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polycystic kidney disease article review

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https://www.readbyqxmd.com/read/27862900/long-term-renal-allograft-survival-after-sequential-liver-kidney-transplantation-from-a-single-living-donor
#1
Kumiko Kitajima, Yuichi Ogawa, Katsuyuki Miki, Kotaro Kai, Akihito Sannomiya, Kazuhiro Iwadoh, Toru Murakami, Ichiro Koyama, Ichiro Nakajima, Shohei Fuchinoue
Background Combined liver-kidney transplantation (CLKT) is well-established as a definitive therapy with the potential to provide complete recovery for certain liver-kidney diseases, although the results might be contingent on the cause of transplantation. The purposes of the present study were to review the long-term outcome of renal allografts in CLKT patients from single living donors and to investigate the beneficial factors, compared with solitary renal transplantation. Materials and Methods Thirteen patients underwent sequential liver (LTx) and kidney (KTx) transplantations from single living donors...
November 10, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/27811548/current-recommendations-for-treating-autosomal-dominant-polycystic-kidney-disease
#2
Becky Ness, Kathryn Stovall
Autosomal dominant polycystic kidney disease is the most common inherited kidney disease in the United States, causing hypertension, cerebral aneurysms, nephrolithiasis, and kidney failure. This article reviews the pathogenesis, diagnosis, associated complications, and current treatment recommendations for the condition.
December 2016: JAAPA: Official Journal of the American Academy of Physician Assistants
https://www.readbyqxmd.com/read/27665410/-polycystic-kidney-disease-and-kidney-transplantation
#3
X Tillou, M-O Timsit, F Sallusto, T Culty, G Verhoest, A Doerfler, R Thuret, F Kleinclauss
OBJECTIVES: To perform a state of the art about autosomal dominant polykystic kidney disease (ADPKD), management of its urological complications and end stage renal disease treatment modalities. MATERIAL AND METHODS: An exhaustive systematic review of the scientific literature was performed in the Medline database (http://www.ncbi.nlm.nih.gov) and Embase (http://www.embase.com) using different associations of the following keywords (MESH): "autosomal dominant polykystic kidney disease", "complications", "native nephrectomy", "kidney transplantation"...
September 21, 2016: Progrès en Urologie
https://www.readbyqxmd.com/read/27629138/autosomal-dominant-polycystic-kidney-disease-diagnosed-in-utero-review
#4
Magdalena Nowak, Hubert Huras, Marcin Wiecheć, Robert Jach, Małgorzata Radoń-Pokracka, Joanna Górecka
Autosomal dominant polycystic kidney disease (ADPKD) is one of most common inherited renal diseases. It is estimated that very early onset ADPKD affects even 2% patients. The purpose of this article is to provide a comprehensive review of genetics, prenatal diagnosis and prognosis in very early onset autosomal dominant polycystic kidney disease.
2016: Ginekologia Polska
https://www.readbyqxmd.com/read/27627216/nephroprotective-effects-of-metformin-in-diabetic-nephropathy
#5
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/27067213/-genetics-and-genetic-counseling
#6
Claudia Izzi, Francesca Liut, Nadia Dallera, Cinzia Mazza, Riccardo Magistroni, Gianfranco Savoldi, Francesco Scolari
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most frequent genetic disease, characterized by progressive development of bilateral renal cysts. Two causative genes have been identified: PKD1 and PKD2. ADPKD phenotype is highly variable. Typically, ADPKD is an adult onset disease. However, occasionally, ADPKD manifests as very early onset disease. The phenotypic variability of ADPKD can be explained at three genetic levels: genic, allelic and gene modifier effects. Recent advances in molecular screening for PKD gene mutations and the introduction of the new next generation sequencing (NGS)- based genotyping approach have generated considerable improvement regarding the knowledge of genetic basis of ADPKD...
March 2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/27046073/pancreatic-cysts-in-autosomal-dominant-polycystic-kidney-disease-prevalence-and-association-with-pkd2-gene-mutations
#7
Jin Ah Kim, Jon D Blumenfeld, Shalini Chhabra, Silvina P Dutruel, Nanda Deepa Thimmappa, Warren O Bobb, Stephanie Donahue, Hanna E Rennert, Adrian Y Tan, Ashley E Giambrone, Martin R Prince
Purpose To define the magnetic resonance (MR) imaging prevalence of pancreatic cysts in a cohort of patients with autosomal dominant polycystic kidney disease (ADPKD) compared with a control group without ADPKD that was matched for age, sex, and renal function. Materials and Methods In this HIPAA-compliant, institutional review board-approved study, all patients with ADPKD provided informed consent; for control subjects, informed consent was waived. Patients with ADPKD (n = 110) with mutations identified in PKD1 or PKD2 and control subjects without ADPKD or known pancreatic disease (n = 110) who were matched for age, sex, estimated glomerular filtration rate, and date of MR imaging examination were evaluated for pancreatic cysts by using axial and coronal single-shot fast spin-echo T2-weighted images obtained at 1...
