keyword
MENU ▼
Read by QxMD icon Read
search

autosomal dominant polycystic kidney

keyword
https://www.readbyqxmd.com/read/29150246/development-of-the-autosomal-dominant-polycystic-kidney-disease-impact-scale-a-new-health-related-quality-of-life-instrument
#1
Dorothee Oberdhan, Jason C Cole, Holly B Krasa, Rebecca Cheng, Frank S Czerwiec, Ron D Hays, Arlene B Chapman, Ronald D Perrone
BACKGROUND: The impact of autosomal dominant polycystic kidney disease (ADPKD) on health-related quality of life (HRQoL) is not well understood due to a lack of instruments specific to the condition. STUDY DESIGN: Content for a new self-administered patient-reported outcome (PRO) questionnaire to assess ADPKD-related HRQoL was developed through clinical expert and patient focus group discussions. The new PRO instrument was administered to study patients with ADPKD to evaluate its reliability and validity...
November 14, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29146141/the-myth-of-water-and-salt-from-aquaretics-to-tenapanor
#2
REVIEW
Luca Visconti, Valeria Cernaro, Sebastiano Calimeri, Antonio Lacquaniti, Francesca De Gregorio, Carlo Alberto Ricciardi, Viviana Lacava, Domenico Santoro, Michele Buemi
The impact of water intake has been studied in several renal diseases. For example, increasing water intake is useful to prevent primary and secondary nephrolithiasis. In autosomal dominant polycystic kidney disease, arginine vasopressin (AVP) is involved in the progression of the disease, and water intake could play a therapeutic role by inhibiting the synthesis of AVP, but its efficacy is still controversial. Conversely, the use of aquaretics, which are antagonists of AVP V2 receptors, results in the reduction of the increase rate of total kidney volume with a slower decline of glomerular filtration rate...
November 14, 2017: Journal of Renal Nutrition
https://www.readbyqxmd.com/read/29145907/-autosomal-dominant-polycystic-kidney-disease-should-patients-young-adult-relatives-be-screened-or-not
#3
B J Kramers, M Storm, R T Gansevoort
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disease, with a global prevalence of 10 per 10,000. It is characterized by the formation of numerous cysts in both kidneys, and leads to renal function loss; the majority of patients will eventually need renal replacement therapy. It is possible to screen patients' presymptomatic family members from a young age, but this has not historically been recommended as until recently there were no treatment options. This year, the vasopressin V2 receptor antagonist tolvaptan was approved for prescription in ADPKD, to slow the rate of renal function decline...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/29145282/autosomal-dominant-polycystic-kidney-disease-combined-with-hypertrophic-cardiomyopathy-a-case-report
#4
Yingjing Shen, Chenggang Xu
INTRODUCTION: This report describes the novel sampling of autosomal dominant polycystic kidney disease (ADPKD) combined with hypertrophic cardiomyopathy (HCM). SYMPTOMS AND CLINICAL FINDINGS: A 48-year-old Chinese man presented with anasarca, hypourocrinia, gross hematuria, and weight gain by 10 kg subsequently developed acute kidney injury after struck by acute respiratory distress syndrome, really a threat to his heart. DIAGNOSES: Abdominal ultrasound revealed multiple small cysts in both kidneys, with the right kidney measuring 11...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29142975/sclerostin%C3%A2-a-debutant-on-the-autosomal-dominant-polycystic-kidney-disease-scene
#5
Magdalena Jankowska, Mathias Haarhaus, Abdul Rashid Qureshi, Bengt Lindholm, Pieter Evenepoel, Peter Stenvinkel
Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disease originating from a mutation in genes encoding polycystin 1 and 2. Recent evidence suggests that these polycystins mediate mechanosensation not only in the primary cilium of kidney cells but also in bone cells. The Wnt/β-catenin signaling pathway plays a central role in mechanotransduction in osteocytes. Mechanical unloading causes the upregulation of the Wnt inhibitor sclerostin. We tested the hypothesis that ADPKD associates with higher circulating sclerostin levels...
May 2017: KI Reports
https://www.readbyqxmd.com/read/29142972/a-drug-development-tool-for-trial-enrichment-in-patients-with-autosomal-dominant-polycystic-kidney-disease
#6
Ronald D Perrone, Mohamad-Samer Mouksassi, Klaus Romero, Frank S Czerwiec, Arlene B Chapman, Berenice Y Gitomer, Vicente E Torres, Dana C Miskulin, Steve Broadbent, Jean F Marier
Introduction: Total kidney volume (TKV) is a promising imaging biomarker for tracking and predicting the natural history of patients with autosomal dominant polycystic kidney disease. Methods: A drug development tool was developed by linking longitudinal TKV measurements to the probability of a 30% decline of estimated glomerular filtration rate (eGFR) or end-stage renal disease. Drug development tools were developed based on observational data collected over multiple decades for an eGFR decline and end-stage renal disease in 641 and 866 patients with autosomal dominant polycystic kidney disease, respectively...
