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hyperoxaluria

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https://www.readbyqxmd.com/read/28435765/chronic-nephropathy-from-dietary-hyperoxaluria-sustained-improvement-of-renal-function-after-dietary-intervention
#1
Yijuan Sun, Bruce L Horowitz, Karen S Servilla, Joanna R Fair, Darlene Vigil, Kavitha Ganta, Larry Massie, Antonios H Tzamaloukas
A 56-year-old man with stable chronic kidney disease (CKD) for two years following a single episode of calcium oxalate urolithiasis developed progressive elevation of his serum creatinine concentration. Urinalysis revealed pyuria and white cell casts, a few red blood cells, minimal proteinuria, and no crystals. Urine culture was sterile. Gallium scintigraphy was consistent with interstitial nephritis. Proton pump inhibitor intake was discontinued, and a short course of oral corticosteroids was initiated. Percutaneous kidney biopsy, performed because of the continued deterioration of renal function to a minimum estimated glomerular filtration rate (eGFR) value of 15 mL/min per 1...
March 20, 2017: Curēus
https://www.readbyqxmd.com/read/28425073/molecular-therapy-of-primary-hyperoxaluria
#2
Cristina Martin-Higueras, Armando Torres, Eduardo Salido
During the last few decades, the molecular understanding of the mechanisms involved in primary hyperoxalurias (PHs) has set the stage for novel therapeutic approaches. The availability of PH mouse models has facilitated preclinical studies testing innovative treatments. PHs are autosomal recessive diseases where the enzymatic deficit plays a central pathogenic role. Thus, molecular therapies aimed at restoring such deficit or limiting the consequences of the metabolic derangement could be envisioned, keeping in mind the specific challenges posed by the cell-autonomous nature of the deficiency...
April 19, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28420322/epidemiology-of-paediatric-renal-stone-disease-a-22-year-single-centre-experience-in-the-uk
#3
Naomi Issler, Stephanie Dufek, Robert Kleta, Detlef Bockenhauer, Naima Smeulders, William Van't Hoff
BACKGROUND: Whilst still rare, the incidence of paediatric stone disease is increasing in developed countries and it is important to evaluate the aetiology. We set up a dedicated renal stone service for children combining medical and surgical expertise in 1993 and now have a large case series of children to investigate the epidemiology. METHODS: A retrospective hospital note review of children presenting with kidney stones during the last 22 years (1993-2015) was conducted...
April 18, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28405703/metabolic-syndrome-contributes-to-renal-injury-mediated-by-hyperoxaluria-in-a-murine-model-of-nephrolithiasis
#4
Javier Sáenz-Medina, E Jorge, C Corbacho, M Santos, A Sánchez, P Soblechero, E Virumbrales, E Ramil, M J Coronado, I Castillón, D Prieto, J Carballido
Metabolic syndrome (MS) individuals have a higher risk of developing chronic kidney disease through unclear pathogenic mechanisms. MS has been also related with higher nephrolithiasis prevalence. To establish the influence of MS on renal function, we designed a murine model of combined metabolic syndrome and hyperoxaluria. Four groups of male Sprague-Dawley rats were established: (1) control group (n = 10) fed with standard chow; (2) stone former group (SF) (n = 10) fed with standard chow plus 0.75% ethylene glycol administered in the drinking water; (3) metabolic syndrome group (MS) (n = 10), fed with 60% fructose diet; (4) metabolic syndrome + stone former group (MS + SF) (n = 10), 60% fructose diet and 0...
April 12, 2017: Urolithiasis
https://www.readbyqxmd.com/read/28402768/primary-hyperoxaluria
#5
Dapeng Jiang, Hongquan Geng
A 3-year-old boy presented to the pediatric urology clinic with a 6-month history of gross hematuria and intermittent abdominal pain. Urinalysis revealed red cells, white cells, and 3+ protein. The serum creatinine level was 0.9 mg per deciliter (80 μmol per liter; normal range in 3-year-old..
April 13, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28392511/combination-of-vitamin-e-and-vitamin-c-alleviates-renal-function-in-hyperoxaluric-rats-via-antioxidant-activity
#6
Orapun Jaturakan, Thasinas Dissayabutra, Narongsak Chaiyabutr, Anusak Kijtawornrat, Piyaratana Tosukhowong, Anudep Rungsipipat, Thumnoon Nhujak, Chollada Buranakarl
Hyperoxaluria and oxidative stress are risk factors in calcium oxalate (CaOx) stone formation. Supplement with antioxidant could be effective in prevention of recurrent stone formation. The present study aims to evaluate the protective effects of vitamin E and vitamin C in hyperoxaluric rat. The experiment was performed in rats for 21 days. Rats were divided into 5 groups as follows: control (group 1, n=8), hyperoxaluric rats (group 2, n=8), hyperoxaluric rats with vitamin E supplement (group 3, n=7), hyperoxaluric rats with vitamin C supplement (group 4, n=7) and hyperoxaluric rats with vitamin E and C supplement (group 5, n=7)...
