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hyperoxaluria

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https://www.readbyqxmd.com/read/27995107/evaluating-the-effectiveness-of-adding-magnesium-chloride-to-conventional-protocol-of-citrate-alkali-therapy-on-kidney-stone-size
#1
Hassan Niroomand, Amin Ziaee, Keivan Ziaee, Alaleh Gheissari
BACKGROUND: Potassium citrate (K-Cit) is one of the therapeutic solutions broadly used in patients with urolithiasis. However, recent studies have shown that it is not so effective. Therefore, the goal of our study was to evaluate the effect of a combination of K-Cit - MgCl2 oral supplements, on urinary stone size. MATERIALS AND METHODS: This study was performed on 70 asymptomatic urolithiasis cases. The supplements included K-Cit and magnesium chloride (MgCl2), purchased from (Merck Company, Germany)...
2016: Advanced Biomedical Research
https://www.readbyqxmd.com/read/27994868/outcomes-of-living-kidney-donors-with-medullary-sponge-kidney
#2
Wisit Cheungpasitporn, Charat Thongprayoon, Brady A Brabec, Wonngarm Kittanamongkolchai, Stephen B Erickson
BACKGROUND: Patients with medullary sponge kidney (MSK) commonly encounter recurrent nephrolithiasis. The existing knowledge on safety of donors with MSK has not been studied. METHODS: We conducted a retrospective cohort study at a tertiary referral hospital to assess the outcomes of living kidney donors with MSK. All adults with MSK (N = 26) who underwent nephrectomy as living kidney donors between January 2000 and September 2014 were included. Non-donors with MSK (N = 78) were randomly selected by matching the year of birth and the comorbidity score with a ratio of 1:3 for comparison...
December 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27993722/use-of-polymer-conjugates-for-the-intraperoxisomal-delivery-of-engineered-human-alanine-glyoxylate-aminotransferase-as-a-protein-therapy-for-primary-hyperoxaluria-type-i
#3
Alessandro Roncador, Elisa Oppici, Marina Talelli, Amaya Niño Pariente, Marta Donini, Stefano Dusi, Carla Borri Voltattorni, María J Vicent, Barbara Cellini
Alanine:glyoxylate aminotransferase (AGT) is a liver peroxisomal enzyme whose deficit causes the rare disorder Primary Hyperoxaluria Type I (PH1). We now describe the conjugation of poly(ethylene glycol)-co-poly(L-glutamic acid) (PEG-PGA) block-co-polymer to AGT via the formation of disulfide bonds between the polymer and solvent-exposed cysteine residues of the enzyme. PEG-PGA conjugation did not affect AGT structural/functional properties and allowed the enzyme to be internalized in a cellular model of PH1 and to restore glyoxylate-detoxification...
December 18, 2016: Nanomedicine: Nanotechnology, Biology, and Medicine
https://www.readbyqxmd.com/read/27960020/-the-hyperoxalurias
#4
Martino Marangella, Michele Petrarulo, Francesca Bermond, Cristina Marcuccio, Corrado Vitale
Oxalate (Ox) is an end-product of metabolism, important for poor solubility of its calcium salt in biological fluids. Ox can therefore be found in about 70% of urinary calculi. Hyperoxaluria (HOx) defined as Ox exceeding 0.5 mmol)/day, may cause nephrolithiasis/nephrocalcinosis and may be classified as dietary (DH), enteric (EH) or primary (PH). Fractional intestinal absorption of Ox is less than 10%, but increases to over 20% at calcium intakes below 200 mg/day. DH is often related to low-calcium diets. EH is caused by non-absorbed fatty acids which bind to calcium and lower its concentration in the intestinal lumen...
2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/27936931/characterization-of-urolithiasis-in-patients-following-lower-urinary-tract-reconstruction-with-intestinal-segments
#5
Tracy Marien, Jennifer Robles, Trisha M Kammann, Mustafa Kadihasanoglu, Davis P Viprakasit, S Duke Herrell, Nicole L Miller
PURPOSE: Urinary diversion and augmentation cystoplasty are associated with long-term complications, including metabolic derangements, infectious complications, and urolithiasis. The aim of this series was to characterize upper and lower urinary tract (LUT) calculi in this population. METHODS: A retrospective chart review was performed on all patients with a history of urinary diversion or augmentation cystoplasty who subsequently underwent treatment for urolithiasis between January 1998 and May 2015...
December 20, 2016: Journal of Endourology
https://www.readbyqxmd.com/read/27935012/mutational-analysis-of-agxt-in-tunisian-population-with-primary-hyperoxaluria-type-1
#6
Saoussen M'dimegh, Asma Omezzine, Ibtihel M'barek, Amira Moussa, Sameh Mabrouk, Hayet Kaarout, Geneviéve Souche, Jalel Chemli, Sabra Aloui, Cécile Aquaviva-Bourdain, Abdellatif Achour, Saoussen Abroug, Ali Bouslama
BACKGROUND: Primary hyperoxaluria type 1 (PH1) is an autosomal recessive metabolic disorder caused by inherited mutations in the AGXT gene encoding liver peroxisomal alanine:glyoxylate aminotransferase (AGT). PH1 is a clinically and genetically heterogeneous disorder. The aim of our study was to analyze and characterize the mutational spectrum of PH1 in Tunisian patients. MATERIALS AND METHODS: Molecular studies of 146 Tunisian patients suspected with PH were performed by PCR/Restriction fragment length polymorphism (RFLP) to detect seven mutations described as the most common...
December 9, 2016: Annals of Human Genetics
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
#7
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
#8
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...
December 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
#9
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
#10
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
#11
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
#12
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...
December 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/27872836/urolithiasis-in-inflammatory-bowel-disease-and-bariatric-surgery
#13
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
#14
REVIEW
S Adibul Hasan Rizvi, Sajid Sultan, Mirza Naqi Zafar, Bashir Ahmed, Sadaf Aba Umer, 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...
December 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
#15
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)...
December 2016: International Journal of Surgery
https://www.readbyqxmd.com/read/27847289/nephrolithiasis-after-bariatric-surgery-a-review-of-pathophysiologic-mechanisms-and-procedural-risk
#16
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...
December 2016: International Journal of Surgery
https://www.readbyqxmd.com/read/27845599/n-acetylcysteine-protects-against-star-fruit-induced-acute-kidney-injury
#17
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
#18
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)...
January 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/27815516/systemic-oxalosis-in-hyperoxaluria-type-1
#19
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
#20
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...
December 2016: International Journal of Surgery
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