839 results match your criteria: "Acquired Cystic Kidney Disease"

Article Synopsis
  • * The study analyzed 33 RCC samples from ESRD patients, focusing on genomic features and their correlation with tumor type and dialysis duration through techniques like whole-genome sequencing and transcriptome analysis.
  • * Findings showed that certain genomic changes in RCC from ESRD patients were similar to those seen in sporadic cases, highlighting a link between prolonged dialysis, unique mutational signatures, and specific genomic alterations tied to the development of cancer.
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Orthotopic ureterocele with concurrent ureteral urothelial carcinoma in a dog.

Vet Med Sci

September 2022

Department of Veterinary Surgery, College of Veterinary Medicine, Konkuk University, Seoul, Republic of Korea.

Article Synopsis
  • A ureterocele is a rare condition where the last part of the ureter swells inside the bladder, either in a normal position or an abnormal one, and its cause is not fully understood.
  • Urothelial carcinoma is common in dogs' urinary tracts, with most cases being invasive, but a non-invasive type exists that stays within the ureteral mucosa without spreading.
  • A 7-year-old Maltese dog with symptoms like lethargy and frequent urination was found to have an orthotopic ureterocele and hydronephrosis, leading to surgery where a non-invasive cancer was discovered, but the dog recovered well with no complications afterward.
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Article Synopsis
  • Various cystic and fibrocystic lesions in the liver can be either acquired or genetic and may appear as single or multiple occurrences.
  • While the structure and associated malformations of ductal plate development are documented, the genetic causes of many related disorders remain largely unknown.
  • Some liver disorders can lead to complications like portal hypertension or malignancy, but recent research is beginning to clarify their genetic basis and the overall understanding of these conditions.
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Purpose: Acquired cystic kidney disease (ACKD) is commonly seen in patients with end-stage renal disease (ESRD), and patients with ACKD have an increased risk of renal cell carcinoma (RCC). Acquired cystic disease-associated RCC (ACD-RCC) was incorporated into the 2016 World Health Organization Classification. This study aims to describe the imaging features of ACD-RCC, which are not well reported previously.

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Objective To determine the frequency of acquired cystic kidney disease (ACKD) in children on chronic hemodialysis. Material and methods In this single-center cross-sectional study, 150 children were included who were on chronic hemodialysis for six months. Ultrasound was done to see the renal cysts.

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Circulating blood is filtered across the glomerular barrier to form an ultrafiltrate of plasma in the Bowman's space. The volume of glomerular filtration adjusted by time is defined as the glomerular filtration rate (GFR), and the total GFR is the sum of all single-nephron GFRs. Thus, when the single-nephron GFR is increased in the context of a normal number of functioning nephrons, single glomerular hyperfiltration results in 'absolute' hyperfiltration in the kidney.

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Renal Cell Carcinoma in End-Stage Renal Disease: A Review and Update.

Biomedicines

March 2022

Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Main Building, Houston, TX 77030, USA.

Renal cell carcinoma (RCC) occurring in the setting of end-stage renal disease (ESRD) shows unique clinicopathological characteristics. The two most frequent types of ESRD-associated RCC are acquired cystic kidney disease-associated renal cell carcinoma (ACKD-RCC) and clear-cell papillary renal cell carcinoma (ccpRCC). Other types of RCC also occur in ESRD, albeit with different frequencies from the non-ESRD general population.

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Cancer Screening in End-Stage Kidney Disease.

Adv Chronic Kidney Dis

September 2021

Department of Medicine, Renal Service, Memorial Sloan Kettering Cancer Center, New York, NY.

Article Synopsis
  • Patients with end-stage kidney disease (ESKD) have a higher incidence of cancer than the general population, but cancer screening is often not cost-effective for them due to low life expectancy and the risks of treatment.
  • Screening may only be beneficial for certain groups, such as those on peritoneal dialysis, home hemodialysis, and specific ethnicities or high-risk individuals, including those with genetic predispositions or prior exposure to harmful substances.
  • The review will cover cancer prevalence in ESKD patients, factors affecting risk, the effectiveness of screening, and specific cases where screening might be advantageous.
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Objective: To distinguish benign from malignant cystic renal lesions (CRL) using a contrast-enhanced CT-based radiomics model and a clinical decision algorithm.

Methods: This dual-center retrospective study included patients over 18 years old with CRL between 2005 and 2018. The reference standard was histopathology or 4-year imaging follow-up.

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Purpose: Acquired cystic kidney disease-associated renal cell carcinoma (ACKD-RCC) is a relatively recently described entity with scarce literature describing its imaging appearance (1, 2). The purpose of this study was to determine typical and potentially unique features of ACKD-RCC on CT scan that could aid lesion identification.

Materials And Methods: A retrospective review of the CT scans of 24 patients with 29 histologically proven ACKD-RCC lesions was performed.

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Acquired cystic disease-associated renal cell carcinoma (ACD-RCC) is the major RCC subtype in patients with end-stage renal disease, specifically those with ACD on dialysis. Three patients with a total of eight tumors have been selected. The aim of this study was to analyze clinicopathologic, immunohistochemical, and prognostic features of eight ACD-RCCs.

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Background: Long-term use of proton pump inhibitors (PPIs) is associated with some safety issues. In this study, data mining was carried out to discover the potential association between renal neoplasms and PPIs.

Research Design And Methods: Neoplasms signals of PPIs were detected in the Food and Drug Administration Adverse Event Reporting System from 2014 to 2020 by examining the reporting odds ratio.

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We described a hemodialysis patient with spontaneous renal rupture of acquired cystic kidney disease (ACKD) after off-pump coronary artery bypass grafting(OPCAB). A 53-year-old man was transfer to our hospital with a diagnosis of unstable angina pectoris. He became ventilated due to congestive heart failure 3 days and underwent OPCAB on schedule 6 days after hospitalization.

