Abstract：
:Nephropathic cystinosis is a multisystem autosomal recessive disease caused by cystine accumulation, which is usually treated by oral cysteamine. In order to determine long-term effects of this therapy, we enrolled 86 adult patients (mean age 26.7 years) diagnosed with nephropathic cystinosis, 75 of whom received cysteamine. Therapy was initiated at a mean age of 9.9 years with a mean duration of 17.4 years. By last follow-up, 78 patients had end-stage renal disease (mean age 11.1 years), 62 had hypothyroidism (mean age 13.4), 48 developed diabetes (mean age 17.1 years), and 32 had neuromuscular disorders (mean age 23.3 years). Initiating cysteamine therapy before 5 years of age significantly decreased the incidence and delayed the onset of end-stage renal disease, and significantly delayed the onset of hypothyroidism, diabetes, and neuromuscular disorders. The development of diabetes and hypothyroidism was still significantly delayed, however, in patients in whom therapy was initiated after 5 years of age, compared with untreated patients. The life expectancy was significantly improved in cysteamine-treated versus untreated patients. Thus, cysteamine decreases and delays the onset of complications and improves life expectancy in cystinosis. Hence, cysteamine therapy should be introduced as early as possible during childhood and maintained lifelong.
journal_name
Kidney Intjournal_title
Kidney internationalauthors
Brodin-Sartorius A,Tête MJ,Niaudet P,Antignac C,Guest G,Ottolenghi C,Charbit M,Moyse D,Legendre C,Lesavre P,Cochat P,Servais Adoi
10.1038/ki.2011.277subject
Has Abstractpub_date
2012-01-01 00:00:00pages
179-89issue
2eissn
0085-2538issn
1523-1755pii
S0085-2538(15)55267-8journal_volume
81pub_type
杂志文章abstract：:We report the stepwise application of the RIFLE classification in 155,624 admissions in the UK Intensive Care National Audit & Research Centre Case Mix Programme database. The assumptions required to define RIFLE and their relationship with renal replacement therapy (RRT) and ICU mortality were assessed. Previous repo...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.2015.70
更新日期：2015-08-01 00:00:00
abstract：:X-ray films of the hand skeleton (mammography technique), serum chemistry, and quantitative bone histology (micromorphometry of undecalcified sections, iliac crest spongiosa) were compared in 25 patients on maintenance hemodialysis. The X-ray findings correlated better with serum PTH levels than with bone histology. O...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1978.45
更新日期：1978-04-01 00:00:00
abstract：:Human mesangial cells (MC) in culture, when stimulated by interleukin 1 alpha(IL-1 alpha) or tumour necrosis factor (TNF alpha), but not with lipopolysaccharide (LPS), express interleukin 8 (IL-8) mRNA, and both cell associated and extracellular IL-8. Dexamethasone treatment of mesangial cells induced partial inhibiti...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1991.184
更新日期：1991-07-01 00:00:00
abstract：:The neonatal proximal tubule has a lower rate of bicarbonate absorption than that of adults. This is due, in part, to a lower rate of apical membrane Na+/H+ antiporter activity. The purpose of these studies was to examine if thyroid hormone could be a factor in the maturational increase in Na+/H+ antiporter activity. ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.1998.00879.x
更新日期：1998-05-01 00:00:00
abstract：:This report of the NIH-supported National Continuous Ambulatory Peritoneal Dialysis (CAPD) Registry summarizes data on 7,404 patients treated with CAPD during the three-year period 1981 through 1983, or nearly one-half of patients treated in the USA who were treated with CAPD during this time. While age and diabetes m...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1985.141
更新日期：1985-08-01 00:00:00
abstract：:McAdoo et al. propose that patients with both circulating antiglomerular basement membrane antibody and antineutrophil cytoplasm antibody demonstrate a phenotype that lies between that of single-positive antiglomerular basement membrane disease and antineutrophil cytoplasm antibody-associated vasculitis. Specifically,...
journal_title：Kidney international
pub_type： 评论,杂志文章
doi：10.1016/j.kint.2017.04.024
更新日期：2017-09-01 00:00:00
abstract：BACKGROUND:Disorders that manifest hypokalemic metabolic alkalosis, such as Bartter's syndrome and Gitelman's syndrome, are caused by the malfunction of renal tubular electrolyte transporters. Bartter's syndrome may be linked to dysfunction of Na-K-2Cl cotransporter (NKCC2), renal outer medullary K channel (ROMK), or C...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.2003.00163.x
更新日期：2003-09-01 00:00:00
abstract：:Dysfunction and loss of podocytes (glomerular epithelial cells) are the hallmarks of focal segmental glomerulosclerosis (FSGS). In recent years, activation and proliferation of parietal epithelial cells (PECs) have been increasingly appreciated in FSGS. The functional role of PECs in FSGS is still a hotly debated issu...
