1,725 results match your criteria: "Cystic Diseases of the Kidney"
Intestinal Barrier Function Declines During Polycystic Kidney Disease Progression.
Am J Physiol Renal Physiol
December 2024
Section of Cardio-Renal Physiology and Medicine, Division of Nephrology, Department of Medicine, University of Alabama at Birmingham, Birmingham, AL, United States.
Most patients with autosomal dominant polycystic kidney disease (ADPKD) develop kidney cysts due to germline mutations. In the kidney, loss impairs epithelial cell integrity and increases macrophage infiltration, contributing to cyst growth. Despite its role as the body's largest inflammatory cell reservoir, it has yet to be elucidated whether a similar phenotype presents in the intestines.
View Article and Find Full Text PDFCorrection to: Localized cystic disease of the kidney: study of 14 cases and review of the literature.
Virchows Arch
December 2024
Department of Pathology and Laboratory Medicine, Albany Medical Center, 43 New Scotland Avenue, Room F110G3, Albany, NY, USA.
Cysticercosis, although reported in several parts of the body, especially the liver, lesions occurring in the kidney are particularly rare. We report a 58-year-old female patient with intermittent epigastric pain, who was found to have a huge occupancy of the right kidney on computed tomography (CT) and magnetic resonance imaging (MRI), which was confirmed to have a predominantly cystic component, with no clear enhancement on CT images after injection of contrast medium, and significant enhancement of the peritoneum and septum visible on MRI. This case reports a case and its rarity of cysticercosis occurring in the kidney.
View Article and Find Full Text PDFThe cystogenic effects of ouabain in autosomal dominant polycystic kidney disease require cell caveolae.
Exp Cell Res
January 2025
Department of Cell Biology and Physiology and the Kidney Institute, University of Kansas Medical Center, Kansas City, KS, USA. Electronic address:
Article Synopsis
- Ouabain, a hormone, speeds up the progression of autosomal dominant polycystic kidney disease (ADPKD) by increasing cyst area and fibrosis specifically in ADPKD mice due to its interaction with Na,K-ATPase (NKA).
- Researchers created a mouse model with a knockout of caveolin-1 (CAV1), the main structural protein of caveolae, to investigate the role of these structures in ouabain's effect on ADPKD.
- The study found that without caveolae, the ADPKD mice did not show increased cyst progression or cellular changes in response to ouabain, indicating that caveolae play a crucial role in NKA signaling and the advancement of
Mucinous Borderline Tumors of the Pelvicalyceal System: Report of Two Tumors With Molecular Findings and Review of Literature.
Int J Surg Pathol
November 2024
Department of Urology, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
Mucinous borderline tumors of the pelvicalyceal system are exceedingly rare tumors with very little information regarding their clinicopathological characteristics and molecular underpinnings in the published literature. Herein, we report two mucinous borderline tumors and review the literature pertaining to these tumors. The patients were women who presented with flank pain without any history of nephrolithiasis.
View Article and Find Full Text PDFUltrasound evaluation of kidney and liver involvement in Bardet-Biedl syndrome.
Orphanet J Rare Dis
November 2024
Children's Hospital, Pediatrics II, Pediatric Nephrology, University of Duisburg-Essen, Essen, Germany.
Article Synopsis
Outcome of delayed presentation in patients with giant renal cell carcinoma: A case report.
Int J Surg Case Rep
December 2024
Department of Immunology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Department of Biomedical Science, Faculty of Medicine, Universitas Surabaya, Indonesia.
Article Synopsis
Detectable Concentrations With Inhaled Tobramycin in Critically Ill Infants and Children Following Implementation of Standardized Protocol.
Ann Pharmacother
September 2024
Department of Pharmacy, Clinical and Administrative Sciences, College of Pharmacy, The University of Oklahoma, Oklahoma City, OK, USA.
Article Synopsis
- A study was conducted on ventilator-associated tracheitis or pneumonia in mechanically ventilated children using inhaled tobramycin at specific intervals to assess drug levels and kidney function.
