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Pediatric nephrology

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359 papers 100 to 500 followers
By P O Pediatrics, Nephrology
https://www.readbyqxmd.com/read/27757584/proteinuria-in-dent-disease-a-review-of-the-literature
#1
Youri van Berkel, Michael Ludwig, Joanna A E van Wijk, Arend Bökenkamp
BACKGROUND: Dent disease is a rare X-linked recessive proximal tubulopathy caused by mutations in CLCN5 (Dent-1) or OCRL (Dent-2). As a rule, total protein excretion (TPE) is low in tubular proteinuria compared with glomerular disease. Several authors have reported nephrotic-range proteinuria (NP) and glomerulosclerosis in Dent disease. Therefore, we aimed to analyze protein excretion in patients with documented CLCN5 or OCRL mutations in a systematic literature review. DESIGN: PubMed and Embase were searched for cases with documented CLCN5 or OCRL mutations and (semi-)quantitative data on protein excretion...
October 18, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27367750/failure-of-calcineurin-inhibitor-tacrolimus-weaning-randomized-trial-in-long-term-stable-kidney-transplant-recipients
#2
Emilie Dugast, Jean-Paul Soulillou, Yohann Foucher, Emmanuelle Papuchon, Pierrick Guerif, Chloé Paul, David Riochet, Mélanie Chesneau, Anne Cesbron, Karine Renaudin, Magali Giral, Sophie Brouard
Long-term renal transplant outcome is limited by side effects of immunosuppressive drugs, particularly CNI. We assumed that some patients selected for a "low immunological risk of rejection" could be eligible and benefit from a CNI weaning strategy. We designed a prospective, randomized, multicenter, double-blind placebo-controlled clinical study (Eudract: 2010-019574-33) to analyse the benefit-risk ratio of tacrolimus weaning on highly selected patients (>= 4 years of transplantation, normal histology, stable graft function, no anti-HLA immunization)...
July 1, 2016: American Journal of Transplantation
https://www.readbyqxmd.com/read/27652838/clinical-features-and-outcomes-of-diffuse-endocapillary-proliferation-henoch-sch%C3%A3-nlein-purpura-nephritis-in-children
#3
Haidong Fu, Jianhua Mao, Yanping Xu, Weizhong Gu, Xiujuan Zhu, Aimin Liu
OBJECTIVE: To investigate the outcomes of childhood diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis (DEP-HSPN) in response to early diagnosis and prompt treatment. METHODS: Eleven cases of DEP-HSPN in children were investigated in comparison to HSPN without diffuse endocapillary proliferation (non-DEP-HSPN). RESULTS: DEP-HSPN had a higher prevalence of nephrotic syndrome but a lower prevalence of hematuria compared to non-DEP-HSPN...
September 2016: Clinics
https://www.readbyqxmd.com/read/27468975/acidosis-and-urinary-calcium-excretion-insights-from-genetic-disorders
#4
R Todd Alexander, Emmanuelle Cordat, Régine Chambrey, Henrik Dimke, Dominique Eladari
Metabolic acidosis is associated with increased urinary calcium excretion and related sequelae, including nephrocalcinosis and nephrolithiasis. The increased urinary calcium excretion induced by metabolic acidosis predominantly results from increased mobilization of calcium out of bone and inhibition of calcium transport processes within the renal tubule. The mechanisms whereby acid alters the integrity and stability of bone have been examined extensively in the published literature. Here, after briefly reviewing this literature, we consider the effects of acid on calcium transport in the renal tubule and then discuss why not all gene defects that cause renal tubular acidosis are associated with hypercalciuria and nephrocalcinosis...
December 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/27536669/complement-in-lupus-nephritis-new-perspectives
#5
REVIEW
Lihua Bao, Patrick N Cunningham, Richard J Quigg
BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disorder caused by loss of tolerance to self-antigens, the production of autoantibodies and deposition of complement-fixing immune complexes (ICs) in injured tissues. SLE is characterized by a wide range of clinical manifestations and targeted organs, with lupus nephritis being one of the most serious complications. The complement system consists of three pathways and is tightly controlled by a set of regulatory proteins to prevent injudicious complement activation on host tissue...
