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hyperoxaluria

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https://www.readbyqxmd.com/read/27924398/a-randomised-phase-i-ii-trial-to-evaluate-the-efficacy-and-safety-of-orally-administered-oxalobacter-formigenes-to-treat-primary-hyperoxaluria
#1
Bernd Hoppe, Patrick Niaudet, Rémi Salomon, Jérôme Harambat, Sally-Anne Hulton, William Van't Hoff, Shabbir H Moochhala, Georges Deschênes, Elisabeth Lindner, Anna Sjögren, Pierre Cochat
BACKGROUND: Primary hyperoxaluria (PH) is a rare, genetic disorder which involves the overproduction of endogenous oxalate, leading to hyperoxaluria, recurrent urolithiasis and/or progressive nephrocalcinosis and eventually resulting in kidney failure and systemic oxalosis. The aim of this trial was to investigate whether treatment involving an oxalate-metabolising bacterium (Oxalobacter formigenes) could reduce urinary oxalate excretion in PH patients. METHODS: The efficacy and safety of O...
December 6, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27915025/molecular-analysis-of-the-agxt-gene-in-patients-suspected-with-hyperoxaluria-type-1-and-three-novel-mutations-from-turkey
#2
Emel Isiyel, Sevcan A Bakkaloglu Ezgu, Salim Caliskan, Sema Akman, Ipek Akil, Yilmaz Tabel, Nurver Akinci, Elif Bahat Ozdogan, Ahmet Ozel, Fehime Kara Eroglu, Fatih S Ezgu
Primary hyperoxaluria type 1 (PH1) is a rare, autosomal recessive disease, caused by the defect of AGXT gene encoding hepatic peroxisomal alanine glyoxylateaminotransferase (AGT). This enzyme is responsible for the conversion of glyoxylate to glycine. The diagnosis of PH1 should be suspected in infants and children with nephrocalcinosis or nephrolithiasis. Early diagnosis and treatment is crucial in preventing disease progression to end stage kidney disease (ESKD). In this study, AGXT gene sequence analyses were performed in 82 patients who were clinically suspected (hyperoxaluria and nephrolithiasis or nephrocalcinosis with or without renal impairment) to have PH1...
November 1, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/27913853/the-role-of-intestinal-oxalate-transport-in-hyperoxaluria-and-the-formation-of-kidney-stones-in-animals-and-man
#3
REVIEW
Jonathan M Whittamore, Marguerite Hatch
The intestine exerts a considerable influence over urinary oxalate in two ways, through the absorption of dietary oxalate and by serving as an adaptive extra-renal pathway for elimination of this waste metabolite. Knowledge of the mechanisms responsible for oxalate absorption and secretion by the intestine therefore have significant implications for understanding the etiology of hyperoxaluria, as well as offering potential targets for future treatment strategies for calcium oxalate kidney stone disease. In this review, we present the recent developments and advances in this area over the past 10 years, and put to the test some of the new ideas that have emerged during this time, using human and mouse models...
December 2, 2016: Urolithiasis
https://www.readbyqxmd.com/read/27904915/studies-using-a-porcine-model-what-insights-into-human-calcium-oxalate-stone-formation-mechanisms-has-this-model-facilitated
#4
REVIEW
Kristina L Penniston, Sutchin R Patel, Denise J Schwahn, Stephen Y Nakada
Animal models are useful in the study of many human diseases. Our current understanding of the biological, physiological, and biochemical aspects of hyperoxaluria and calcium oxalate urolithiasis has been greatly informed by studies using animals. Recently, limitations in the extrapolation to humans of research results derived from laboratory rodents have been identified. The use in biomedical research of a variety of organisms, including large animals, is increasingly encouraged. The purpose of this article is to review the use of pigs in biomedical and stone research, to provide a rationale for using pigs in metabolic stone research, and to describe our 8-year experience in developing a porcine platform for studying hyperoxaluria and calcium oxalate urolithiasis...
