keyword
https://read.qxmd.com/read/38245210/rationale-and-design-of-the-nephrotic-syndrome-study-network-neptune-match-in-glomerular-diseases-designing-the-right-trial-for-the-right-patient-today
#21
JOURNAL ARTICLE
Howard Trachtman, Hailey Desmond, Amanda L Williams, Laura H Mariani, Sean Eddy, Wenjun Ju, Laura Barisoni, Heather K Ascani, Wendy R Uhlmann, Cathie Spino, Lawrence B Holzman, John R Sedor, Crystal Gadegbeku, Lalita Subramanian, Chrysta C Lienczewski, Tina Manieri, Scott J Roberts, Debbie S Gipson, Matthias Kretzler
Glomerular diseases are classified using a descriptive taxonomy that is not reflective of the heterogeneous underlying molecular drivers. This limits not only diagnostic and therapeutic patient management, but also impacts clinical trials evaluating targeted interventions. The Nephrotic Syndrome Study Network (NEPTUNE) is poised to address these challenges. The study has enrolled >850 pediatric and adult patients with proteinuric glomerular diseases who have contributed to deep clinical, histologic, genetic, and molecular profiles linked to long-term outcomes...
February 2024: Kidney International
https://read.qxmd.com/read/38233720/pediatric-contributions-and-lessons-learned-from-the-neptune-cohort-study
#22
REVIEW
Zubin J Modi, Yan Zhai, Jennifer Yee, Hailey Desmond, Wei Hao, Matthew G Sampson, Christine B Sethna, Chia-Shi Wang, Debbie S Gipson, Howard Trachtman, Matthias Kretzler
Primary glomerular diseases are rare entities. This has hampered efforts to better understand the underlying pathobiology and to develop novel safe and effective therapies. NEPTUNE is a rare disease network that is focused on patients of all ages with minimal change disease, focal segmental glomerulosclerosis, and membranous nephropathy. It is a longitudinal cohort study that collects detailed demographic, clinical, histopathologic, genomic, transcriptomic, and metabolomic data. The goal is to develop a molecular classification for these disorders that supersedes the traditional pathological features-based schema...
January 18, 2024: Pediatric Nephrology
https://read.qxmd.com/read/38204270/cyclophosphamide-toxicity-in-pediatric-nephrotic-syndrome-patient-a-case-report-and-literature-review
#23
Soumya Patil, Mahantesh V Patil, Apoorva Bagalkotkar, Shashikala Wali
BACKGROUND: Primary membranous nephropathy is a rare presentation in children. Patients unresponsive to steroids and experiencing frequent relapse are considered steroid-resistant. They often require complex treatment regimens consisting of immunosuppressants like cyclophosphamide, tacrolimus, and cyclosporin A. CASE: In the present case, a 5-year-old child was suffering from steroid-resistant nephrotic syndrome for the past 10 months. He was initially treated with prednisolone 20mg but was subsequently found to be steroid-resistant...
January 9, 2024: Current Drug Safety
https://read.qxmd.com/read/38195515/the-diagnosis-and-treatment-of-septic-hip-with-osteonecrosis-of-the-femoral-head
#24
JOURNAL ARTICLE
MingYang Li, ZhenShuai Shao, HaoXiang Zhu, YongTao Zhang
This article aims to provide clinical doctors with references for the diagnosis and treatment of osteonecrosis of the femoral head (ONFH) accompanied with septic hip by summarizing and analyzing clinical data and postoperative follow-up information of patients treated with two-stage arthroplasty. We retrospectively analyzed ten patients who underwent two-stage arthroplasty in our hospital due to ONFH accompanied with septic hip. The diagnosis of septic hip includes erythrocyte sedimentation rate (ESR) > 30 mm/h, C-reactive protein (CRP) > 10 mg/L, pus-like synovial fluid, positive microbiological culture, and the findings of septic arthritis on magnetic resonance imaging (MRI) scan...
January 9, 2024: Journal of Orthopaedic Surgery and Research
https://read.qxmd.com/read/38183071/repeated-implants-failure-in-young-patient-with-idiopathic-nephrotic-syndrome-a-case-report-with-brief-review-of-the-literature
#25
JOURNAL ARTICLE
Lamees R Alssum
BACKGROUND: Nephrotic syndrome is a chronic disorder characterized by heavy proteinuria, hypoalbuminemia, hyperlipidemia, and edema. Idiopathic minimal-change disease is the most common form encountered in children. Corticosteroids are the cornerstone for the treatment of idiopathic nephrotic syndrome (INS), with different regimens depending on the response to therapy and frequency of relapses. This case report presents complications after implant treatment in patient with INS. CASE PRESENTATION: 20 years old female patient presented for implant consultation...
