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https://www.readbyqxmd.com/read/28937086/pulmonary-thromboembolism-as-first-presentation-of-childhood-membranous-nephropathy
#1
Mohit Madken, Shankar Prasad Nagaraju, Ravindra Prabhu Attur, Mahesha Vankalakunti
Nephrotic syndrome is associated with an increased risk of thromboembolism. Pulmonary thromboembolism has been described in nephrotic syndrome with or without deep vein thrombosis. In this case report, we describe an unusual first presentation of childhood membranous nephropathy with massive pulmonary thrombus with pulmonary infarction with right renal vein thrombosis.
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28937085/galloway-mowat-syndrome-unusual-form-of-nephrotic-syndrome-in-adolescent
#2
G Diwakar Naidu, Pathapati Deepthi, K RajaKarthik, S Sriram, G Swarnalatha, T Gangadhar
Galloway-Mowat syndrome (GMS), also acknowledged as Microcephaly-Hiatal hernia nephrotic syndrome, is an uncommon genetic disorder inherited as an autosomal recessive trait usually seen before two years of life. It is an exceptional multisystem genetic disorder with a collection of skeletal, neurological, facial, gastrointestinal, growth, and renal abnormalities. This case report describes GMS in a girl, suffering from developmental delay, stunted growth, and various dysmorphic features, in whom nephrotic syndrome became apparent at adolescent age...
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28937067/histopathological-patterns-of-renal-diseases-in-egyptian-children-a-single-center-experience
#3
Maher Ahmed Abdel-Hafez, Hend Abdel-Nabi, Mohamed El-Gamasy, Hossam Zayton, Ibrahim Nassar
The present cross-sectional, retrospective study was aimed to determine the histopathological spectrum of renal diseases in Egyptian children and to evaluate the indications, safety, and efficacy of percutaneous renal biopsy (PRB) in a large tertiary center in Egypt. PRBs performed at the Department of Pediatrics, Tanta University Hospital over a period of nine years (from January 2007 to December 2015) were included. Light microscopic (LM) examination was performed in all cases while immunofluorescence and electron microscopic examination were performed in selected cases...
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28933341/c4d-expressing-glomerulopathy-and-proteinuria-post-transplantation-of-a-%C3%A2-too-big-for-size-mismatched-kidney-allograft-an-unusual-case-with-good-outcome%C3%A2
#4
Francois Gougeon, Alexei V Mikhailov, Keisha Gibson, Tomasz Kozlowski, Harsharan K Singh, Volker Nickeleit
A 5-year-old severely growth-retarded child with tubulointerstitial, oliguric end-stage renal disease received an adult-size kidney transplant. Three years post grafting under standard triple immunosuppression (mycophenolate mofetil, tacrolimus, and prednisone) de novo nephrotic range proteinuria without the nephrotic syndrome developed. Graft function was normal (serum creatinine: 0.2 - 0.3 mg/dL), there were no donor-specific HLA antibodies (DSA), and the urine sediment was inactive. Two biopsies collected 3 and 4 years post-transplantation showed severe glomerular capillary wall remodeling and associated pseudolinear C4d staining as morphologic correlates for the proteinuria...
September 21, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28921387/analysis-of-24-genes-reveals-a-monogenic-cause-in-11-1-of-cases-with-steroid-resistant-nephrotic-syndrome-at-a-single-center
#5
Weizhen Tan, Svjetlana Lovric, Shazia Ashraf, Jia Rao, David Schapiro, Merlin Airik, Shirlee Shril, Heon Yung Gee, Michelle Baum, Ghaleb Daouk, Michael A Ferguson, Nancy Rodig, Michael J G Somers, Deborah R Stein, Asaf Vivante, Jillian K Warejko, Eugen Widmeier, Friedhelm Hildebrandt
BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) is the second most frequent cause of end-stage renal disease (ESRD) among patients manifesting at under 25 years of age. We performed mutation analysis using a high-throughput PCR-based microfluidic technology in 24 single-gene causes of SRNS in a cohort of 72 families, who presented with SRNS before the age of 25 years. METHODS: Within an 18-month interval, we obtained DNA samples, pedigree information, and clinical information from 77 consecutive children with SRNS from 72 different families seen at Boston Children's Hospital (BCH)...
September 18, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28916546/approach-to-membranous-lupus-nephritis-a-survey-of-pediatric-nephrologists-and-pediatric-rheumatologists
#6
Alexis Boneparth, Suhas M Radhakrishna, Larry A Greenbaum, Eric Yen, Daryl M Okamura, Jennifer C Cooper, Sherene Mason, Deborah M Levy, Sangeeta D Sule, Paul T Jensen, Cagri Yildirim-Toruner, Stacy P Ardoin, Scott E Wenderfer
OBJECTIVE: To describe treatment practices for childhood pure membranous lupus nephritis (MLN). METHODS: Survey study of Childhood Arthritis and Rheumatology Research Alliance and American Society of Pediatric Nephrology members. RESULTS: There were 117 respondents who completed the survey (60 pediatric nephrologists, 57 pediatric rheumatologists). Steroids and nonsteroid immunosuppression (NSI) were routinely used by the majority for MLN...