- The main goal was to find how many patients had tobramycin levels above 0.5 µg/mL, while secondary goals included comparing patient characteristics and tracking any instances of acute kidney injury (AKI).
- Out of 44 patients studied, 68% had detectable drug levels and 20.5% experienced AKI, but no significant differences were found in demographics or medical details between those with and without detectable levels or AKI.
Localized cystic disease of the kidney: study of 14 cases and review of the literature.
Virchows Arch
September 2024
Department of Pathology and Laboratory Medicine, Albany Medical Center, 43 New Scotland Avenue, Room F110G3, Albany, NY, USA.
Article Synopsis
- Localized cystic disease of the kidney (LCDK) is a rare, non-progressive condition that can be mistaken for cancer, potentially leading to unnecessary surgeries.
- In a study of 14 patients (ages 3-79, mostly middle-aged), most cases presented as unilateral and many patients experienced flank pain or had incidental findings.
- The lesions were multilocular and benign, lacking malignant features, and a review of 75 other cases supports the idea that LCDK should be included in the differential diagnosis for cystic kidney lesions.
Article Synopsis
- - The LITESPARK-004 study is testing the hypoxia-inducible factor-2α inhibitor, belzutifan, for its effectiveness in treating tumors associated with von Hippel-Lindau disease, specifically focusing on patients with central nervous system (CNS) haemangioblastomas after additional follow-up.
- - A total of 61 patients, with a majority having CNS haemangioblastomas, were enrolled in this phase 2 study across multiple cancer centers in four countries, and they received oral belzutifan until there were unacceptable side effects or disease progression.
- - The study assesses the antitumor activity by evaluating patient responses using two different methods based on tumor measurements and is ongoing, with results still being
An Uncommon Case of Sinonasal Adenoid Cystic Carcinoma Metastatic to the Kidney Treated with Metastasectomy.
J Kidney Cancer VHL
August 2024
Division of Urologic Oncology, The Ohio State University Comprehensive Cancer Center, Columbus, OH.
Adenoid cystic carcinoma (ACC) is a rare tumor, accounting for 1% of all head and neck cancers, with an aggressive nature characterized by local recurrence, delayed metastasis, and survival of less than 50% at 10 years. This is a case of biopsy-proven ACC to the kidney, 1 of 29 known occurrences, managed by metastasectomy by robotic-assisted nephrectomy, with plans for resection of lung metastasis. Thirteen years after diagnosis of sinonasal ACC treated with resection, the patient presented with shortness of breath.
View Article and Find Full Text PDFKidney After Lung Transplants or Combined Kidney-Lung Transplantation: A Single-Center Retrospective Cohort Study.
Ann Transplant
August 2024
Department of Urology, Foch Hospital, Suresnes, France.
Article Synopsis
- End-stage renal disease poses challenges for lung transplant patients, leading to the consideration of combined kidney-lung transplantation (CKLT) and kidney after lung transplantation (KALT) as potential solutions.
- A study reviewing outcomes of 26 patients showed that CKLT had a 30-day and 1-year survival rate of 75.6%, while no KALT patients died during follow-up, highlighting KALT's safety.
- The study found that kidney graft functions were similar between both strategies, but CKLT's success is significantly impacted by post-lung transplant complications, emphasizing the need for careful consideration of transplant options.
Impaired Glucose Metabolism, Primary Cilium Defects, and Kidney Cystogenesis in Glycogen Storage Disease Type Ia.
J Am Soc Nephrol
December 2024
Université Claude Bernard Lyon 1, INSERM, UMR_S1213, NUDICE, Villeurbanne, France.
Article Synopsis
Autoptic aspects in a case of autosomal dominant polycystic kidney disease.
Clin Ter
August 2024
Institute of Legal Medicine, Department of Medical and Surgical Sciences, "Magna Graecia" University of Catanzaro, Catanzaro, Italy.
Background: Polycystic kidney disease is a cystic genetic disease. There are two forms: an autosomal dominant one, more common and typical of adults, and an autosomal recessive one, rarer and present in childhood. The autosomal dominant form is caused by genetic mutations of the PKD1 gene in 85% of cases and of PKD2 in 10-15% of cases.
