752 results match your criteria Acquired Cystic Kidney Disease

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

Biomedicines 2022 Mar 11;10(3). Epub 2022 Mar 11.

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. Read More

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Cancer Screening in End-Stage Kidney Disease.

Adv Chronic Kidney Dis 2021 09;28(5):502-508.e1

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

The incidence of cancer is higher in patients with end-stage kidney disease (ESKD) than among the general population. Despite this, screening for cancer is generally not cost-effective and may worsen quality of life in these patients. This is due to high mortality rates (patients are not living long enough to reap the benefits of screening), the inaccuracy of cancer screening tests, and the increased risks associated with therapy in patients with ESKD. Read More

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September 2021

Malignancy risk stratification of cystic renal lesions based on a contrast-enhanced CT-based machine learning model and a clinical decision algorithm.

Eur Radiol 2022 Jun 23;32(6):4116-4127. Epub 2022 Jan 23.

Department of Diagnostic Radiology, McGill University, Montreal, Canada.

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. Read More

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CT features of acquired cystic kidney disease-associated renal cell carcinoma.

Clin Imaging 2022 Mar 5;83:83-86. Epub 2022 Jan 5.

Department of Pathology, Memorial Sloan Kettering Hospital, New York, NY, United States of America.

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. Read More

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Acquired cystic disease-associated renal cell carcinoma: A clinicopathologic study of eight tumors with clinical follow-up.

Indian J Cancer 2021 Oct-Dec;58(4):608-614

Departments Chair of Pathomorphology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia, Katowice, Poland.

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. Read More

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February 2022

Proton pump inhibitors and increased reporting odds of renal neoplasms: FAERS-based adverse event data mining and analysis.

Expert Opin Drug Saf 2022 May 29;21(5):707-713. Epub 2021 Dec 29.

Department of Oncology, The Fifth Affiliated Hospital of Zhengzhou University, Zhengzhou, P. R. China.

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. Read More

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[Spontaneous Renal Rupture of Acquired Cystic Kidney Disease after Coronary Artery Bypass Grafting:Report of a Case].

Kyobu Geka 2021 Nov;74(12):1032-1035

Department of Cardiovascular Surgery, Aomori City Hospital, Aomori, Japan.

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. Read More

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November 2021

Detection of and Somatic Variants in Autosomal Dominant Polycystic Kidney Cyst Epithelial Cells by Whole-Genome Sequencing.

J Am Soc Nephrol 2021 Oct 29. Epub 2021 Oct 29.

Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, New York

Background: Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by the development of multiple cysts in the kidneys. It is often caused by pathogenic mutations in and genes that encode polycystin proteins. Although the molecular mechanisms for cystogenesis are not established, concurrent inactivating germline and somatic mutations in and have been previously observed in renal tubular epithelium (RTE). Read More

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October 2021

A human multi-lineage hepatic organoid model for liver fibrosis.

Nat Commun 2021 10 22;12(1):6138. Epub 2021 Oct 22.

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. Read More

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October 2021

A case of colonoscopy-induced Wunderlich's syndrome in a hemodialysis patient: coincidence or association?

J Bras Nefrol 2021 Sep 13. Epub 2021 Sep 13.

Notre Dame Maritime Hospital, Department of Urology, Byblos, Lebanon.

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. Read More

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September 2021

Renal artery embolization for spontaneous hemorrhage in patients with acquired cystic kidney disease: A 20-year single-center experience.

Pak J Med Sci 2021 Jul-Aug;37(4):1111-1117

Hee Ho Chu, MD. Department of Radiology and Research Institute of Radiology, Asan Medical Center, Seoul, Korea.

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. Read More

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December 2020

An intracapsular nephrectomy for the acquired cystic disease-associated renal cell carcinoma in renal transplant allograft: A clinical case report.

Medicine (Baltimore) 2021 May;100(19):e25858

Department of Urology.

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. Read More

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Comparable survival outcome between acquired cystic disease associated renal cell carcinoma and clear cell carcinoma in patients with end-stage renal disease: a multi-institutional central pathology study.

Pathology 2021 Oct 2;53(6):720-727. Epub 2021 May 2.

Department of Surgical Pathology, Aichi Medical University Hospital, Nagakute, Japan.

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. Read More

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October 2021

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

Kidney Int 2021 09 30;100(3):650-659. Epub 2021 Apr 30.

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.

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. Read More

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September 2021

Renal Cell Carcinoma and Kidney Transplantation: A Narrative Review.

Transplantation 2022 01;106(1):e52-e63

Department of Transplantation Medicine, Oslo University Hospital, Oslo, Norway.

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. Read More

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January 2022

Spontaneous renal hemorrhage in acquired cystic kidney disease.