September 2015: Kidney Diseases
https://www.readbyqxmd.com/read/27493007/the-need-for-genetic-study-to-diagnose-some-cases-of-distal-renal-tubular-acidosis
#6
Manuel Heras Benito, Miguel A Garcia-Gonzalez, María Valdenebro Recio, Álvaro Molina Ordás, Ramiro Callejas Martínez, María Astrid Rodríguez Gómez, Leonardo Calle García, Lisbeth Sousa Silva, María José Fernández-Reyes Luis
We describe the case of a young woman who was diagnosed with advanced kidney disease, with an incidental finding of nephrocalcinosis of unknown aetiology, having been found asymptomatic throughout her life. The genetic study by panels of known genes associated with tubulointerstitial disease allowed us to discover autosomal dominant distal renal tubular acidosis associated with a de novo mutation in exon 14 of the SLC4A1 gene, which would have been impossible to diagnose clinically due to the advanced nature of the kidney disease when it was discovered...
September 2016: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/27583915/the-effect-of-calcineurin-inhibitors-in-the-treatment-of-iga-nephropathy-a-systematic-review-and-meta-analysis-prisma
#7
Wei Peng, Yi Tang, Zheng Jiang, Zi Li, Xuhua Mi, Wei Qin
BACKGROUND: Immunoglobin A nephropathy (IgAN), the most prevalent form of primary glomerulonephritis, represents the leading cause of kidney failure among East Asian populations. Immunosuppressive treatment regimen, except for a 6-month trial of corticosteroids, has not been approved by the KDIGO guideline yet. Specific and effective treatment is still lacking. We decided to evaluate the efficacy and safety of the calcineurin inhibitors (CNIs) in the treatment of IgAN. METHODS: Database from the Cochrane library, PubMed, Embase, CBM, CNKI, and CENTRAL databases were searched and reviewed up to March 2016...
August 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27338776/eular-era-edta-recommendations-for-the-management-of-anca-associated-vasculitis
#8
M Yates, R A Watts, I M Bajema, M C Cid, B Crestani, T Hauser, B Hellmich, J U Holle, M Laudien, M A Little, R A Luqmani, A Mahr, P A Merkel, J Mills, J Mooney, M Segelmark, V Tesar, K Westman, A Vaglio, N Yalçındağ, D R Jayne, C Mukhtyar
In this article, the 2009 European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated. The 2009 recommendations were on the management of primary small and medium vessel vasculitis. The 2015 update has been developed by an international task force representing EULAR, the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based upon evidence from systematic literature reviews, as well as expert opinion where appropriate...
September 2016: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27461851/phosphate-binding-agents-in-adults-with-ckd-a-network-meta-analysis-of-randomized-trials
#9
Suetonia C Palmer, Sharon Gardner, Marcello Tonelli, Dimitris Mavridis, David W Johnson, Jonathan C Craig, Richard French, Marinella Ruospo, Giovanni F M Strippoli
BACKGROUND: Guidelines preferentially recommend noncalcium phosphate binders in adults with chronic kidney disease (CKD). We compare and rank phosphate-binder strategies for CKD. STUDY DESIGN: Network meta-analysis. SETTING & POPULATION: Adults with CKD. SELECTION CRITERIA FOR STUDIES: Randomized trials with allocation to phosphate binders. INTERVENTIONS: Sevelamer, lanthanum, iron, calcium, colestilan, bixalomer, nicotinic acid, and magnesium...
July 22, 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/27384690/rare-case-of-nephrocalcinosis-in-a-14-year-old-girl-questions
#10
Omar Bjanid, Piotr Adamczyk, Małgorzata Stojewska, Dagmara Roszkowska-Bjanid, Magdalena Paszyna-Grześkowiak, Agnieszka Jędzura, Joanna Oświęcimska, Katarzyna Ziora, Aurelia Morawiec-Knysak, Maria Szczepańska
A 14-year-old Caucasian girl with a history of primary hypoparathyroidism and unstable calcium and phosphorus levels and on ongoing treatment was admitted to the Department of Pediatric Nephrology because of the onset of nephrocalcinosis and difficulties achieving normocalcemia. Coexistence of hypoparathyroidism, oral candidiasis, dental enamel hypoplasia, and subclinical Hashimoto's disease was strongly suggestive for autoimmune polyglandular syndrome (APS) type I. One of the clinical implications of this diagnosis is the high probability of future occurrence of adrenal insufficiency and hence the importance of maintaining a high level of suspicion in case of the onset of symptoms like weakness, fainting, hypotonia, or hyperkaliemia...
