Abstract：
:Primary hyperoxalurias are rare recessive inherited inborn errors of glyoxylate metabolism. They are responsible for progressive renal involvement, which further lead to systemic oxalate deposition, which can even occur in infants. Primary hyperoxaluria type 1 is the most common form in Europe and is due to alanine-glyoxylate aminostransferase deficiency, a hepatic peroxisomal pyridoxin-dependent enzyme. Therefore primary hyperoxaluria type 1 is responsible for hyperoxaluria leading to aggressive stone formation and nephrocalcinosis. As glomerular filtration rate decreases, systemic oxalate storage occurs throughout all the body, and mainly in the skeleton. The diagnosis is first based on urine oxalate measurement, then on genotyping, which may also allow prenatal diagnosis to be proposed. Conservative measures - including hydration, crystallization inhibitors and pyridoxine - are safe and may allow long lasting renal survival, provided it is given as soon as the diagnosis has been even suspected. No dialysis procedure can remove enough oxalate to compensate oxalate overproduction from the sick liver, therefore a combined liver and kidney transplantation should be planned before advanced renal disease has occurred, in order to limit/avoid systemic oxalate deposition. In the future, primary hyperoxaluria type 1 may benefit from hepatocyte transplantation, chaperone molecules, etc.
journal_name
Nephrol Therjournal_title
Nephrologie & therapeutiqueauthors
Cochat P,Fargue S,Bacchetta J,Bertholet-Thomas A,Sabot JF,Harambat Jdoi
10.1016/j.nephro.2011.03.004subject
Has Abstractpub_date
2011-07-01 00:00:00pages
249-59issue
4eissn
1769-7255issn
1872-9177pii
S1769-7255(11)00074-5journal_volume
7pub_type
杂志文章abstract：AIM:To investigate risk factors of renal complications in glycogen storage disease type I, in order to identify practical implications for renal preservation. METHODS:A retrospective study of 38 patients with glycogen storage disease type I. RESULTS:The patients studied were 8.6 years old in average (1.5 to 22 years)...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2014.12.007
更新日期：2015-07-01 00:00:00
abstract：:The overall risk of cancer in the kidney transplant patient is three to five times higher than the risk in the general population, and the risk of cutaneous epitheliomas, the most frequent, is multiplied by 100. There are many causes of posttransplantation cancer involving modifications in the immunosurveillance of ne...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：10.1016/S1769-7255(08)74238-X
更新日期：2008-10-01 00:00:00
abstract：:High blood pressure during renal disease is highly prevalent and an important risk factor for cardiovascular disease and renal progression. Its optimal management is therefore necessary to improve the prognosis of patients. Several trials concerning the blood pressure target in patients with chronic non-diabetic kidne...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：10.1016/j.nephro.2018.01.001
更新日期：2018-11-01 00:00:00
abstract：:Cohort studies, mainly US, show that vitamin D deficiency is more common in African-American population. Social and environmental factors play a role but the difference in skin color is essential. Despite low 25-hydroxyvitamin D concentrations, a lower risk of fragility or fracture exists in these populations. Vitamin...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：10.1016/j.nephro.2013.03.012
更新日期：2013-11-01 00:00:00
abstract：BACKGROUND:Online hemodiafiltration (oHDF) is increasingly used in children; we treated 28 children since 2009, adapting this technique to pediatric patients. METHODS:In this service evaluation audit, we assessed plasma electrolytes to evaluate the evolution of total (tCa) and ionized (iCa) during a session, as well a...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2015.03.006
更新日期：2015-11-01 00:00:00
abstract：:While the CKD-EPI (for Chronic Kidney Disease Epidemiology) equation is now implemented worldwide, utilization of the Cockcroft formula is still advocated by some physicians for drug dosage adjustment. Justifications for this recommendation are that the Cockcroft formula was preferentially used to determine dose adjus...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2015.07.472
更新日期：2016-02-01 00:00:00
abstract：:About an observation of aspergillus sinusitis in an hemodialyzed patient, correlation between chronic maxillary sinusitis, its aspergillus origin and previous dental treatments is emphasized. Eventual severe complications then evolved in immunocompromised patients justify having the knowledge of this possible patholog...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2006.01.003
更新日期：2006-05-01 00:00:00
abstract：:Chronic kidney disease is known to be associated with phosphate retention. The mechanisms are complex and the early increase in serum phosphate levels in chronic kidney disease is not strictly related to the dietary phosphate load or to the degree of phosphate retention. It also implicates the activity of intestinal s...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：10.1016/j.nephro.2017.01.002
更新日期：2017-04-01 00:00:00
abstract：:Cytomegalovirus is common in adult recipients (prevalence of 40-90%). Children are typically seronegative but immunosuppression may prone to primary-infection or viral reactivation, with potentially severe consequences. CMV infection incidence in pediatric kidney transplant recipients has seldom been investigated. The...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2018.04.003