September 2016: Radiology
https://www.readbyqxmd.com/read/26599520/a-systematic-review-to-determine-the-most-effective-interventions-to-increase-water-intake
#8
Teresa X W Chua, Neha S Prasad, Gopala K Rangan, Margaret Allman-Farinelli, Anna M Rangan
BACKGROUND: Maintaining adequate fluid intake has been hypothesized to be beneficial for the progression of chronic kidney disease (CKD). The aim of this study was to undertake a systematic review to determine the most effective interventions to increase water intake. METHODS: Six electronic databases were searched from 1910 until March 2015 in the English language. Additional sources through hand-searches, expert recommendations and reviews were checked. Intervention studies increasing water intake in adults through non-pharmacological methods were eligible for inclusion...
October 2016: Nephrology
https://www.readbyqxmd.com/read/26328773/therapeutic-targets-for-polycystic-kidney-disease
#9
REVIEW
Patricia D Wilson
INTRODUCTION: Polycystic kidney disease (PKD) is a common genetic disease in which renal enlargement and loss of function is caused by progressive expansion of tubular cysts. To reverse the detrimental effects of PKD gene mutation(s) and to slow cystic expansion, new drug therapies are required. AREAS COVERED: The underlying cell biology leading to identification of molecular targets for PKD is reviewed. Specific focus is on studies published at the early pre-clinical level...
2016: Expert Opinion on Therapeutic Targets
https://www.readbyqxmd.com/read/26275819/a-systematic-review-of-the-predictors-of-disease-progression-in-patients-with-autosomal-dominant-polycystic-kidney-disease
#10
REVIEW
Claire Woon, Ashleigh Bielinski-Bradbury, Karl O'Reilly, Paul Robinson
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterised by progressive renal cyst formation leading to renal failure in the majority of patients. The likelihood and rate of ADPKD progression is difficult to predict and there is a clear need to identify prognostic indicators that could be used to anticipate ADPKD progression, to aid the management of patients in clinical practice. METHODS: A systematic literature review was conducted to identify publications detailing the natural history of ADPKD, including diagnosis, prognosis and progression...
2015: BMC Nephrology
https://www.readbyqxmd.com/read/25774748/risk-factors-for-progression-in-adpkd
#11
REVIEW
Ahsan Alam
PURPOSE OF REVIEW: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. This article will describe the factors associated with both functional and structural evidence of disease progression. It will also review the results of recent clinical trials that have shown an impact on markers of disease progression. RECENT FINDINGS: A variety of prognostic factors have been described that relate to a decline in glomerular filtration rate or an increase in total cyst or kidney volumes...
May 2015: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/25634914/sudden-death-of-a-patient-with-polycystic-kidneys-due-to-acute-inferior-vena-cava-thrombosis
#12
Riccardo Tamburrini, Zubir Ahmed, Jon van der Walt, David Goldsmith
A management algorithm for large renal cyst in autosomal dominant polycystic kidney disease (ADPKD) is lacking despite the potential to cause widespread medical and surgical complications. We report the case of a 37-year-old gentleman with ADPKD and large (>5 cm diameter) cysts who suffered sudden death due to autopsy-proven inferior vena cava and pulmonary arterial thrombosis. In this article, we discuss the possible pathophysiological factors at play in this catostrophic complication of ADPKD. We also review available literature to establish the prevalence of such a complication and also establish current thoughts and opinions as to the optimal management strategy for giant cysts in the context of ADPKD...
January 28, 2015: Scottish Medical Journal
https://www.readbyqxmd.com/read/25438190/novel-therapeutic-approaches-to-autosomal-dominant-polycystic-kidney-disease
#13
REVIEW
Wells B LaRiviere, Maria V Irazabal, Vicente E Torres
Autosomal dominant polycystic kidney disease (ADPKD) is an inherited disorder characterized by the progressive growth of renal cysts that, over time, destroy the architecture of the renal parenchyma and typically lead to kidney failure by the sixth decade of life. ADPKD is common and represents a leading cause of renal failure worldwide. Currently, there are no Food and Drug Administration-approved treatments for the disease, and the existing standard of care is primarily supportive in nature. However, significant advances in the understanding of the molecular biology of the disease have inspired investigation into potential new therapies...
