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hyperoxaluria

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https://www.readbyqxmd.com/read/28302239/hyperoxaluria-after-renal-transplantation
#1
Waqar Ahmed, Abad Ur Rehman, Sumit Acharya
Primary hyperoxaluria is a rare autosomal recessive disorder, characterised by precipitation of insoluble oxalate crystals in the joints, kidneys, heart, eyes, skin, nerves, and bone marrow. The patients of primary oxaluria usually present with renal stone/nephrocalcinosis, and isolated kidney transplantation should not be done in these patients. We present a case report of 31-year lady with acute graft dysfunction due to oxaluria with no history of nephrolithiasis/nephrocalcinosis prior to renal transplantation...
March 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28273709/-primary-hyperoxaluria-type-1-a-case-report
#2
(no author information available yet)
No abstract text is available yet for this article.
March 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28271480/dihydrodipicolinate-synthase-structure-dynamics-function-and-evolution
#3
F Grant Pearce, André O Hudson, Kerry Loomes, Renwick C J Dobson
Enzymes are usually comprised of multiple subunits and more often than not they are made up of identical subunits. In this review we examine lysine biosynthesis and focus on the enzyme dihydrodipicolinate synthase in terms of its structure, function and the evolution of its varied number of subunits (quaternary structure). Dihydrodipicolinate synthase is the first committed step in the biosynthesis of lysine, which occurs naturally in plants, bacteria, archaea and fungi, but is not synthesized in mammals. In bacteria, there have been four separate pathways identified from tetrahydrodipicolinate to meso-diaminopimelate, which is the immediate precursor to lysine...
2017: Sub-cellular Biochemistry
https://www.readbyqxmd.com/read/28261895/bilateral-native-nephrectomy-reduces-systemic-oxalate-level-after-combined-liver-kidney-transplant-a-case-report
#4
Vincenzo Villani, Neena Gupta, Nahel Elias, Parsia A Vagefi, James F Markmann, Elahna Paul, Avram Z Traum, Heidi Yeh
Primary hyperoxaluria type 1 (PH1) is a rare liver enzymatic defect that causes overproduction of plasma oxalate. Accumulation of oxalate in the kidney and subsequent renal failure are fatal to PH1 patients often in pediatric age. Combined liver and kidney transplantation is the therapy of choice for end-stage renal disease due to PH1. Levels of plasma oxalate remain elevated for several months after liver transplantation, as the residual body oxalate is slowly excreted. Patients with persistent hyperoxaluria after transplant often require hemodialysis, and accumulation of residual oxalate in the kidney can induce graft dysfunction...
March 5, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28228792/malabsorption-syndrome-as-a-rare-cause-of-nephrocalcinosis
#5
Rui Abreu, Cláudia Bento, Luís Oliveira, Teresa Morgado
Nephrocalcinosis is characterized by calcification of kidney parenchyma and can be caused by an increased amount of calcium, phosphate or oxalate in urinary excretion. We report a 35-year-old female with nephrocalcinosis. She had fitful steatorrhea since last year. Physical examination was normal. Analytic exams found normal renal function and ionogram. Primary hyperparathyroidism, renal tubular acidosis and sarcoidosis were excluded. Urinalysis showed mild hematuria, without proteinuria and 24-hour urine collection exhibited hyperoxaluria...
September 2016: Clinical Cases in Mineral and Bone Metabolism
https://www.readbyqxmd.com/read/28220601/multiple-sclerosis-and-nephrolithiasis-a-matched-case-comparative-study
#6
Vishnu Ganesan, Wen Min Chen, Rajat Jain, Shubha De, Manoj Monga
OBJECTIVE: To compare stone composition and serum/urine biochemistries in stone formers with multiple sclerosis (MS) against stone formers without MS and to examine the association between mobility, methods of bladder emptying, and stone formation. MATERIALS AND METHODS: In this retrospective case-control study, we identified patients diagnosed with multiple sclerosis and kidney stone disease who were seen at our institution between 2001 and 2016. For the first part of the study, up to 2 controls (stone formers without a history of MS) were identified for each case and matched on age, body mass index (BMI), and sex...
February 20, 2017: BJU International
https://www.readbyqxmd.com/read/28217701/gut-microbiota-and-oxalate-homeostasis
#7
Marguerite Hatch
This perspective focuses on how the gut microbiota can impact urinary oxalate excretion in the context of hyperoxaluria, a major risk factor in kidney stone disease. In the genetic disease of Primary Hyperoxaluria Type 1 (PH1), an increased endogenous production of oxalate, due to a deficiency of the liver enzyme alanine-glyoxylate aminotransferase (AGT), results in hyperoxaluria and oxalate kidney stones. The constant elevation in urinary oxalate in PH1 patients ultimately leads to tissue deposition of oxalate, renal failure and death and the only known cure for PH1 is a liver or liver-kidney transplant...
January 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28208534/re-hyperoxaluria-requires-tnf-receptors-to-initiate-crystal-adhesion-and-kidney-stone-disease
#8
Dean G Assimos
No abstract text is available yet for this article.