January 5, 2024: BMC Oral Health
https://read.qxmd.com/read/38161837/retrospective-cross-sectional-study-reviewing-the-effectiveness-of-mycophenolate-mofetil-on-saudi-children-with-nephrotic-syndrome
#26
JOURNAL ARTICLE
Deemah A Aldakheel, Abdulrahman Alamir, Khawla K Almarshad, Zahra A Alsafwani, Roa A Alshaer, Abdullah T Alzulfah, Soud A Al Rasheed
Background Idiopathic nephrotic syndrome (INS) is the most common form of nephrotic syndrome (NS) in children. It is often associated with minimal change disease (MCD). Corticosteroid therapy is the initial treatment, but many patients experience relapses, leading to steroid-dependent nephrotic syndrome (SDNS) or frequently relapsing steroid-sensitive nephrotic syndrome (FR-SSNS). To avoid prolonged steroid use, mycophenolate mofetil (MMF) is used as an immunosuppressive alternative. MMF is safe and effective for treating SDNS and FR-SSNS in children, with studies showing reduced relapse rates...
November 2023: Curēus
https://read.qxmd.com/read/38142038/post-transplant-recurrence-of-focal-segmental-glomerular-sclerosis-consensus-statements
#27
JOURNAL ARTICLE
Rupesh Raina, Swathi Jothi, Dieter Haffner, Michael Somers, Guido Filler, Prabhav Vasistha, Ronith Chakraborty, Ron Shapiro, Parmjeet S Randhawa, Rulan Parekh, Christopher Licht, Timothy Bunchman, Sidharth Sethi, Guneive Mangat, Joshua Zaritsky, Franz Schaefer, Bradley Warady, Sharon Bartosh, Mignon McCulloch, Khalid Alhasan, Agnieszka Swiatecka-Urban, William E Smoyer, Anil Chandraker, Hui Kim Yap, Vivekanand Jha, Arvind Bagga, Jai Radhakrishnan
Focal segmental glomerular sclerosis (FSGS) is 1 of the primary causes of nephrotic syndrome in both pediatric and adult patients, which can lead to end-stage kidney disease. Recurrence of FSGS after kidney transplantation significantly increases allograft loss, leading to morbidity and mortality. Currently, there are no consensus guidelines for identifying those patients who are at risk for recurrence or for the management of recurrent FSGS. Our work group performed a literature search on PubMed/Medline, Embase, and Cochrane, and recommendations were proposed and graded for strength of evidence...
December 22, 2023: Kidney International
https://read.qxmd.com/read/38136965/nup85-as-a-neurodevelopmental-gene-from-podocyte-to-neuron
#28
REVIEW
Antonella Gambadauro, Giuseppe Donato Mangano, Karol Galletta, Francesca Granata, Antonella Riva, Laura Massella, Isabella Guzzo, Giovanni Farello, Giovanna Scorrano, Ludovica Di Francesco, Giulio Di Donato, Carolina Ianni, Armando Di Ludovico, Saverio La Bella, Pasquale Striano, Stephanie Efthymiou, Henry Houlden, Rosaria Nardello, Roberto Chimenz
Pathogenic gene variants encoding nuclear pore complex (NPC) proteins were previously implicated in the pathogenesis of steroid-resistant nephrotic syndrome (SRNS). The NUP85 gene, encoding nucleoporin, is related to a very rare form of SRNS with limited genotype-phenotype information. We identified an Italian boy affected with an SRNS associated with severe neurodevelopmental impairment characterized by microcephaly, axial hypotonia, lack of achievement of motor milestones, and refractory seizures with an associated hypsarrhythmic pattern on electroencephalography...
November 27, 2023: Genes
https://read.qxmd.com/read/38123711/kidney-biopsy-findings-in-children-with-diabetes-mellitus
#29
JOURNAL ARTICLE
Lasanthi Weerasooriya, Alexander J Howie, Matthew P Wakeman, Susan Cavanagh, David V Milford
BACKGROUND: Diabetic nephropathy may begin in childhood, but clinical kidney disease ascribable to this is uncommon in children with type 1 (insulin dependent) diabetes mellitus. METHODS: We reviewed our experience of kidney biopsies in children with type 1 diabetes mellitus. RESULTS: Between 1995 and 2022, there were biopsies in 17 children, with various clinical indications for kidney biopsy, making this the largest series of biopsies in diabetic children with clinical kidney abnormalities...