July 6, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27384692/rare-case-of-nephrocalcinosis-in-a-14-year-old-girl-answers
#11
Omar Bjanid, Piotr Adamczyk, Małgorzata Stojewska, Dagmara Roszkowska-Bjanid, Magdalena Paszyna-Grześkowiak, Agnieszka Jędzura, Joanna Oświęcimska, Katarzyna Ziora, Aurelia Morawiec-Knysak, Maria Szczepańska
No abstract text is available yet for this article.
July 6, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27416024/corticosteroid-treatment-influences-ta-proteinuria-and-renal-survival-in-iga-nephropathy
#12
Cristina Sarcina, Carmine Tinelli, Francesca Ferrario, Bianca Visciano, Antonello Pani, Annalisa De Silvestri, Ilaria De Simone, Lucia Del Vecchio, Veronica Terraneo, Silvia Furiani, Gaia Santagostino, Enzo Corghi, Claudio Pozzi
The clinical course of IgA nephropathy (IgAN) and its outcome are extremely variable. Proteinuria at baseline has been considered one of the most important risk factors. More recently, mean proteinuria of follow-up (time-average proteinuria: TAp) was described as a stronger marker of renal survival, suggesting to consider it as a marker of disease activity and response to treatment. We evaluated predictors of renal survival in IgAN patients with different degrees of renal dysfunction and histological lesions, focusing on the role of the therapy in influencing TAp...
2016: PloS One
https://www.readbyqxmd.com/read/27368023/devastating-renal-outcome-caused-by-skin-infection-with-methicillin-resistant-staphylococcus-aureus-a-case-report
#13
Jun-Hua Liang, Yu-Wei Fang, An-Hung Yang, Ming Hsien Tsai
Methicillin-resistant Staphylococcus aureus (MRSA) is an emerging pathogen that infects the skin and soft tissue. However, there are few reports of renal complications from MRSA involving immunoglobulin (Ig)A-dominated rapidly progressive glomerulonephritis (GN). Favorable renal outcomes from IgA GN are achieved by administering timely therapy. In the present study, we describe the case of a healthy young woman suffering from a cutaneous MRSA infection that initially presented with gross hematuria. Six months after eradicating the infection, severe impairment of renal function was noted because of intractable nausea and vomiting...
June 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27274828/a-novel-heterozygous-mutation-in-the-atp6v0a4-gene-encoding-the-v-atpase-a4-subunit-in-an-adult-patient-with-incomplete-distal-renal-tubular-acidosis
#14
Eri Imai, Shuzo Kaneko, Takayasu Mori, Tomokazu Okado, Shinichi Uchida, Yusuke Tsukamoto
A 40-year-old Japanese man who had a medical history of hypokalemic periodic paralysis 4 months prior was hospitalized to undergo a cholecystectomy. Hypokalemia, nephrocalcinosis and alkaluria suggesting distal renal tubular acidosis (dRTA) were detected, but metabolic acidosis was not evident. An ammonium chloride/furosemide-fludrocortisone/bicarbonate loading test demonstrated a remarkable disability in urinary H(+) excretion. A novel heterozygous mutation in the ATP6V0A4 gene encoding the vacuolar H(+)-ATPase (V-ATPase) a4 subunit p...
June 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27274823/revisiting-post-infectious-glomerulonephritis-in-the-emerging-era-of-c3-glomerulopathy
#15
Mazdak A Khalighi, Shihtien Wang, Kammi J Henriksen, Margret Bock, Mahima Keswani, Shane M Meehan, Anthony Chang
BACKGROUND: Post-infectious glomerulonephritis (PIGN) is an immune complex-mediated glomerular injury that typically resolves. Dominant C3 deposition is characteristic of PIGN, but with the emergence of C3 glomerulonephritis (C3GN) as a distinct entity, it is unclear how the pathologic similarities between PIGN and C3GN should be reconciled. Therefore, nephrologists and nephropathologists need additional guidance at the time of biopsy. METHODS: We studied 23 pediatric and young adult patients diagnosed with PIGN...
