keyword
https://read.qxmd.com/read/38529548/exploring-multiple-endocrinological-issues-and-dysautonomia-in-a-rare-case-hypoparathyroidism-in-mirage-syndrome
#1
JOURNAL ARTICLE
Sirmen Kızılcan Çetin, Elif Özsu, Zeynep Şıklar, Hasan Fatih Çakmaklı, Gizem Şenyazar, Zehra Aycan, Serdar Ceylaner, Merih Berberoğlu
MIRAGE syndrome is a rare multisystemic disorder characterized by various manifestations, such as myelodysplasia, susceptibility to infections, growth retardation, adrenal hypoplasia, genital anomalies, and enteropathy. In the literature, there have been rare cases of dysautonomia. We present a 6.5-year-old girl, who was first admitted to our department with short stature. On follow up, she exhibited multiple endocrinological issues, including transient hypothyroidism, primary hypoparathyroidism and dysautonomia, along with multisystem involvement...
March 26, 2024: Journal of Clinical Research in Pediatric Endocrinology
https://read.qxmd.com/read/38526665/lymphatic-vessels-in-patients-with%C3%A2-crescentic-glomerulonephritis-association-with-renal-pathology-and-prognosis
#2
JOURNAL ARTICLE
Danni Hu, Zheng Wang, Shujie Wang, Yueqiang Li, Guangchang Pei, Rui Zeng, Gang Xu
BACKGROUND: Various immune cells, including T cells, B cells, macrophages, and neutrophils contribute to the development of crescentic glomerulonephritis. Previous animal studies have suggested that lymphangiogenesis is involved in the migration of inflammatory cells and the activation of adaptive immunity. However, the extent of the association between lymphatic vessels and crescentic glomerulonephritis severity and prognosis remains unknown. METHODS AND RESULTS: In this study, we assessed lymphatic vessel density in 71 patients with crescentic glomerulonephritis who underwent renal biopsies between June 2017 and June 2022...
March 25, 2024: Journal of Nephrology
https://read.qxmd.com/read/38523389/a-case-of-crescentic-glomerulonephritis-with-exacerbation-of-pre-existing-iga-nephropathy-after-covid-19
#3
Esra Karabağ Yılmaz, Seha Saygılı, Gülüstan Musayeva, Rüveyda Gülmez, Ayşe Ağbaş, Yasemin Özlük, Nur Canpolat
BACKGROUND: Relapses or new-onset IgA nephropathy (IgAN) have been documented in patients after vaccination against SARS-CoV-2; however, only one adult patient has been reported in whom pre-existing IgAN worsened during coronavirus disease 2019 (COVID-19). CASE: We present the first pediatric case with biopsy-proven IgAN and genetically confirmed Alport syndrome, who developed end-stage kidney disease after an exacerbation of IgAN associated with COVID-19. The patient`s basal serum creatinine was 0...
2024: Turkish Journal of Pediatrics
https://read.qxmd.com/read/38522954/the-association-between-monocyte-to-high-density-lipoprotein-cholesterol-ratio-and-chronic-kidney-disease-in-a-chinese-adult-population-a-cross-sectional-study
#4
JOURNAL ARTICLE
Lingling Xu, Dongling Li, Zongwei Song, Jin Liu, Yang Zhou, Junwei Yang, Ping Wen
BACKGROUND: Monocyte to high-density lipoprotein cholesterol ratio (MHR) was confirmed as a novel inflammatory marker and strongly associated with the risk of several diseases. This study aimed to investigate the relationship between MHR and chronic kidney disease (CKD) in a Chinese adult population. METHODS: In this cross-sectional study, 232,775 community-dwelling adults in Binhai who completed health checkups in 2021 were enrolled. Participants were categorized based on the MHR quartiles...
