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https://www.readbyqxmd.com/read/28077374/parallel-microarray-profiling-identifies-erbb4-as-a-determinant-of-cyst-growth-in-adpkd-and-a-prognostic-biomarker-for-disease-progression
#1
Andrew J Streets, Tajdida A Magayr, Linghong Huang, Laura Vergoz, Sandro Rossetti, Roslyn J Simms, Peter C Harris, Dorien J M Peters, Albert Cm Ong
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the fourth most common cause of end-stage renal disease. The disease course can be highly variable and treatment options are limited. To identify new therapeutic targets and prognostic biomarkers of disease, we conducted parallel discovery microarray profiling in normal and diseased human PKD1 cystic kidney cells. A total of 1515 genes and 5 miRNA were differentially expressed by more than two-fold in PKD1 cells. Functional enrichment analysis identified 30 dysregulated signalling pathways including the epidermal growth factor (EGF) receptor pathway...
January 11, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28050435/mucinous-cystadenocarcinoma-in-a-horse-shoe-kidney-masquerading-as-giant-hydronephrosis-a-case-report-diagnostic-challenges-lessons-learnt-and-review-of-literature
#2
Leena Dennis Joseph, Sriram Krishnamoorthy, Rajendiran Swaminathan, Gokul Kripesh, Hariharasudhan Sekar
Primary epithelial tumour of the renal pelvis is a rare entity. So far, very few cases are reported in literature. Of these, mucin secreting adenocarcinomas are distinctly rare. However, mucinous cystadenocarcinoma, that too arising in a horse shoe kidney is extremely rare to be reported. Mucinous cystadenomas and carcinomas arising from the renal pelvis are regarded to be secondary to a metaplastic change in the urothelium. Here, we present a case of a 51-year-old male, who complained of abdominal pain, palpable abdominal mass, haematuria along with passage of mucoid material in the urine, with a very poorly functioning kidney...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28050373/histopathologic-findings-in-autopsies-with-emphasis-on-interesting-and-incidental-findings-a-pathologist-s-perspective
#3
Sapna Patel, B R Rajalakshmi, G V Manjunath
INTRODUCTION: Autopsy aids to the knowledge of pathology by unveiling the rare lesions which are a source of learning from a pathologist's perspective Some of them are only diagnosed at autopsy as they do not cause any functional derangement. This study emphasizes the various incidental lesions which otherwise would have been unnoticed during a person's life. AIM: The aim of this study was to determine the spectrum of histopathological findings including neoplastic lesions related or unrelated to the cause of death...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28042792/-cystic-nephroma-report-of-two-cases-and-bibliographic-review
#4
E Rodríguez-Zarco, S Pabon-Carrasco, C Romero-Abrio, F J Vázquez-Ramírez
OBJECTIVE: Multicystic nephroma (multilocular cystic nephroma, multilocular cyst) is a relatively rare benign neoplasm of the kidney. Most patients are asymptomatic and tumours are usually discovered incidentally. METHODS: Between 2010 and 2015, 2 patients with cystic nephroma at our institution were diagnosed and treated. Our study includes two new cases of cystic nephroma and a review of the literature about the differential diagnosis of a cystic renal mass. RESULTS: In this report we present two cases of multilocular cystic nephroma in a 75-year-old-female and a 33-year-old female...
December 2016: Archivos Españoles de Urología
https://www.readbyqxmd.com/read/28032311/prognostic-factors-for-renal-cell-carcinoma-subtypes-diagnosed-according-to-the-2016-who-renal-tumor-classification-a-study-involving-928-patients
#5
Levente Kuthi, Alex Jenei, Adrienn Hajdu, István Németh, Zoltán Varga, Zoltán Bajory, László Pajor, Béla Iványi
The morphotype and grade of renal cell carcinoma (RCC) in 928 nephrectomies were reclassified according to the 2016 WHO classification in order to analyze the distribution and outcomes of RCC subtypes in Hungary, to assess whether microscopic tumor necrosis is an independent prognostic factor in clear cell RCC, and to study whether a two-tiered grading (low/high) for clear cell and papillary RCC provides similar prognostic information to that of the four-tiered ISUP grading system. 83.4% of the cohort were clear cell, 6...