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Article Synopsis
  • - Autosomal dominant polycystic kidney disease (ADPKD) is a genetic condition causing multiple cysts in kidneys, primarily due to mutations in the PKD1 and PKD2 genes that code for polycystin proteins.
  • - Researchers analyzed DNA from 90 kidney cysts from 24 patients, discovering that 93% of these cysts had harmful somatic mutations in PKD1 or PKD2, mainly resulting in significant gene alterations like truncations.
  • - The study suggests that cyst formation in ADPKD follows a cellular recessive mechanism, implicating both inherited and acquired mutations in the PKD1 and PKD2 genes within kidney cyst cells.
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A human multi-lineage hepatic organoid model for liver fibrosis.

Nat Commun

October 2021

Department of Anesthesiology, Pain and Perioperative Medicine, Stanford, CA, 94305, USA.

To investigate the pathogenesis of a congenital form of hepatic fibrosis, human hepatic organoids were engineered to express the most common causative mutation for Autosomal Recessive Polycystic Kidney Disease (ARPKD). Here we show that these hepatic organoids develop the key features of ARPKD liver pathology (abnormal bile ducts and fibrosis) in only 21 days. The ARPKD mutation increases collagen abundance and thick collagen fiber production in hepatic organoids, which mirrors ARPKD liver tissue pathology.

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Wunderlich syndrome, or spontaneous renal hemorrhage (SRH), is a rare condition encountered in patients undergoing chronic hemodialysis (HD) usually attributed to acquired cystic kidney disease (ACKD) among other causes. In the literature, colonoscopy is associated with splenic injuries, and renal hemorrhage has not been previously described. Management can range from conservative treatment to angiographic embolization or exploration and nephrectomy.

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Objectives: To evaluate the safety and effectiveness of transcatheter arterial embolization for controlling spontaneous hemorrhage in patients with acquired cystic kidney disease (ACKD).

Methods: This retrospective study included 18 patients with ACKD (M:F=13:5; mean age, 56 years) who underwent renal artery embolization to control spontaneous hemorrhage between January 2001 and September 2020. The underlying etiology and clinical presentations were reviewed and previous computed tomography (CT) findings were analyzed.

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Rationale: Acquired cystic disease-associated renal cell carcinoma (ACKD-RCC) is a unique subtype of renal cell carcinoma (RCC) and is found exclusively in patients with end-stage renal disease. We report a case of intracapsular nephrectomy (ICAN) of renal allograft with ACKD-RCC. To our knowledge, this is the first case in Asia of ICAN of renal allograft to treat ACKD-RCC.

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Acquired cystic disease (ACD) associated renal cell carcinoma (RCC) is designated as a new subtype unique to patients with end-stage renal disease (ESRD) according to the 2016 World Health Organization (WHO) classification. However, the oncological outcomes of the prognostic factors for patients with this subtype are not fully understood. In the present study, we compared the survival of ACD associated RCC patients who underwent nephrectomy with that of patients with other histological subtypes who developed ESRD.

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Refining genotype-phenotype correlations in 304 patients with autosomal recessive polycystic kidney disease and PKHD1 gene variants.

Kidney Int

September 2021

Department of Pediatrics, University Hospital Cologne and University of Cologne, Faculty of Medicine, Cologne, Germany; Center for Rare Diseases, University Hospital Cologne and Medical Faculty, University of Cologne, Cologne, Germany; Center for Molecular Medicine Cologne, University of Cologne, Faculty of Medicine, University Hospital Cologne, Cologne, Germany. Electronic address:

Autosomal recessive polycystic kidney disease (ARPKD) is a severe disease of early childhood that is clinically characterized by fibrocystic changes of the kidneys and the liver. The main cause of ARPKD are variants in the PKHD1 gene encoding the large transmembrane protein fibrocystin. The mechanisms underlying the observed clinical heterogeneity in ARPKD remain incompletely understood, partly due to the fact that genotype-phenotype correlations have been limited to the association of biallelic null variants in PKHD1 with the most severe phenotypes.

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Kidney transplant recipients (KTRs) are at increased risk of developing renal cell carcinoma (RCC). The cancer can be encountered at different steps in the transplant process. RCC found during work-up of a transplant candidate needs treatment and to limit the risk of recurrence usually a mandatory observation period before transplantation is recommended.

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We performed computed tomography every year and pointed out the development of kidney atrophy and a cystic lesion in relation to prolonged hemodialysis, which may be cause of spontaneous renal hemorrhage.

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A 48-year-old man with histories of IgA nephropathy for 33 years, hemodialysis for 29 years, and a kidney transplant from a deceased donor 5 years ago was admitted to our institute complaining of high fever and back pain. Although repeated follow-up of computed tomography failed to detect any de novo issues, he was eventually diagnosed as a renal cell carcinoma with multiple metastases, developing from his native-acquired cystic disease kidney with multiple cysts using a positron emission tomography. We should be cautious of de novo renal cell carcinoma in kidney transplantation recipients, and careful follow-up might be helpful to detect it.

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The Genitourinary Pathology Society (GUPS) reviewed recent advances in renal neoplasia, particularly post-2016 World Health Organization (WHO) classification, to provide an update on existing entities, including diagnostic criteria, molecular correlates, and updated nomenclature. Key prognostic features for clear cell renal cell carcinoma (RCC) remain WHO/ISUP grade, AJCC/pTNM stage, coagulative necrosis, and rhabdoid and sarcomatoid differentiation. Accrual of subclonal genetic alterations in clear cell RCC including SETD2, PBRM1, BAP1, loss of chromosome 14q and 9p are associated with variable prognosis, patterns of metastasis, and vulnerability to therapies.

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