8,940 results match your criteria: "Focal Segmental Glomerulosclerosis"

Objective: The objective of this study is to investigate the diagnostic utility of microRNAs (miRNAs) for distinguishing between urine samples from patients with Diabetic Kidney Disease (DKD) and those with Focal Segmental Glomerulosclerosis (FSGS).

Methods: In this multicentric, cross-sectional investigation, we enrolled patients diagnosed with DKD, individuals with primary biopsy-proven FSGS, and healthy controls. The top 5 miRNAs (hsa-mir-21, hsa-mir-30a, hsa-mir-193a, hsa-mir-196a, hsa-mir-200a) were selected to quantify miRNAs in urine samples.

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A subgroup of children with frequently-relapsing, steroid-dependent nephrotic syndrome relapse during B-cell depletion after rituximab. A 15-year-old boy with focal segmental glomerulosclerosis became rituximab-refractory after 5 courses of treatments, with a relapse-free period shortened to 1 month. Circulating total and memory B-cells were undetectable at the time of relapse.

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Bi- and Monoallelic Variants and Chronic Kidney Disease in West Africans.

N Engl J Med

October 2024

From the Department of Pediatrics, Duke University Medical Center, Durham (R.A.G.), and the Departments of Medicine (B.I.F.) and Biochemistry (N.D.P.), Wake Forest University School of Medicine, Winston-Salem - both in North Carolina; the Department of Medicine, University of Nigeria, Nsukka (I.U.), the Department of Medicine, College of Medicine, University of Ibadan, Ibadan (S. Ajayi, Y.R., A.D.A., A. Asinobi, O. Amodu, B.L.S.), the Department of Medicine, University of Ilorin, Ilorin (T.O.), the Department of Medicine, Obafemi Awolowo University, Ile-Ife (F.A.), the Department of Medicine, University of Abuja, Abuja, Nigeria (M. Mamven), the Department of Medicine, College of Medicine, University of Lagos, Lagos (T.A.), the Department of Medicine, College of Medicine, Lagos State University, Ojo (A.S., O. Awobusuyi), the Department of Medicine, Usmanu Danfodiyo University, Sokoto (M. Makusidi), Nnamdi Azikiwe University Teaching Hospital, Nnewi (U.O.), Delta State University Teaching Hospital, Oghara (O.O.), and Aminu Kano Teaching Hospital, Kano (A. Abdu) - all in Nigeria; the Department of Medicine, University of Ghana Medical School (C.O., M. Matekole, V.A., V.B., D.A.), and Noguchi Memorial Institute for Medical Research, University of Ghana (A.N., A.G.), Accra, the Department of Medicine, University of Cape Coast, Cape Coast (I.E.), and Kwame Nkrumah University of Science and Technology, Kumasi (J.P.-R., S. Antwi) - all in Ghana; the Basic Research Laboratory, Frederick National Laboratory for Cancer Research, National Cancer Institute, Frederick (C.A.W.), the Center for Research on Genomics and Global Health, National Human Genome Research Institute (A.A.A.), and the Division of Kidney, Urologic and Digestive Disease, National Institute of Diabetes and Digestive Kidney Diseases (P.L.K.), National Institutes of Health, Bethesda - all in Maryland; the Departments of Human Genetics (D.B.), Medicine (M.K.), and Pathology (J.B.H.), University of Michigan Medical School, Ann Arbor; the Parkinson School of Health Sciences and Public Health, Loyola University, Chicago (R.C., B.T.); the Department of Medicine, Boston University School of Medicine (T.O.I.), the Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School (M.R.P.), and the Departments of Pathology (B.C.) and Medicine (W.W.), Massachusetts General Hospital and Harvard Medical School - all in Boston; the Department of Medicine, College of Medicine, University of Arizona, Tucson (F.C.B.); the Departments of Biostatistics and Data Science (M.P., J.S.) and Medicine (C.I.A., J.W., A.O.), University of Kansas Medical Center, Kansas City, Kansas; and the Department of Medicine and Pediatrics, Women's College Hospital, Hospital for Sick Children and University of Toronto, Toronto (R.S.P.).

