2,866 results match your criteria Hyperoxaluria


Outcomes of liver-kidney transplantation in patients with primary hyperoxaluria: an analysis of the scientific registry of transplant recipients database.

BMC Gastroenterol 2020 Jul 3;20(1):208. Epub 2020 Jul 3.

Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Key Laboratory of Combined Multi-Organ Transplantation, Ministry of Public Health Key Laboratory of Organ Transplantation, Zhejiang University, No.79 Qingchun Road, Hangzhou, 310003, Zhejiang Province, China.

Background: Primary hyperoxaluria (PH) is an inherited disease lacking of hepatic oxalic acid metabolic enzymes which could lead to irreverisible renal damage. Currently, liver-kidney transplantation is a curative but highly invasive therapy used to treat patients with PH. However, limited studies have focused on combined liver-kidney transplantation (CLKT) and sequential liver and kidney transplantation (SLKT) in patients with PH. Read More

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http://dx.doi.org/10.1186/s12876-020-01349-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7333252PMC

Examination of the eye and retinal alterations in primary hyperoxaluria type 1.

Nephrol Dial Transplant 2020 Jun 24. Epub 2020 Jun 24.

Department of Pediatric Nephrology, University Children's Hospital Essen, Essen, Germany.

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http://dx.doi.org/10.1093/ndt/gfaa101DOI Listing

The influence of dysbiosis on kidney stones that risk up renal cell carcinoma (RCC).

Semin Cancer Biol 2020 Jun 20. Epub 2020 Jun 20.

Department of Biotechnology, Himachal Pradesh University, Summer Hill, Shimla, 171 005 India. Electronic address:

Kidney stone is a common urological condition, the prevalence and incidence of which has escalated in the last few years due to dietary habits and other related medical conditions such as obesity and diabetes mellitus. It is a chronic disease which leads to loss of kidney function(s) and nephrectomy. Chronic kidney stone disease has been shown to be associated with transitional cell carcinoma (TCC) or renal cell carcinoma (RCC) and kidney tumors have been found to be more frequent among patients with kidney stones. Read More

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http://dx.doi.org/10.1016/j.semcancer.2020.06.011DOI Listing

Primary hyperoxaluria Type 1: A case report in an extended family with a novel AGXT gene mutation.

Medicine (Baltimore) 2020 Jun;99(25):e20371

Faculty of Medicine, Cairo University, Cairo, Egypt.

Introduction: Primary hyperoxaluria type 1 (PH1) is a genetic autosomal recessively inherited disorder due to mutation in the alanine-glyoxylate aminotransferase (AGXT) gene. It usually presents in children with nephrolithiasis and/or nephrocalcinosis and progressive renal function impairment and end stage renal disease (ESRD).

Patient Concerns: A 13 years old Saudi boy with history of recurrent urolithiasis since the age of 2 years presented to us with picture of ESRD. Read More

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http://dx.doi.org/10.1097/MD.0000000000020371DOI Listing

Characteristics of the genotype and phenotype in Chinese primary hyperoxaluria type 1 populations.

Urolithiasis 2020 Jun 18. Epub 2020 Jun 18.

Department of Urology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing, 100050, China.

The aim of our study is to explore the relationship between genotype and phenotype in Chinese PH1 patients and determine the putative mutation hotspot regions. This was a retrospective study regarding 13 Chinese PH1 patients. And all sporadic published researches of Chinese PH1 populations were searched and enrolled based on the inclusive standard. Read More

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http://dx.doi.org/10.1007/s00240-020-01201-xDOI Listing

Re: Endpoints for Clinical Trials in Primary Hyperoxaluria.

Authors:
Dean G Assimos

J Urol 2020 Jun 18:101097JU000000000000117302. Epub 2020 Jun 18.

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http://dx.doi.org/10.1097/JU.0000000000001173.02DOI Listing

Investigational Therapies for Primary Hyperoxaluria.

Bioconjug Chem 2020 Jun 29. Epub 2020 Jun 29.

Institute of Pharmaceutical Sciences, Department of Chemistry and Applied Biosciences, ETH Zurich, 8093 Zurich, Switzerland.