View Article and Find Full Text PDFLocalized cystic kidney disease: a case report unveiling clinical and histopathological challenges.
Autops Case Rep
June 2024
Mount Sinai Medical Center, Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Miami Beach, FL, USA.
Article Synopsis
- Localized cystic kidney disease (LCKD) is a specific kidney disorder marked by cysts in certain kidney areas, demonstrated in a case of a 41-year-old man with urinary symptoms and hematuria.
- Initial diagnosis revealed a urinary tract infection, but follow-up imaging showed a concerning 2.6 cm cystic lesion in the right kidney, leading to surgical intervention.
- The report highlights LCKD's benign nature, differences from progressive renal diseases, various symptoms, diagnostic methods, and similarities in tissue findings with conditions like polycystic kidney disease.
Incidental finding of cardiac hydatid cyst during autopsy.
Autops Case Rep
June 2024
All India Institute of Medical Sciences, Department of Forensic Medicine and Toxicology, Patna, Bihar, India.
Article Synopsis
- Hydatidosis, or echinococcosis, is a worldwide parasitic disease caused by ingesting echinococcal eggs, with higher incidence in states like Andhra Pradesh and Tamil Nadu in India.
- Dogs are the main hosts, while humans and livestock serve as intermediate hosts, with the disease often affecting the liver and lungs, though cardiac cases are rare.
- A notable case involves a 26-year-old male who died from electrical injuries, where an incidental finding of a hydatid cyst was discovered during autopsy on the heart wall.
Synovial Sarcoma of the Kidney: Diagnostic Pitfalls in a Case with Myxoid Monophasic Differentiation and No Epithelial Biomarkers Expression.
Int J Mol Sci
July 2024
Pathology Unit, Vanvitelli University Hospital, 80138 Naples, Italy.
Article Synopsis
- * Primary renal synovial sarcomas can appear similar to other kidney tumors, like nephroblastoma, and may exhibit distinct features such as myxoid stroma and specific immunohistochemical positivity.
- * The case discussed highlights the importance of molecular analysis in diagnosis and notes that, despite advanced disease, chemotherapy led to an unexpectedly positive response in the patient.
Expanding the phenotypic spectrum of CC2D2A-related ciliopathies: a rare homozygous nonsense variant in a patient with suspected nephronophthisis.
Eur J Hum Genet
September 2024
Department of Human Genetics, McGill University, Montreal, Canada.
Article Synopsis
- Biallelic pathogenic variants in the CC2D2A gene are linked to ciliopathies like Joubert and Meckel syndrome, which commonly affect kidney function, but isolated cases of kidney disease such as nephronophthisis had not been documented prior to this study.* -
- An adult presenting symptoms of nephronophthisis was found to have a rare homozygous nonsense variant in the CC2D2A gene, specifically c.100 C > T, which impacts kidney-specific transcripts but not those in other affected tissues like the cerebellum or liver.* -
- Analysis revealed that this variant may allow for partial translation re-initiation, suggesting a potential escape from normal decay
Identification and Properties of TRPV4 Mutant Channels Present in Polycystic Kidney Disease Patients.
Function (Oxf)
September 2024
Departamento de Neurociencia Cognitiva, Instituto de Fisiología Celular, Universidad Nacional Autónoma de México, Ciudad de México 04510, Mexico.
Article Synopsis
- * TRPV4 and TRPP2 channels interact in kidney cell physiology, and mutations in TRPV4 could affect kidney function, potentially worsening PKD severity.
- * PKD patients with TRPV4 mutations may face increased risks of requiring dialysis or kidney transplants compared to those without these mutations.
Rapamycin-encapsulated nanoparticle delivery in polycystic kidney disease mice.
Sci Rep
July 2024
Division of Nephrology, Department of Medicine, Section of Cardio-Renal Physiology and Medicine, McCallum Basic Health Science Building, University of Alabama at Birmingham, Room 533, 1918 University Blvd, Birmingham, AL, 35233, USA.