更新日期：2019-03-01 00:00:00
abstract：:A patient is admitted in hospital to explore a nephrotic proteinuria associated with milky urine. This is explained by a chyluria (presence of lymphatic fluid in the urines), which is due to a pyelolymphatic fistula probably linked to a lymphatic filariasis. Usually, the diagnosis of chyluria can be confirmed by the p...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：10.1016/j.nephro.2009.06.003
更新日期：2009-12-01 00:00:00
abstract：BACKGROUND:Self-care education can play an important role in improving the quality of life in hemodialysis patients. PURPOSE:The purpose of this study was to determine the effect of self-care education with teach back method on the quality of life in hemodialysis patients. METHODS:In this pre- and post-test clinical ...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2020.01.002
更新日期：2020-07-01 00:00:00
abstract：:Buerger's disease or thombo-angiitis obliterans disease is a small vessel's vasculitis, frequently observed in young and smoker's males. Diagnosis is based on both clinical and radiological arguments. There is no specific treatment designed for this disease. We report the case of 43 years old patient presenting with a...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2017.10.006
更新日期：2018-05-01 00:00:00
abstract：:Continuous renal replacement therapy (CRRT) with a high cutoff (HCO) membrane is proposed for septic shock as a blood purification technique. The aim of this therapy is to modulate the immune response through the increase of the clearances of the inflammatory mediators as compared to standard CRRT. The use of HCO memb...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2013.07.370
更新日期：2013-12-01 00:00:00
abstract：:Since the introduction of the first magnesium pharmaceutical specialty in 1928, multiple medical indications were attributed to magnesium supplementation, despite rigorous scientific researches, leading to a very confuse therapeutic chapter. Real hypomagnesemia, acute or chronic, is rare, however oral magnesium drugs ...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：10.1016/j.nephro.2013.09.004
更新日期：2014-02-01 00:00:00
abstract：:Mitochondrial disorders are genetic defects of oxidative phosphorylation. Oxidative phosphorylation takes place in the mitochondrial inner membrane and consists of the oxidation of fuel molecules by oxygen and the concomitant energy transduction into ATP. The mitochondrial respiratory chain is a complex metabolic path...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2012.10.004
更新日期：2013-04-01 00:00:00
abstract：:Low salt diet for dialysis patients was recommended at the very beginning of dialysis treatment. It was issued from Kempner and Guyton studies who have established the relationship between salt, hypertension, and the role of the kidney in sodium balance. This recommendation vanished from dialysis literature during the...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/s1769-7255(07)80022-8
更新日期：2007-09-01 00:00:00
abstract：:Overweight, as well as obesity have become mass phenomena with an ever increasing prevalence in most countries all over the world, and are associated with a greater cardiovascular risk and mortality. Weight excess is usually directly related to an additional risk of morbidity and mortality in the general population. I...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：10.1016/S1769-7255(09)75167-3
更新日期：2009-07-01 00:00:00
abstract：:Hypertension in subjects on long term dialysis is frequent. Its origins are found in extracellular volume overload, which is complicated by increased peripheral arterial resistance. The latter is affected by many systems, including that of renin-angiotensin, endothelin, nitric oxide, the sympathetic nervous system, an...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：10.1016/s1769-7255(07)80630-4
更新日期：2007-10-01 00:00:00
abstract：AIM:Post-streptococcal glomerulonephritis (PSGN) is a frequent cause of acute nephritis in children. This study aimed to describe the epidemiology, clinical characteristics and outcomes of PSGN and look for predictor's factors of severity. METHODS:A 12-year retrospective review of case notes and laboratory data was co...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2018.04.001
更新日期：2018-12-01 00:00:00
abstract：:For ten years, an increase in the number of elderly patients on renal transplant waiting lists has occured. In an attempt to close the widening gap between supply and demand and because the demand for kidneys for younger patients already surpasses the supply, transplant physicians nowadays accept organs from older don...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2005.12.004
更新日期：2006-02-01 00:00:00
abstract：:Nephrologists should be aware the fact that the angioedema is a common side effect not only under angiotensin-converting enzyme (ACE) inhibitors treatment but also under sartans therapy. The frequency of angioedema under ACE inhibitors is estimated at 1 to 7 per thousand. The physiopathology of ACE angioedema implicat...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：10.1016/j.nephro.2007.03.003
更新日期：2007-06-01 00:00:00
abstract：:Clinical nephrology in the european french-speaking countries made great strides just after the 2nd World War. The "Société de Pathologie Rénale" was founded and assembles French, Belgian and Swiss nephrologists. The first studies were physiopathological and bore on acute uremia and the associated hydromineral disorde...