Recent years have brought exciting new insights in the field of primary hyperoxaluria (PH), both on a basic research level as well as through the progress of novel therapeutics in clinical development. To date, very few supportive measures are available for patients suffering from PH, which, together with the severity of the disorder, make disease management challenging. Basic and clinical research and development efforts range from correcting the underlying gene mutations, preventing calcium oxalate crystal-induced kidney damage, to the administration of probiotics favoring the intestinal secretion of excess oxalate. Read More

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http://dx.doi.org/10.1021/acs.bioconjchem.0c00268DOI Listing

Case Report: Acute kidney failure leading to permanent haemodialysis due to hyperoxaluria following one-anastomosis gastric bypass-related rapid weight loss.

F1000Res 2020 26;9:155. Epub 2020 Feb 26.

General Surgery Departement, Desio Hospital, Desio, Italy, 20843, Italy.

The one-anastomosis gastric bypass (OAGB) has been proven to provide good weight loss, comorbidity improvement, and quality of life with follow-up longer than five years. Although capable of improving many obesity-related diseases, OAGB is associated with post-operative medical complications mainly related to the induced malabsorption. A 52-year-old man affected by nephrotic syndrome due to a focal segmental glomerulosclerosis underwent OAGB uneventfully. Read More

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http://dx.doi.org/10.12688/f1000research.22109.2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7265574.2PMC
February 2020

Genetics of kidney stone disease.

Nat Rev Urol 2020 Jul 12;17(7):407-421. Epub 2020 Jun 12.

Academic Endocrine Unit, Radcliffe Department of Medicine, University of Oxford, Oxford, UK.

Kidney stone disease (nephrolithiasis) is a common problem that can be associated with alterations in urinary solute composition including hypercalciuria. Studies suggest that the prevalence of monogenic kidney stone disorders, including renal tubular acidosis with deafness, Bartter syndrome, primary hyperoxaluria and cystinuria, in patients attending kidney stone clinics is ∼15%. However, for the majority of individuals, nephrolithiasis has a multifactorial aetiology involving genetic and environmental factors. Read More

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http://dx.doi.org/10.1038/s41585-020-0332-xDOI Listing

A rare cause of nephrocalcinosis in an infant: Answers.

Pediatr Nephrol 2020 Jun 4. Epub 2020 Jun 4.

Pediatric Gastroenterology, Hepatology and Nutrition Unit, Division of Pediatrics, Hospital Regional Universitario de Málaga, Málaga, Spain.

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http://dx.doi.org/10.1007/s00467-020-04615-2DOI Listing

Plasma oxalate: comparison of methodologies.

Urolithiasis 2020 May 29. Epub 2020 May 29.

Infection and Immunity, University College London, London, UK.

Measurement of oxalate in the blood is essential for monitoring primary hyperoxaluria patients with progressive renal impairment and on dialysis prior to transplantation. As no external quality assurance scheme is available for this analyte, we conducted a sample exchange scheme between six laboratories specifically involved with the investigation of primary hyperoxaluria to compare results. The methodologies compared were gas chromatography/mass spectrometry (GCMS), ion chromatography with mass spectrometry (ICMS), and enzymatic methods using oxalate oxidase and spectrophotometry. Read More

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http://dx.doi.org/10.1007/s00240-020-01197-4DOI Listing

CRISPR/Cas9-mediated metabolic pathway reprogramming in a novel humanized rat model ameliorates primary hyperoxaluria type 1.

Kidney Int 2020 May 25. Epub 2020 May 25.

Department of Pediatric Urology, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, 1665 KongJiang Road, Shanghai, 200092, China; Children's Stone Treatment Center of National Health and Family Planning Commission of the People's Republic of China, 1665 KongJiang Road, Shanghai, 200092, China. Electronic address:

Primary hyperoxaluria type I is caused by mutations in the alanine glyoxylate aminotransferase gene (AGXT), leading to accumulation of glyoxylate and subsequent production of oxalate and urolithiasis. Here, we generated a novel rat model of primary hyperoxaluria type I that carries a D205N mutation in the partially humanized Agxt gene through the CRISPR/Cas9 system. The AgxtD205N mutant rats showed undetectable alanine glyoxylate aminotransferase protein expression, developed hyperoxaluria at 1 month of age and exhibited severe renal calcium oxalate deposition after ethylene glycol challenge. Read More

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http://dx.doi.org/10.1016/j.kint.2020.04.049DOI Listing

Stiripentol identifies a therapeutic target to reduce oxaluria.

