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Nephrotic syndrome

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https://www.readbyqxmd.com/read/28341877/short-courses-of-daily-prednisolone-during-upper-respiratory-tract-infections-reduce-relapse-frequency-in-childhood-nephrotic-syndrome
#1
Asiri S Abeyagunawardena, R S Thalgahagoda, Pathum V Dissanayake, Shamali Abeyagunawardena, Y A Illangasekera, Umeshi I Karunadasa, Richard S Trompeter
BACKGROUND: Relapses of childhood nephrotic syndrome (NS) are frequently precipitated by viral upper respiratory tract infections (URTIs). A review of the literature reveals that in patients with steroid-dependent NS on alternate day corticosteroids, a short course of daily corticosteroid therapy during the course of an URTI may reduce relapse frequency. OBJECTIVE: To assess the effect of a short course of low-dose corticosteroid therapy during the course of an URTI on relapse frequency in patients with steroid-sensitive NS who have not been taking any treatment for a minimum period of 3 months...
March 24, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28340492/hemolysis-elevated-liver-enzymes-low-platelets-syndrome-superimposed-on-hemolytic-uremic-syndrome
#2
Inês Martins, Madalena Gomes Conceição, Paulo Pereira Gomes, Nuno Clode
A pregnancy complicated by typical hemolytic uremic syndrome (HUS) and hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome is reported. At 20 weeks of gestation, a case of HUS was diagnosed, with Shiga toxin-producing Escherichia coli identified. Plasmapheresis allowed continuation of the pregnancy for 5 weeks. Superimposed preeclampsia and HELLP syndrome were diagnosed after the establishment of nephrotic range proteinuria, hypertension and recurrence of hemolysis. This is a singular case, as it demonstrates that HELLP syndrome can superimpose upon HUS, a fact that can impact future research on reproductive immunology...
March 24, 2017: Revista Brasileira de Ginecologia e Obstetrícia
https://www.readbyqxmd.com/read/28339906/interstitial-fibrosis-scored-on-whole-slide-digital-imaging-of-kidney-biopsies-is-a-predictor-of-outcome-in-proteinuric-glomerulopathies
#3
Laura H Mariani, Sebastian Martini, Laura Barisoni, Pietro A Canetta, Jonathan P Troost, Jeffrey B Hodgin, Matthew Palmer, Avi Z Rosenberg, Kevin V Lemley, Hui-Ping Chien, Jarcy Zee, Abigail Smith, Gerald B Appel, Howard Trachtman, Stephen M Hewitt, Matthias Kretzler, Serena M Bagnasco
Background.: Interstitial fibrosis (IF), tubular atrophy (TA) and interstitial inflammation (II) are known determinants of progression of renal disease. Standardized quantification of these features could add value to current classification of glomerulopathies. Methods.: We studied 315 participants in the Nephrotic Syndrome Study Network (NEPTUNE) study, including biopsy-proven minimal change disease (MCD = 98), focal segmental glomerulosclerosis (FSGS = 121), membranous nephropathy (MN = 59) and IgA nephropathy (IgAN = 37)...
February 27, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28339709/clinicopathologic-correlations-of-renal-pathology-in-adult-population-of-poland
#4
Agnieszka Perkowska-Ptasinska, Artur Bartczak, Malgorzata Wagrowska-Danilewicz, Agnieszka Halon, Krzysztof Okon, Aldona Wozniak, Marian Danilewicz, Henryk Karkoszka, Andrzej Marszalek, Jolanta Kowalewska, Andrzej Mroz, Agnieszka Korolczuk, Andrzej Oko, Alicja Debska-Slizien, Beata Naumnik, Zbigniew Hruby, Marian Klinger, Kazimierz Ciechanowski, Marek Myslak, Wladyslaw Sulowicz, Andrzej Rydzewski, Andrzej Wiecek, Jacek Manitius, Tadeusz Gregorczyk, Stanislaw Niemczyk, Michal Nowicki, Ryszard Gellert, Tomasz Stompor, Monika Wieliczko, Krzysztof Marczewski, Leszek Paczek, Olga Rostkowska, Dominika Deborska-Materkowska, Grazyna Bogdanowicz, Andrzej Milkowski, Magdalena Durlik
Background: This is the first report on the epidemiology of biopsy-proven kidney diseases in Poland. Methods: The Polish Registry of Renal Biopsies has collected information on all (n = 9394) native renal biopsies performed in Poland from 2009 to 2014. Patients' clinical data collected at the time of biopsy, and histopathological diagnoses were used for epidemiological and clinicopathologic analysis. Results: There was a gradual increase in the number of native renal biopsies performed per million people (PMP) per year in Poland in 2009-14, starting from 36 PMP in 2009 to 44 PMP in 2014...
