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Chronic kidney polycystic

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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
#1
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...
November 24, 2016: Journal of Vascular Research
https://www.readbyqxmd.com/read/27798096/chronic-pancreatitis-and-lipomatosis-are-associated-with-defective-function-of-ciliary-genes-in-pancreatic-ductal-cells
#2
Cécile Augereau, Louis Collet, Pierfrancesco Vargiu, Carmen Guerra, Sagrario Ortega, Frédéric P Lemaigre, Patrick Jacquemin
Genetic diseases associated with defects in primary cilia are classified as ciliopathies. Pancreatic lesions and ductal cysts are found in patients with ciliopathic polycystic kidney diseases suggesting a close connection between pancreatic defects and primary cilia. Here we investigate the role of two genes whose deletion is known to cause primary cilium defects, namely Hnf6 and Lkb1, in pancreatic ductal homeostasis. We find that mice with postnatal duct-specific deletion of Hnf6 or Lkb1 show duct dilations...
October 7, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/27776979/chronic-kidney-disease-impairs-renal-nerve-and-haemodynamic-reflex-responses-to-vagal-afferent-input-through-a-central-mechanism
#3
Ibrahim M Salman, Cara M Hildreth, Jacqueline K Phillips
We investigated age- and sex-related changes in reflex renal sympathetic nerve activity (RSNA) and haemodynamic responses to vagal afferent stimulation in a rodent model of chronic kidney disease (CKD). Using anaesthetised juvenile (7-8weeks) and adult (12-13weeks) Lewis Polycystic Kidney (LPK) and Lewis control rats of either sex (n=63 total), reflex changes in RSNA, heart rate (HR) and mean arterial pressure (MAP) to vagal afferent stimulation (5-s train, 4.0V, 2.0-ms pulses, 1-16Hz) were measured. In all groups, stimulation of the vagal afferents below 16Hz produced frequency-dependent reductions in RSNA, HR and MAP, while a 16Hz stimulus produced an initial sympathoinhibition followed by sympathoexcitation...
October 11, 2016: Autonomic Neuroscience: Basic & Clinical
https://www.readbyqxmd.com/read/27766197/uremic-lung-the-calcified-cauliflower-sign-in-the-end-stage-renal-disease
#4
Giuseppe Dilorenzo, Michele Telegrafo, Giuseppe Marano, Michele De Ceglie, Amato Antonio Stabile Ianora, Giuseppe Angelelli, Marco Moschetta
Metastatic pulmonary calcification (MPC) is a rare pathological condition consisting of lung calcium salt deposits which commonly occurs in patients affected by chronic kidney disease probably for some abnormalities in calcium and phosphate metabolism. CT represents the technique of choice for detecting MPC findings including ground glass opacities and partially calcified nodules or consolidations. We present a case of MCP in a patient affected by hepato-renal autosomic-dominant polycystic disease; chest CT revealed extensive lobar-segmental parenchymal calcification with a peculiar cauliflower shape which we called "calcified cauliflower" sign...
2016: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27761278/autosomal-dominant-polycystic-disease-is-associated-with-depressed-levels-of-soluble-tumor-necrosis-factor-related-apoptosis-inducing-ligand
#5
Funda Sarı, Arzu Didem Yalçın, Gizem Esra Genç, Metin Sarıkaya, Atıl Bisgin, Ramazan Çetinkaya, Saadet Gümüşlü
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by multiple, large renal cysts and impaired kidney function. Although the reason for the development of kidney cysts is unknown, ADPKD is associated with cell cycle arrest and abundant apoptosis of renal tubular epithelial cells. AIMS: We asked whether serum-soluble TNF-related apoptosis-inducing ligand (sTRAIL) might underlie ADPKD. STUDY DESIGN: Case-control study...