December 2024: Renal Failure
https://read.qxmd.com/read/38520631/de-novo-posttransplant-membranous-nephropathy-after-covid-19-vaccination-9%C3%A2-years-after-renal-transplantation-in-a-patient-with-polycystic-kidney-disease
#5
JOURNAL ARTICLE
Miruzato Fukuda, Takayoshi Yokoyama, Katsuyuki Miki, Masayuki Yamanouchi, Daisuke Ikuma, Hiroki Mizuno, Yuki Oba, Noriko Inoue, Akinari Sekine, Kiho Tanaka, Eiko Hasegawa, Tatsuya Suwabe, Takehiko Wada, Kei Kono, Keiichi Kinowaki, Kenichi Ohashi, Yutaka Yamaguchi, Yuki Nakamura, Yasuo Ishii, Naoki Sawa, Yoshifumi Ubara
A 63-year-old man with polycystic kidney disease underwent kidney transplantation from his wife. Nine years later, after the first and second doses of the COVID-19 vaccination, he developed proteinuria, hematuria, and elevated C-reactive protein. Kidney biopsy 7 months after the initial appearance of proteinuria showed immunoglobulin (Ig)-G granular stain, predominantly IgG1, and spike formation in the glomerular basement membrane. Electron microscopy revealed mainly subepithelial deposits, which corresponds to membranous nephropathy (MN) stage 3 of the Ehrenreich-Churg classification indicating chronic disease, but it also showed electron-dense deposits and endothelial damage...
March 23, 2024: CEN Case Reports
https://read.qxmd.com/read/38519988/tocilizumab-in-chronic-active-antibody-mediated-rejection-rationale-and-protocol-of-an-in-progress-randomized-controlled-open-label-multi-center-trial-intercept-study
#6
JOURNAL ARTICLE
Lillian Streichart, Marie Felldin, Jana Ekberg, Lars Mjörnstedt, Per Lindnér, Annette Lennerling, Verena Bröcker, Johan Mölne, Jan Holgersson, Kristien Daenen, Lars Wennberg, Tomas Lorant, Seema Baid-Agrawal
BACKGROUND: Chronic active antibody-mediated rejection (caAMR) in kidney transplants is associated with irreversible tissue damage and a leading cause of graft loss in the long-term. However, the treatment for caAMR remains a challenge to date. Recently, tocilizumab, a recombinant humanized monoclonal antibody directed against the human interleukin-6 (IL-6) receptor, has shown promise in the treatment of caAMR. However, it has not been systematically investigated so far underscoring the need for randomized controlled studies in this area...
March 22, 2024: Trials
https://read.qxmd.com/read/38519234/oligonephronia-another-piece-in-the-ckdu-jigsaw
#7
JOURNAL ARTICLE
Wendy E Hoy, John F Bertram
Rates of chronic kidney disease of unknown etiology are high in Aguascalientes, Mexico. Kidneys of adolescents are small by ultrasonography, compatible with oligonephronia, whereas proteinuria and higher estimated glomerular filtration rates and blood pressures among those with relatively higher kidney volumes probably flag relatively greater degrees of compensatory hypertrophy. Glomerulomegaly and podocytopathy, and later segmental glomerulosclerosis in biopsies, suggest a cascade driven by nephron deficiency...
April 2024: Kidney International
https://read.qxmd.com/read/38519232/autosomal-dominant-chronic-tubulointerstitial-nephropathy-do-not-forget-amyloidosis
#8
JOURNAL ARTICLE
Frank Bridoux, Samih H Nasr
Amyloidosis is a rare cause of inherited kidney disease, with most variants responsible for prominent glomerular involvement. In this issue, Kmochová et al. reported the first description of autosomal dominant medullary amyloidosis due to apolipoprotein A4 variants, resulting in slowly progressive chronic kidney disease with minimal proteinuria. Combining next-generation sequencing with histopathological studies incorporating Congo red staining and mass spectrometry should be considered in the diagnostic workup of hereditary tubulointerstitial disorders not identified after routine genetic testing...