Curr Opin Nephrol Hypertens 2020 Jul;29(4):394-399

Sorbonne Université, INSERM UMR S 1155, APHP Service des Explorations Fonctionnelles Multidisciplinaires, Hôpital Tenon, Paris, France.

Purpose Of Review: Oxalate is a metabolic end-product promoting the formation of calcium oxalate crystals in urine. Massive urine oxalate excretion occurs in genetic diseases, mainly primary hyperoxaluria type I and II, threatening renal function. Ethylene glycol poisoning may induce the precipitation of calcium oxalate crystals in renal tubules, leading to acute renal failure. Read More

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http://dx.doi.org/10.1097/MNH.0000000000000621DOI Listing

Kidney stones: KI at the crossroads of nephrology and urology.

Kidney Int 2020 Jun;97(6):1070-1073

Department of Medicine, Division of Nephrology, Duke University and Duke Clinical Research Institute, Durham, North Carolina, USA. Electronic address:

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http://dx.doi.org/10.1016/j.kint.2020.03.019DOI Listing

Plasma Oxalate as a Predictor of Kidney Function Decline in a Primary Hyperoxaluria Cohort.

Int J Mol Sci 2020 May 20;21(10). Epub 2020 May 20.

Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN 55905, USA.

This retrospective analysis investigated plasma oxalate (POx) as a potential predictor of end-stage kidney disease (ESKD) among primary hyperoxaluria (PH) patients. PH patients with type 1, 2, and 3, age 2 or older, were identified in the Rare Kidney Stone Consortium (RKSC) PH Registry. Since POx increased with falling estimated glomerular filtration rate (eGFR), patients were stratified by chronic kidney disease (CKD) subgroups (stages 1, 2, 3a, and 3b). Read More

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http://dx.doi.org/10.3390/ijms21103608DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7279271PMC

Dietary Recommendations for Bariatric Patients to Prevent Kidney Stone Formation.

Nutrients 2020 May 16;12(5). Epub 2020 May 16.

Nephrology Division, Universidade Federal de São Paulo, São Paulo 04023-062, Brazil.

Bariatric surgery (BS) is one of the most common and efficient surgical procedures for sustained weight loss but is associated with long-term complications such as nutritional deficiencies, biliary lithiasis, disturbances in bone and mineral metabolism and an increased risk of nephrolithiasis, attributed to urinary metabolic changes resultant from low urinary volume, hypocitraturia and hyperoxaluria. The underlying mechanisms responsible for hyperoxaluria, the most common among all metabolic disturbances, may comprise increased intestinal oxalate absorption consequent to decreased calcium intake or increased dietary oxalate, changes in the gut microbiota, fat malabsorption and altered intestinal oxalate transport. In the current review, the authors present a mechanistic overview of changes found after BS and propose dietary recommendations to prevent the risk of urinary stone formation, focusing on the role of dietary oxalate, calcium, citrate, potassium, protein, fat, sodium, probiotics, vitamins D, C, B6 and the consumption of fluids. Read More

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http://dx.doi.org/10.3390/nu12051442DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7284744PMC

Stiripentol fails to lower plasma oxalate in a dialysis-dependent PH1 patient.

Pediatr Nephrol 2020 May 16. Epub 2020 May 16.

Departments of Pediatric Gastroenterology, Nephrology and Metabolic Diseases, Charité University Medicine, Berlin, Germany.

Background: Primary hyperoxaluria type 1 (PH1) is a multisystemic metabolic disorder caused by an excessive production of oxalate by the liver. The majority of patients presenting in early infancy have end-stage renal disease (ESRD). While awaiting the results of sRNAi trials, the current standard treatment is combined liver-kidney transplantation. Read More

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http://dx.doi.org/10.1007/s00467-020-04585-5DOI Listing

Lessons from rodent gastric bypass model of enteric hyperoxaluria.

Curr Opin Nephrol Hypertens 2020 Jul;29(4):400-406

Department of Urology, College of Medicine, University of Florida, Gainesville, Florida, USA.