September 2016: Balkan Medical Journal
https://www.readbyqxmd.com/read/27748299/influence-of-angiotensin-converting-enzyme-ace-gene-rs4362-polymorphism-on-the-progression-of-kidney-failure-in-patients-with-autosomal-dominant-polycystic-kidney-disease-adpkd
#6
Gnanasambandan Ramanathan, Santu Ghosh, Ramprasad Elumalai, Soundararajan Periyasamy, Bhaskar V K S Lakkakula
BACKGROUND & OBJECTIVES: Autosomal dominant polycystic kidney disease (ADPKD) is an inherited systemic disorder, characterized by the fluid filled cysts in the kidneys leading to end stage renal failure in later years of life. Hypertension is one of the major factors independently contributing to the chronic kidney disease (CKD) progression. The renin-angiotensin aldosterone system (RAAS) genes have been extensively studied as hypertension candidate genes. The aim of the present study was to investigate the role of angiotensin converting enzyme tagging - single nucleotide polymorphisms (ACE tag-SNPs) in progression of CKD in patients with ADPKD...
June 2016: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/27733443/high-serum-soluble-%C3%AE-klotho-levels-in-patients-with-autosomal-dominant-polycystic-kidney-disease
#7
Funda Sari, Ayca Inci, Suleyman Dolu, Hamit Yasar Ellidag, Ramazan Cetinkaya, Fettah Fevzi Ersoy
This study aims to determine fibroblast growth factor-23 and soluble α-Klotho levels in patients with autosomal dominant polycystic kidney disease. A total of 76 patients with autosomal dominant polycystic kidney disease and 32 healthy volunteers were included in the study. Serum fibroblast growth factor-23 and soluble α-Klotho levels were measured with ELISA kits. Parathyroid hormone, phosphate, calcium, creatinine, 25-hydroxyvitamin D3 levels, urinary protein to creatinine ratio and estimated glomerular filtration rate were also measured or calculated...
October 12, 2016: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
https://www.readbyqxmd.com/read/27733241/chronic-kidney-disease-in-the-neonate-etiologies-management-and%C3%A2-outcomes
#8
Jason Misurac
Neonatal chronic kidney disease (CKD) occurs with an estimated incidence of 1 in 10,000 live births, whereas the incidence of neonatal end-stage renal disease (ESRD) is about 7.1 per million age-related population. The most frequent etiologies are renal hypoplasia/dysplasia, posterior urethral valves, and other congenital anomalies of the kidney and urinary tract. Other etiologies include polycystic kidney disease, cortical necrosis, and renal vascular thrombosis. Management of CKD focuses primarily on replacing renal functions such as erythropoietin, 1,25-hydroxylation of vitamin D, electrolyte homeostasis/excretion, and, in ESRD, waste product removal...
October 9, 2016: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/27704174/cftr-pharmacology
#9
Olga Zegarra-Moran, Luis J V Galietta
CFTR protein is an ion channel regulated by cAMP-dependent phosphorylation and expressed in many types of epithelial cells. CFTR-mediated chloride and bicarbonate secretion play an important role in the respiratory and gastrointestinal systems. Pharmacological modulators of CFTR represent promising drugs for a variety of diseases. In particular, correctors and potentiators may restore the activity of CFTR in cystic fibrosis patients. Potentiators are also potentially useful to improve mucociliary clearance in patients with chronic obstructive pulmonary disease...
October 4, 2016: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/27703559/risk-factors-of-end-stage-renal-disease-in-peshawar-pakistan-odds-ratio-analysis
#10
Salahuddin Khan, Tariq Hussain, Najma Salahuddin, Salahuddin Mehreen
AIM: The basic aim of this study was to discover the association of End Stage Renal Disease (ESRD) with various risk factors. End Stage Renal Failure is the last stage of the chronic renal failure in which kidneys become completely fail to function. MATERIALS AND METHODS: The data were collected from the patients of renal diseases from three major hospitals in Peshawar, Pakistan. Odds ratio analysis was performed to examine the relationship of ESRD (a binary response variable) with various risk factors: Gender, Diabetic, Hypertension, Glomerulonephritis, Obstructive Nephropathy, Polycystic kidney disease, Myeloma, SLE Nephritis, Heredity, Hepatitis, Excess use of Drugs, heart problem and Anemia...