April 2024: Kidney International
https://read.qxmd.com/read/38519229/charting-new-frontiers-in-iga-nephropathy-a-paradigm-shift-toward-precision-medicine-and-early-intervention
#9
JOURNAL ARTICLE
Haresh Selvaskandan, Jonathan Barratt
Advancements in glomerular transcriptomics offer a promising avenue toward precision medicine in IgA nephropathy. Traditional prognostic biomarkers, including proteinuria, blood pressure, and histomorphometry, fall short at capturing the complexity of IgA nephropathy and can only crudely guide therapeutic decisions. This issue needs to be addressed urgently as pathway-specific treatments become available. Glomerular transcriptomics, although technically challenging, offers an opportunity to refine prognostic precision and identify therapeutic targets, even when apparent risk of disease progression is low...
April 2024: Kidney International
https://read.qxmd.com/read/38517255/effect-of-allogeneic-hematopoietic-stem-cell-transplantation-on-sickle-cell-disease-related-organ-complications-a-systematic-review-and-meta-analysis
#10
REVIEW
Elisabeth Dovern, Mesire Aydin, Michael R DeBaun, Komeil Alizade, Bart J Biemond, Erfan Nur
Sickle cell disease (SCD)-related organ complications are a major cause of morbidity and mortality in patients with SCD. We sought to assess whether hematopoietic stem cell transplantation (HSCT) stabilizes, attenuates, or exacerbates organ decline. We performed a systematic review and meta-analysis of trials investigating organ function before and after HSCT in patients with SCD. We searched MEDLINE/PubMed and EMBASE up to September 21, 2023. Continuous data were expressed as standardized mean difference (SMD) and pooled in a weighted inverse-variance random-effects model; binomial data were expressed as risk ratio (RR) using the Mantel-Haenszel random-effects meta-analyses...
March 22, 2024: American Journal of Hematology
https://read.qxmd.com/read/38514826/clinical-and-histopathological-characteristics-of-primary-focal-segmental-glomerulosclerosis-in-turkish-adults
#11
JOURNAL ARTICLE
Ilhan Kurultak, Ozkan Gungor, Savas Ozturk, Ahmet Burak Dirim, Necmi Eren, Ezgi Yenigün, Elbis Ahbab Dal, Mevlut Tamer Dincer, Feyza Bora, Suat Akgur, Abdullah Sumnu, Belda Dursun, Savas Sipahi, Hakki Cetinkaya, Idris Sahin, Garip Sahin, Murvet Yilmaz, Bulent Vatansever, Emre Aydın, Memnune Sena Ulu, Ali Gundogdu, Sedat Ustundag, Hayriye Sayarlioglu, Gizem Kumru, Omer C Elcioglu, Zeki Aydın, Nedim Yılmaz Selcuk, Ceren Onal Guclu, Meric Oruc, Mehmet Kucuk, Nimet Aktas, Ulver Derici, Gultekin Suleymanlar
The data regarding primary FSGS (pFSGS) from different parts of the world differ. While the prevalence of pFSGS has been increasing in Western countries like the USA, it follows an inconsistent trend in Europe and Asia and a decreasing trend in Far Eastern countries such as China in the last two decades. There are undetermined factors to explain those national and geographic discrepancies. Herein, we aimed to reveal the current prevalence with clinical and histopathological characteristics of pFSGS in Turkish adults...
March 21, 2024: Scientific Reports
https://read.qxmd.com/read/38512375/pathogenic-pathways-of-renal-damage-in-fabry-nephropathy-interplay-between-immune-cell-infiltration-apoptosis-and-fibrosis
#12
JOURNAL ARTICLE
Constanza Bondar, Maria de Los Angeles de Bolla, Pablo Neumann, Antonio Pisani, Sandro Feriozzi, Paula Adriana Rozenfeld
BACKGROUND: Fabry nephropathy is a consequence of the deposition of globotriaosylceramide, caused by deficient GLA enzyme activity in all types of kidney cells. These deposits are perceived as damage signals leading to activation of inflammation resulting in renal fibrosis. There are few studies related to immunophenotype characterization of the renal infiltrate in kidneys in patients with Fabry disease and its relationship to mechanisms of fibrosis. This work aims to quantify TGF-β1 and active caspase 3 expression and to analyze the profile of cells in inflammatory infiltration in kidney biopsies from Fabry naïve-patients, and to investigate correlations with clinical parameters...
