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https://www.readbyqxmd.com/read/29146214/-treatments-of-steroid-dependent-nephrotic-syndrome-in-children
#1
A Couderc, E Bérard, V Guigonis, I Vrillon, J Hogan, V Audard, V Baudouin, C Dossier, O Boyer
Primary nephrotic syndrome (NS) is the most common glomerular disease in children. It is characterized by massive proteinuria and hypoalbuminemia. It typically has a sudden onset and more than 70% of patients will experience at least one relapse. An immunological origin has long been postulated, although the precise molecular mechanisms underlying the disease remain debated. Steroids are the first-line therapy with cumulative dose and duration of initial treatment varying among countries. Steroid-sparing agents may be indicated in case of steroid-dependency or frequent relapses...
November 13, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29142939/negative-staining-for-col4a5-correlates-with-worse-prognosis-and-more-severe-ultrastructural-alterations-in-males-with-alport-syndrome
#2
Samar M Said, Mary E Fidler, Anthony M Valeri, Brooke McCann, Wade Fiedler, Lynn D Cornell, Mariam Priya Alexander, Ahmed M Alkhunaizi, Anne Sullivan, Carl H Cramer, Marie C Hogan, Samih H Nasr
Introduction: Alport syndrome (AS) is a genetic disorder characterized by progressive hematuric nephropathy with or without sensorineural hearing loss and ocular lesions. Previous studies on AS included mostly children. Methods: To determine the prognostic value of loss of staining for collagen type IV alpha 5 (COL4A5) and its relationship with the ultrastructural glomerular basement membrane alterations, we performed direct immunofluorescence using a mixture of fluorescein isothiocyanate-conjugated and Texas-red conjugated antibodies against COL4A5 and COL4A2, respectively, on renal biopsies of 25 males with AS (including 16 who were diagnosed in adulthood)...
January 2017: KI Reports
https://www.readbyqxmd.com/read/29130775/results-of-native-and-transplant-kidney-biopsies-of-children-in-a-single-center-over-a-15-years-period
#3
Emel Isiyel, Kibriya Fidan, Bahar Buyukkaragoz, Meltem Akcaboy, Yasar Kandur, Ipek Isik Gonul, Necla Buyan, Sevcan Bakkaloglu, Oguz Soylemezoglu
Renal biopsy is an important diagnostic procedure in pediatric nephrology. This study retrospectively investigates the indications, results and complications in a single tertiary children's hospital in Turkey. We evaluated the native and transplant kidney biopsies in Gazi University Pediatric Nephrology Department between 2001 and 2015. A total of 196 biopsies (144 natives and 52 transplants) were included into the study. The mean age of the patients was respectively 10.8 ± 3.5, 13.9 ± 1.5 years...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/29106598/safety-and-effectiveness-of-restrictive-eculizumab-treatment-in-atypical-haemolytic-uremic-syndrome
#4
Kioa L Wijnsma, Caroline Duineveld, Elena B Volokhina, Lambertus P van den Heuvel, Nicole C A J van de Kar, Jack F M Wetzels
Background: Atypical haemolytic uremic syndrome (aHUS) is a rare but severe form of thrombotic microangiopathy as a consequence of complement dysregulation. aHUS has a poor outcome with high mortality and >50% of patients developing end-stage renal disease. Since the end of 2012, these outcomes have greatly improved with the introduction of eculizumab. Currently the duration of treatment is debated. Most guidelines advise lifelong treatment. However, there is no hard evidence to support this advice...
July 4, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29101676/long-term-follow-up-of-paediatric-mefv-carriers
#5
Balahan Makay, Nesrin Gülez
Although familial Mediterranean fever (FMF) is inherited autosomal recessively, some heterozygotes may express disease phenotype and require therapy. To date, there is no study in the literature about how to follow-up Mediterranean fever (MEFV) heterozygotes who do not fulfil FMF criteria in the paediatric age group. This study aims to share a single-centre experience of the long-term clinical and laboratory follow-up of paediatric MEFV carriers. We reviewed the charts of 69 children who were heterozygous for MEFV variants...