November 30, 2016: Urolithiasis
https://www.readbyqxmd.com/read/27882414/systemic-endothelial-function-measured-by-flow-mediated-dilation-is-impaired-in-patients-with-urolithiasis
#5
Esin Yencilek, Hakan Sarı, Faruk Yencilek, Ezgi Yeşil, Hasan Aydın
Some in vitro and animal studies have shown endothelial dysfunction in hyperoxaluria models indicating its role in pathogenesis of urolithiasis and relation to CVD. The aim of this study was to investigate endothelial function in patients with urolithiasis in relation to urinary stone risk factors and metabolic parameters. A total of 120 subjects without any known CVD (60 with urolithiasis and 60 healthy subjects) were included into study. Fasting blood and 24-h urine samples were collected to study metabolic parameters (glucose and lipids) and urine stone risk factors (oxalate, citrate, uric acid, and calcium, pH)...
November 23, 2016: Urolithiasis
https://www.readbyqxmd.com/read/27876212/alteration-in-oxidative-nitrosative-imbalance-histochemical-expression-of-osteopontin-and-antiurolithiatic-efficacy-of-xanthium-strumarium-l-in-ethylene-glycol-induced-urolithiasis
#6
Padma Nibash Panigrahi, Sahadeb Dey, Monalisa Sahoo, Shyam Sundar Choudhary, Sumit Mahajan
Xanthium strumarium has traditionally been used in the treatment of urolitiasis especially by the rural people in India, but its antiurolithiatic efficacy was not explored scientifically till now. Therefore, the present study was designed to validate the ethnic practice scientifically, and explore the possible antiurolithiatic effect to rationalize its medicinal use. Urolitiasis was induced in hyperoxaluric rat model by giving 0.75% ethylene glycol (EG) for 28days along with 1% ammonium chloride (AC) for first 14days...
November 18, 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/27872836/urolithiasis-in-inflammatory-bowel-disease-and-bariatric-surgery
#7
Agapios Gkentzis, Michael Kimuli, Jon Cartledge, Olivier Traxer, Chandra Shekhar Biyani
AIM: To analyse current literature focusing on pathogenesis and therapeutic aspects of urolithiasis with inflammatory bowel disease (IBD) and following bariatric surgery. METHODS: A systematic literature search was performed using PubMed, supplemented with additional references. Studies assessing the association of IBD or bariatric surgery with renal stones in both paediatric and adulthood were included. RESULTS: Certain types of stones are seen more frequently with IBD...
November 6, 2016: World Journal of Nephrology
https://www.readbyqxmd.com/read/27865972/paediatric-urolithiasis-in-emerging-economies
#8
REVIEW
S Adibul Hasan Rizvi, Sajid Sultan, Mirza Naqi Zafar, Sadaf Aba Umar, Bashir Ahmed, S A Anwar Naqvi
BACKGROUND: Paediatric urolithiasis remains endemic in low resource countries. This review highlights the epidemiology, causation and management of urolithiasis in an Asian country in the context of emerging economies. METHODS: A literature review of recent articles with key words paediatric urolithiasis, developing countries, endemic stone disease, stone composition, metabolic risk factors, management of paediatric urolithiasis was undertaken and 51 relevant articles were selected with the main focus on experience of this center in managing stone disease in the last two decades...
November 16, 2016: International Journal of Surgery
https://www.readbyqxmd.com/read/27847291/rna-interference-in-the-treatment-of-renal-stone-disease-current-status-and-future-potentials
#9
REVIEW
Kyle D Wood, Ross P Holmes, John Knight
Recent advances in RNA interference (RNAi) delivery and chemistry have resulted in the development of more than 20 RNAi-based therapeutics, several of which are now in Phase III trials. The most advanced clinical trials have utilized modifications such as lipid nanoparticles and conjugation to N-acetyl galactosamine to treat liver specific diseases. Recent reports have suggested that reducing endogenous oxalate synthesis by RNAi may be a safe and effective therapy for patients with the rare disease, Primary Hyperoxaluria (PH)...