J Gen Fam Med 2021 Mar 24;22(2):94-95. Epub 2020 Nov 24.

Division of Regeneration and Medicine Medical Center for Translational and Clinical Research Hiroshima University Hospital Hiroshima Japan.

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. Read More

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Renal Cell Carcinoma on the Native Kidney Following Kidney Transplantation.

Transplant Proc 2021 May 11;53(4):1268-1271. Epub 2021 Mar 11.

The Second Department of Internal Medicine, University of Toyama, Toyama, Japan.

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. Read More

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New developments in existing WHO entities and evolving molecular concepts: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.

Mod Pathol 2021 07 4;34(7):1392-1424. Epub 2021 Mar 4.

Department of Pathology MD Anderson Cancer Center, Houston, TX, USA.

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. Read More

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Differential diagnosis of perinatal Bartter, Bartter and Gitelman syndromes.

Clin Kidney J 2021 Jan 25;14(1):36-48. Epub 2020 Oct 25.

Department of Pediatrics, Kings County Hospital, Brooklyn, NY, USA.

The common finding of hypokalemic alkalosis in several unrelated disorders may confound the early diagnosis of salt-losing tubulopathy (SLT). Antenatal Bartter syndrome (BS) must be considered in idiopathic early-onset polyhydramnios. Fetal megabladder in BS may allow its distinction from third-trimester polyhydramnios that occurs in congenital chloride diarrhea (CCD). Read More

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January 2021

Distal Tubular Hyperplasia: A Proposal for a Unique Form of Renal Tubular Proliferation Distinct From Papillary Adenoma.

Am J Surg Pathol 2021 04;45(4):516-522

Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN.

We identified an unusual pattern of renal tubular proliferation associated with chronic renal disease, found in 23 patients, diffusely (n=12), or focally (n=11). Incidence was 5% of end-stage renal disease kidneys from one institution (8/177) and 7/23 patients with acquired cystic kidney disease-associated renal cell carcinoma from another. Most (19 patients) had 1 or more neoplasms including papillary (n=9), acquired cystic kidney disease (n=8), clear cell (n=4), or clear cell papillary (n=3) renal cell carcinoma. Read More

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Histomorphology and Immunohistochemistry of a Congenital Nephromegaly Demonstrate Concurrent Features of Heritable and Acquired Cystic Nephropathies in a Girgentana Goat ().

Case Rep Vet Med 2021 19;2021:8749158. Epub 2021 Jan 19.

Center of Clinical Veterinary Medicine, Institute for Veterinary Pathology, Ludwig-Maximilians-University, Veterinärstraße 13, Munich 80539, Germany.

Polycystic kidney diseases (PKD) represent frequent congenital and adult nephropathies in humans and domestic animals. This report illustrates an uncommon state of congenital PKD in a girgentana goat (). A stillborn female goat kid was submitted for postmortem examination and underwent macroscopic and microscopic examination. Read More

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January 2021

Paneth-like cells in renal cell carcinomas and in cysts associated with acquired cystic kidney disease: Clinicopathologic analysis, comparative study and description of precursor lesions.

Ann Diagn Pathol 2021 Apr 23;51:151707. Epub 2021 Jan 23.

Department of Pathology, Charles University in Prague, Faculty of Medicine in Pilsen and University Hospital Pilsen, Czech Republic. Electronic address:

Paneth-like cells (PLCs) are different from Paneth cells (PCs) and contain Paneth-like granules, which have been reported in non-neoplastic conditions and in neoplasms of various organs. PLCs have been reported in clear cell renal cell carcinoma (CCRCC), but not in non-CCRCC, including acquired cystic disease-associated renal cell carcinoma (ACD-RCC). We analyzed clinicopathological features of 24 acquired cystic disease-associated renal cell carcinoma (ACD-RCC) with PLCs (ACD-RCCP+) and compared with those of 23 ACD-RCCs without PLCs (ACD-RCCP-). Read More

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[Renal cell carcinoma in candidates for renal transplantation and recipients of a kidney transplant: The French guidelines from CTAFU].

Prog Urol 2021 Jan;31(1):18-23

Comité de transplantation et d'insuffisance rénale chronique de l'association française d'urologie (CTAFU), maison de l'urologie, 11, rue Viète, 75017 Paris, France; Inserm, équipe labellisée par la ligue contre le cancer, université de Paris, PARCC, 56, rue Leblanc, 75015 Paris, France; Service d'urologie et transplantation rénale, hôpital européen Georges-Pompidou, hôpital Necker, Assistance publique-Hôpitaux de Paris, 20, rue Leblanc, 75015 Paris, France. Electronic address:

Objective: To define guidelines for the management of renal cell carcinoma of the native kidney (NKRCC) in kidney transplant (KTx) recipients and renal cell carcinoma (RCC) in end-stage renal disease (ESRD) patients candidates for renal transplantation.