March 21, 2024: Journal of Nephrology
https://read.qxmd.com/read/38512269/development-and-validation-of-staging-systems-for-aa-amyloidosis
#13
JOURNAL ARTICLE
Marco Basset, Stefan O Schönland, Laura Obici, Janine Günther, Eloisa Riva, Tobias Dittrich, Paolo Milani, Virginia Valeria Ferretti, Ettore Pasquinucci, Andrea Foli, Christoph Kimmich, Martina Nanci, Claudia Bellofiore, Francesca Benigna, Jörg Beimler, Pietro Benvenuti, Francesca Fabris, Roberta Mussinelli, Mario Nuvolone, Catherine Klersy, Riccardo Albertini, Giampaolo Merlini, Ute Hegenbart, Giovanni Palladini, Norbert Blank
BACKGROUND: The kidney is involved in almost 100% of cases of AA amyloidosis, a rare disease caused by persistent inflammation with long overall survival but frequent progression to end-stage kidney failure . Identification of patients with advanced disease at diagnosis is difficult, given the absence of validated staging systems. METHODS: Newly diagnosed patients with AA amyloidosis from the Pavia (n=233, testing cohort) and Heidelberg (n=243, validation cohort) centers were included in the study...
March 21, 2024: Journal of the American Society of Nephrology: JASN
https://read.qxmd.com/read/38511980/renal-transplant-in-elderly-end-stage-renal-disease-patients-impact-of-comorbidities-and-posttransplant-adverse-events-on-outcomes
#14
JOURNAL ARTICLE
Mohamed Yehia Hamdy, Hayam El Aggan, Iman El Gohary, Ahmed Halawa
OBJECTIVES: Elderly renal transplant continues to be debated because of age-related factors affecting transplant success and long-term prognosis. We investigated the effects ofrecipient age and predictors of renal transplant outcomes in elderly renal transplant recipients. MATERIALS AND METHODS: We retrospectively analyzed 506 patients who had a first renal transplant between January 2010 and December 2020; there were 165 recipients aged ≥60 years (elderly) and 341 recipients aged <60 years (young)...
February 2024: Experimental and Clinical Transplantation
https://read.qxmd.com/read/38511256/augmented-mannose-binding-lectin-levels-in-primary-membranous-nephropathy-a-pilot-study
#15
JOURNAL ARTICLE
Deeksha Pal, Neeraj Inamdar, Prabhjot Kaur, Manphool Singhal, Anupam Lal, Ujjwal Gorsi, Ritambhra Nada, Harbir S Kohli, Vinod Kumar, Raja Ramachandran
There is evidence to suggest that M-type phospholipase A2 (PLA2R) antibodies activate the mannose-binding lectin (MBL) cascade, resulting in glomerular damage and proteinuria in patients with primary membranous nephropathy (PMN). Furthermore, there are few reports indicating that aberrant MBL activation is associated with endothelial dysfunction and accelerated atherosclerosis. While PMN is a common cause of adult nephrotic syndrome, and patients are at increased risk of cardiovascular disease (CVD), there is a lack of research that explores the factors that contribute to this condition...
March 21, 2024: Nephrology
https://read.qxmd.com/read/38508877/renin-angiotensin-aldosterone-system-blockers-effect-in-chronic-kidney-disease-progression-in-hypertensive-elderly-patients-without-proteinuria-proercan-trial
#16
JOURNAL ARTICLE
A M García-Prieto, Ú Verdalles, A P de José, D Arroyo, I Aragoncillo, D Barbieri, R E Camacho, M Goicoechea
INTRODUCTION: Evidence about nefroprotective effect with RAAS blockers in elderly patients with chronic kidney disease (CKD) without proteinuria is lacking. The primary outcome of our study is to evaluate the impact of RAAS blockers in CKD progression in elderly patients without proteinuria. MATERIALS AND METHODS: Multicenter open-label, randomized controlled clinical trial including patients over 65 year-old with hypertension and CKD stages 3-4 without proteinuria...