Purpose Of Review: The aim of the article is to review studies on bone health and oxalate metabolism/therapeutics in the obese rodent model of Roux-en-Y gastric bypass (RYGB) and examine pathways to decrease procedural morbidity.

Recent Findings: Compared with controls, RYGB rodents have up to 40-fold more fat in their stool (steatorrhea) which positively correlates to increased urinary oxalate. These unabsorbed intestinal fatty acids bind calcium and prevent gut calcium oxalate formation, increasing soluble luminal oxalate availability and absorption (enteric hyperoxaluria). Read More

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http://dx.doi.org/10.1097/MNH.0000000000000613DOI Listing

Metabolic evaluation in urolithiasis - study of the prevalence of metabolic abnormalities in a tertiary centre.

Cent European J Urol 2020 25;73(1):55-61. Epub 2020 Feb 25.

Department of Urology, Centro Hospitalar Universitário São João, Porto, Portugal.

Introduction: Metabolic abnormalities are one of the most important risk factors for urinary stone disease. Our objective was to determine the prevalence of metabolic abnormalities in patients referred to the urolithiasis outpatient clinic of a tertiary centre.

Material And Methods: We performed a cross-sectional study evaluating 67 patients referred to the urolithiasis outpatient clinic. Read More

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http://dx.doi.org/10.5173/ceju.2020.0051DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7203776PMC
February 2020

Diffuse Hypermetabolic Bone Marrow Infiltration in Severe Primary Hyperoxaluria on FDG PET.

Clin Nucl Med 2020 Jun;45(6):e296-e298

From the Nuclear Medicine Department, Bichat Claude Bernard Hospital, Assistance Publique Hôpitaux de Paris, Inserm UMR-S 1148, Paris Diderot University.

A 24-year-old man, with type 1 primary hyperoxaluria (diagnosed at age 20 years after repeated renal lithiasis, due to a I244T mutation frequently encountered in Mediterranean countries) complicated by end-stage renal failure requiring dialysis, was admitted for pancytopenia, refractory to erythropoietin injections. On clinical examination, he presented a hepatosplenomegaly without palpable adenopathy. F-FDG PET/CT revealed intense and diffuse bone marrow uptake in the axial skeleton and preferential long bone metaphyseal uptake. Read More

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http://dx.doi.org/10.1097/RLU.0000000000003047DOI Listing

Relationship of endoscopic lesions of the renal papilla with type of renal stone and 24 h urine analysis.

BMC Urol 2020 Apr 25;20(1):46. Epub 2020 Apr 25.

Hospital Universitari Son Espases, Ctra. Valldemossa, 79, Palma de Mallorca, Spain.

Background: Our purpose was to study the relationship of the 3 different types of endoscopic calcifications of the renal papilla (Randall's plaque, intratubular calcification, papillary crater) with the type of stone and urine analysis.

Methods: This prospective study examined 41 patients (age range: 18 to 80 years) who received retrograde intrarenal surgery (RIRS) for renal lithiasis (mean stone size: 15.3 ± 7. Read More

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http://dx.doi.org/10.1186/s12894-020-00615-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7183647PMC

Inhibitors of Calcium Oxalate Crystallization for the Treatment of Oxalate Nephropathies.

Adv Sci (Weinh) 2020 Apr 27;7(8):1903337. Epub 2020 Feb 27.

Institute of Pharmaceutical Sciences Department of Chemistry and Applied Biosciences ETH Zurich 8093 Zurich Switzerland.

Calcium oxalate (CaOx) crystal-induced nephropathies comprise a range of kidney disorders, for which there are no efficient pharmacological treatments. Although CaOx crystallization inhibitors have been suggested as a therapeutic modality already decades ago, limited progress has been made in the discovery of potent molecules with efficacy in animal disease models. Herein, an image-based machine learning approach to systematically screen chemically modified -inositol hexakisphosphate (IP6) analogues is utilized, which enables the identification of a highly active divalent inositol phosphate molecule. Read More

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http://dx.doi.org/10.1002/advs.201903337DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7175250PMC

Urinary monocyte chemoattractant protein 1 associated with calcium oxalate crystallization in patients with primary hyperoxaluria.