April 2015: Translational Research: the Journal of Laboratory and Clinical Medicine
https://www.readbyqxmd.com/read/25155726/neonatal-polycystic-kidney-disease
#14
REVIEW
Priya Verghese, Yosuke Miyashita
This article provides an up-to-date comprehensive review and summary on neonatal polycystic kidney disease (PKD) with emphasis on the differential diagnosis, clinical manifestations, diagnostic techniques, and potential therapeutic approaches for the major causes of neonatal PKD, namely hereditary disease, including autosomal recessive and autosomal dominant PKD and nonhereditary PKD, with particular emphasis on multicystic dysplastic kidney. A brief overview of obstructive cystic dysplasia and simple and complex cysts is also included...
September 2014: Clinics in Perinatology
https://www.readbyqxmd.com/read/24463189/mechanisms-and-management-of-hypertension-in-autosomal-dominant-polycystic-kidney-disease
#15
REVIEW
Frederic Rahbari-Oskoui, Olubunmi Williams, Arlene Chapman
Autosomal dominant polycystic kidney disease (ADPKD) is the most commonly inherited kidney disease, characterized by progressive cyst growth and renal enlargement, resulting in renal failure. Hypertension is common and occurs early, prior to loss of kidney function. Whether hypertension in ADPKD is a primary vasculopathy secondary to mutations in the polycystin genes or secondary to activation of the renin-angiotensin-aldosterone system by cyst expansion and intrarenal ischemia is unclear. Dysregulation of the primary cilium causing endothelial and vascular smooth muscle cell dysfunction is a component of ADPKD...
December 2014: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/24370765/renal-relevant-radiology-radiologic-imaging-in-autosomal-dominant-polycystic-kidney-disease
#16
REVIEW
Frederic Rahbari-Oskoui, Ankush Mittal, Pardeep Mittal, Arlene Chapman
Autosomal-dominant polycystic kidney disease is a systemic disorder and the most common hereditary renal disease, which is characterized by cyst growth, progressive renal enlargement, and development of renal failure. The cystic nature of autosomal dominant polycystic kidney disease and its renal and extrarenal complications (kidney stones, cyst hemorrhage, intracerebral aneurysm, liver cysts, cardiac valve abnormalities, etc.) give radiologic imaging studies a central role in the management of these patients...
February 2014: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/24335972/strategies-targeting-camp-signaling-in-the-treatment-of-polycystic-kidney-disease
#17
REVIEW
Vicente E Torres, Peter C Harris
Polycystic kidney disease (PKD) is a leading cause of ESRD worldwide. In PKD, excessive cell proliferation and fluid secretion, pathogenic interactions of mutated epithelial cells with an abnormal extracellular matrix and alternatively activated interstitial macrophages, and the disruption of mechanisms controlling tubular diameter contribute to cyst formation. Studies with animal models suggest that several diverse pathophysiologic mechanisms, including dysregulation of intracellular calcium levels and cAMP signaling, mediate these cystogenic mechanisms...
January 2014: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/24246705/-treatment-of-autosomal-dominant-polycystic-kidney-disease
#18
REVIEW
Roser Torra
Autosomal dominant polycystic kidney disease is the most frequent hereditary kidney disease. However it lacks a specific treatment. Its prevalence is 1/800 and causes the need for renal replacement therapy in 8-10% of patients on dialysis or kidney transplant. It is caused by mutations in the PKD1 and PKD2 genes, which cause a series of alterations in the polycystic cells, which have become therapeutic targets. There are many molecules that are being tested to counteract the alterations of these therapeutic targets...
January 21, 2014: Medicina Clínica
https://www.readbyqxmd.com/read/24246056/evidence-for-a-role-of-proteins-lipids-and-phytochemicals-in-the-prevention-of-polycystic-kidney-disease-progression-and-severity
#19
REVIEW
Kaitlin H Maditz, Joseph C Gigliotti, Janet C Tou
Polycystic kidney disease (PKD) is a heritable disease characterized by renal cysts and is a leading cause of end-stage renal disease. Dietary intervention offers a potentially efficacious, cost-effective, and safe therapeutic option for PKD. The aim of this article was to review studies investigating the effect of dietary components on PKD and potential mechanisms of action. Low-protein diets are commonly recommended for PKD patients, but inconsistent findings in human and animal PKD studies suggest that the type rather the amount of protein may be of greater importance...
December 2013: Nutrition Reviews
https://www.readbyqxmd.com/read/24099828/renal-transplantation-and-polycystic-surgical-considerations
#20
REVIEW
O Rodríguez-Faba, A Breda, H Villavicencio
BACKGROUND: The indication and timing of nephrectomy in patients with autosomal dominant polycystic kidney disease (ADPKD) remain controversial, especially in patients who are candidates to renal transplantation (RT). The main surgical options such as unilateral vs. bilateral nephrectomy, nephrectomy before vs. after RT, or simultaneous nephrectomy and transplantation, are herein discussed. OBJECTIVE: Evidence acquisition of the best surgical management available for ADPKD in the context of kidney transplantation...
January 2014: Actas Urologicas Españolas
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