March 19, 2024: Hipertensión y Riesgo Vascular
https://read.qxmd.com/read/38504059/obesity-related-glomerulopathy-is-associated-with-elevated-wt1-expression-in-podocytes
#17
JOURNAL ARTICLE
Sneha Jakhotia, Rajesh Kavvuri, Sumathi Raviraj, Somorita Baishya, Anil Kumar Pasupulati, G Bhanuprakash Reddy
BACKGROUND: The prevalence of obesity is increasing worldwide at an alarming rate. In addition to the increased incidence of cardiovascular and metabolic diseases, obesity is the most potent risk factor for developing chronic kidney disease (CKD). Although systemic events such as hemodynamic factors, metabolic effects, and lipotoxicity were implicated in the pathophysiology of obesity-related glomerulopathy (ORG) and kidney dysfunction, the precise mechanisms underlying the association between obesity and CKD remain unexplored...
March 19, 2024: International Journal of Obesity
https://read.qxmd.com/read/38498672/risk-factors-for-lower-renal-compensation-after-nephrectomy-an-analysis-of-living-kidney-donors-in-an-amazonian-cohort
#18
JOURNAL ARTICLE
Luan Moraes Ferreira, Gisela Gomes Batista, Leoneide Érica Maduro Bouillet, Emanuel Pinheiro Esposito
INTRODUCTION: Living donor kidney transplantation is considered the ideal renal replacement therapy because it has a lower complication rate and allows an efficient response to the high demand for grafts in the healthcare system. Careful selection and adequate monitoring of donors is a key element in transplantation. Individuals at greater risk of developing kidney dysfunction after nephrectomy must be identified. OBJECTIVE: To identify risk factors associated with a renal compensation rate (CR) below 70% 12 months after nephrectomy...
2024: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://read.qxmd.com/read/38496471/prevalence-of-biomarkers-and-associated-factors-for-chronic-kidney-disease-in-adult-diabetic-out-patients-in-a-tertiary-hospital-in-eastern-uganda-a-cross-sectional-study
#19
Moses Kirya, Denis Bwayo, Michael E Otim, Paul Bukhota Mutoo, J Peter M Masaba, Ambrose Okibure, Richard Katuramu
Background Chronic kidney disease (CKD) is one of the most common complications of Diabetes Mellitus (DM). DM contributes to about 66% of CKD cases globally. CKiiiD results in increased morbidity and mortality and advanced stages often require renal replacement therapy that is unaffordable for the majority of the patients. Developing countries have scanty data regarding CKD burden in diabetic patients. OBJECTIVES This study aimed at determining the prevalence of biomarkers for CKD and associated factors among diabetic patients attending the adult diabetic clinic of Mbale Regional Referral Hospital (MRRH)...
March 5, 2024: Research Square
https://read.qxmd.com/read/38488435/identification-of-novel-pathogenic-variants-of-cubn-in-patients-with-isolated-proteinuria
#20
JOURNAL ARTICLE
Huihui Yang, Lanfen He, Hongjian Gong, Chunhui Wan, Juanjuan Ding, Panli Liao, Xiaowen Wang
BACKGROUND: Although proteinuria is long recognized as an independent risk factor for progressive chronic kidney diseases, not all forms of proteinuria are detrimental to kidney function, one of which is isolated proteinuria caused by cubilin (CUBN)-specific mutations. CUBN encodes an endocytic receptor, initially found to be responsible for the Imerslund-Gräsbeck syndrome (IGS; OMIM #261100) characterized by a combined phenotype of megaloblastic anemia and proteinuria. METHODS: After analyzing their clinical and pathological characterizations, next-generation sequencing for renal disease genes or whole-exome sequencing (WES) was performed on four patients with non-progressive isolated proteinuria...
March 2024: Molecular Genetics & Genomic Medicine
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