November 3, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29090653/tuberculous-pyelonephritis-in-children-three-case-reports
#6
Nimisha Arora, Abhijeet Saha, Manpreet Kaur
Tuberculous pyelonephritis is rare in children; three case reports are presented. Case 1 was an 11-year-old girl with a previous history of pulmonary tuberculosis who presented with flank pain and fever for 10 days. An ultrasound suggested focal pyelonephritis, and a contrast-enhanced computed tomography (CECT) scan demonstrated acute focal pyelonephritis and a perinephric collection. Mycobacterium tuberculosis was cultured in the urine. She responded well to anti-tuberculous treatment (ATT). Case 2 was a 13-year-old boy who presented with fever, haematuria, burning micturition, proteinuria (3+) and cervical lymphadenopathy...
November 1, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/29077646/high-incidence-of-proteinuria-in-children-with-chronic-intestinal-failure-under-long-term-parenteral-nutrition
#7
Heiko Billing, Anna Traunspurger, Ekkehard Sturm, Andreas Busch
Long-term home parenteral nutrition (LTPN) in children with chronic intestinal failure (CIF) is associated with renal complications such as fluid and electrolyte imbalances, nephrocalcinosis, nephrolithiasis and chronic kidney disease (CKD). The etiology of CIF-associated nephropathy is multifactorial. The aim of this study was to evaluate renal involvement under LTPN. In this study 50 patients with CIF, median age 4.2 years (1.4 - 9.3, 23 female) were included. Kidney involvement was a frequent finding in this study...
October 27, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29072675/outcomes-of-prenatally-diagnosed-solitary-functioning-kidney-during-early-life
#8
M Davidovits, R Cleper, N Eizenberg, O Hocherman, R Mashiach
OBJECTIVE: To evaluate outcomes of congenital solitary functioning kidney (SFK) in early childhood. STUDY DESIGN: A retrospective study of 32 children diagnosed in utero with SFK owing to unilateral renal agenesis or multicystic dysplastic kidney and followed for 1 to 11.5 years. RESULTS: SFK length was in the compensatory hypertrophy range in 45% of fetal sonographic evaluations from mid-pregnancy, and in 85% on postnatal follow-up. Glomerular filtration rate was below normal range in 44...
October 26, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/29071540/current-and-emerging-treatment-strategies-for-children-with-progressive-chiasmatic-hypothalamic-glioma-diagnosed-as-infants-a-web-based-survey
#9
Amedeo A Azizi, Antoinette Y N Schouten-van Meeteren
Treatment of infant hypothalamic chiasmatic glioma (iCHG) is challenging, about 30% of the children progress during chemotherapy. Despite subsequent treatments the 5 year overall-survival rate is only 70%. This study investigates treatment strategies currently applied for progressive iCHG. A web-based questionnaire was sent out to the members of the SIOPE Brain Tumour Group asking for current second and third line strategies at progression during and after the end of first line therapy. The questionnaire was answered by 47 paediatric oncologists from 15 countries...
October 25, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29063524/on-the-relationship-between-proteinuria-and-plasma-phosphate
#10
Sophie de Seigneux, Alexandra Wilhelm-Bals, Marie Courbebaisse
Albuminuria is strongly associated with renal and cardiovascular outcomes independently of renal function level. However, the pathophysiology of these associations is debated. In chronic kidney disease (CKD), phosphate retention participates in cardiovascular events and increased cardiovascular mortality. We hypothesised that albuminuria may modulate tubular phosphate handling by the kidney. To verify this hypothesis, we first studied the association between phosphataemia and albuminuria in children with nephrotic syndrome and in adults with CKD...
October 24, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/29055396/role-of-gut-microbiota-in-idiopathic-nephrotic-syndrome-in-children
#11
Kazunari Kaneko, Shoji Tsuji, Takahisa Kimata
Nephrotic syndrome characterized by heavy proteinuria and edema is the most common chronic kidney disease in children. It is classified into three categories, of which the idiopathic type accounts for the vast majority of cases. As indicated by the name, the etiology of idiopathic nephrotic syndrome remains unknown though it has been suggested that impaired T cell function is involved. Recently, evidence has mounted to suggest that dysfunction in regulatory T cells plays an important role in the development of allergic disease, a recognized comorbid condition for children with idiopathic nephrotic syndrome...