journal_title：Kidney international
pub_type： 评论,杂志文章
doi：10.1038/ki.2013.66
更新日期：2013-06-01 00:00:00
abstract：:The parathyroid hormone response to hypocalcemia was investigated in hemodialysis patients with osteomalacia and compared to those with osteitis fibrosa. Hypocalcemia was induced during hemodialysis by employing a dialysate devoid of calcium. Patients with osteomalacia had a blunted maximum amino terminal parathyroid ...
journal_title：Kidney international
pub_type： 临床试验,杂志文章
doi：10.1038/ki.1983.168
更新日期：1983-09-01 00:00:00
abstract：:Obesity could affect associations between creatinine generation, estimated body surface area, and excretory burden, with effects on chronic kidney disease assessment. We therefore examined the impact of obesity on the performances of estimated glomerular filtration rate (eGFR), the urine albumin:creatinine ratio (ACR)...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.2014.112
更新日期：2014-12-01 00:00:00
abstract：:We have shown that cisplatin inhibits fatty acid oxidation, and that fibrate treatment ameliorates renal function by preventing the inhibition of fatty acid oxidation and proximal tubule cell death. Urine samples of mice treated with single injection of cisplatin (20 mg/kg body weight) were collected for 3 days and an...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/sj.ki.5000433
更新日期：2006-06-01 00:00:00
abstract：:Twelve patients with progressive renal failure were placed on a very low protein diet supplemented by an essential amino acid-keto acid mixture for six to twelve months. Total daily intake was 0.04 g nitrogen/kg and 50 kcal/kg. Eight subjects had a significant change in the slope of reciprocal plasma creatinine, becom...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1986.99
更新日期：1986-05-01 00:00:00
abstract：BACKGROUND:The new oral microemulsion formulation of cyclosporine (Neoral) possesses superior pharmacokinetics to the conventional formulation, Sandimmun (SIM), providing more complete and predictable absorption, and less pharmacokinetic variability. METHODS:The safety and tolerability of Neoral, together with the inc...
journal_title：Kidney international
pub_type： 临床试验,杂志文章,多中心研究,随机对照试验
doi：10.1046/j.1523-1755.1998.00042.x
更新日期：1998-09-01 00:00:00
abstract：:The incidence of malnutrition disorders in chronic kidney disease (CKD) appears unchanged over time, whereas patient-care and dialysis techniques continue to progress. Despite some evidence for cost-effective treatments, there are numerous caveats to applying these research findings on a daily care basis. There is a s...
journal_title：Kidney international
pub_type： 杂志文章,评审
doi：10.1038/ki.2011.118
更新日期：2011-08-01 00:00:00
abstract：BACKGROUND:IgA nephropathy is the most common glomerular disease. Mechanisms leading to its occurrence and controlling the evolution of the disease remain largely unknown. Various genetic factors have been found, mostly implicating immunologically relevant genes (IgH, TCR, human lymphocyte antigen, and complement loci)...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.2000.00253.x
更新日期：2000-09-01 00:00:00
abstract：BACKGROUND:Renal inflammation is regulated by a network of local and systemic mediators. Of them, transforming growth factor-beta1 (TGF-beta 1) and glucocorticoids play an important role in deactivating monocytes/macrophages. We examined the hypothesis that TGF-beta 1 effects may be partially achieved through modulatio...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.63.6s.3.x
更新日期：2003-06-01 00:00:00
abstract：:The renal effects of a single intravenous dose of two different E. coli lipopolysaccharides (LPS 0111:B4 and LPS 0127:B8), at the same dose of 100 micrograms/kg, were evaluated in euvolemic Munich-Wistar (MW) rats by whole kidney clearance techniques and micropuncture studies. Following LPS infusion, a significant dec...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1989.232
更新日期：1989-10-01 00:00:00
abstract：:Effect of insulin-like growth factor-1 (IGF) on the synthesis of glomerular proteoglycans (PGs) in an ex vivo recirculating organ perfusion system was investigated. Kidneys were perfused with a medium (approximately 80 ml) containing [35S]-sulfate (250 microCi/ml) and IGF (62.5 to 625 ng/ml). After radiolabeling, a sm...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1992.188
更新日期：1992-05-01 00:00:00
abstract：:Interstitial monocytic infiltration of the kidney occurs within hours of acute kidney injury and is an important determinant of functional decline and fibrosis. Li et al. used several surface markers to distinguish between dendritic cells and inflammatory monocytes following acute kidney injury and to identify two che...