Background: Apolipoprotein L1 gene () variants are risk factors for chronic kidney disease (CKD) among Black Americans. Data are sparse on the genetic epidemiology of CKD and the clinical association of variants with CKD in West Africans, a major group in the Black population.

Methods: We conducted a case-control study involving participants from Ghana and Nigeria who had CKD stages 2 through 5, biopsy-proven glomerular disease, or no kidney disease.

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Focal segmental glomerulosclerosis (FSGS) is a major cause of pediatric kidney failure. Most cases of FSGS in children are idiopathic and have a high risk of post-transplantation recurrence and graft loss. Common treatments for recurrent FSGS (rFSGS) post-transplantation include plasmapheresis, immunoadsorption, and/or immunomodulatory therapy.

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An Updated Comprehensive Review on Diseases Associated with Nephrotic Syndromes.

Biomedicines

October 2024

Department II of Internal Medicine, Center for Molecular Medicine Cologne, Faculty of Medicine, University Hospital Cologne, University of Cologne, 50937 Cologne, Germany.

Article Synopsis
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Article Synopsis
  • - Focal Segmental Glomerulosclerosis (FSGS) is a complex kidney disorder that leads to nephrotic syndrome and can cause kidney failure in both children and adults.
  • - The Kidney Health Initiative is exploring the potential of using proteinuria (excess protein in urine) as a key indicator for measuring treatment outcomes in FSGS clinical trials.
  • - While total remission of proteinuria seems to correlate strongly with better kidney health, more research is necessary to establish how to best evaluate treatment effects, with ongoing efforts to consolidate data from various studies.
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  • - The study analyzed changes in the molecular characteristics of podocytes in two kidney conditions: primary focal segmental glomerulosclerosis (pFSGS) and IgA nephropathy (IgAN), using kidney samples from patients and negative controls.
  • - Results showed that pFSGS patients had significantly higher protein levels in their urine and distinct alterations in podocytes compared to IgAN and negative controls, including decreased WT1 expression and increased desmin expression.
  • - The findings suggest that these alterations may indicate a genomic reprogramming in podocytes and their surrounding cells, which could contribute to the pathogenesis of these kidney diseases.
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Background: Podocyte loss occurs in both primary and secondary glomerular diseases, leading to the progression of kidney disease. A large body of evidence suggests that apoptosis and detachment are the mechanisms mediating the reduction in podocyte numbers in glomerular diseases. Recent studies demonstrate a renal protective effect of protein S (PS) through the activation of Tyro3, one of the TAM receptors.

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Alport syndrome is a rare kidney disease typically more severe in males due to its X-linked inheritance. However, female patients with heterozygous X-linked Alport syndrome (XLAS) can develop renal failure over time, necessitating accurate pathologic assessment for effective therapy. A key pathologic finding in female patients with XLAS is the mosaic pattern of partial loss of α5 chains of type IV collagen.

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Article Synopsis
  • The study reports on a case of steroid-resistant nephrotic syndrome in a 9-month-old infant caused by novel gene mutations, highlighting the clinical and genetic features of Pierson syndrome.
  • Retrospective analysis included clinical presentation, genetic mutation features, and prognosis, supported by literature searches in various medical databases.
  • The findings revealed specific genetic mutations, common histopathological results, and a trend toward severe outcomes in affected patients, with a significant proportion progressing to end-stage kidney disease.
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Introduction: Lymphocele is a common complication post-kidney transplantation, influenced by various factors including surgical technique, graft vessel count, operator experience, body mass index, ischemia time, and immunotherapy regimens.

Project Aims: The purpose of this study was to evaluate lymphocele risk factors, particularly focusing on the role of end-stage kidney disease.

Design: A retrospective study was conducted on renal transplant recipients from a single center (March 2020 to December 2022).