BMC Nephrol 2020 Apr 15;21(1):133. Epub 2020 Apr 15.

Division of Nephrology and Hypertension, Department of Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.

Background: Patients with primary hyperoxaluria (PH) often develop kidney stones and chronic kidney disease. Noninvasive urine markers reflective of active kidney injury could be useful to gauge the effectiveness of ongoing treatments.

Methods: A panel of biomarkers that reflect different nephron sites and potential mechanisms of injury (clusterin, neutrophil gelatinase-associated lipocalin (NGAL), 8-isoprostane (8IP), monocyte-chemoattractant protein 1(MCP-1), liver-type fatty acid binding protein (L-FABP), heart-type fatty acid binding protein (H-FABP), and osteopontin (OPN)) were measured in 114 urine specimens from 30 PH patients over multiple visits. Read More

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http://dx.doi.org/10.1186/s12882-020-01783-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7161151PMC

Oxidative stress and endoplasmic stress in calcium oxalate stone disease: the chicken or the egg?

Free Radic Res 2020 Apr 15;54(4):244-253. Epub 2020 Apr 15.

Department of Physiology and Pharmacology "Vittorio Erspamer", Sapienza University of Rome, Rome, Italy.

Crystal modulators play a significant role in the formation of calcium oxalate stone disease. When renal cells are subjected to oxalate stress, the loss in cell integrity leads to exposure of multiple proteins that assist and/or inhibit crystal attachment and retention. Contact between oxalate and calcium oxalate with urothelium proves fatal to cells as a result of reactive oxygen species generation and onset of oxidative stress. Read More

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http://dx.doi.org/10.1080/10715762.2020.1751835DOI Listing

The Efficacy of Polyunsaturated Fatty Acids as Protectors against Calcium Oxalate Renal Stone Formation: A Review.

Nutrients 2020 Apr 12;12(4). Epub 2020 Apr 12.

University Stone Centre, Department of Urology, University Hospital of Bonn, 53127 Bonn, Germany.

In the pathogenesis of hypercalciuria and hyperoxaluria, n-6 polyunsaturated fatty acids (PUFAs) have been implicated by virtue of their metabolic links with arachidonic acid (AA) and prostaglandin PGE. Studies have also shown that n-3 PUFAs, particularly those in fish oil-eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA)-can serve as competitive substrates for AA in the n-6 series and can be incorporated into cell membrane phospholipids in the latter's place, thereby reducing urinary excretions of calcium and oxalate. The present review interrogates several different types of study which address the question of the potential roles played by dietary PUFAs in modulating stone formation. Read More

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http://dx.doi.org/10.3390/nu12041069DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7230958PMC

Plasma oxalate levels in primary hyperoxaluria type I show significant intra-individual variation and do not correlate with kidney function.

Pediatr Nephrol 2020 Jul 9;35(7):1227-1233. Epub 2020 Apr 9.

Division of Pediatric Nephrology, Department of Pediatrics, University Children's Hospital Bonn, Bonn, Germany.

Background: Primary hyperoxalurias are rare diseases with endogenous overproduction of oxalate, thus leading to hyperoxaluria, hyperoxalemia, urolithiasis, and/or nephrocalcinosis and eventually early kidney failure. Plasma oxalate (POx) is an important diagnostic parameter in clinical studies on primary hyperoxaluria (PH). This is especially the case in kidney failure, where urinary parameters are no longer suitable. Read More

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http://dx.doi.org/10.1007/s00467-020-04531-5DOI Listing

A comprehensive analysis of sialolith proteins and the clinical implications.

Clin Proteomics 2020 31;17:12. Epub 2020 Mar 31.

7Department of Otolaryngology Head Neck Surgery, Louisiana State University Medical School Health Sciences Center, 533 Bolivar St. Suite 566, New Orleans, LA 70112 USA.

Background: Sialolithiasis or salivary gland stones are associated with high clinical morbidity. The advances in the treatment of sialolithiasis has been limited, however, by our understanding of their composition. More specifically, there is little information regarding the formation and composition of the protein matrix, the role of mineralogical deposition, or the contributions of cell epithelium and secretions from the salivary glands. Read More

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http://dx.doi.org/10.1186/s12014-020-09275-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7110646PMC

No stone unturned: The epidemiology and outcomes of paediatric urolithiasis in Manchester, United Kingdom.

