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Pediatric nephrology

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389 papers 100 to 500 followers
By P O Pediatrics, Nephrology
https://www.readbyqxmd.com/read/30453889/renal-outcomes-of-stop-igan-trial-patients-in-relation-to-baseline-histology-mest-c-scores
#1
Judith Isabel Schimpf, Till Klein, Christina Fitzner, Frank Eitner, Stefan Porubsky, Ralf-Dieter Hilgers, Jürgen Floege, Hermann-Josef Groene, Thomas Rauen
BACKGROUND: The Oxford classification of IgA nephropathy (IgAN) defines histologic criteria (MEST-C) that provide prognostic information based on the kidney biopsy. There are few data on the predictive impact of this classification in randomized clinical trial settings. METHODS: We performed an exploratory analysis of MEST-C scores in 70 available renal biopsies from 162 randomized STOP-IgAN trial participants and correlated the results with clinical outcomes. Analyses were performed by researchers blinded to the clinical outcome of the patients...
November 19, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/30249419/urine-sediment-examination-in-the-diagnosis-and-management-of-kidney-disease-core-curriculum-2019
#2
REVIEW
Corey Cavanaugh, Mark A Perazella
Automated urine technology and centralized laboratory testing are becoming the standard for providing urinalysis data to clinicians, including nephrologists. This trend has had the unintended consequence of making examination of urine sediment by nephrologists a relatively rare event. In addition, the nephrology community appears to have lost interest in and forgotten the utility of provider-performed urine microscopy. However, it is critical to remember that urine sediment examination remains a time-honored test that provides a wealth of information about the patient's underlying kidney disease...
September 21, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/30393837/physiological-regulation-of-phosphate-by-vitamin-d-parathyroid-hormone-pth-and-phosphate-pi
#3
REVIEW
Grégory Jacquillet, Robert J Unwin
Inorganic phosphate (Pi) is an abundant element in the body and is essential for a wide variety of key biological processes. It plays an essential role in cellular energy metabolism and cell signalling, e.g. adenosine and guanosine triphosphates (ATP, GTP), and in the composition of phospholipid membranes and bone, and is an integral part of DNA and RNA. It is an important buffer in blood and urine and contributes to normal acid-base balance. Given its widespread role in almost every molecular and cellular function, changes in serum Pi levels and balance can have important and untoward effects...
November 5, 2018: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/30309322/rituximab-plasma-exchange-and-immunoglobulins-an-ineffective-treatment-for-chronic-active-antibody-mediated-rejection
#4
Gastón J Piñeiro, Erika De Sousa-Amorim, Manel Solé, José Ríos, Miguel Lozano, Frederic Cofán, Pedro Ventura-Aguiar, David Cucchiari, Ignacio Revuelta, Joan Cid, Eduard Palou, Josep M Campistol, Federico Oppenheimer, Jordi Rovira, Fritz Diekmann
BACKGROUND: Chronic active antibody-mediated rejection (c-aABMR) is an important cause of allograft failure and graft loss in long-term kidney transplants. METHODS: To determine the efficacy and safety of combined therapy with rituximab, plasma exchange (PE) and intravenous immunoglobulins (IVIG), a cohort of patients with transplant glomerulopathy (TG) that met criteria of active cABMR, according to BANFF'17 classification, was identified. RESULTS: We identified 62 patients with active c-aABMR and TG (cg ≥ 1)...
October 11, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/30343732/immunosuppressive-treatment-for-proliferative-lupus-nephritis-summary-of-a-cochrane-review
#5
David J Tunnicliffe, Suetonia C Palmer
No abstract text is available yet for this article.
November 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/30139458/hypokalemic-distal-renal-tubular-acidosis
#6
REVIEW
Patricia G Vallés, Daniel Batlle
Distal renal tubular acidosis (DRTA) is defined as hyperchloremic, non-anion gap metabolic acidosis with impaired urinary acid excretion in the presence of a normal or moderately reduced glomerular filtration rate. Failure in urinary acid excretion results from reduced H+ secretion by intercalated cells in the distal nephron. This results in decreased excretion of NH4 + and other acids collectively referred as titratable acids while urine pH is typically above 5.5 in the face of systemic acidosis. The clinical phenotype in patients with DRTA is characterized by stunted growth with bone abnormalities in children as well as nephrocalcinosis and nephrolithiasis that develop as the consequence of hypercalciuria, hypocitraturia, and relatively alkaline urine...
July 2018: Advances in Chronic Kidney Disease
https://www.readbyqxmd.com/read/30139459/hyperkalemic-forms-of-renal-tubular-acidosis-clinical-and-pathophysiological-aspects
#7
REVIEW
Daniel Batlle, Jose Arruda
In contrast to distal type I or classic renal tubular acidosis (RTA) that is associated with hypokalemia, hyperkalemic forms of RTA also occur usually in the setting of mild-to-moderate CKD. Two pathogenic types of hyperkalemic metabolic acidosis are frequently encountered in adults with underlying CKD. One type, which corresponds to some extent to the animal model of selective aldosterone deficiency (SAD) created experimentally by adrenalectomy and glucocorticoid replacement, is manifested in humans by low plasma and urinary aldosterone levels, reduced ammonium excretion, and preserved ability to lower urine pH below 5...