Method: A review of the literature following a systematic approach (Medline) was conducted by the CTAFU to report renal cell carcinoma epidemiology, screening, diagnosis and management in KTx candidates and recipients. References were assessed according to a predefined process to propose recommendations with the corresponding levels of evidence. Read More

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January 2021

Genetic identification of inherited cystic kidney diseases for implementing precision medicine: a study protocol for a 3-year prospective multicenter cohort study.

BMC Nephrol 2021 01 6;22(1). Epub 2021 Jan 6.

Department of Internal Medicine, Seoul National University College of Medicine, Seoul, South Korea.

Background: Inherited cystic kidney disease is a spectrum of disorders in which clusters of renal cysts develop as the result of genetic mutation. The exact methods and pipelines for defining genetic mutations of inherited cystic kidney disease are not clear at this point. This 3-year, prospective, multicenter, cohort study was designed to set up a cohort of Korean patients with inherited cystic kidney disease, establish a customized genetic analysis pipeline for each disease subtype, and identify modifying genes associated with the severity of the disease phenotype. Read More

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January 2021

The Relationship Between Simple Renal Cysts and Renal Function in Patients With Type 2 Diabetes.

Front Physiol 2020 15;11:616167. Epub 2020 Dec 15.

Hunan Key Laboratory of Kidney Disease and Blood Purification, Department of Nephrology, The Second Xiangya Hospital, Central South University, Changsha, China.

: Simple renal cysts (SRCs) are the most common acquired cystic kidney disease, but the relationship between SRCs and renal function has not been clarified in patients with type 2 diabetes mellitus (T2DM). : A retrospective study was conducted to analyze the clinical features of renal cysts and ultrasound data of the kidney in 4,304 patients with T2DM. : The prevalence of SRCs in patients with T2DM was 21. Read More

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December 2020

Reverse Phenotyping Maternal Cystic Kidney Disease by Diagnosis in a Newborn: Case Report and Literature Review on Neonatal Cystic Kidney Diseases.

Acta Med Litu 2021 2;28(2):308-316. Epub 2021 Aug 2.

Faculty of Medicine, Vilnius University, Lithuania Vilnius University Hospital Santaros Klinikos.

Kidney cysts are the most common kidney lesion, while congenital kidney cysts are mostly found in pediatric population. Neonatal kidney cysts can develop due to fetal malformations, rare genetic disorders or can be acquired which is very rare. Kidney cysts may be the only isolated finding or be part of the overall phenotype. Read More

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[Atypical renal cysts: a clinicopathological and molecular analysis of six cases].

Zhonghua Bing Li Xue Za Zhi 2020 Dec;49(12):1249-1254

Department of Pathology, Zhejiang Provincial People's Hospital, People's Hospital of Hangzhou Medical College, Hangzhou 310014, China.

To investigate the clinicopathological characteristics and molecular genetics of atypical renal cysts. Six cases of atypical renal cysts were collected from Zhejiang Provincial People's Hospital, Hangzhou, China, between February 2014 and February 2019. The clinicopathological characteristics and disease progression were analyzed. Read More

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December 2020

Developmental Renal Glomerular Defects at the Origin of Glomerulocystic Disease.

Cell Rep 2020 10;33(4):108304

Université de Paris, Institut Necker-Enfants Malades (INEM), Epigenetics and Development Team, INSERM U1151, CNRS UMR 8253, 75015 Paris, France. Electronic address:

The architecture of renal glomeruli is acquired through intricate and still poorly understood developmental steps. In our study we identify a crucial glomerular morphogenetic event in nephrogenesis that drives the remodeling/separation of the prospective vascular pole (the future entrance of the glomerular arterioles) and the urinary pole (the tubular outflow). We demonstrate that this remodeling is genetically programmed. Read More

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October 2020

Acquired Cystic Disease-Associated Renal Cell Carcinoma Extending to the Renal Pelvis Mimicking Urothelial Carcinoma on Computed Tomography (CT): Two Case Reports.

Am J Case Rep 2020 Oct 19;21:e926630. Epub 2020 Oct 19.

Department of Radiology, National Defense Medical College, Tokorozawa, Saitama, Japan.

BACKGROUND Acquired cystic disease-associated renal cell carcinoma (ACD-associated RCC), which was added to the 2016 World Health Organization classification, is the most common subtype of RCC in patients undergoing long-term dialysis. ACD-associated RCC is underrecognized and reports of computed tomography (CT) and magnetic resonance imaging findings for the lesion are sparse. Similar to urothelial carcinoma, ACD-associated RCC is poorly to slightly enhanced on dynamic CT. Read More

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October 2020