March 2017: Journal of Urology
https://www.readbyqxmd.com/read/28202121/-oliguria-and-acute-renal-dysfunction-in-a-six-month-old-infant
#9
Ya-Jie Cui, Chun-Lan Song, Yi-Bing Cheng
The infant (a girl aged 6 months) was admitted to the hospital because of oliguria and acute renal dysfunction. The laboratory examination results showed serious metabolic acidosis and increased blood urea nitrogen and serum creatinine levels. The patient continued to be anuric after 10 days of treatment with continuous renal replacement therapy (CRRT). she died a day later. The family history showed that the patient's sister died of acute renal failure 6 months after birth. The genomic sequencing results showed AGXT mutation in the patient and confirmed the diagnosis of primary hyperoxaluria type 1 (PH1)...
February 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28185107/protective-impact-of-resveratrol-in-experimental-rat-model-of-hyperoxaluria
#10
Taylan Oksay, Sedat Yunusoğlu, Mustafa Calapoğlu, I Aydın Candan, İbrahim Onaran, Osman Ergün, Alper Özorak
PURPOSE: Resveratrol (RES) is a polyphenol with antioxidant, anti-inflammatory, and many other physiological effects on tissues. In the present study, the effect of resveratrol in hyperoxaluria driven nephrolithiasis/nephrocalcinosis is investigated. METHODS: Wistar-Albino rats of 250-300 g (male, n = 24) were included in the present study. The rats were randomized into three groups: Group 1 consisted of the controls (n = 8), Group 2 of hyperoxaluria (1% ethylene glycol (EG), n = 8), and Group 3 of the treatment (1% EG + 10 mg/kg of RES, n = 8) group...
February 9, 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/28161266/clinical-spectrum-of-primary-hyperoxaluria-type%C3%A2-1-experience-of-a-tertiary-center
#11
Neveen A Soliman, Marwa M Nabhan, Safaa M Abdelrahman, Hanan Abdelaziz, Rasha Helmy, Khaled Ghanim, Hafez M Bazaraa, Ahmed M Badr, Omar A Tolba, Magd A Kotb, Khaled M Eweeda, Alaa Fayez
BACKGROUND AND AIM: Primary hyperoxalurias are rare inborn errors of metabolism resulting in increased endogenous production of oxalate that leads to excessive urinary oxalate excretion. Diagnosis of primary hyperoxaluria type 1 (PH1) is a challenging issue and depends on diverse diagnostic tools including biochemical analysis of urine, stone analysis, renal biopsy, genetic studies and in some cases liver biopsy for enzyme assay. We characterized the clinical presentation as well as renal and extrarenal phenotypes in PH1 patients...
February 1, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28124521/forming-a-stone-in-pelviureteric-junction-obstruction-cause-or-effect
#12
Theodora Stasinou, Andreas Bourdoumis, Junaid Masood
OBJECTIVES: To investigate a possible causal relationship for stone formation in pelviureteric junction obstruction and to outline management options. MATERIALS AND METHODS: A literature search and evidence synthesis was conducted via electronic databases in the English language using the key words pelviureteric junction obstruction; urolithiasis; hyperoxaluria; laparoscopic pyeloplasty; flexible nephroscopy; percutaneous nephrolithotomy, alone or in combination...
January 2017: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/28116040/mitotempo-prevents-oxalate-induced-injury-in-nrk-52e-cells-via-inhibiting-mitochondrial-dysfunction-and-modulating-oxidative-stress
#13
Jiaqiao Zhang, Qing Wang, Chuou Xu, Yuchao Lu, Henglong Hu, Baolong Qin, Yufeng Wang, Deng He, Cong Li, Xiao Yu, Shaogang Wang, Jihong Liu
As one of the major risks for urolithiasis, hyperoxaluria can be caused by genetic defect or dietary intake. And high oxalate induced renal epithelial cells injury is related to oxidative stress and mitochondrial dysfunction. Here, we investigated whether MitoTEMPO, a mitochondria-targeted antioxidant, could protect against oxalate mediated injury in NRK-52E cells via inhibiting mitochondrial dysfunction and modulating oxidative stress. MitoSOX Red was used to determine mitochondrial ROS (mtROS) production...
2017: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/28089681/steatorrhea-and-hyperoxaluria-in-severely-obese-patients-before-and-after-roux-en-y-gastric-bypass
#14
Amber M Moreland, Carol A Santa Ana, John R Asplin, Joseph A Kuhn, Ross P Holmes, Jason A Cole, Elizabeth A Odstrcil, Thomas G Van Dinter, Juan G Martinez, John S Fordtran
BACKGROUND AND AIMS: Hyperoxaluria after Roux-en-Y gastric bypass (RYGB) is generally attributed to fat malabsorption. If hyperoxaluria is indeed caused by fat malabsorption, magnitudes of hyperoxaluria and steatorrhea should correlate. Severely obese patients, prior to bypass, ingest excess dietary fat that can produce hyperphagic steatorrhea. The primary objective of the study was to determine whether urine oxalate excretion correlates with elements of fat balance in severely obese patients before and after RYGB...
January 12, 2017: Gastroenterology
https://www.readbyqxmd.com/read/27995107/evaluating-the-effectiveness-of-adding-magnesium-chloride-to-conventional-protocol-of-citrate-alkali-therapy-on-kidney-stone-size
#15
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
#16
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
#17
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
#18
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
#19
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...
March 2017: Journal of Endourology
https://www.readbyqxmd.com/read/27935012/mutational-analysis-of-agxt-in-tunisian-population-with-primary-hyperoxaluria-type-1
#20
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
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