May 2017: KI Reports
https://www.readbyqxmd.com/read/29142971/total-kidney-volume-is-a-prognostic-biomarker-of-renal-function-decline-and-progression-to-end-stage-renal-disease-in%C3%A2-patients-with-autosomal-dominant-polycystic-kidney-disease
#7
Ronald D Perrone, Mohamad-Samer Mouksassi, Klaus Romero, Frank S Czerwiec, Arlene B Chapman, Berenice Y Gitomer, Vicente E Torres, Dana C Miskulin, Steve Broadbent, Jean F Marier
Introduction: Autosomal dominant polycystic kidney disease is the most common hereditary kidney disease. TKV is a promising imaging biomarker for tracking and predicting the natural history of autosomal dominant polycystic kidney disease. The prognostic value of TKV was evaluated, in combination with age and eGFR, for the outcomes of 30% decline in eGFR and progression to ESRD. Observational data including 2355 patients with TKV measurements were available. Methods: Multivariable Cox models were developed to assess the prognostic value of age, TKV, height-adjusted TKV, eGFR, sex, race, and genotype for the probability of a 30% decline in eGFR or ESRD...
May 2017: KI Reports
https://www.readbyqxmd.com/read/29142941/the-longitudinal-study-of-liver-cysts-in%C3%A2-patients-with-autosomal-dominant-polycystic-kidney-disease-and-polycystic-liver-disease
#8
Ryo Matsuura, Kenjiro Honda, Yoshifumi Hamasaki, Kent Doi, Eisei Noiri, Masaomi Nangaku
Introduction: Although polycystic liver disease (PCLD) is one of the extrarenal complications in patients with autosomal dominant polycystic kidney disease (ADPKD), longitudinal changes and the association with total liver volume (TLV) have not been clearly elucidated yet. Methods: Patients with ADPKD were chosen who underwent computed tomography or magnetic resonance imaging twice or more during August 2003 through December 2015. TLV, each cyst volume, and the proportion of parenchyma were measured...
January 2017: KI Reports
https://www.readbyqxmd.com/read/29135414/retroperitoneoscopic-nephrectomy-for-huge-autosomal-dominant-polycystic-kidney-disease-using-morcellator
#9
Dong Sup Lee, Hee Youn Kim, Seung-Ju Lee
No abstract text is available yet for this article.
November 17, 2017: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/29133541/autosomal-dominant-polycystic-kidney-disease
#10
Matthew B Lanktree, Arlene B Chapman
No abstract text is available yet for this article.
November 13, 2017: CMAJ: Canadian Medical Association Journal, Journal de L'Association Medicale Canadienne
https://www.readbyqxmd.com/read/29123425/long-term-safety-profile-of-tolvaptan-in-autosomal-dominant-polycystic-kidney-disease-patients-tempo-extension-japan-trial
#11
Satoru Muto, Tadashi Okada, Moriyoshi Yasuda, Hidetsugu Tsubouchi, Koji Nakajima, Shigeo Horie
Aim: The aim of this trial (ClinicalTrials.gov identifier: NCT01280721) was to investigate the long-term safety profile of tolvaptan in Japanese patients with autosomal dominant polycystic kidney disease (ADPKD). Methods: This open-label multicenter trial was conducted to examine adverse drug reactions (ADRs) related to tolvaptan up to an additional 3 years in 135 Japanese patients who participated in the Tolvaptan Efficacy and Safety in Management of Autosomal Dominant Polycystic Kidney Disease and its Outcomes (TEMPO) 3:4 trial at doses of 60-120 mg/d...
2017: Drug, Healthcare and Patient Safety
https://www.readbyqxmd.com/read/29121521/induced-pluripotent-stem-cells-derived-from-an-autosomal-dominant-polycystic-kidney-disease-patient-carrying-a-pkd1-q533x-mutation
#12
Jia-Jung Lee, Ming-Ching Ho, Ching-Ying Huang, Cheng-Hao Wen, Yu-Che Cheng, Yu-Hung Hsu, Daw-Yang Hwang, Huai-En Lu, Hung-Chun Chen, Patrick C H Hsieh
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most prevalent monogenic kidney disorder leading to kidney failure. We generated induced pluripotent stem cells (iPSCs) from a 37-year-old man carrying a PKD1 Q533X mutation who suffered from kidney failure and a myocardial infarction. The iPSCs were reprogrammed from the patient's peripheral blood mononuclear cells using the Sendai virus system, and were confirmed to possess the specific PKD1 Q533X mutation and normal karyotype. Pluripotency was confirmed using in vitro and in vivo assays...
October 28, 2017: Stem Cell Research
https://www.readbyqxmd.com/read/29118087/overweight-and-obesity-are-predictors-of-progression-in-early-autosomal-dominant-polycystic-kidney-disease
#13
Kristen L Nowak, Zhiying You, Berenice Gitomer, Godela Brosnahan, Vincente E Torres, Arlene B Chapman, Ronald D Perrone, Theodore I Steinman, Kaleab Z Abebe, Frederic F Rahbari-Oskoui, Alan S L Yu, Peter C Harris, Kyongtae T Bae, Marie Hogan, Dana Miskulin, Michel Chonchol
The association of overweight/obesity with disease progression in patients with autosomal dominant polycystic kidney disease (ADPKD) remains untested. We hypothesized that overweight/obesity associates with faster progression in early-stage ADPKD. Overall, 441 nondiabetic participants with ADPKD and an eGFR>60 ml/min per 1.73 m(2) who participated in the Halt Progression of Polycystic Kidney Disease Study A were categorized on the basis of body mass index (BMI; calculated using nonkidney and nonliver weight) as normal weight (18...