December 21, 2023: Pediatric Nephrology
https://read.qxmd.com/read/38117311/biomarkers-of-histologic-severity-in-children-with-severe-or-atypical-acute-post-streptococcal-glomerulonephritis
#30
JOURNAL ARTICLE
William Wong, Chanel Prestidge, Jonathan Zwi, Dug Yeo Han
BACKGROUND: Acute post-streptococcal glomerulonephritis (APSGN) is a common cause of acute kidney injury (AKI) in children; however, in a small subgroup, the presentation is one of rapidly progressive glomerulonephritis (RPGN) deteriorating kidney function associated with severe oligo-anuria or a mixed nephritic-nephrotic picture. This study reviewed potential clinical and laboratory factors which may assist the treating clinician to identify patients at high risk of severe disease. METHODS: All kidney biopsies for APSGN performed between 1996 and 2020 were obtained from a departmental biopsy database...
December 20, 2023: Pediatric Nephrology
https://read.qxmd.com/read/38110745/tumor-necrosis-factor-alpha-gene-polymorphism-affects-the-pattern-of-idiopathic-nephrotic-syndrome-in-kuwaiti-arab-children
#31
JOURNAL ARTICLE
Amal A Al-Eisa, Maysoun Al Rushood, Sumedha Kashyap, Mohammad Z Haider
OBJECTIVES: TNF-α is a pro-inflammatory cytokine that has been implicated in many inflammatory diseases, but its association with idiopathic nephrotic syndrome (INS) is poorly understood. This study looked for an association of TNF-α gene polymorphisms with INS, as well as its effect on steroid responsiveness among Kuwaiti Arab children. METHODS: Genotypes of the TNF-a gene polymorphisms were analyzed using polymerase chain reaction-restriction fragment length polymorphism in 151 INS Kuwaiti Arab patients and 64 age and sex-matched controls...
December 6, 2023: Journal of Tropical Pediatrics
https://read.qxmd.com/read/38106587/podocyte-infolding-glomerulopathy-a-special-morphology-of-podocyte-injury-caused-by-heterogeneous-diseases
#32
JOURNAL ARTICLE
Ling Hong, Lin Wang, Honglei Wang, Qihua Wang, Shicong Yang, Tian Tian, Tianjiao Cui, Shuling Yue, Xiaotao Hou, Zhihua Zheng, Wenfang Chen
INTRODUCTION: Podocyte infolding glomerulopathy (PIG) is a newly recognized rare glomerular injury. The clinical significance and mechanism of this injury pattern remains unclear. METHODS: We conducted a retrospective study of renal biopsies from January 2018 to December 2020 in Kingmed Diagnostics. The renal biopsy features and clinical data were reviewed. Laser scanning microdissection and mass spectrometry (LMD/MS) was conducted to analyze the potential mechanism...
December 2023: KI Reports
https://read.qxmd.com/read/38072955/the-epidemiology-of-primary-fsgs-including-cluster-analysis-over-a-20-year-period
#33
JOURNAL ARTICLE
Thomas McDonnell, Joshua Storrar, Rajkumar Chinnadurai, Calvin Heal, Constantina Chrysochou, James Ritchie, Francesco Rainone, Dimitrios Poulikakos, Philip Kalra, Smeeta Sinha
INTRODUCTION: Focal segmental glomerulosclerosis (FSGS) is one of the leading causes of nephrotic syndrome in adults. This epidemiological study describes a renal centre's 20-year experience of primary FSGS. METHODS: Patients were identified with a diagnosis of primary FSGS after exclusion of known secondary causes. In this retrospective observational study, data was collected for baseline demographics, immunosuppression and outcomes. A two-step cluster analysis was used to identify natural groupings within the dataset...
December 10, 2023: BMC Nephrology
https://read.qxmd.com/read/38065629/urinary-loss-of-thyroid-hormones-an-issue-to-remember
#34
REVIEW
Ismael Capel, Loreley Betancourt, María Florencia Luchtenberg, Andreea Muntean, Valeria Arsentales, Judith Jover, Mercedes Rigla
A 45-year-old woman suffering from primary hypothyroidism, previously well substituted with levothyroxine, was urgently referred from Primary Care to Endocrinology due to very elevated thyrotropin, free thyroxine at low limit of normality, very high cholesterol and generalised oedema. Hypothyroidism was suspected as the main aetiology of this clinical condition. A detailed examination showed nephrotic range proteinuria and the patient was finally diagnosed with lupus nephritis. Urinary loss of thyroid hormones, fundamentally linked to their transport proteins, in patients affected by nephrotic syndrome is sometimes a forgotten phenomenon and one which should be considered in patients with increased levothyroxine requirements...