November 12, 2016: International Journal of Surgery
https://www.readbyqxmd.com/read/27847289/nephrolithiasis-after-bariatric-surgery-a-review-of-pathophysiologic-mechanisms-and-procedural-risk
#10
REVIEW
Umer Hasan Bhatti, Andrew J Duffy, Kurt Eric Roberts, Amir Hafeez Shariff
Obesity alone is a known risk factor for nephrolithiasis, and bariatric surgery has been linked to a higher incidence of post-operative new-onset nephrolithiasis. The mean interval from bariatric surgery to diagnosis of nephrolithiasis, ranges from 1.5 to 3.6 years. The stone risk is greatest for purely malabsorptive procedures, intermediate for Roux-en-Y gastric bypass and lowest for purely restrictive procedures (laparoscopic adjustable gastric banding, laparoscopic sleeve gastrectomy) where it approaches or is reduced below that of non-operative obese controls...
November 12, 2016: International Journal of Surgery
https://www.readbyqxmd.com/read/27845599/n-acetylcysteine-protects-against-star-fruit-induced-acute-kidney-injury
#11
Maria Heloisa Massola Shimizu, Pedro Henrique França Gois, Rildo Aparecido Volpini, Daniele Canale, Weverton Machado Luchi, Leila Froeder, Ita Pfeferman Heilberg, Antonio Carlos Seguro
BACKGROUND: Star fruit (SF) is a popular fruit, commonly cultivated in many tropical countries, that contains large amount of oxalate. Acute oxalate nephropathy and direct renal tubular damage through release of free radicals are the main mechanisms involved in SF-induced acute kidney injury (AKI). The aim of this study was to evaluate the protective effect of N-acetylcysteine (NAC) on SF-induced nephrotoxicity due to its potent antioxidant effect. MATERIALS AND METHODS: Male Wistar rats received SF juice (4 mL/100 g body weight) by gavage after a 12 h fasting and water deprivation...
November 15, 2016: Renal Failure
https://www.readbyqxmd.com/read/27844104/primary-hyperoxaluria-spectrum-of-clinical-and-imaging-findings
#12
Sara B Strauss, Temima Waltuch, William Bivin, Frederick Kaskel, Terry L Levin
Primary hyperoxaluria is a rare autosomal recessive inborn error of metabolism with three known subtypes. In primary hyperoxaluria type 1, the most common of the subtypes, a deficiency in the hepatic enzymes responsible for the metabolism of glycoxylate to glycine, leads to excessive levels of glyoxylate, which is converted to oxalate. The resultant elevation in serum and urinary oxalate that characterizes primary hyperoxaluria leads to calcium oxalate crystal deposition in multiple organ systems (oxalosis)...
November 14, 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/27815516/systemic-oxalosis-in-hyperoxaluria-type-1
#13
Marília Rodrigues, José Carlos Cardoso, José A P da Silva
No abstract text is available yet for this article.
November 3, 2016: Rheumatology
https://www.readbyqxmd.com/read/27815184/the-primary-hyperoxalurias-a-practical-approach-to-diagnosis-and-treatment
#14
REVIEW
Sally-Anne Hulton
Although the primary hyperoxalurias (PH) are rare disorders, they are of considerable clinical importance in relation to calcium oxalate urolithiasis and as a cause of renal failure worldwide. Three distinct disorders have been described at the molecular level. The investigation of any child or adult presenting with urinary tract stones or nephrocalcinosis, must exclude PH as an underlying cause. This paper provides a practical approach to the investigation and diagnosis of PH, indicating the importance of distinguishing between the PH types for the purposes of targeting appropriate therapy...
November 1, 2016: International Journal of Surgery
https://www.readbyqxmd.com/read/27795638/bilateral-nephrocalcinosis-in-primary-hyperoxaluria-type-1
#15
C A Mansoor, A Jemshad, D S Milliner, N K N Bhushan
No abstract text is available yet for this article.