J Pediatr Urol 2020 Jun 19;16(3):372.e1-372.e7. Epub 2020 Mar 19.

Royal Manchester Children's Hospital, Manchester, UK.

Background: The epidemiology and risk factors for paediatric urolithiasis (UL) in developed countries are evolving, with increasing rates of metabolic stone-formers. In the United Kingdom (UK), only a single London cohort has been studied in the past three decades. Regional disease patterns across the UK remain unknown. Read More

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http://dx.doi.org/10.1016/j.jpurol.2020.03.009DOI Listing

Etiological Profile of Nephrocalcinosis in Children from Southern India.

Indian Pediatr 2020 May 12;57(5):415-419. Epub 2020 Mar 12.

Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India.

Objective: To study the etiological profile and patterns of clinical presentation of nephrocalcinosis.

Methods: In this observational study, patients 18 years or younger, referred to the pediatric nephrology clinic with nephrocalcinosis were evaluated for etiology. Symptoms/signs at presentation, estimated glomerular filtration rate (eGFR) at presentation and follow-up, and growth parameters were recorded. Read More

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Characterising a healthy adult with a rare HAO1 knockout to support a therapeutic strategy for primary hyperoxaluria.

Elife 2020 Mar 24;9. Epub 2020 Mar 24.

Blizard Institute and Institute for Population Health Sciences, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, United Kingdom.

By sequencing autozygous human populations, we identified a healthy adult woman with lifelong complete knockout of (expected ~1 in 30 million outbred people). (glycolate oxidase) silencing is the mechanism of lumasiran, an investigational RNA interference therapeutic for primary hyperoxaluria type 1. Her plasma glycolate levels were 12 times, and urinary glycolate 6 times, the upper limit of normal observed in healthy reference individuals (n = 67). Read More

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http://dx.doi.org/10.7554/eLife.54363DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7108859PMC
March 2020
8.519 Impact Factor

Traditional Medications Mixed with Ethylene Glycol in a Nigerian Patient on Hemodialysis.

Cureus 2020 Feb 11;12(2):e6950. Epub 2020 Feb 11.

Nephrology, Baylor College of Medicine, Houston, USA.

Unregulated traditional medications and their solvents are nephrotoxic. We present a case of a 49-year-old Nigerian male with a 10-year history of diabetes mellitus and hypertension who was ingesting a traditional, herbal medication as an aphrodisiac for erectile dysfunction. He had a rapid decline in kidney function over a period of one year and the patient commenced thrice weekly hemodialysis. Read More

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http://dx.doi.org/10.7759/cureus.6950DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7067357PMC
February 2020

Primary Hyperoxaluria: The Patient and Caregiver Perspective.

Clin J Am Soc Nephrol 2020 Mar 12. Epub 2020 Mar 12.

Oxalosis and Hyperoxaluria Foundation, New York, New York.

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http://dx.doi.org/10.2215/CJN.13831119DOI Listing

Endpoints for Clinical Trials in Primary Hyperoxaluria.

Clin J Am Soc Nephrol 2020 Mar 12. Epub 2020 Mar 12.

Division of Nephrology, Mayo Clinic, Rochester, Minnesota.

Patients with primary hyperoxaluria experience kidney stones from a young age and can develop progressive oxalate nephropathy. Progression to kidney failure often develops over a number of years, and is associated with systemic oxalosis, intensive dialysis, and often combined kidney and liver transplantation. There are no therapies approved by the Food and Drug Association. Read More

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http://dx.doi.org/10.2215/CJN.13821119DOI Listing

Alteration of the gut microbiota by vinegar is associated with amelioration of hyperoxaluria-induced kidney injury.

Food Funct 2020 Mar 11;11(3):2639-2653. Epub 2020 Mar 11.

Department of Urology and Guangdong Key Laboratory of Urology, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong, China510230.