December 2023: Endocrinología, diabetes y nutrición
https://read.qxmd.com/read/38054033/longitudinal-analysis-of-prophylactic-anticoagulation-in-primary-nephrotic-syndrome-low-incidence-of-thromboembolic-complications
#35
JOURNAL ARTICLE
Thomas McDonnell, John Hartemink, Omar Ragy, Katherine Parker, Meshaal Shukkur, Jecko Thachil, Durga Kanigicherla
INTRODUCTION: Thromboembolic events (TEEs) are a serious and potentially fatal complication of nephrotic syndrome (NS). Despite this, there is a lack of evidence examining the benefits of prophylactic anticoagulation (PAC) in NS. It was our objective to review the risk factors, rates of TEEs, and patterns of PAC in patients with primary NS, with the aim to provide a pragmatic approach to PAC in primary NS. METHODS: This is a retrospective longitudinal cohort study of adult patients with primary NS...
2023: Glomerular diseases
https://read.qxmd.com/read/38042380/experimental-models-for-elderly-patients-with-membranous-nephropathy-application-and-advancements
#36
REVIEW
Xinyue Tang, Haoran Dai, Yuehong Hu, Wenbin Liu, Qihan Zhao, Hanxue Jiang, Zhendong Feng, Naiqian Zhang, Hongliang Rui, Baoli Liu
Membranous nephropathy (MN) occurs predominantly in middle-aged and elderly individuals and ranks among the most prevalent etiologies of elderly nephrotic syndrome. As an autoimmune glomerular disorder characterized by glomerular basement membrane thickening and immune complex deposition, conventional MN animal models, including the Heymann nephritis rat model and the c-BSA mouse model, have laid a foundation for MN pathogenesis research. However, differences in target antigens between rodents and humans have impeded this work...
November 30, 2023: Experimental Gerontology
https://read.qxmd.com/read/38007057/proteinuria-selectivity-index-in-renal-disease
#37
REVIEW
Wen Liu, Zhi-Hang Su, Qi-Jun Wan
One of the main barriers to early detection and subsequent prevention of kidney diseases is the accessibility and feasibility of testing, especially in urine research. The proteinuria selectivity index (PSI or SI) is a method used to assess changes in glomerular permeability in glomerular diseases. It describes the pattern of proteinuria by comparing the clearance rates of large molecular proteins and transferrin, categorizing it as selective or non-selective. PSI is widely applied for kidney disease classification, prediction of corticosteroid efficacy, and prognosis...
November 23, 2023: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://read.qxmd.com/read/37973491/drug-induced-glomerular-diseases
#38
JOURNAL ARTICLE
Anne-Sophie Garnier, Hélène Laubacher, Marie Briet
Drug-induced kidney diseases represent a wide range of diseases that are responsible for a significant proportion of all acute kidney injuries and chronic kidney diseases. In the present review, we focused on drug-induced glomerular diseases, more precisely podocytopathies - minimal change diseases (MCD), focal segmental glomerulosclerosis (FSGS) - and membranous nephropathies (MN), from a physiological and a pharmacological point of view. The glomerular filtration barrier is composed of podocytes that form foot processes tightly connected and directly in contact with the basal membrane and surrounding capillaries...
October 31, 2023: Thérapie
https://read.qxmd.com/read/37971268/pattern-of-renal-diseases-among-children-attending-paediatric-nephrology-clinic-federal-teaching-hospital-gombe
#39
R Aliu, I Jalo, L Olatoke, I Asabe, K S Hassan, A Bello, M A Danimoh
INTRODUCTION: Children present with a spectrum of renal diseases depending on age, sex, and geographic location among other factors. With the absence of a paediatric renal registry in Nigeria, this will provide part of the regional data necessary for the Nigerian renal registry. METHODOLOGY: A retrospective study where cases of renal diseases that presented in a nephrology clinic over a 2-year period were retrieved from the nurses' and doctors' records and analysed...
November 10, 2023: West African Journal of Medicine
https://read.qxmd.com/read/37921337/current-approaches-to-overcome-recurrent-focal-segmental-glomerulosclerosis-after-kidney-transplantation
#40
REVIEW
Syeda Behjat Ahmad, Yonatan Peleg, Wooin Ahn
PURPOSE OF REVIEW: Recurrent focal segmental glomerulosclerosis (FSGS) presents with nephrotic syndrome shortly after kidney transplantation. This review will overview the role of circulating permeability factors in disease pathogenesis and treatment options for recurrent FSGS. RECENT FINDINGS: Novel circulating permeability factors have been identified in serum samples. Current research is focused on detection of permeability factors as a marker of treatment response...
January 1, 2024: Current Opinion in Nephrology and Hypertension
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