February 11, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28337616/follow-up-results-of-patients-with-adck4-mutations-and-the-efficacy-of-coq10-treatment
#5
Mustafa Atmaca, Bora Gulhan, Emine Korkmaz, Mihriban Inozu, Oguz Soylemezoglu, Cengiz Candan, Aysun Karabay Bayazıt, Ahmet Midhat Elmacı, Gonul Parmaksiz, Ali Duzova, Nesrin Besbas, Rezan Topaloglu, Fatih Ozaltin
BACKGROUND: ADCK4-related glomerulopathy is an important differential diagnosis in adolescents with steroid-resistant nephrotic syndrome (SRNS) and/or chronic kidney disease (CKD) of unknown origin. We screened adolescent patients to determine the frequency of ADCK4 mutation and the efficacy of early CoQ10 administration. METHODS: A total of 146 index patients aged 10-18 years, with newly diagnosed non-nephrotic proteinuria, nephrotic syndrome, or chronic renal failure and end-stage kidney disease (ESKD) of unknown etiology were screened for ADCK4 mutation...
March 24, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28336816/glomerular-density-and-volume-in-renal-biopsy-specimens-of-children-with-proteinuria-relative-to-preterm-birth-and-gestational-age
#6
Kentaro Koike, Yohei Ikezumi, Nobuo Tsuboi, Go Kanzaki, Kotaro Haruhara, Yusuke Okabayashi, Takaya Sasaki, Makoto Ogura, Akihiko Saitoh, Takashi Yokoo
BACKGROUND AND OBJECTIVES: A low total nephron number, which is associated with low birth weight (LBW), may indicate increased susceptibility to early-onset renal diseases in children. However, few studies have assessed renal biopsy findings in LBW children. We examined the relationship between LBW and glomerular density (GD) and/or glomerular volume (GV) in renal biopsy samples as a surrogate for total nephron number. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Renal biopsy findings of children of LBW were compared with those of age-matched control subjects of normal birth weight (NBW) who were histopathologically diagnosed with FSGS or minimal change nephrotic syndrome (MCNS) from 1995 to 2011...
March 23, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28335748/steroid-resistant-nephrotic-syndrome-as-the-initial-presentation-of-nail-patella-syndrome-a-case-of-a-de-novo-lmx1b-mutation
#7
Tomohiro Nakata, Ryo Ishida, Yuu Mihara, Atsuko Fujii, Yoshimoto Inoue, Tetsuro Kusaba, Tsuyoshi Isojima, Yutaka Harita, Chiaki Kanda, Sachiko Kitanaka, Keiichi Tamagaki
BACKGROUND: Nail-patella syndrome (NPS) is an autosomal dominant disorder caused by mutations in the LMX1B gene and is characterized by nail dysplasia, skeletal abnormalities, and nephropathy. We herein report a case of steroid-resistant nephrotic syndrome (SRNS) prior to overt orthopedic symptoms in a patient with NPS. CASE PRESENTATION: A 24-year-old woman presented to our hospital with knee pain. She had poorly developed nails, hypoplastic patellas, dislocation of the elbows, and iliac horns in the pelvis...
March 23, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28326197/childhood-nephrotic-syndrome-management-and-outcome-a-single-center-retrospective-analysis
#8
Chia-Shi Wang, Jia Yan, Robert Palmer, James Bost, Mattie Feasel Wolf, Larry A Greenbaum
There is a paucity of information on outpatient management and risk factors for hospitalization and complications in childhood nephrotic syndrome (NS). We described the management, patient adherence, and inpatient and outpatient usage of 87 pediatric NS patients diagnosed between 2006 and 2012 in the Atlanta Metropolitan Statistical Area. Multivariable analyses were performed to examine the associations between patient characteristics and disease outcome. We found that 51% of the patients were treated with two or more immunosuppressants...
2017: International Journal of Nephrology
https://www.readbyqxmd.com/read/28321320/calcineurin-inhibitors-in-the-treatment-of-primary-focal-segmental-glomerulosclerosis-a-systematic-review-and-meta-analysis-of-the-literature
#9
REVIEW
Louis-Philippe Laurin, Patrick H Nachman, Bethany J Foster
PURPOSE OF REVIEW: Primary focal segmental glomerulosclerosis (FSGS) is the most common cause of nephrotic syndrome in adults. Glucocorticoids have been evaluated in the treatment of primary FSGS in numerous retrospective studies. Evidence suggesting a role for including calcineurin inhibitors (CNIs) in early therapy remains limited. The aim of this study was to systematically review the literature examining the efficacy of CNIs in the treatment of primary FSGS both as first-line therapy and as an adjunctive agent in steroid-resistant patients, with respect to remission in proteinuria and renal survival...