Hyperoxaluria is well known to cause renal injury and end-stage kidney disease. Previous studies suggested that the renal function of rats with hyperoxaluria was improved after dietary vinegar intake. However, its underlying mechanisms remain largely unknown. Read More

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http://dx.doi.org/10.1039/c9fo02172hDOI Listing

Reducing urinary oxalate by simultaneous using Sankol herbal drop with oxalate-degrading bacteria.

Iran J Microbiol 2019 Dec;11(6):460-467

Department of Microbiology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.

Background And Objectives: Oxalate degrading bacteria and herbal extracts are new strategy for reducing hyperoxaluria. In Iranian traditional medicine, Sankol oral drop is widely used as an antispasmodic drug to reduce stones from urinary tract. This study aimed to evaluate the synergistic effect of oxalate-degrading bacteria and Sankol oral drop in reducing urinary oxalate in rat model. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7048961PMC
December 2019

Anticalculi Activity of Apigenin and Celery ( L.) Extract in Rats Induced by Ethylene Glycol-Ammonium Chloride.

J Pharm Bioallied Sci 2019 Dec 30;11(Suppl 4):S556-S561. Epub 2019 Dec 30.

Department of Pharmaceutics and Pharmaceutical Technology, Padjadjaran University, Sumedang, Indonesia.

Objective: Kidney stones (nephrolithiasis) is one of the kidney diseases in the form of stones that contain crystal and organic matrix components. It is one of the most common diseases of the urinary tract. Calcium stone is the most important type of stone (80%) found in the case of kidney stones. Read More

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http://dx.doi.org/10.4103/jpbs.JPBS_202_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7020841PMC
December 2019

Oxalobacter formigenes treatment combined with intensive dialysis lowers plasma oxalate and halts disease progression in a patient with severe infantile oxalosis.

Pediatr Nephrol 2020 Jun 27;35(6):1121-1124. Epub 2020 Feb 27.

Center for Rare Diseases Bonn (ZSEB), University of Bonn, Bonn, Germany.

Background: Infantile oxalosis, the most devastating form of primary hyperoxaluria type 1 (PH1), often leads to end-stage renal disease (ESRD) during the first weeks to months of life.

Case-diagnosis: Here, we report the outcome of the therapeutic use of Oxalobacter formigenes (Oxabact OC5; OxThera AB, Stockholm, Sweden) in a female infant with PH1 who exhibited severely elevated plasma oxalate (Pox) levels, pronounced nephrocalcinosis, anuretic end-stage renal disease, and retinal oxalate deposits. Following the diagnosis of PH1 at an age of 8 weeks, a combined regimen of daily peritoneal dialysis, daily pyridoxine treatment and hemodialysis (3 times a week) was unable to reduce the pronounced hyperoxalemia. Read More

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http://dx.doi.org/10.1007/s00467-019-04463-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7184045PMC

[Clinical analysis of seven cases with primary hyperoxaluria type 1 in children].

Zhonghua Er Ke Za Zhi 2020 Feb;58(2):129-134

Department of Nephrology, Guangzhou Women and Children's Medical Center, Guangzhou 510120, China.

To investigate the clinical, imaging and molecular characteristics of primary hyperoxaluria type 1 (PH1) in children and to sum up existing evidence for further understanding the phenotype-genotype correlation of infantile PH1. This retrospective analysis was based on the medical records of children with PH1 diagnosed by gene test in the Department of Nephrology, Guangzhou Women and Children's Medical Center from June 2016 to May 2019. Targeted exome sequencing was performed on tubular disease-related genes of the probands and Sanger sequencing was conducted to validate suspected pathogenic variants of family members. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2020.02.012DOI Listing
February 2020

Clinical features of genetically confirmed patients with primary hyperoxaluria identified by clinical indication versus familial screening.

Kidney Int 2020 Apr 13;97(4):786-792. Epub 2019 Dec 13.

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.

Primary hyperoxaluria is a rare monogenic disorder characterized by excessive hepatic production of oxalate leading to recurrent nephrolithiasis, nephrocalcinosis, and progressive kidney damage. Most patients with primary hyperoxaluria are diagnosed after clinical suspicion based on symptoms. Since some patients are detected by family screening following detection of an affected family member, we compared the clinical phenotype of these two groups. Read More

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http://dx.doi.org/10.1016/j.kint.2019.11.023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7175669PMC

Primary hyperoxaluria type 2 successfully treated with combined liver-kidney transplantation after failure of isolated kidney transplantation.