April 8, 2017: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/28387228/calcium-oxalate-crystals-and-oxalate-induce-an-epithelial-to-mesenchymal-transition-in-the-proximal-tubular-epithelial-cells-contribution-to-oxalate-kidney-injury
#7
Marcia Bastos Convento, Edson Andrade Pessoa, Edgar Cruz, Maria Aparecida da Glória, Nestor Schor, Fernanda Teixeira Borges
TGF-β1 is the main mediator of epithelial-to-mesenchymal transition (EMT). Hyperoxaluria induces crystalluria, interstitial fibrosis, and progressive renal failure. This study analyzed whether hyperoxaluria is associated with TGF-β1 production and kidney fibrosis in mice and if oxalate or calcium oxalate (CaOx) could induce EMT in proximal tubule cells (HK2) and therefore contribute to the fibrotic process. Hyperoxaluria was induced by adding hydroxyproline and ethylene glycol to the mice's drinking water for up to 60 days...
April 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28356078/calcium-oxalate-crystal-related-kidney-injury-in-a-patient-receiving-roux-en-y-hepaticojejunostomy-due-to-gall-bladder-cancer
#8
Jun-Li Tsai, Shang-Feng Tsai
BACKGROUND: Calcium oxalate nephropathy is rare in current practice. It was a common complication during jejunoileal bypass, but much less seen in modern gastric bypass surgery for morbid obesity. The major cause of it is enteric hyperoxaluria. CASE PRESENTATION: We report on a patient here with acute kidney disease due to calcium oxalate nephropathy, rather than the conditions mentioned above. The male patient received a Roux-en Y hepaticojejunostomy and common bile duct drainage...
March 29, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28352011/biopsy-of-small-kidneys-a-safe-and-a-useful-guide-to-potentially-treatable-kidney-disease
#9
Kamel El-Reshaid, Wael El-Reshaid, Dalal Al-Bader, Jozsef Varro, John Madda, Hosameldin Tawfik Sallam
Over the past four years, all patients with unexplained rapid progression of their renal disease were subjected to kidney biopsy, despite their small size (<9 cm), to define its etiology. Children, pregnant women, morbidly obese patients, and those with an unstable cardiovascular state, septicemia, bleeding diathesis as well as those kidney size with size <6 cm were excluded from the study. Doppler ultrasound was used to exclude renovascular/ischemic nephropathy. The procedure was performed by an interventional radiologist using a biopsy gun technique and under ultrasound guidance...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28346094/drug-library-screening-for-the-identification-of-ionophores-that-correct-the-mistrafficking-disorder-associated-with-oxalosis-kidney-disease
#10
Shurong Hou, Franck Madoux, Louis Scampavia, Jo Ann Janovick, P Michael Conn, Timothy P Spicer
Primary hyperoxaluria is the underlying cause of oxalosis and is a life-threatening autosomal recessive disease, for which treatment may require dialysis or dual liver-kidney transplantation. The most common primary hyperoxaluria type 1 (PH1) is caused by genetic mutations of a liver-specific enzyme alanine:glyoxylate aminotransferase (AGT), which results in the misrouting of AGT from the peroxisomes to the mitochondria. Pharmacoperones are small molecules with the ability to modify misfolded proteins and route them correctly within the cells, which may present an effective strategy to treat AGT misrouting in PH1 disorders...
January 1, 2017: SLAS Discovery
https://www.readbyqxmd.com/read/28340800/long-term-results-of-living-donors-in-simultaneous-kidney-and-liver-transplantations
#11
T Unek, T Egeli, M Özbilgin, A Çelik, K Atilla, C Ağalar, N Ç Arslan, S Karademir, S Bora, H Gülay, Z S Derici, I Astarcıoğlu
INTRODUCTION: Because of the shortage of organs available for transplantation, living related sequential transplantation with the use of liver and a kidney from the same donor has emerged as a reasonable therapeutic alternative. However, there is insufficient literature about the complications that living donors experience after simultaneous kidney and liver transplantations. METHODS: From December 2001 to October 2009, 5 living donors provided simultaneous donation of livers and kidneys and 1 living donor donated first her kidney and then her liver...
April 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28302239/hyperoxaluria-after-renal-transplantation
#12
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
#13
(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
#14
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
#15
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...
May 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28228792/malabsorption-syndrome-as-a-rare-cause-of-nephrocalcinosis
#16
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
#17
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
#18
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
#19
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
#20
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
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