September 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28915908/advances-in-paediatrics-in-2016-current-practices-and-challenges-in-allergy-autoimmune-diseases-cardiology-endocrinology-gastroenterology-infectious-diseases-neonatology-nephrology-neurology-nutrition-pulmonology
#7
REVIEW
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Silvia Montella, Sergio Bernasconi
This review reports main progresses in various pediatric issues published in Italian Journal of Pediatrics and in international journals in 2016. New insights in clinical features or complications of several disorders may be useful for our better understanding. They comprise severe asthma, changing features of lupus erythematosus from birth to adolescence, celiac disease, functional gastrointestinal disorders, Moebius syndrome, recurrent pneumonia. Risk factors for congenital heart defects, Kawasaki disease have been widely investigated...
September 16, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28915836/changes-in-dna-methylation-in-na%C3%A3-ve-t-helper-cells-regulate-the-pathophysiological-state-in-minimal-change-nephrotic-syndrome
#8
Yasuko Kobayashi, Akira Aizawa, Takumi Takizawa, Katsuhide Igarashi, Izuho Hatada, Hirokazu Arakawa
BACKGROUND: DNA methylation plays a crucial role in regulating transcription, and changes in DNA methylation affect gene expression and disease development. Minimal change nephrotic syndrome (MCNS) has been reported to involve immunological disturbances. Since the characteristic features of the disease include recurrent relapse and sex and age preference, the disease pathogenesis may be partly related to epigenetic changes. However, little is known about these changes. METHODS: We analyzed genome-wide DNA methylation using the microarray-based integrated analysis of methylation by isoschizomers method...
September 15, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28914167/nephrotic-syndrome-in-infants-and-children-pathophysiology-and-management
#9
Mallory L Downie, Claire Gallibois, Rulan S Parekh, Damien G Noone
Nephrotic syndrome is defined by nephrotic-range proteinuria (≥40 mg/m(2)/hour or urine protein/creatinine ratio ≥200 mg/mL or 3+ protein on urine dipstick), hypoalbuminaemia (<25 g/L) and oedema. This review focuses on the classification, epidemiology, pathophysiology, management strategies and prognosis of idiopathic nephrotic syndrome of childhood, and includes a brief overview of the congenital forms.
September 15, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28906386/development-and-validation-of-a-prediction-score-system-in-lupus-nephritis
#10
Yi Tang, Wei Qin, Wei Peng, Ye Tao
The risk assessment for developing end-stage renal disease (ESRD) remains unclear in patients with lupus nephritis (LN). The purpose of this study was to develop and validate a prediction rule for estimating the individual risk of ESRD in patients with LN using clinical and pathological data.A total of 599 patients with LN diagnosed by renal biopsy between June 2009 and June 2014 in West China Hospital of Sichuan University were retrospectively followed. Patients were randomly divided into derivation cohort (n = 379) and validation cohort (n = 220)...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28904948/recent-progress-in-deciphering-the-etiopathogenesis-of-primary-membranous-nephropathy
#11
REVIEW
Andreas Kronbichler, Jun Oh, Björn Meijers, Gert Mayer, Jae Il Shin
Primary membranous nephropathy (MN) is the leading cause of nephrotic syndrome in adults. Discovery of several antibodies has contributed to an increased understanding of MN. Antibodies against the M-type phospholipase A2 receptor (PLA2R) are present in 50-100% with primary MN and are associated with a lower frequency of spontaneous remission. High levels are linked with a higher probability of treatment resistance, higher proteinuria, and impaired renal function, as well as a more rapid decline of kidney function during follow-up...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28904877/long-term-repeated-rituximab-treatment-for-childhood-steroid-dependent-nephrotic-syndrome
#12
Ji Hyun Kim, Eujin Park, Hye Sun Hyun, Myung Hyun Cho, Yo Han Ahn, Hyun Jin Choi, Hee Gyung Kang, Il-Soo Ha, Hae Il Cheong
BACKGROUND: Rituximab (RTX) can be used as a rescue therapy for steroid-dependent nephrotic syndrome (SDNS). However, the efficacy and safety of long-term, repeated use of RTX are not established. This study was conducted to assess the efficacy and safety of long-term, repeated RTX treatment in children. METHODS: Eighteen consecutive child patients with SDNS who were treated with three or more cycles of RTX for one year or longer were recruited, and their medical records were retrospectively reviewed...