2017: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/28321309/recurrent-podocytopathy-in-a-patient-with-systemic-lupus-erythematosus
#10
Shereen Paramalingam, Daniel D Wong, Gursharan K Dogra, Johannes C Nossent
Podocytopathy in systemic lupus erythematosus is characterised by diffuse foot process effacement without significant peripheral capillary wall immune deposits as seen on electron microscopy. Lupus podocytopathy falls outside the scope of the current International Society of Nephrology and the Renal Pathology Society classification of lupus nephritis. We present a case of relapsing podocytopathy with nephrotic syndrome occurring simultaneously with two extra-renal and serological disease flares, which makes it likely that podocytopathy was related to systemic lupus erythematosus activity...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28321019/myocardial-infarction-in-a-young-man-with-nephrotic-syndrome
#11
Zhenliang Chu, Hang Zhu, Bin Zhang, Liqin Jiang
A 26-year-old man diagnosed with nephrotic syndrome (NS) 2 years previously presented with chest pain. An electrocardiogram (ECG) performed at a local hospital showed ST-elevation in chest leads. Cardiac troponin-I was significantly positive. Echocardiography revealed mild regional wall-motion abnormalities in the heart apex. Seven days later, angiography (CAG) revealed a thrombus in the left anterior descending branch (LAD). Tirofiban was injected into the LAD for thromboclasis. ECG after CAG showed the ST-segment was much lower than before...
March 17, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28318625/mycophenolate-mofetil-is-inferior-to-tacrolimus-in-sustaining-remission-in-children-with-idiopathic-steroid-resistant-nephrotic-syndrome
#12
Aditi Sinha, Aarti Gupta, Mani Kalaivani, Pankaj Hari, Amit K Dinda, Arvind Bagga
Studies of nephrotic syndrome show that substitution of calcineurin inhibitors by mycophenolate mofetil (MMF) enables sustained remission and corticosteroid sparing and avoids therapy associated adverse effects. However, controlled studies in patients with steroid resistance are lacking. Here we examined the effect of switching from therapy with tacrolimus to MMF on disease course in an open-label, one-to-one randomized, controlled trial on children (one to 18 years old), recently diagnosed with steroid-resistant nephrotic syndrome, at a referral center in India...
March 15, 2017: Kidney International
https://www.readbyqxmd.com/read/28314927/enhanced-podocyte-vesicle-transport-in-the-nephrotic-rat
#13
Akihiro Tojo, Saaya Hatakeyama, Satoshi Kinugasa, Satoru Fukuda, Toshio Sakai
Albumin endocytosis is enhanced in the podocytes of minimal change nephrotic syndrome. We investigated that the endocytic vesicle transport in the podocyte using three-dimensional observation in a rat model of puromycin aminonucleoside (PAN)-induced nephrotic syndrome. At day 7, Evans Blue-labeled albumin was intravenously injected in PAN rats, and one kidney was fixed for a morphological analysis; the other was used for the isolation of glomeruli through sieving and protein analyses. Evans Blue-labeled albumin was found to accumulate in an increased number of vesicles in the podocytes of PAN rat...
March 17, 2017: Medical Molecular Morphology
https://www.readbyqxmd.com/read/28314744/randomised-controlled-trial-comparing-ofatumumab-to-rituximab-in-children-with-steroid-dependent-and-calcineurin-inhibitor-dependent-idiopathic-nephrotic-syndrome-study-protocol
#14
Pietro Ravani, Alice Bonanni, Gian Marco Ghiggeri
INTRODUCTION: Oral steroids induce remission in about 90% of children with idiopathic nephrotic syndrome (INS), which is characterised by severe proteinuria and hypoalbuminaemia. Some children become steroid-dependent (SD) and require addition of calcineurin inhibitors (CNI) to maintain remission. Since these oral agents are toxic, alternative interventions are needed for long-term treatment. The anti-CD20 antibody rituximab has shown promising steroid-sparing properties in clinical trials, but benefits are less convincing in complicated forms of SD-INS...
