However, the impact of the method used to assess renal biopsies, i.e., light microscopy (LM), immunofluorescence (IF), and electron microscopy (EM), on the occurrence of a difficult biopsy classification in the native kidneys of pediatric nephrotic patients is unknown. A 12-month-old Japanese boy was diagnosed with nephrotic syndrome (NS); he was administered prednisolone (60 mg/m2 /day), and a continuous albumin infusion was started...
September 2017: Case Reports in Nephrology and Dialysis
Kapil Bhalla, Deepali Garg, Mahima Rajan, Jaya Shankar Kaushik, Geeta Gathwala
Nephrotic syndrome is a prothrombotic state with predisposition to venous sinus thrombosis and arterial vascular stroke. Watershed infarcts in junction of arterial territory develop in hypotensive hypovolemic state. These border zone infarcts are usually bilateral in the absence of unilateral arterial stenosis or microembolism. We report a 6-year-girl of frequently relapsing nephrotic syndrome who developed sudden onset hemiparesis with aphasia. Magnetic resonance (MR) imaging brain revealed unilateral watershed infarct in territory between the major cerebral arterial vessels with evidence of restricted diffusion and normal vessel anatomy on MR angiography...
January 2018: Journal of Natural Science, Biology, and Medicine
Alice Bonanni, Marta Calatroni, Matteo D'Alessandro, Sara Signa, Enrica Bertelli, Michela Cioni, Eddi Di Marco, Roberto Biassoni, Gianluca Caridi, Giulia Ingrasciotta, Roberta Bertelli, Armando Di Donato, Maurizio Bruschi, Alberto Canepa, Giorgio Piaggio, Pietro Ravani, Gian Marco Ghiggeri
AIMS: Anti-CD20 antibodies are increasingly being used to treat idiopathic nephrotic syndrome (INS) in children. While they may allow steroid and calcineurin-inhibitor withdrawal, repeated infusions of anti-CD20 antibodies are often required to maintain remission. Data on their potential toxicity in INS are needed to consider repeated infusions. METHODS: We reported data on side effects related to the use of rituximab (a chimeric anti-body; 130 pts) and ofatumumab (a humanized anti-body; 37 pts) in children with INS (steroid-dependent and steroid/calcineurin-inhibitor dependent disease) treated in a national referral center during a 9-year period (400 treatments; follow-up 1-9 years)...
February 13, 2018: British Journal of Clinical Pharmacology
Kirtisudha Mishra, Sandeep Kumar Kanwal, Sushma Veeranna Sajjan, Vikram Bhaskar, Bimbadhar Rath
BACKGROUND AND OBJECTIVE: Nearly 50% of the children with steroid sensitive nephrotic syndrome (SSNS) have a frequently relapsing (FR) or steroid dependent (SD) course, experiencing steroid toxicities and complications of immunosuppression. The study aimed to compare parameters between children with infrequent relapsing (IFR) and FR/SD nephrotic syndrome and to identify the factors associated with a FR/SD course. METHODS: A retrospective analysis of medical records from 2009 to 2014, of children with SSNS attending the pediatric nephrology clinic in a tertiary care medical college and hospital...
February 8, 2018: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
Moglie Le Quintrec, Anne-Laure Lapeyraque, Arnaud Lionet, Anne-Laure Sellier-Leclerc, Yahsou Delmas, Véronique Baudouin, Eric Daugas, Stéphane Decramer, Leila Tricot, Mathilde Cailliez, Philippe Dubot, Aude Servais, Catherine Mourey-Epron, Franck Pourcine, Chantal Loirat, Véronique Frémeaux-Bacchi, Fadi Fakhouri
BACKGROUND: Cases reports and small series of patients with C3 glomerulopathy have reported variable efficacy of eculizumab. STUDY DESIGN: Case series of C3 glomerulopathy. SETTING & PARTICIPANTS: Pediatric and adult patients with C3 glomerulopathy treated with eculizumab between 2010 and 2016 were identified through the C3 glomerulopathy French registry database, and a questionnaire was sent to participating French pediatric and adult nephrology centers, as well as one pediatric referral center in Québec, Canada...