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Article Synopsis
  • Rituximab is effective for treating minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) in patients with frequently relapsing nephrotic syndrome, but does not work for everyone, particularly those with rapid relapses or no remission after treatment.
  • This study analyzed the use of obinutuzumab, a new anti-CD20 antibody, in 11 adult patients who had not responded well to standard treatments, focusing on relapse rates and safety.
  • Results indicated that obinutuzumab was mostly effective, with a median relapse-free time of over 12 months and more than half of the patients able to stop using corticosteroids and other immunosuppressants within three months, although
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Article Synopsis
  • The study investigated the effectiveness and safety of rituximab for treating childhood steroid-resistant nephrotic syndrome (SRNS) across 28 pediatric nephrology centers globally.
  • Researchers found that children with a longer history of calcineurin inhibitors (CNIs) treatment before rituximab had lower remission rates compared to those with shorter treatment periods.
  • Overall, rituximab showed enhanced remission rates in a portion of SRNS patients, was mostly safe, and achieving complete remission was linked to better kidney survival outcomes.
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  • Glomerulonephritis (GN) is a leading cause of end-stage renal disease and kidney transplants, with potential for recurrence in transplanted kidneys, prompting a study on its characteristics in these patients.
  • A retrospective study of 109 transplant patients revealed that the most common recurring types of GN were IgA nephropathy (GNIgA) and focal segmental glomerulosclerosis (FSGS), which were also associated with significant kidney dysfunction.
  • The study found that GNIgA recurred mainly after five years post-transplant, while FSGS recurrence occurred much sooner, within 1 to 6 months.
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IgA Nephropathy Secondary to COVID-19 Infection - A Case Report.

J Community Hosp Intern Med Perspect

July 2024

Department of Nephrology, Washington Nephrology, Baltimore, MD, USA.

Article Synopsis
  • ! A 73-year-old African American woman with COVID-19 developed acute kidney injury (AKI) and proteinuria, leading to a renal biopsy that confirmed IgA nephropathy. * ! Her diagnosis of IgA nephropathy was identified several weeks after her COVID-19 infection, which is unusual compared to typical cases that present with immediate symptoms. * ! This case emphasizes the complexity of kidney issues related to COVID-19 and stresses the importance of careful monitoring for renal complications in such patients, particularly when symptoms are atypical. *
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Clinical relevance of proteinuria selectivity index and fractional excretion of sodium in patients with nephrotic syndrome.

Sci Rep

October 2024

Division of Nephrology, Endocrinology and Metabolism, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-Ku, Tokyo, 160-8582, Japan.

Article Synopsis
  • The study investigates the potential of the Proteinuria Selectivity Index (PSI) and fractional excretion of sodium (FENa) as tools for classifying nephrotic syndrome and predicting treatment responses.
  • It involved a retrospective analysis of 611 patients who underwent kidney biopsies, categorizing their conditions and determining optimal cutoffs for PSI and FENa to predict complete remission.
  • The findings indicate that both low PSI and FENa are associated with a higher likelihood of achieving complete remission, suggesting their clinical relevance in nephrotic syndrome management.
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Article Synopsis
  • * Recent studies indicate that sphingosine 1-phosphate (S1P) signaling plays a significant role in kidney fibrosis and the progression of specific types of CKD, such as diabetic nephropathy and lupus nephritis.
  • * There is potential for new therapies targeting S1P signaling, with research suggesting benefits from modulators like fingolimod and promising applications in disease progression monitoring, particularly in Fabry disease and diabetic nephropathy.
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  • Alport syndrome (AS) is a common inherited kidney disease that requires early diagnosis for better outcomes; this study assessed the awareness and understanding of AS among Korean clinicians, patients, and caregivers.
  • An online survey of 103 participants revealed that many clinicians had limited experience with AS and frequently overlooked it as a potential diagnosis, while patients and caregivers expressed concerns about delayed diagnoses and lack of support.
  • The findings highlight a gap in the adoption of recent guidelines for AS in clinical practice in Korea, indicating the need for improved recommendations and a registry to enhance patient care.
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Pathological diagnosis of Alport syndrome.

Kidney Res Clin Pract

August 2024

Department of Pathology, Yonsei University College of Medicine, Seoul, Republic of Korea.

Article Synopsis
  • - Alport syndrome (AS) is a genetic kidney disorder caused by faulty COL4A3, COL4A4, and COL4A5 genes, leading to structural problems in the kidney's filtering membranes.
  • - Standard tests show vague results, but electron microscopy and type IV collagen staining help differentiate AS subtypes based on how the traits are inherited.
  • - Genetic testing, especially next-generation sequencing, is crucial as it can detect various mutations linked to AS, improving diagnosis and understanding.
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