December 28, 2016: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28009031/contemporary-national-trends-of-cystic-fibrosis-hospitalizations-and-co-morbidities-in-the-united-states
#6
Kshitij Chatterjee, Abhinav Goyal, Nishi Shah, Krishna Kakkera, Rajani Jagana, Paula Anderson
INTRODUCTION: Cystic fibrosis (CF) is a life-limiting multisystemic genetic disease. Patients with CF have a high rate of hospitalization. We attempt to ascertain national trends of inpatient stays, prevalence of various co-morbidities during hospitalizations, outcomes and discharge disposition among CF patients. MATERIAL AND METHODS: Data from the National Inpatient Sample (NIS) was used to identify all hospitalizations of patients with CF and their demographic characteristics from 2003 to 2013...
2016: Adv Respir Med
https://www.readbyqxmd.com/read/28007907/h255y-and-k508r-missense-mutations-in-tumour-suppressor-folliculin-flcn-promote-kidney-cell-proliferation
#7
Hisashi Hasumi, Yukiko Hasumi, Masaya Baba, Hafumi Nishi, Mitsuko Furuya, Cathy D Vocke, Martin Lang, Nobuko Irie, Chiharu Esumi, Maria J Merino, Takashi Kawahara, Yasuhiro Isono, Kazuhide Makiyama, Andrew C Warner, Diana C Haines, Ming-Hui Wei, Berton Zbar, Herbert Hagenau, Lionel Feigenbaum, Keiichi Kondo, Noboru Nakaigawa, Masahiro Yao, Adam R Metwalli, W Marston Linehan, Laura S Schmidt
Germline H255Y and K508R missense mutations in the folliculin (FLCN) gene have been identified in patients with bilateral multifocal (BMF) kidney tumours and clinical manifestations of Birt-Hogg-Dubé (BHD) syndrome, or with BMF kidney tumours as the only manifestation; however, their impact on FLCN function remains to be determined. In order to determine if FLCN H255Y and K508R missense mutations promote aberrant kidney cell proliferation leading to pathogenicity, we generated mouse models expressing these mutants using BAC recombineering technology and investigated their ability to rescue the multi-cystic phenotype of Flcn-deficient mouse kidneys...
December 22, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/28007903/autophagy-activators-suppress-cystogenesis-in-an-autosomal-dominant-polycystic-kidney-disease-model
#8
Ping Zhu, Cynthia J Sieben, Xiaolei Xu, Peter C Harris, Xueying Lin
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in either PKD1 or PKD2 It is one of the most common heritable human diseases with eventual development of renal failure; however, effective treatment is lacking. While inhibition of mechanistic target of rapamycin (mTOR) effectively slows cyst expansions in animal models, results from clinical studies are controversial, prompting further mechanistic studies of mTOR-based therapy. Here, we aim to establish autophagy, a downstream pathway of mTOR, as a new therapeutic target for PKD...
December 22, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/27999716/primary-cerebral-echinoccocosis-in-a-child-case-report-surgical-technique-technical-pitfalls-and-video-atlas
#9
Ahmed M A Altibi, Raed A H Qarajeh, Telmo A B Belsuzarri, Walid Maani, Tareq M A Kanaan
BACKGROUND: Hydatid disease is a life-threatening parasitic infestation caused by Echinococcus granulosus. Infection with E. granulosus typically results in the formation of hydatid cysts in the liver, lungs, kidney, and spleen. Primary intracranial hydatid cyst disease is extremely rare. Here, we are reporting an unusual case of Echinococcus, where the only identifiable lesion was a hydatid cyst in the brain without liver or lung involvement. We are also providing a description for the surgical technique used to remove the cyst, highlighting the possible surgical pitfalls...
2016: Surgical Neurology International
https://www.readbyqxmd.com/read/27986536/unusual-cystic-mass-of-retrovesical-space-a-retrovesical-kidney
#10
Youness El Harrech, Driss Touiti
Retrovesical cysts are very rare, with more reported cases being hydatid or seminal cyst. To our knowledge, no case of pelvic kidney revealed as cystic mass of the retrovesical space has been reported. We provide images of a direct retrovesical kidney in a 24-year-old patient who presented with lower urinary tract symptoms.