September 15, 2016: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/27689416/attitudes-in-patients-with-autosomal-dominant-polycystic-kidney-disease-toward-prenatal-diagnosis-and-preimplantation-genetic-diagnosis
#11
Oscar Swift, Enric Vilar, Belinda Rahman, Lucy Side, Daniel P Gale
AIMS: No recommendations currently exist regarding implementation of both prenatal diagnosis and preimplantation genetic diagnosis (PGD) for autosomal dominant polycystic kidney disease (ADPKD). This study evaluated attitudes in ADPKD patients with either chronic kidney disease (CKD) stages I-IV or end-stage renal failure (ESRF) toward prenatal diagnosis and PGD. METHODS: Ninety-six ADPKD patients were recruited from an outpatient clinic, wards, and dialysis units...
September 30, 2016: Genetic Testing and Molecular Biomarkers
https://www.readbyqxmd.com/read/27683096/sudden-onset-of-parathyroid-hormone-independent-severe-hypercalcemia-from-reversal-of-tumoral-calcinosis-in-a-dialysis-patient
#12
Fareed B Kamar, Bikaramjit Mann, Gregory Kline
BACKGROUND: Tumoral calcinosis is a rare manifestation of extraskeletal calcification, featuring large calcified cystic masses in the periarticular regions of large joints. In chronic kidney disease (CKD), this disorder is thought to evolve through a chronically elevated calcium-phosphorus solubility product leading to calcium precipitation in soft tissue. Treating tumoral calcinosis in these patients involves interventions to lower the calcium-phosphorus product such as reduction in vitamin D therapy and intensive hemodialysis regimens...
September 29, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27662885/health-related-quality-of-life-across-all-stages-of-autosomal-dominant-polycystic-kidney-disease
#13
Daniel Eriksson, Linda Karlsson, Oskar Eklund, Hans Dieperink, Eero Honkanen, Jan Melin, Kristian Selvig, Johan Lundberg
BACKGROUND: A limited number of studies have assessed health-related quality of life (HRQoL) in autosomal dominant polycystic kidney disease (ADPKD). Results to date have been conflicting and studies have generally focused on patients with later stages of the disease. This study aimed to assess HRQoL in ADPKD across all stages of the disease, from patients with early chronic kidney disease (CKD) to patients with end-stage renal disease. METHODS: A study involving cross-sectional patient-reported outcomes and retrospective clinical data was undertaken April-December 2014 in Denmark, Finland, Norway and Sweden...
September 23, 2016: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/27651963/an-atypical-presentation-of-a-male-with-oral-facial-digital-syndrome-type-1-related-ciliopathy
#14
Sheena Sharma, Jennifer M Kalish, Ethan M Goldberg, Francis Jeshira Reynoso, Madhura Pradhan
Background. Oral-facial-digital syndrome type 1 (OFD1) is a rare condition with X-linked dominant inheritance caused by mutations in the Cxorf5 (OFD1) gene. This gene encodes the OFD1 protein located within centrosomes and basal bodies of primary cilia. Approximately 15-50% of patients with OFD1 progress to end-stage kidney disease following development of polycystic changes within the kidneys. This condition almost always causes intrauterine lethality in males. Description of Case Diagnosis and Treatment. A Caucasian male aged 9 years and 9 months presented with increased urinary frequency, increased thirst, and decreased appetite...
2016: Case Reports in Nephrology
https://www.readbyqxmd.com/read/27642786/-autosomal-polycystic-kidney-disease
#15
Frank Strutz
Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenetic kidney disease. The clinical course is highly variable, association to certain genetic mutations only weak. Great progress has been made in recent years in determining the pathophysiology of the disease. Diagnosis of ADPKD is almost always possible by ultrasound, genetic examination is confined to selected cases. In addition, imaging is important for prognosis where MRI and computer tomography are superior for determination of total kidney volume...