Rapamycin slows cystogenesis in murine models of polycystic kidney disease (PKD) but failed in clinical trials, potentially due to insufficient drug dosing. To improve drug efficiency without increasing dose, kidney-specific drug delivery may be used. Mesoscale nanoparticles (MNP) selectively target the proximal tubules in rodents.
View Article and Find Full Text PDFContrast-enhanced ultrasound (CEUS): applications from the kidneys to the bladder.
Abdom Radiol (NY)
November 2024
Department of Radiology, University of Washington, Seattle, Washington, 98195, USA.
Contrast-enhanced ultrasound (CEUS) is an advanced ultrasound (US) technique utilizing ultrasound contrast agents (UCAs) to provide detailed visualization of anatomic and vascular architecture, including the depiction of microcirculation. CEUS has been well-established in echocardiography and imaging of focal hepatic lesions and recent studies have also shown the utility of CEUS in non-hepatic applications like the urinary system. The updated guidelines by the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) from 2018 describe the use of CEUS for non-hepatic applications.
View Article and Find Full Text PDFEfficacy of F-Fluorodeoxyglucose positron emission tomography/computed tomography for detecting renal cell carcinoma in patients with end-stage renal disease.
Jpn J Radiol
October 2024
Department of Diagnostic Radiology and Nuclear Medicine, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan.
Article Synopsis
- Dialysis patients face a heightened risk of developing renal cell carcinoma (RCC), but distinguishing RCC from benign cysts can be challenging with standard imaging techniques like CT and ultrasound.
- This study conducted between 2012 and 2016 used FDG-PET/CT to screen for RCC in 150 participants with end-stage renal disease, with evaluations made by three radiologists.
- The results showed that FDG-PET/CT had a high sensitivity (100%) and negative predictive value (100%), ultimately diagnosing RCC in seven out of twenty patients identified as positive for malignancy.
Application of virtual endoscopic imaging to parapelvic cyst incision by flexible ureteroscopy: a multicenter retrospective cohort study.
Minerva Urol Nephrol
August 2024
Department of Urology, Affiliated Jinhua Hospital, Zhejiang University School of Medicine, Jinhua, Zhejiang, China -
Article Synopsis
- A 3D reconstruction of the kidney and cysts using 3D Slicer software was created to assist in performing flexible ureteroscopic procedures on parapelvic cysts with a holmium laser.
- A retrospective study involving 59 patients showed that the group using 3D imaging had significantly shorter operation times (about 39 minutes) compared to those who didn't use it (about 51 minutes).
- Although the new technique reduced operation time, there were no significant differences in other postoperative outcomes like fever or hospital stays, indicating more research is needed for long-term effectiveness.
Carbonic Anhydrase 2 Deletion Delays the Growth of Kidney Cysts Whereas Foxi1 Deletion Completely Abrogates Cystogenesis in TSC.
Int J Mol Sci
April 2024
Research Services, New Mexico Veterans Health Care System, Albuquerque, NM 87108, USA.
Article Synopsis
- Tuberous sclerosis complex (TSC) is linked to kidney issues, specifically renal cysts and benign tumors, which can lead to kidney failure.
- Research shows inactivation of carbonic anhydrase 2 reduces cyst formation, while deletion of the Foxi1 gene nearly eliminates cysts in knockout mice.
- By 110 days, knockout mice showed significant cyst development, correlating with increased Foxi1 expression and mTORC1 activation, unlike double-knockout mice (dKO) that had no cysts at either 47 or 110 days.
Article Synopsis
- HNF1B mutations are a leading genetic cause of congenital kidney and urinary tract abnormalities, prompting a study of 16 pediatric patients to better understand its clinical and genetic features.
- The research found that 81.3% of patients had prenatal kidney abnormalities, with kidney cysts being the most prevalent, and identified missense variants as the most common genetic issues linked to these conditions.
- The study suggests that patients with missense variants are at a greater risk for chronic kidney disease (CKD), while urinary system abnormalities like vesicoureteral reflux are also common in this group.