journal_title：Nephrologie & therapeutique
pub_type： 历史文章,杂志文章
doi：10.1016/j.nephro.2009.01.005
更新日期：2009-06-01 00:00:00
abstract：AIM:The aim of the study is to analyse haemodialysis patients' prescriptions accordance with legal registrations in 64 chronic haemodialysis patients during a one-month follow-up period. RESULTS:Patients are taking 9.6+/-3 different drugs. A mean of 2.4+/-1.3 prescriptions per patient disagree with the recommended dru...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,多中心研究
doi：10.1016/j.nephro.2005.08.002
更新日期：2005-10-01 00:00:00
abstract：INTRODUCTION:Morocco stays far behind other countries in the domain of organ donation and transplantation. Improving the knowledge of Moroccan students, about organ donation and transplantation, can be a key factor in the development of transplant activity. The aim of this study is to evaluate the knowledge, attitudes ...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2015.02.003
更新日期：2015-07-01 00:00:00
abstract：:In France, 1 adult out of 3 is affected by hypertension and only 1 hypertensive out of 4 achieves blood pressure targets (<140/90mmHg). This proportion is significantly better in similar countries (e.g. England, Germany, the USA). Nephrologists are particularly concerned since although more than 90 % of Chronic Kidney...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2020.10.001
更新日期：2020-11-01 00:00:00
abstract：:Due to a national theoretical training provided to all resident for many years and the ongoing discussions to change the organization of the resident training of specialized study diploma (SSD) of nephrology, a survey was done to assess the perception of skills acquired in the management of different clinical situatio...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2017.01.026
更新日期：2017-12-01 00:00:00
abstract：UNLABELLED:Ethylene glycol is present predominantly in antifreeze,and in industrial solvents. Accidental ingestion of ethylene glycol is relatively rare, but may be potentially lethal. It results in a depression of the central nervous system, a severe metabolic acidosis and an acute renal failure by tubular precipitati...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2008.12.003
更新日期：2009-06-01 00:00:00
abstract：:The optimal method to assess the adequacy of peritoneal dialysis therapies is controversial. Today, the adequacy must not be considered as a number or a concept assessed only by two parameters (total KT/V urea and total solute clearance) but defined by many more items. In the absence of data, based on theoretical cons...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2013.05.005
更新日期：2013-11-01 00:00:00
abstract：:Chronic kidney disease (CKD) is a worldwide public health problem. Little is known about its burden in Africa. This paper reviews the knowledge of CKD in Kinshasa, summarizing four studies undertaken in the general population and traditional health system of Kinshasa. CKD was defined by either kidney damage (proteinur...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,meta分析
doi：10.1016/j.nephro.2010.03.008
更新日期：2010-07-01 00:00:00
abstract：:The management of lupus nephritis remains a clinical problem in children as in adults. Corticosteroids, cyclophosphamide and azathioprine have been used with satisfactory response, but the most important problems are their potential toxicities. Therefore, we evaluate the use of mycophenolate mofetil (MMF) as a new age...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2010.07.001
更新日期：2010-12-01 00:00:00