July 2018: Advances in Chronic Kidney Disease
https://www.readbyqxmd.com/read/30139463/incomplete-distal-renal-tubular-acidosis-and-kidney-stones
#8
REVIEW
Daniel G Fuster, Orson W Moe
Renal tubular acidosis (RTA) is comprised of a diverse group of congenital or acquired diseases with the common denominator of defective renal acid excretion with protean manifestation, but in adults, recurrent kidney stones and nephrocalcinosis are mainly found in presentation. Calcium phosphate (CaP) stones and nephrocalcinosis are frequently encountered in distal hypokalemic RTA type I. Alkaline urinary pH, hypocitraturia, and, less frequently, hypercalciuria are the tripartite lithogenic factors in distal RTA (dRTA) predisposing to CaP stone formation; the latter 2 are also commonly encountered in other causes of urolithiasis...
July 2018: Advances in Chronic Kidney Disease
https://www.readbyqxmd.com/read/30343957/intravenous-sodium-bicarbonate-in-treating-patients-with-severe-metabolic-acidemia
#9
EDITORIAL
Jeffrey A Kraut, Nicolaos E Madias
No abstract text is available yet for this article.
October 18, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29975121/should-we-really-stop-treating-patients-with-iga-nephropathy-with-steroids
#10
REVIEW
F Locatelli, L Del Vecchio, C Ponticelli
IgA nephropathy (IgAN) is the most common primary glomerulonephritis all over the world. Once considered as a benign disease, today the scientific community is aware that a significant percentage of patients eventually progress to end-stage kidney disease (ESKD). The rate of progression is often very slow. Since 1980s, several therapeutic attempts have been made with steroids. Despite different molecules, doses, and lengths of treatment, the majority of uncontrolled and controlled studies found benefits in terms of proteinuria reduction and reduction of the risk of ESKD...
June 1, 2018: Physiology International
https://www.readbyqxmd.com/read/29998120/iga-nephropathy-a-european-perspective-in-the-corticosteroid-treatment
#11
REVIEW
Rosanna Coppo
Background: IgA nephropathy (IgAN) is detected in Europe in 22% of glomerular diseases diagnosed by biopsy. The frequency of IgAN as cause of ESRD in Europe has increased in the last decades, accounting for 35% of young and adult transplanted patients. These data justify the interest for risk factors and a possible therapeutic approach. Summary: Insight into a European perspective of IgAN was allowed by the multicenter study VALIGA, on 1,147 patients, almost all Caucasians, with follow-up of 4...
June 2018: Kidney Diseases
https://www.readbyqxmd.com/read/29292861/antibody-mediated-rejection-new-approaches-in-prevention-and-management
#12
R A Montgomery, A Loupy, D L Segev
Despite the success of desensitization protocols, antibody-mediated rejection (AMR) remains a significant contributor to renal allograft failure in patients with donor-specific antibodies. Plasmapheresis and high-dose intravenous immunoglobulin have proved to be effective treatments to prevent and treat AMR, but irreversible injury in the form of transplant glomerulopathy can commonly manifest months to years later. There is an unmet need to improve the outcomes for patients at risk for AMR. Updated Banff criteria now take into account the increasing understanding of the complex and heterogeneous nature of AMR phenotypes, including the timing of rejection, subclinical and chronic AMR, C4d-negative AMR, and antibody-mediated vascular rejection...
January 2018: American Journal of Transplantation
https://www.readbyqxmd.com/read/29724182/favorable-effect-of-bortezomib-in-dense-deposit-disease-associated-with-monoclonal-gammopathy-a-case-report
#13
Shuma Hirashio, Ayaka Satoh, Takahiro Arima, Kouichi Mandai, Tadasuke Awaya, Kumi Oshima, Shigeo Hara, Takao Masaki
BACKGROUND: Complement component 3 (C3) glomerulopathy, which includes dense deposit disease (DDD) and C3 glomerulonephritis, is caused by dysregulation of the alternative complement pathway. In most cases, C3 glomerulopathy manifests pathologically with membranoproliferative glomerulonephritis-like features. An association between C3 glomerulopathy and monoclonal gammopathy was recently reported in several cases, raising the possibility that C3 glomerulopathy is the underlying pathological process in monoclonal gammopathy of renal significance...