Am J Transplant 2020 06 19;20(6):1752-1753. Epub 2020 Mar 19.

Department of Nephrology and Organ Transplantation, CHU Rangueil, Toulouse, France.

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http://dx.doi.org/10.1111/ajt.15829DOI Listing

[Primary hyperoxaluria: case report and therapeutic perspectives].

G Ital Nefrol 2020 Feb 12;37(1). Epub 2020 Feb 12.

UOSD Nefrologia e dialisi, P.O. Piedimonte Matese, Azienda Sanitaria Locale Caserta.

Primary hyperoxaluria (PH) is a rare genetic disorder with autosomal recessive transmission, characterized by high endogenous production and markedly excessive urinary excretion of oxalate (Ox). It causes the accumulation of calcium oxide crystals in organs and tissues including bones, heart, arteries, skin and kidneys, where it may cause oxalo-calcic nephrolithiasis, nephrocalcinosis and chronic renal failure. Some forms are secondary to enteric diseases, drugs or dietetic substances, while three primitive forms, caused by various enzymatic defects, are currently known: PH1, PH2 and PH3. Read More

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February 2020

[Genetic aspects of primary hyperoxaluria: epidemiology, ethiology, pathogenesis, and clinical signs of the disorder].

Urologiia 2019 12(6):125-130

I.M. Sechenov First Moscow State Medical University of Ministry of Public Health of the Russian Federation, Moscow, Russia.

Primary hyperoxaluria is a group of rare inherited diseases characterized by impaired oxalate metabolism with the early manifestation of urolithiasis and the development of the chronic kidney disease. The mutations in the AGXT, GRHPR, HOGA1 genes are attributable for different types of primary hyperoxaluria leading to the dysfunction of specific enzymes involved in the oxalate metabolism. The article summary the current data on the epidemiology, genetic and biochemical aspects of pathogenesis of the primary hyperoxaluria types 1-3. Read More

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December 2019

2019 Update in basic kidney research: microbiota in chronic kidney disease, controlling autoimmunity, kidney inflammation and modelling the glomerular filtration barrier.

Nephrol Dial Transplant 2020 01;35(1):4-9

Renal Division, Department of Medicine IV, University Hospital, LMU Munich, München, Germany.

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http://dx.doi.org/10.1093/ndt/gfz219DOI Listing
January 2020

Importance of Assessing Compliance with Conservative Treatment of Primary Hyperoxaluria Type 1: A Case Report of a Patient with I244T/c.969-3C>G Mutation.

Perm J 2020 30;24. Epub 2019 Dec 30.

Pediatric Nephrology Department, Hospital Universitario La Paz, Madrid, Spain.

Introduction: Primary hyperoxaluria type 1 (PH1) is an autosomal recessive inherited disorder that progresses to end-stage renal disease. Patients experience excessive urinary oxalate excretion, which causes nephrocalcinosis and recurrent urolithiasis. When the glomerular filtration rate declines, calcium oxalate accumulates in extrarenal tissues, causing end-organ damage. Read More

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http://dx.doi.org/10.7812/TPP/19.136DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6972639PMC
December 2019

Future treatments for hyperoxaluria.

Curr Opin Urol 2020 Mar;30(2):171-176

University of Alabama at Birmingham School of Medicine, Birmingham, Alabama, USA.

Purpose Of Review: The review of potential therapies in the treatment of hyperoxaluria is timely, given the current excitement with clinical trials and the mounting evidence of the importance of oxalate in both kidney stone and chronic kidney disease.

Recent Findings: Given the significant contribution of both endogenous and dietary oxalate to urinary oxalate excretions, it is not surprising therapeutic targets are being studied in both pathways. This article covers the existing data on endogenous and dietary oxalate and the current targets in these pathways. Read More

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http://dx.doi.org/10.1097/MOU.0000000000000709DOI Listing

When the Cause Is Not Crystal Clear.

N Engl J Med 2020 Jan;382(1):74-78

From Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool, United Kingdom.

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http://dx.doi.org/10.1056/NEJMcps1809996DOI Listing
January 2020