March 17, 2017: BMJ Open
https://www.readbyqxmd.com/read/28314579/keeping-the-customers-stratified-moving-toward-genetics-based-treatment-options-in-childhood-ns
#15
Megan Puckelwartz, H William Schnaper
In this issue, Bierzynska et al. report the results of analyzing a cohort of children with steroid-resistant nephrotic syndrome (SRNS) of childhood, correlating genetic studies with clinical outcomes. This approach has the potential to advance both our research into the causes and treatments of childhood SRNS and our ability to stratify patients into groups that are likely to be responsive to specific treatments. Here, we discuss some of the strengths and limitations of this report.
April 2017: Kidney International
https://www.readbyqxmd.com/read/28303389/variability-of-diagnostic-criteria-and-treatment-of-idiopathic-nephrotic-syndrome-across-european-countries
#16
Georges Deschênes, Marina Vivarelli, Licia Peruzzi
The aim of the surveys conducted by the Idiopathic Nephrotic Syndrome Working Group of the ESPN was to study the possible variability of treatment in Europe at different stages of the disease by means of questionnaires sent to members of the Working Group. Four surveys have been completed: treatment of the first flare, treatment of the first relapse and the issue of steroid dependency, use of rituximab, and the management of steroid-resistant patients. A uniform treatment of the first flare was applied in only three countries, and ten additional centers have adopted one of the three main protocols...
March 16, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28299461/efficacy-of-higher-dose-levamisole-in-maintaining-remission-in-steroid-dependant-nephrotic-syndrome
#17
Asiri S Abeyagunawardena, Umeshi Karunadasa, Heshan Jayaweera, Shenal Thalgahagoda, Sampath Tennakoon, Shamali Abeyagunawardena
OBJECTIVE: Levamisole (LEV) has been used successfully on an alternate-day regime of 2.5 mg/kg in steroid-dependant nephrotic syndrome (SDNS) to maintain remission. This pilot study was carried out between 2010 and 2015 at a single center in Sri Lanka to evaluate the efficacy of LEV prescribed at 2.5 mg/kg daily, which is double the alternate-day dose. METHODS: Sequential children with SDNS, relapsing more than twice in the preceding 12 months and previously treated with LEV and low-dose alternate-day prednisolone (0...
March 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28299339/cytoplasmic-localization-of-wt1-and-decrease-of-mirna-16-1-in-nephrotic-syndrome
#18
Pablo Zapata-Benavides, Mariela Arellano-Rodríguez, Juan José Bollain-Y-Goytia, Moisés Armides Franco-Molina, Gloria Azucena Rangel-Ochoa, Esperanza Avalos-Díaz, Rafael Herrera-Esparza, Cristina Rodríguez-Padilla
Nephrotic syndrome (NS) is a glomerular disease that is defined by the leakage of protein into the urine and is associated with hypoalbuminemia, hyperlipidemia, and edema. Steroid-resistant NS (SRNS) patients do not respond to treatment with corticosteroids and show decreased Wilms tumor 1 (WT1) expression in podocytes. Downregulation of WT1 has been shown to be affected by certain microRNAs (miRNAs). Twenty-one patients with idiopathic NS (68.75% were SSNS and 31.25% SRNS) and 10 healthy controls were enrolled in the study...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28298181/early-onset-of-adck4-glomerulopathy-with-renal-failure-a-case-report
#19
Ksenija Lolin, Benedetta D Chiodini, Elise Hennaut, Brigitte Adams, Karin Dahan, Khalid Ismaili
BACKGROUND: We present a rare early presentation of a ADCK4-related glomerulopathy. This case is of interest as potentially treatable if genetic results are timely obtained. CASE PRESENTATION: We report the case of a 5-year-old boy who was identified with significant proteinuria by a urinary routine screening program for school children. Physical examination revealed dysplastic ears and abnormal folded pinna. Albumin level was 41 g/L (39-53 g/L), and urine proteins/creatinine ratio was 2...
March 16, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28296375/-endomyocardial-fibrosis-associated-to-antiphospholipid-syndrome-case-report-and-review
#20
Carlos A Parquet, Jorge Curotto-Grasiosi, Rogelio A Machado, Bruno Peressotti, Matías B Padilla, Alberto L Bolaño
A 29-year-old man with a history of seizures, was admitted due to an episode of unconsciousness recovered and hypertension with renal disfunction. The electrocardiogram mimicked a hypertrophic cardiomyopathy, but, by Doppler echocardiography, this was discarded because it suggested endomyocardial fibrosis which was confirmed by cardiac magnetic resonance imaging with late enhancement. Since the episode of unconsciousness, brain imaging studies were performed showing vascular sequelae and microangiopathic lesions...
March 2017: Revista Médica del Instituto Mexicano del Seguro Social
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