journal_title：Kidney international
pub_type： 评论,杂志文章
doi：10.1038/ki.2008.555
更新日期：2008-12-01 00:00:00
abstract：:To determine the prognosis and risk factors for ischemic heart disease in chronic uremia, a cohort of 432 dialysis patients were followed prospectively from start of dialysis therapy until death or renal transplantation. Baseline demographic, clinical and echocardiographic data were obtained. After the initiation of d...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1996.201
更新日期：1996-05-01 00:00:00
abstract：:The renal growth hormone--insulin-like growth factor-I system in acute ischemic renal failure. Recovery from acute tubular necrosis (ATN) is accelerated by IGF-I therapy. Furthermore, the local renal growth hormone-IGF-I system may participate in the natural repair. We examined the IGF-I system in rat kidneys subjecte...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1995.230
更新日期：1995-06-01 00:00:00
abstract：:A newly described glomerulotubular feedback loop may explain the relationship between glomerular damage, epitope spreading, tubulointerstitial nephritis, proteinuria as a progression factor, and the importance of the local milieu in kidney damage. It also opens the horizons for exciting innovative approaches to therap...
journal_title：Kidney international
pub_type： 杂志文章,评审
doi：10.1038/ki.2009.489
更新日期：2010-03-01 00:00:00
abstract：:Australia's Indigenous people have high rates of chronic kidney disease and kidney failure. To define renal disease among these people, we reviewed 643 renal biopsies on Indigenous people across Australia, and compared them with 249 biopsies of non-Indigenous patients. The intent was to reach a consensus on pathologic...
journal_title：Kidney international
pub_type： 杂志文章,多中心研究
doi：10.1038/ki.2012.307
更新日期：2012-12-01 00:00:00
abstract：:To define the pattern of recovery from postischemic acute renal failure (ARF), we performed clearance and micropuncture studies at intervals of 1, 2, 4, and 8 weeks following 60 min of complete unilateral renal artery occlusion in the rat. At 1 week, the inulin clearance (CIn) of the postischemic kidney was less than ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1979.112
更新日期：1979-08-01 00:00:00
abstract：:Membranous nephropathy is characterized by deposition of immune complexes along the glomerular basement membrane. PLA2R and THSD7A are target antigens in 70% and 1-5% of primary membranous nephropathy cases, respectively. In the remaining cases, the target antigen is unknown. Here, laser microdissection of glomeruli f...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1016/j.kint.2019.09.014
更新日期：2020-01-01 00:00:00
abstract：:Acute phosphate nephropathy (APhN) is a clinical pathological entity characterized by acute and subsequent chronic renal failure following exposure to oral sodium phosphate (OSP) bowel purgatives. Renal biopsy findings include acute and chronic tubular injury with prominent tubular and interstitial calcium phosphate d...
journal_title：Kidney international
pub_type： 杂志文章,评审
doi：10.1038/ki.2009.308
更新日期：2009-11-01 00:00:00
abstract：:The high serum fibroblast growth factor 23 (FGF23) levels in patients with acute kidney injury (AKI) and chronic kidney disease (CKD) are associated with increased morbidity and mortality. Mice with folic acid-induced AKI had an increase in bone FGF23 mRNA expression together with an increase in serum FGF23 and severa...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1016/j.kint.2018.02.026
更新日期：2018-08-01 00:00:00
abstract：:Tuberous sclerosis complex is a genetic disorder characterized by hamartomatous lesions in multiple organs, frequently involving the kidney. We conducted a retrospective review of the clinical and radiographic records of 167 patients with tuberous sclerosis to determine the frequency of renal disease, the likelihood o...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/sj.ki.5001853
更新日期：2006-11-01 00:00:00
abstract：BACKGROUND:The prevalence of cardiovascular disease (CVD) and inflammation is high in patients with end-stage renal disease (ESRD). Adiponectin is an adipocytokine that may have significant anti-inflammatory and anti-atherosclerotic effects. Low adiponectin levels have previously been found in patients with high risk f...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1111/j.1523-1755.2004.00370.x
更新日期：2004-01-01 00:00:00
abstract：:The possibility that hypercalciuria could cause calcium stone formation through a mechanism other than by increasing urinary saturation of stone-forming calcium salts was explored. The effect of increasing calcium concentration on the inhibitor activity against the spontaneous precipitation of calcium oxalate was exam...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1988.100
更新日期：1988-05-01 00:00:00