February 8, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Rozita Hoseini, Kamran Sabzian, Hasan Otukesh, Nazanin Zafaranloo, Parsa Panahi, Nahid Rahimzadeh, Shahrbanoo Nakhaie, Mohsen Akhavan Sepehi
INTRODUCTION: There is evidence of the effectiveness of rituximab in treatment of nephrotic syndrome in children. The present study aimed to assess safety and the therapeutic effectiveness of rituximab in steroid- and cyclosporine-resistant pediatric nephrotic syndrome. MATERIALS AND METHODS: Forty-three children with steroid- and cyclosporine-resistant or steroid- and cyclosporine-dependent noncongenital nephrotic syndrome were included in the study to receive intravenous rituximab, 375 mg/m2/wk, for 4 weeks...
January 2018: Iranian Journal of Kidney Diseases
Yuan Yang, Li Zhao, Li Xiao, Yumei Liang, Chang Wang, Xiao Fu, Xuejing Zhu, Shuguang Yuan, Jianling Zhu, Xiaoping Zhu, Yinghong Liu, Jun Li, Jian Luo, Fuyou Liu, Lin Sun
BACKGROUND/AIMS: The present study aimed to explore the equivalence of CHL and tacrolimus (TAC), despite reports regarding the efficacy and safety of TAC in treating SRNS patients. METHODS: A retrospective cohort study of CHL or TAC treatment was performed by collecting the medical records of SRNS patients with a pathological classification of focal segmental glomurular sclerosis (FSGS) or membranous nephropathy (MN) from December 2008 to December 2014 in a 3A grade hospital in southern China...
January 24, 2018: Kidney & Blood Pressure Research
Markus J Kemper, Thomas J Neuhaus
Many patients with steroid-sensitive nephrotic syndrome develop a relapsing course; therefore, alternative treatment may be necessary to avoid steroid toxicity. In this issue, a multicenter controlled study in relapsing steroid-sensitive nephrotic syndrome shows the effectiveness of levamisole. Time to first relapse was significantly increased compared with placebo. In addition, possible differential treatment effects were suggested for subgroups: patients with frequent relapses might have a superior response to those with steroid dependency...
February 2018: Kidney International
Maria-Carmen Serrano-Perez, Frances C Tilley, Fabien Nevo, Christelle Arrondel, Selim Sbissa, Gaëlle Martin, Kalman Tory, Corinne Antignac, Géraldine Mollet
Podocin is a key component of the slit diaphragm in the glomerular filtration barrier, and mutations in the podocin-encoding gene NPHS2 are a common cause of hereditary steroid-resistant nephrotic syndrome. A mutant allele encoding podocin with a p.R138Q amino acid substitution is the most frequent pathogenic variant in European and North American children, and the corresponding mutant protein is poorly expressed and retained in the endoplasmic reticulum (ER) both in vitro and in vivo To better understand the defective trafficking and degradation of this mutant, we generated human podocyte cell lines stably expressing podocinwt or podocinR138Q Although it has been proposed that podocin has a hairpin topology, we present evidence for podocinR138Q N-glycosylation, suggesting that most of the protein has a transmembrane topology...
January 30, 2018: Journal of Biological Chemistry
Giovanni Conti, Dominique De Vivo, Claudia Fede, Stefania Arasi, Angela Alibrandi, Roberto Chimenz, Domenico Santoro
BACKGROUND: Low birth weight (LBW) is associated with reduced nephron endowment. Clinical-pathologic features of post adaptive focal segmental glomerulosclerosis (FSGS) have been observed in subjects with prematurity and very LBW. METHODS: We aimed to investigate the correlation between LBW and outcome in a cohort of 89 children with idiopathic nephrotic syndrome (NS) (2-12 years-old at onset, followed for > 3 years), of whom 21 with LBW (birth weight < 10th percentile for gestational age, gender, ethnicity, and maternal parity or birth weight < 2500 g)...
January 19, 2018: Journal of Nephrology
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