September 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27627216/nephroprotective-effects-of-metformin-in-diabetic-nephropathy
#16
Sreenithya Ravindran, Vinitha Kuruvilla, Kerry Wilbur, Shankar Munusamy
Metformin, a well-known anti-diabetic agent, is very effective in lowering blood glucose in patients with type 2 diabetes with minimal side-effects. Metformin is also being recommended in the treatment of obesity and polycystic ovary syndrome. Metformin elicits its therapeutic effects mainly via activation of AMP-activated kinase (AMPK) pathway. Renal cells under hyperglycemic or proteinuric conditions exhibit inactivation of cell defense mechanisms such as AMPK and autophagy, and activation of pathologic pathways such as mammalian target of rapamycin (mTOR), endoplasmic reticulum (ER) stress, epithelial-to-mesenchymal transition (EMT), oxidative stress, and hypoxia...
September 14, 2016: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/27559537/growth-in-children-with-autosomal-recessive-polycystic-kidney-disease-in-the-ckid-cohort-study
#17
Erum A Hartung, Katherine M Dell, Matthew Matheson, Bradley A Warady, Susan L Furth
BACKGROUND: Previous studies have suggested that some children with autosomal recessive polycystic kidney disease (ARPKD) have growth impairment out of proportion to their degree of chronic kidney disease (CKD). The objective of this study was to systematically compare growth parameters in children with ARPKD to those with other congenital causes of CKD in the chronic kidney disease in Children (CKiD) prospective cohort study. METHODS: Height SD scores (z-scores), proportion of children with severe short stature (z-score < -1...
2016: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/27550645/risk-of-cancer-in-patients-with-polycystic-kidney-disease-a-propensity-score-matched-analysis-of-a-nationwide-population-based-cohort-study
#18
Tung-Min Yu, Ya-Wen Chuang, Mei-Ching Yu, Cheng-Hsu Chen, Cheng-Kuang Yang, Shih-Ting Huang, Cheng-Li Lin, Kuo-Hsiung Shu, Chia-Hung Kao
BACKGROUND: Data for the risk of any solid cancer in patients with polycystic kidney disease are scarce. Therefore, we did a nationwide cohort study in Taiwan to establish the risk of cancer in patients with polycystic kidney disease without either chronic kidney disease or end-stage renal disease. METHODS: From inpatient claims of the Taiwan National Health Insurance Research Database, we included patients aged 20 years and older and diagnosed with polycystic kidney disease between January, 1998 and December, 2010, in the polycystic kidney disease cohort...
October 2016: Lancet Oncology
https://www.readbyqxmd.com/read/27538311/-cardiorenal-syndromes-definition-and-classification
#19
Bruno Moulin
Cardiorenal syndromes refer to clinical and metabolic consequences of acute and chronic heart failure or kidney disease on other organ. Recent studies have further clarified the pathophysiological mechanisms behind the different types of cardiorenal syndromes and propose a new classification. The cardiorenal syndrome type 1 corresponds to an acute heart failure (cardiogenic shock, acute decompensated congestive heart failure) which induces acute renal dysfunction. In the cardiorenal syndrome type 2 heart failure is chronic (congestive heart failure) and induces chronic kidney damages in the long-term...
June 2016: La Revue du Praticien
https://www.readbyqxmd.com/read/27525098/a-kidney-shaped-polycystic-mass-on-the-back-of-a-hemodialysis-patient
#20
Julia Volkmann, Johannes Nordlohne, Roland Schmitt, Sibylle von Vietinghoff
Multicystic back masses can be of infectious, metastatic, or local pre- or malignant origin. We present a case of a rapidly evolving mass in a hemodialysis patient with severe "chronic kidney disease-associated mineral bone disease" (CKD-MBD), that also highlights limitations of chest x-ray for diagnosis of bone disease.
August 2016: Clinical Case Reports
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