May 3, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29321782/functional-characterization-of-the-disease-associated-n-terminal-complement-factor-h-mutation-w198r
#14
Marcell Cserhalmi, Barbara Uzonyi, Nicolas S Merle, Dorottya Csuka, Edgar Meusburger, Karl Lhotta, Zoltán Prohászka, Mihály Józsi
Dysregulation of the complement alternative pathway is involved in the pathogenesis of several diseases, including the kidney diseases atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G). In a patient, initially diagnosed with chronic glomerulonephritis, possibly C3G, and who 6 years later had an episode of aHUS, a heterozygous missense mutation leading to a tryptophan to arginine exchange (W198R) in the factor H (FH) complement control protein (CCP) 3 domain has previously been identified...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29497606/network-for-early-onset-cystic-kidney-diseases-a-comprehensive-multidisciplinary-approach-to-hereditary-cystic-kidney-diseases-in-childhood
#15
Jens Christian König, Andrea Titieni, Martin Konrad
Hereditary cystic kidney diseases comprise a complex group of genetic disorders representing one of the most common causes of end-stage renal failure in childhood. The main representatives are autosomal recessive polycystic kidney disease, nephronophthisis, Bardet-Biedl syndrome, and hepatocyte nuclear factor-1beta nephropathy. Within the last years, genetic efforts have brought tremendous progress for the molecular understanding of hereditary cystic kidney diseases identifying more than 70 genes. Yet, genetic heterogeneity, phenotypic variability, a lack of reliable genotype-phenotype correlations and the absence of disease-specific biomarkers remain major challenges for physicians treating children with cystic kidney diseases...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29340311/hyponatremia-and-the-brain
#16
REVIEW
Fabrice Gankam Kengne, Guy Decaux
Hyponatremia is defined by low serum sodium concentration and is the most common electrolyte disorder encountered in clinical practice. Serum sodium is the main determinant of plasma osmolality, which, in turn, affects cell volume. In the presence of low extracellular osmolality, cells will swell if the adaptation mechanisms involved in the cell volume maintenance are inadequate. The most dramatic effects of hyponatremia on the brain are seen when serum sodium concentration decreases in a short period, allowing little or no adaptation...
January 2018: KI Reports
https://www.readbyqxmd.com/read/29344504/new-findings-on-the-pathogenesis-of-distal-renal-tubular-acidosis
#17
REVIEW
Francesco Trepiccione, Federica Prosperi, Luigi Regenburgh de la Motte, Christian A Hübner, Regine Chambrey, Dominique Eladari, Giovambattista Capasso
Background: Distal renal tubular acidosis (dRTA) is characterized by an impairment of the urinary acidification process in the distal nephron. Complete or incomplete metabolic acidosis coupled with inappropriately alkaline urine are the hallmarks of this condition. Genetic forms of dRTA are caused by loss of function mutations of either SLC4A1 , encoding the AE1 anion exchanger, or ATP6V1B1 and ATP6V0A4 , encoding for the B1 and a4 subunits of the vH+ ATPase, respectively. These genes are crucial for the function of A-type intercalated cells (A-IC) of the distal nephron...
December 2017: Kidney Diseases
https://www.readbyqxmd.com/read/28701203/discovery-of-a-novel-dominant-mutation-in-the-ren-gene-after-forty-years-of-renal-disease-a-case-report
#18
Rhian L Clissold, Helen C Clarke, Olivera Spasic-Boskovic, Kim Brugger, Stephen Abbs, Coralie Bingham, Charles Shaw-Smith
BACKGROUND: Heterozygous mutations in the gene encoding renin (REN) cause autosomal dominant tubulointerstitial kidney disease (ADTKD), early-onset anaemia and hyperuricaemia; only four different mutations have been described in the published literature to date. We report a novel dominant REN mutation discovered in an individual after forty years of renal disease. CASE PRESENTATION: A 57 year old Caucasian woman with chronic kidney disease stage five was reviewed in a regional joint renal genetics clinic...
July 12, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28781372/uromodulin-from-physiology-to-rare-and-complex-kidney-disorders
#19
REVIEW
Olivier Devuyst, Eric Olinger, Luca Rampoldi
Uromodulin (also known as Tamm-Horsfall protein) is exclusively produced in the kidney and is the most abundant protein in normal urine. The function of uromodulin remains elusive, but the available data suggest that this protein might regulate salt transport, protect against urinary tract infection and kidney stones, and have roles in kidney injury and innate immunity. Interest in uromodulin was boosted by genetic studies that reported involvement of the UMOD gene, which encodes uromodulin, in a spectrum of rare and common kidney diseases...
September 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/29217307/autosomal-dominant-tubulointerstitial-kidney-disease-due-to-muc1-mutation
#20
Samuel Mon-Wei Yu, Anthony J Bleyer, Kisra Anis, Leal Herlitz, Martina Živná, Helena Hůlková, Glen S Markowitz, Belinda Jim
Mucin 1 kidney disease, previously referred to as medullary cystic kidney disease type 1, is a rare hereditary kidney disease. It is one of several diseases now termed autosomal dominant tubulointerstitial kidney disease, as proposed by a KDIGO (Kidney Disease: Improving Global Outcomes) consensus report in 2014. Autosomal dominant tubulointerstitial kidney diseases share common clinical findings, such as autosomal dominant inheritance, bland urinary sediment, absent to mild proteinuria, and progressive loss of kidney function...
April 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
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