October 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/29022223/acute-kidney-injury-in-pregnancy-and-the-role-of-underlying-ckd-a-point-of-view-from-m%C3%A3-xico
#12
REVIEW
Margarita Ibarra-Hernández, Oralia Alejandra Orozco-Guillén, María Luz de la Alcantar-Vallín, Ruben Garrido-Roldan, María Patricia Jiménez-Alvarado, Kenia Benitez Castro, Francisco Villa-Villagrana, Mario Borbolla, Juan Manuel Gallardo-Gaona, Guillermo García-García, Norberto Reyes-Paredes, Giorgina Barbara Piccoli
The incidence of acute kidney injury in pregnancy (P-AKI) in developed countries is significantly lower than in developing ones, where it is estimated to range between 4 and 26%. Mortality in cases of P-AKI requiring dialysis is high, varying from 20 to 80%. In developing countries, clinical decisions are often based on the availability of services and not on needs. Prenatal surveillance in Mexico does not include serum creatinine, limiting the potential for early diagnosis of AKI and CKD and their differential diagnosis...
October 11, 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/29020719/diagnosis-and-management-of-nephrotic-syndrome
#13
Oonagh McCloskey, Alexander P Maxwell
Nephrotic syndrome is defined by a triad of clinical features: oedema, substantial proteinuria (> 3.5 g/24 hours) and hypoalbuminaemia (< 30 g/L). It is often associated with hyperlipidaemia, thromboembolism and an increased risk of infection. Nephrotic syndrome develops following pathological injury to renal glomeruli. This may be a primary problem, with a disease specific to the kidneys, or secondary to a systemic disorder such as diabetes mellitus. The most common cause in children is minimal change glomerulonephritis...
February 2017: Practitioner
https://www.readbyqxmd.com/read/29019060/reports-of-three-cases-with-the-initial-presentation-of-mesenteric-vasculitis-in-children-with-system-lupus-erythematous
#14
REVIEW
Yuan Liu, Jia Zhu, Jian Ming Lai, Xue Feng Sun, Jun Hou, Zhi Xuan Zhou, Xin Yu Yuan
We reviewed three cases of systemic lupus erythematosus (SLE) in children with mesenteric vasculitis (LMV) as initial presentation and analysed their clinical characteristics to improve the understanding of this disease. Three patients with SLE were admitted to our hospital and initially presented with gastrointestinal symptoms. We retrospectively analysed their clinical data, including clinical presentations, laboratory results, images and short- and long-term treatment outcomes. (1) All three children were school-age girls...
October 10, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28994226/proteinuria-in-pediatric-renal-transplant-recipients
#15
Songül Yılmaz, Z Birsin Özçakar, Aysel Taktak, Eda Didem Kurt-Şükür, Nilgün Çakar, Fatoş Yalçınkaya
Proteinuria has been shown to be an important and potentially treatable risk factor for graft loss. The aim of this study was to evaluate prevalence, etiology, and outcome of proteinuria during the follow-up of children with renal transplantation. We retrospectively reviewed the files of renal transplanted children between 2006 and 2016 in our center. All patients were interpreted with respect to the demographic data and clinical and laboratory features including information about proteinuria. Chi-square test and Mann-Whitney U test were used for analysis...
October 9, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28992235/treatment-by-immunoadsorption-for-recurrent-focal-segmental-glomerulosclerosis-after-paediatric-kidney-transplantation-a-multicentre-french-cohort
#16
Lise Allard, Theresa Kwon, Saoussen Krid, Justine Bacchetta, Arnaud Garnier, Robert Novo, Georges Deschenes, Rémi Salomon, Gwenaëlle Roussey, Emma Allain-Launay
Background: Primary focal segmental glomerulosclerosis (FSGS) frequently recurs after kidney transplantation (KTx) in children. This can lead to delayed graft loss. As the management of children with recurrent FSGS is not well established, apheresis strategies could be a cornerstone to control the disease. Immunoadsorption (IA) is a recent apheresis therapy. There have been few studies examining IA in this setting. We report the results of IA for management of recurrent FSGS after KTx in children in France...