December 13, 2016: Urology
https://www.readbyqxmd.com/read/27940671/case-report-cystinuria-and-polycystic-kidney-disease
#11
Ajaydeep Sidhu, Angela Mittal, Xamayta Negroni-Balasquide, Alex Constantinescu, Kristin Kozakowski
Cystinuria and polycystic kidney disease are 2 genetic disorders that affect the genitourinary tract but rarely together. This case report presents 2 pediatric patients diagnosed with polycystic kidney disease and cystinuria requiring surgical treatment. Both subjects presented acutely with stone disease. Imaging studies and stone analysis established the diagnoses. Although coexistence of these 2 conditions is rare, cystinuria should be considered in the differential diagnosis when evaluating patients with cystic disease who develop renal calculi...
December 2016: Pediatrics
https://www.readbyqxmd.com/read/27936068/correction-an-empirical-biomarker-based-calculator-for-cystic-index-in-a-model-of-autosomal-recessive-polycystic-kidney-disease-the-nieto-narayan-formula
#12
(no author information available yet)
[This corrects the article DOI: 10.1371/journal.pone.0163063.].
2016: PloS One
https://www.readbyqxmd.com/read/27921040/new-insights-into-the-molecular-mechanisms-targeting-tubular-channels-transporters-in-pkd-development
#13
REVIEW
Ming Wu, Shengqiang Yu
BACKGROUND: Autosomal dominant polycystic kidney disease (PKD) or autosomal recessive PKD is caused by a mutation in the PKD1, PKD2 or PKHD1 gene, which encodes polycystin-1, polycystin-2 or fibrocystin, respectively. Embryonic and postnatal mutation studies show that transport or channel function is dysregulated before the initiation of cystogenesis, suggesting that the abnormality of transport or channel function plays a critical role in the pathology of PKD. SUMMARY: Polycystin-2 by itself is a calcium-permeable cation channel, and its channel function can be regulated by polycystin-1 or fibrocystin...
October 2016: Kidney Diseases
https://www.readbyqxmd.com/read/27921039/clinical-manifestation-and-management-of-adpkd-in-western-countries
#14
REVIEW
Claudia Sommerer, Martin Zeier
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease in Western countries. The prevalence is between 2.4/10,000 and 3.9/10,000. ADPKD represents a systemic disease resulting in deterioration in renal function. Until now, mutations in two genes (PKD1 and PKD2) have been identified. Recently, the European Medicines Agency (EMA) approved the use of the vasopressin V2 receptor antagonist tolvaptan to slow the progression of cyst development and renal insufficiency connected with ADPKD in adult patients with chronic kidney disease stages 1-3 at initiation of treatment with evidence of rapidly progressing disease...
October 2016: Kidney Diseases
https://www.readbyqxmd.com/read/27920423/de-novo-renal-neoplasia-after-kidney-transplantation-according-to-new-2016-who-classification-of-renal-tumors
#15
Albino Eccher, Luigino Boschiero, Brett Delahunt, Luca Cima, Francesca Fior, Francesco Nacchia, Momo Rostand, Amedeo Carraro, Umberto Tedeschi, Gianluigi Zaza, Marilena Casartelli Liviero, Laura Zampicinini, Marco Chilosi, Giuseppe Feltrin, Claudio Rago, Antonietta D'Errico, Claudio Ghimenton, Guido Martignoni, Matteo Brunelli
BACKGROUND De novo renal neoplasia developing after kidney transplantation at Verona Kidney Transplant Center were reviewed according to new 2016 WHO Renal Tumor Classification. MATERIAL AND METHODS Primary renal tumors developed in native or transplanted kidneys de novo following renal transplantation were retrieved and histologically reviewed by three expert uropathologists. Immunoexpression of the diagnostic antigens CD13, CD10, CK7, CK34bE12, AMACR, CAIX, AE1/AE3, CK14, GATA-3, HMB-45, cathepsin-k, S100A1, and parvalbumin was assessed...