June 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27188503/elevated-blood-pressure-is-not-associated-with-accelerated-glomerular-filtration-rate-decline-in-the-general-non-diabetic-middle-aged-population
#16
Bjørn O Eriksen, Vidar T N Stefansson, Trond G Jenssen, Ulla D Mathisen, Jørgen Schei, Marit D Solbu, Tom Wilsgaard, Toralf Melsom
Although hypertension is a risk factor for end-stage renal disease, this complication develops in only a minority of hypertensive patients. Whether non-malignant hypertension itself is sufficient to cause reduced glomerular filtration rate (GFR) is unclear. Therefore, we investigated whether elevated blood pressure (BP) was associated with accelerated GFR decline in the general population. The study was based on the Renal Iohexol Clearance Survey in Tromsø 6 (RENIS-T6), which included a representative sample of 1594 individuals aged 50 to 62 years from the general population without baseline diabetes or kidney or cardiovascular disease...
August 2016: Kidney International
https://www.readbyqxmd.com/read/27195820/short-and-long-term-effects-of-the-use-of-raas-blockers-immediately-after-renal-transplantation
#17
Christos Chatzikyrkou, Jenny Eichler, Annika Karch, Christian Clajus, Florian Gunnar Scurt, Wolf Ramackers, Frank Lehner, Jan Menne, Hermann Haller, Peter R Mertens, Mario Schiffer
BACKGROUND: The efficacy and safety of renin angiotensin aldosterone system blockers (RAASB's) if introduced immediately after renal transplantation have not been extensively investigated. METHODS: The medical charts of 142 kidney transplant recipients who received a RAASB in the early postoperative period and of 114 matched controls were analyzed. The RAASB was given primarily for blood pressure control. RESULTS: 117 patients continued to receive and 50 controls remained continuously free of the RAASB in the first year...
May 19, 2016: Blood Pressure
https://www.readbyqxmd.com/read/27226969/protracted-clinical-course-of-postinfectious-glomerulonephritis-in-a-previously-healthy-child
#18
Camilla Grøndahl, Søren Rittig, Johan Vestergaard Povlsen, Kostantinos Kamperis
Acute postinfectious glomerulonephritis (PIGN) affects children typically after upper respiratory tract or skin infections with streptococci but can complicate the course of other infections. In children, it is generally a self-limiting disease with excellent prognosis. This paper reports a previously healthy 4-year-old boy who experienced a protracted course of PIGN with persisting episodes of gross haematuria, proteinuria, decreased complement C3c levels but normal P-creatinine levels. Due to the protracted course and the nephrotic-range proteinuria, a renal biopsy was performed 6 months after the initial presentation and the overall pathology was consistent with acute endocapillary glomerulonephritis...
January 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27247958/mutations-in-atp6v1b1-and-atp6v0a4-genes-cause-recessive-distal-renal-tubular-acidosis-in-mexican-families
#19
Laura I Escobar, Christopher Simian, Cyrielle Treard, Donia Hayek, Carolina Salvador, Norma Guerra, Mario Matos, Mara Medeiros, Sandra Enciso, María Dolores Camargo, Rosa Vargas-Poussou
BACKGROUND: Autosomal recessive distal renal tubular acidosis (dRTA) is a rare disease characterized by a hyperchloremic metabolic acidosis with normal anion gap, hypokalemia, hypercalciuria, hypocitraturia, nephrocalcinosis, and conserved glomerular filtration rate. In some cases, neurosensorial deafness is associated. dRTA is developed during the first months of life and the main manifestations are failure to thrive, vomiting, dehydration, and anorexia. METHODS: Nine unrelated families were studied: seven children, a teenager, and an adult with dRTA...
May 2016: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/27178679/multifocal-epstein-barr-virus-negative-posttransplantation-lymphoproliferative-disorder-treated-with-reduction-of%C3%A2-immunosuppression
#20
Akinori Miyazono, Yasuhiro Okamoto, Hironobu Nagasako, Yuko Hamasaki, Seiichiro Shishido, Takako Yoshioka, Yoshifumi Kawano
Posttransplantation lymphoproliferative disorder (PTLD) is associated with significant mortality in kidney transplant recipients. PTLD cases associated with poor prognostic factors that are refractory to reduction of immunosuppression generally require chemotherapy and immunotherapy. We present a patient with PTLD who achieved complete remission after reduction of immunosuppression alone despite having a poor prognosis. A boy with a mutation in the WT1 gene developed bilateral Wilms tumor at 15 months and received a kidney transplant at the age of 4 years...
September 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
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