July 28, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28990100/low-molecular-weight-heparin-may-benefit-nephrotic-remission-in-steroid%C3%A2-sensitive-nephrotic-syndrome-via-inhibiting-elastase
#17
Songhui Zhai, Lijuan Hu, Lin Zhong, Yuhong Tao, Zheng Wang
Low molecular weight heparin (LMWH) has a structure similar to heparan sulfate, which exerts anti‑inflammatory effects via inhibiting elastase (Ela) activity. Release of Ela along the glomerular capillary wall may induce glomerular injury and proteinuria. The present study aimed to investigate the influence of LMWH on steroid‑sensitive nephrotic syndrome (SSNS) and the potential underlying mechanism. A total of 40 SSNS patients and 20 healthy controls were recruited. SSNS patients were treated with LMWH and prednisone simultaneously (LMWH+pred group) or with prednisone alone (pred group)...
December 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28983704/presentation-of-pediatric-henoch-sch%C3%A3-nlein-purpura-nephritis-changes-with-age-and-renal-histology-depends-on-biopsy-timing
#18
Imke Hennies, Charlotte Gimpel, Jutta Gellermann, Kristina Möller, Brigitte Mayer, Katalin Dittrich, Anja K Büscher, Matthias Hansen, Wiebke Aulbert, Elke Wühl, Richard Nissel, Gessa Schalk, Lutz T Weber, Michael Pohl, Simone Wygoda, Rolf Beetz, Günter Klaus, Henry Fehrenbach, Sabine König, Hagen Staude, Ortraud Beringer, Martin Bald, Ulrike Walden, Christian von Schnakenburg, Gunhard Bertram, Michael Wallot, Karsten Häffner, Thorsten Wiech, Peter F Hoyer, Martin Pohl
BACKGROUND: This study correlates the clinical presentation of Henoch-Schönlein purpura nephritis (HSPN) with findings on initial renal biopsy. METHODS: Data from 202 pediatric patients enrolled in the HSPN registry of the German Society of Pediatric Nephrology reported by 26 centers between 2008 and 2014 were analyzed. All biopsy reports were re-evaluated for the presence of cellular crescents or chronic pathological lesions (fibrous crescents, glomerular sclerosis, tubular atrophy >5%, and interstitial fibrosis >5%)...
October 5, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28982638/prospective-cohort-analyzing-risk-factors-for-chronic-kidney-disease-progression-in-children
#19
Vera M S Belangero, Liliane C Prates, Andreia Watanabe, Benita S G Schvartsman, Paula Nussenzveig, Natalia A Cruz, Ana L S Abreu, Isabel P Paz, Inalda Facincani, Fernanda E C Morgantetti, Andreia O Silva, Olberes V B Andrade, Maria F C Camargo, Paulo C Koch Nogueira
OBJECTIVE: To identify risk factors for chronic kidney disease progression in Brazilian children and to evaluate the interactions between factors. METHODS: This was a multicenter prospective cohort in São Paulo, involving 209 children with CKD stages 3-4. The study outcome included: (a) death, (b) start of kidney replacement therapy, (c) eGFR decrease >50% during the followup. Thirteen risk factors were tested using univariate regression models, followed by multivariable Cox regression models...
October 2, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/28971258/prevalence-and-associated-factors-of-renal-dysfunction-and-proteinuria-in-cyanotic-congenital-heart-disease
#20
Nattaphorn Hongsawong, Prapimdaw Khamdee, Suchaya Silvilairat, Wattana Chartapisak
BACKGROUND: Cyanotic nephropathy (CN), seen in 30-50% of patients with congenital cyanotic heart disease (CCHD), affects both tubular and glomerular function, resulting in proteinuria and azotemia. Microalbuminuria is an early marker for glomerular damage and an independent predictor of progressive renal disease. METHODS: A cross-sectional study was conducted. A total of 116 patients aged 1 month to 15 years with CCHD at Chiang Mai University Hospital between 2015 and 2016 were assessed and 94 patients were enrolled...
October 2, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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