September 2016: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/27781207/reversal-of-gastric-bypass-resolves-hyperoxaluria-and-improves-oxalate-nephropathy-secondary-to-roux-en-y-gastric-bypass
#16
Varun Agrawal, Jonathan B Wilfong, Christopher E Rich, Pamela C Gibson
Hyperoxaluria after Roux-en-Y gastric bypass (RYGB) increases the risk for kidney injury. Medical therapies for hyperoxaluria have limited efficacy. A 65-year-old female was evaluated for acute kidney injury [AKI, serum creatinine (Cr) 2.1 mg/dl, baseline Cr 1.0 mg/dl]. She did not have any urinary or gastrointestinal symptoms or exposure to nephrotoxic agents. Sixteen months prior to this evaluation, she underwent RYGB for morbid obesity. Her examination was unremarkable for hypertension or edema and there was no protein or blood on urine dipstick...
September 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27748900/analysis-of-altered-microrna-expression-profiles-in-the-kidney-tissues-of-ethylene-glycol-induced-hyperoxaluric-rats
#17
Zhuo Liu, Hongyang Jiang, Jun Yang, Tao Wang, Yufeng Ding, Jihong Liu, Shaogang Wang, Zhangqun Ye
Calcium oxalate stones account for >80% of urinary stones, however the mechanisms underlying their formation remains to be elucidated. Hyperoxaluria serves an important role in the pathophysiological process of stone formation. In the present study, differences in the miRNA expression profiles between experimental hyperoxaluric rats and normal rats were analyzed, in order to identify target genes and signaling pathways involved in the pathogenesis of hyperoxaluria. Ethylene glycol and ammonium chloride was fed to male hyperoxaluric rats (EXP) and normal age‑matched male rats (CON)...
November 2016: Molecular Medicine Reports
https://www.readbyqxmd.com/read/27744630/metabolic-risk-factors-in-pediatric-stone-formers-a-report-from-an-emerging-economy
#18
Kiran Imran, Mirza Naqi Zafar, Uzma Ozair, Sadia Khan, Syed Adibul Hasan Rizvi
The goal of this study was to investigate metabolic risk factors in pediatric stone formers in an emerging economy. A prospective, data collection enrolled 250 children age <1-15 years at our center. Risk factors were evaluated by gender and in age groups <1-5, 6-10 and 11-15 years. Patients were evaluated for demographics, blood and 24 h urine for calcium, magnesium, phosphate, uric acid, electrolytes and additional protein, citrate, ammonia and oxalate in urine. All reported values were two sided and statistical significance was considered at p value ≤0...
October 15, 2016: Urolithiasis
https://www.readbyqxmd.com/read/27742850/annals-express-late-diagnosis-of-primary-hyperoxaluria-type-iii-a-case-report
#19
Emmanuel Richard, Jean-Marc Blouin, Jerome Harambat, Brigitte Llanas, Stephane Bouchet, Cecile Acquaviva, Renaud De la Faille
We report the case of a 78-year-old patient with late diagnosis of hyperoxaluria type III (PH3). He developed renal failure after nephrectomy for clear cell papillary renal carcinoma and complained of recurrent urolithiasis for some 30 years whose etiology was never identified. Biochemical laboratory investigations of urine and urolithiasis composition revealed marked hyperoxaluria but normal levels of urinary glyceric and glycolic acid as well as stones of idiopathic calcium-oxalate appearance. Furthermore, the dietary survey showed excessive consumption of food supplements containing massive amounts of oxalate precursors...
October 13, 2016: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/27738124/oxalobacter-formigenes-derived-bioactive-factors-stimulate-oxalate-transport-by-intestinal-epithelial-cells
#20
Donna Arvans, Yong-Chul Jung, Dionysios Antonopoulos, Jason Koval, Ignacio Granja, Mohamed Bashir, Eltayeb Karrar, Jayanta Roy-Chowdhury, Mark Musch, John Asplin, Eugene Chang, Hatim Hassan
Hyperoxaluria is a major risk factor for kidney stones and has no specific therapy, although Oxalobacter formigenes colonization is associated with reduced stone risk. O. formigenes interacts with colonic epithelium and induces colonic oxalate secretion, thereby reducing urinary oxalate excretion, via an unknown secretagogue. The difficulties in sustaining O. formigenes colonization underscore the need to identify the derived factors inducing colonic oxalate secretion. We therefore evaluated the effects of O...
October 13, 2016: Journal of the American Society of Nephrology: JASN
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