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) and were followed during 7.4 ± 4.5 years. Hypercalciuria was detected in 23 patients and was related to acidosis (P=0.028), higher lactate levels (5.9 ± 3.5 versus 3.7 ± 1.7 mmol/L; P=0.013) and smaller height (-2.1 ± 1.5 SD versus -0.8 ± 1.5 SD; P=0.026). Urolithiasis was diagnosed in 7 cases. Glomerular disease (19/38) was more frequent in cases with severe hypertriglyceridemia (P=0.042) and occurred at an older age (P=0.007). Microalbuminuria occurred in 15/31 cases; ACE inhibitors were prescribed in only 8 cases. The frequency of renal complications did not differ according to the diet group (continuous enteral feeding or uncooked starch). Logistic regression concluded as risk factors: lactic acidosis for tubular disease and age>10 years for glomerular disease. CONCLUSIONS:Renal involvement is common in glycogen storage disease type I patients. Tubular abnormalities are precocious, related to lactic acidosis and may be detected by monitoring of urinary calcium. Glomerular hyperfiltration is the first stage of a progressive glomerular disease and is related to age. Practical implications for renal preservation are discussed based on our results and literature.
journal_name
Nephrol Therjournal_title
Nephrologie & therapeutiqueauthors
Ben Chehida A,Bensmaïl T,Ben Rehouma F,Ben Abdelaziz R,Azzouz H,Boudabbous H,Slim Abdelmoula M,Abdelhak S,Kaabachi N,Ben Turkia H,Tebib Ndoi
10.1016/j.nephro.2014.12.007subject
Has Abstractpub_date
2015-07-01 00:00:00pages
240-5issue
4eissn
1769-7255issn
1872-9177pii
S1769-7255(15)00062-0journal_volume
11pub_type
杂志文章abstract：:Definition and classification of acute renal failure evolved in recent years. The acronym "Acute Kidney Injury" replaces "Acute Renal Failure". The RIFLE classification spreads the AKI in three degrees of severity, and two degrees of disease duration. The group Acute Kidney Injury Network refines this classification i...
journal_title：Nephrologie & therapeutique
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journal_title：Nephrologie & therapeutique
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journal_title：Nephrologie & therapeutique
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abstract：:Recombinant human erythropoietin (rHuEPO) has transformed the management chronic renal failure (CKD) and considerably improved the outcome of patients on regular chronic dialysis. However, a significant number of patients fail to respond to high of Erythropoiesis-stimulating agents (ESAs) and several causes of inadequ...
journal_title：Nephrologie & therapeutique
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journal_title：Nephrologie & therapeutique
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abstract：:African-Americans exhibit an excess risk for chronic and end-stage kidney disease compared to the non-African populations. Two APOL1 genetic variants were shown to account for the majority of this racial disparity in glomerulopathies and other non-diabetic kidney disease. The high-risk genotype has only been reported ...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
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abstract：INTRODUCTION:Emphysematous pyelonephritis is a rare and severe form of acute pyelonephritis. It is defined as the presence of gas-producing bacteria in the kidney and in peri-nepheretic areas. We report a case of emphysematous pyelonephritis on a single kidney associated with urolithiasis. OBSERVATION:A 44-year-old wo...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：10.1016/j.nephro.2008.04.005
更新日期：2009-02-01 00:00:00
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：10.1016/j.nephro.2012.09.006
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：10.1016/j.nephro.2008.04.004
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：
更新日期：2006-01-01 00:00:00
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,多中心研究
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更新日期：2005-10-01 00:00:00
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,多中心研究
doi：10.1016/j.nephro.2013.11.004
更新日期：2014-04-01 00:00:00
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journal_title：Nephrologie & therapeutique
pub_type： 历史文章,杂志文章
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更新日期：2009-06-01 00:00:00
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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
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/S1769-7255(08)73645-9
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2009.06.002
更新日期：2009-12-01 00:00:00
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2018.02.003
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abstract：BACKGROUND:The end-stage renal disease (ESRD) in children has special features in terms of etiologies, therapeutic modalities and access to renal transplantation. In Tunisia, there are no data on the epidemiology of ESRD in children. The aim of our study was to describe epidemiology of ESRD among Tunisian children. ME...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,多中心研究
doi：10.1016/j.nephro.2015.09.001
更新日期：2016-06-01 00:00:00
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2020.05.001
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journal_title：Nephrologie & therapeutique
pub_type： 历史文章,杂志文章
doi：10.1016/j.nephro.2012.05.004
更新日期：2012-12-01 00:00:00
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2016.03.004
更新日期：2016-07-01 00:00:00
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：
更新日期：2006-11-01 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：:Plasma exchange is a non-selective apheresis technique that can be performed by filtration or centrifugation allowing rapid purification of high molecular weight pathogens. An immunosuppressive treatment is generally associated to reduce the rebound effect of the purified substance. Substitution solutes such as human ...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2016.12.002
更新日期：2017-02-01 00:00:00
abstract：:Beta cell replacement by pancreas or Langerhans islets transplantation is the only way to restore glucose homeostasis in type 1 diabetic patients. The counterpart is the need for long-term immunosuppression. These transplantations are therefore mainly indicated for patients candidates for kidney transplantation and fo...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2018.02.006
更新日期：2018-04-01 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：: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：:Focal segmental glomerulosclerosis is a common glomerular histological lesion, which is usually characterised by non-nephrotic range proteinuria or nephrotic syndrome. It may be idiopathic or occurs secondarily to drugs, diabetes, obesity or HIV nephropathy and other infections. Dasatinib, a tyrosine kinase inhibitor ...
journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2020.09.007
更新日期：2021-01-08 00:00:00