November 8, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29114798/intensive-blood-pressure-control-in-autosomal-dominant-polycystic-kidney-disease-how-safe-is-it-reply
#14
Wan-Chuan Tsai, Kuo-Liong Chien, Hon-Yen Wu
No abstract text is available yet for this article.
November 1, 2017: JAMA Internal Medicine
https://www.readbyqxmd.com/read/29114789/intensive-blood-pressure-control-in-autosomal-dominant-polycystic-kidney-disease-how-safe-is-it
#15
Mohammadreza Ardalan, Samad E J Golzari
No abstract text is available yet for this article.
November 1, 2017: JAMA Internal Medicine
https://www.readbyqxmd.com/read/29105594/tolvaptan-in-later-stage-autosomal-dominant-polycystic-kidney-disease
#16
Vicente E Torres, Arlene B Chapman, Olivier Devuyst, Ron T Gansevoort, Ronald D Perrone, Gary Koch, John Ouyang, Robert D McQuade, Jaime D Blais, Frank S Czerwiec, Olga Sergeyeva
BACKGROUND: In a previous trial involving patients with early autosomal dominant polycystic kidney disease (ADPKD; estimated creatinine clearance, ≥60 ml per minute), the vasopressin V2-receptor antagonist tolvaptan slowed the growth in total kidney volume and the decline in the estimated glomerular filtration rate (GFR) but also caused more elevations in aminotransferase and bilirubin levels. The efficacy and safety of tolvaptan in patients with later-stage ADPKD are unknown. METHODS: We conducted a phase 3, randomized withdrawal, multicenter, placebo-controlled, double-blind trial...
November 16, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/29105553/tolvaptan-and-autosomal-dominant-polycystic-kidney-disease
#17
EDITORIAL
Julie R Ingelfinger
New England Journal of Medicine, Volume 377, Issue 20, Page 1988-1989, November 2017.
November 16, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/29101551/novel-semi-automated-kidney-volume-measurements-in-autosomal-dominant-polycystic-kidney-disease
#18
Satoru Muto, Haruna Kawano, Shuji Isotani, Hisamitsu Ide, Shigeo Horie
BACKGROUND: We assessed the effectiveness and convenience of a novel semi-automatic kidney volume (KV) measuring high-speed 3D-image analysis system SYNAPSE VINCENT(®) (Fuji Medical Systems, Tokyo, Japan) for autosomal dominant polycystic kidney disease (ADPKD) patients. METHODS: We developed a novel semi-automated KV measurement software for patients with ADPKD to be included in the imaging analysis software SYNAPSE VINCENT(®). The software extracts renal regions using image recognition software and measures KV (VINCENT KV)...
November 3, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29092060/evidence-for-bone-and-mineral-metabolism-alterations-in-children-with-autosomal-dominant-polycystic-kidney-disease
#19
Stéphanie De Rechter, Justine Bacchetta, Nathalie Godefroid, Laurence Dubourg, Pierre Cochat, Julie Maquet, Ann Raes, Jean De Schepper, Pieter Vermeersch, Maria Van Dyck, Elena Levtchenko, Patrick D'Haese, Pieter Evenepoel, Djalila Mekahli
Context: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. Hypophosphatemia was demonstrated in adult patients with preserved renal function, together with high fibroblast growth factor 23 (FGF23) and low soluble Klotho levels. The latter explained the relative FGF23 hyporesponsiveness in this cohort. Objective: Evaluating phosphate and bone mineral metabolism in children with ADPKD compared with what is known in adult ADPKD patients...
November 1, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29062909/congenital-hepatic-fibrosis-in-a-9-year-old-female-patient-a-case-report
#20
Kamil Janowski, Maria Goliszek, Joanna Cielecka-Kuszyk, Irena Jankowska, Joanna Pawłowska
Congenital hepatic fibrosis (CHF) is a rare, autosomal recessive disorder, clinically characterized by hepatic fibrosis and portal hypertension. CHF results from ductal plate malformation (DPM) of the intrahepatic bile ducts. Four clinical forms can be observed: portal hypertensive, cholangitic, mixed and latent. CHF is one of the "fibropolycystic diseases" which also include several conditions with a variety of intrahepatic bile duct dilatation and associated periportal fibrosis such as Caroli disease, autosomal recessive and dominant polycystic kidney disease (ARPKD or ADPKD), Ivemark, Jeune, Joubert, Bardet-Biedl, Meckel-Gruber and Arima syndromes...
September 2017: Clin Exp Hepatol
keyword
keyword
98330
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"