December 6, 2016: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/27899951/the-first-case-of-benign-multicystic-mesothelioma-presenting-as-a-splenic-mass
#16
Antonio D'Antonio, Carlo Baldi, Maria Addesso, Carmine Napolitano
Multicystic mesothelioma (MM) is a relatively rare tumour arising in the pelvic peritoneum of the tuboovarian region of young woman. Exceptionally, MM occurs on the serosal surfaces of various organs including kidney, bladder, lymph nodes, and liver. We report here the first case of MM wherein a 58-year-old woman with a previous history of endometriosis of the right ovary presented with a large multicystic mass of the spleen. The diagnosis of MM was made on a surgical specimen after splenectomy. A histopathologic examination is always necessary for the diagnosis of MM which should be differentiated from other lesions particularly from cystic lymphangioma...
2016: Ecancermedicalscience
https://www.readbyqxmd.com/read/27895019/nephrotoxicity-during-vancomycin-therapy-in-combination-with-piperacillin-tazobactam-or-cefepime
#17
W Cliff Rutter, Jessica N Cox, Craig A Martin, Donna R Burgess, David S Burgess
BACKGROUND: Recent reports have demonstrated that vancomycin (VAN) may lead to an increase in acute kidney injury (AKI) when combined with anti-pseudomonal beta-lactams. This study compared the incidence of AKI associated with VAN plus piperacillin-tazobactam (TZP) or cefepime (FEP). METHODS: This was a retrospective, matched cohort study at an academic medical center between September 2010 and September 2014 including adult patients receiving TZP-VAN or FEP-VAN for at least 48 hours and without severe chronic or structural kidney disease, dialysis, pregnancy, cystic fibrosis, or hospital transfer...
November 28, 2016: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/27894621/glomerulocystic-kidney-presenting-as-a-unilateral-kidney-mass-in-a-newborn-with-tuberous-sclerosis-report-of-a-case-and-review-of-the-literature
#18
Miguel Rito, Rafael Adame Cabrera
Glomerular cysts are defined as a 2-3 times dilation of Bowman spaces and their presence in at least 5% of the glomeruli defines the kidneys as glomerulocystic (GCK). The association between cystic kidney disease and the tuberous sclerosis complex (TSC) is well known, but its presentation as a unilateral mass with glomerulocystic pattern is rare. We describe a case of an infant with a prenatal diagnosis of TSC, with a renal mass that was believed to be a renal tumor. A four-month-old infant with maternal history of TSC and prenatally diagnosed subependymal nodules and a right renal mass underwent nephrectomy...
November 18, 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27894620/clinical-implications-of-a-rare-renal-entity-pleomorphic-hyalinizing-angiectatic-tumor-phat
#19
Cristina Scalici Gesolfo, Vincenzo Serretta, Fabrizio Di Maida, Giulio Giannone, Elisabetta Barresi, Vito Franco, Rodolfo Montironi
Pleomorphic Hyalinizing Angiectatic Tumor (PHAT) is a rare benign lesion characterized by slow growth, infiltrative behavior and high rate of local recurrences. Only one case has been described in retroperitoneum, at renal hilum, but not involving pelvis or parenchyma. Here we present the first case of PHAT arising in the renal parenchyma. A nodular lesion in right kidney lower pole was diagnosed to a 61 year old woman. The patient underwent right nephrectomy. Microscopically, the lesion showed solid and pseudo-cystic components with hemorrhagic areas characterized by aggregates of ectatic blood vessels...
November 17, 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27880955/long-term-angiotensin-ii-receptor-blockade-limits-hypertension-aortic-dysfunction-and-structural-remodeling-in-a-rat-model-of-chronic-kidney-disease
#20
Omar Z Ameer, Mark Butlin, Elena Kaschina, Manuela Sommerfeld, Alberto P Avolio, Jacqueline K Phillips
BACKGROUND/AIMS: Chronic kidney disease (CKD) is associated with large artery remodeling, endothelial dysfunction and calcification, with angiotensin II (Ang II) a known driver of these pathologies. We investigated long-term Ang II type 1 receptor inhibition with valsartan on aortic function and structure in the Lewis polycystic kidney (LPK) rat model of CKD. METHODS: Mixed sex LPK and Lewis control (total n = 28) treated (valsartan 60 mg/kg/day p.o. from 4 to 18 weeks) and vehicle groups were studied...
2016: Journal of Vascular Research
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