September 2017: Kidney Research and Clinical Practice
https://www.readbyqxmd.com/read/28904681/clinicopathological-findings-and-outcome-of-lupus-nephritis-in-tunisian-children-a-review-of-43-patients
#13
Hela Jebali, Meriam Hajji, Lamia Rais, Fethi Ben Hamida, Soumaya Beji, Mohammed Karim Zouaghi
We report clinical and renal histological data, treatment modalities and outcome of 43 Tunisian children with biopsy-proven lupus nephritis seen over a 23-year period. There were 39 girls and 4 boys with a mean age of 12.5 years at diagnosis of lupus nephritis and followed for a mean period of 77 months. Renal symptoms included urinary abnormalities in all patients, hypertension in 40% of cases, nephrotic syndrome in 60% of cases and renal failure in 25% of cases. Class IV and class III nephritis were observed in 48...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28904430/immunohistochemical-analysis-of-anti-phospholipase-a2-receptor-antibody-on-renal-biopsies-a-single-tertiary-care-center-study
#14
A Gudipati, M S Uppin, R K Kalidindi, G Swarnalatha, U Das, G Taduri, S B Raju, L Rajasekhar, Aruna K Prayaga
Membranous nephropathy (MN) is one of the common cause of nephrotic syndrome. The discrimination between primary MN (iMN) and secondary MN is essential because of treatment implications. Immunohistochemical (IHC) evaluation with the help of anti-phospholipase A2 receptor (PLA2R) antibody helps in tissue evaluation of iMN, which is an easy, cost-effective, and pathologist-friendly technique. The study included 82 cases of MN over a period of 3 years. IHC using PLA2R antibody was performed on iMN and secondary cases with adequate tissue...
September 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28898339/-young-woman-daughter-of-a-father-with-alport%C3%A2-s-syndrome-debuts-with-a-impure-nephrotic-syndrome
#15
Fernando González, Gonzalo Méndez, Daniela Navarrete, Emilio Roessler B
No abstract text is available yet for this article.
May 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/28898290/comparative-effectiveness-and-tolerance-of-immunosuppressive-treatments-for-idiopathic-membranous-nephropathy-a-network-meta-analysis
#16
Song Ren, Ying Wang, Li Xian, Tadashi Toyama, Meg Jardine, Guisen Li, Vlado Perkovic, Daqing Hong
BACKGROUND: Immunosuppressive agents in general are shown to prevent renal progression and all-cause mortality in idiopathic membranous nephropathy (IMN) patients with nephrotic syndrome. However, the efficacy and safety of different immunosuppressive treatments have not been systematic assessed and compared. A network meta-analysis was performed to compare different immunosuppressive treatment in IMN. METHODS: Cochrane library, MEDLINE, EMBASE and trial register system were searched for randomized controlled trials reporting the treatments for IMN to May 3, 2016...
2017: PloS One
https://www.readbyqxmd.com/read/28891307/a-comprehensive-narrative-review-of-diagnostic-biomarkers-in-human-primary-membranous-nephropathy
#17
Shiva Kalantari, Mohsen Nafar
Membranous nephropathy (MN) is relatively major cause of nephrotic syndrome in adults which is recognized as an organ-specific autoimmune disease. The etiology of most cases is idiopathic, whereas the secondary MN is caused by systemic autoimmune diseases, infections, medications and malignancies. The idiopathic disease is developed by the formation of sub-epithelial immune complex deposits most likely due to binding the circulating auto-antibodies to intrinsic antigen on podocytes. The major auto antibody is the anti-phospholipase A2 receptor (anti-PLA2R), however, it is not enough sensitive...
September 11, 2017: Biomarkers in Medicine
https://www.readbyqxmd.com/read/28890943/trial-of-amiloride-in-type-2-diabetes-with-proteinuria
#18
Mark L Unruh, V Shane Pankratz, John E Demko, Evan C Ray, Rebecca P Hughey, Thomas R Kleyman
INTRODUCTION: Renal Na(+) retention and extracellular fluid volume expansion are hallmarks of nephrotic syndrome, which occurs even in the absence of activation of hormones that stimulate renal Na(+) transporters. Plasmin-dependent activation of the epithelial Na(+) channel (ENaC) has been proposed to have a role in renal Na(+) retention in the setting of nephrotic syndrome. We hypothesized that the ENaC inhibitor amiloride would be an effective therapeutic agent in inducing a natriuresis and lowering blood pressure in individuals with macroscopic proteinuria...
September 2017: KI Reports
https://www.readbyqxmd.com/read/28890537/nephrotic-syndrome-novel-monogenic-causes-of-galloway-mowat-syndrome
#19
Ellen F Carney
No abstract text is available yet for this article.
September 11, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28890365/detection-and-quantification-of-rituximab-in-the-human-urine
#20
Roland Jacobs, Thais Langer-Jacobus, Michelle Duong, Klaus Stahl, Hermann Haller, Reinhold E Schmidt, Mario Schiffer
B cell depletion by rituximab treatment might be inefficient in patients suffering from nephrotic syndrome. Due to the impaired glomerular filtration barrier a significant portion of the therapeutic antibody might be lost into the urinary space. In order to determine the amount of rituximab in the urine of such patients, CD20+ Daudi cells were stained with the patients' urine followed by a fluorochrome-labeled secondary antibody. Mean fluorescence intensity of that way labeled Daudi cells was determined by flow cytometry...
September 7, 2017: Journal of Immunological Methods
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