Abstract:
:Cystinosis as a clinical entity is a progressive dysfunction of multiple organs caused by the accumulation of cystine in the tissues, leading, for example, to end-stage renal failure, diabetes, hypothyroidism, myopathy, and central nervous system deterioration. Brodin-Sartorius and colleagues present a long-term study on the impact of cysteamine therapy on these complications. The data show that cysteamine improves the outcome and complications of cystinosis but does not prevent them.
journal_name
Kidney Intjournal_title
Kidney internationalauthors
Cherqui Sdoi
10.1038/ki.2011.301subject
Has Abstractpub_date
2012-01-01 00:00:00pages
127-9issue
2eissn
0085-2538issn
1523-1755pii
S0085-2538(15)55268-Xjournal_volume
81pub_type
评论,杂志文章abstract:BACKGROUND:The T allele of the G protein beta3 subunit (GNB3) C825T polymorphism has been associated with increased signal transduction, increased activity of the kidney Na+/H+ exchanger, and also with late-onset essential hypertension. Hypertension is a strong independent risk factor for progression in IgA nephropathy...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1111/j.1523-1755.2004.00734.x
更新日期:2004-07-01 00:00:00
abstract::Gap junctions are present in the juxtaglomerular apparatus enabling intercellular communication. Our study determined the location of different connexin subtypes within the juxtaglomerular apparatus of the rat, and the role of these subtypes in renal hemodynamics through the use of specific mimetic peptides. Immunohis...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5002673
更新日期:2008-02-01 00:00:00
abstract:BACKGROUND:Renal salt wasting and hypotension are some of the frequent complications in patients treated with cis-diamminedichloroplatinum (II) (cDDP), and it is suggested that cDDP produces an abnormality in the renin-angiotensin system. However, not only the underlying mechanism but also prophylactic treatment of thi...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.2000.00307.x
更新日期:2000-10-01 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::We compared the effects of calcitriol and doxercalciferol, in combination with either calcium carbonate or sevelamer, on bone, mineral, and fibroblast growth factor-23 (FGF-23) metabolism in patients with secondary hyperparathyroidism. A total of 60 pediatric patients treated with peritoneal dialysis were randomized t...
journal_title:Kidney international
pub_type: 杂志文章,随机对照试验
doi:10.1038/ki.2010.352
更新日期:2011-01-01 00:00:00
abstract::Lipid abnormalities have been suggested as a major cause of the accelerated atherosclerosis and the high incidence of coronary heart disease in chronic renal failure patients. In the present work the postprandial lipoprotein metabolism was studied in chronic dialysis patients with or without fasting hypertriglyceridem...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1992.411
更新日期:1992-11-01 00:00:00
abstract::This study was done to investigate the mechanisms that underly the changes of renal renin gene expression upon hypoperfusion of one kidney. To this end the left renal arteries of male Sprague-Dawley rats were clipped with 0.2 mm silver clips and renal renin mRNA levels were assayed by RNase protection during the first...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1994.440
更新日期:1994-12-01 00:00:00
abstract::The vascular endothelium normally is maintained in a quiescent state, but under certain conditions it is induced to undergo marked changes in behavior and form new vascular structures. A complex interaction among various growth and differentiation factors and the extracellular milieu regulates this behavior. One serie...
journal_title:Kidney international
pub_type: 杂志文章,评审
doi:10.1038/ki.1993.5
更新日期:1993-01-01 00:00:00
abstract:BACKGROUND:Several examinations have been performed to identify the genes involved in chronic renal failure using 5/6 nephrectomized rats. Recently, many systematic techniques for examining molecular expression have been developed. They might also be effective in elucidating the molecular mechanism of progressive renal...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1111/j.1523-1755.2004.00704.x
更新日期:2004-07-01 00:00:00
abstract:BACKGROUND:Estradiol suppresses the synthesis of type I collagen by murine mesangial cells. However, neither the alpha 1(I) nor the alpha 2(I) collagen gene contains an estrogen-response element. Because estradiol modulates the transcription of several genes that lack an estrogen-response element but contain a regulato...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.1999.00376.x
更新日期:1999-04-01 00:00:00
abstract::Renal iron handling was characterized in three experimental models of the nephrotic syndrome: puromycin aminonucleoside, adriamycin and nephrotoxic serum. In adriamycin-induced nephrotic syndrome, which has previously been shown to result from alterations in pore size of the filtration barrier, the transferrin leak wa...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1990.130
更新日期:1990-06-01 00:00:00
abstract::Phagocyte-derived interleukin-12 (IL-12) is a key cytokine that induces the development of an effective Th1 type immune response in various inflammatory and infectious disorders. To determine the importance of IL-12 in the pathogenesis of autoimmune renal injury we examined the renal production of this heterodimeric c...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1997.10
更新日期:1997-01-01 00:00:00
abstract::Using in situ hybridization and immunocytochemistry we describe the renal localization of the PDGF alpha-receptor. PDGF alpha-receptor mRNA was uniformly present in human metanephric kidney in interstitial cells and vascular arcades that course through the blastema. PDGF alpha-receptor mRNA was present in some mesangi...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1997.157
更新日期:1997-04-01 00:00:00
abstract::Endostatin (EST), an antiangiogenic factor, is enriched in aging kidneys. EST is also an interactive partner of transglutaminase 2 (TG2), an enzyme that cross-links extracellular matrix proteins. Here we tested whether EST and TG2 play a role in the fibrosis of aging. In wild-type mice, aging kidneys exhibited a 2- to...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1016/j.kint.2016.01.030
更新日期:2016-06-01 00:00:00
abstract::The autoregulatory ability of the renal vascular system in the split hydronephrotic kidney was quantitated with intravital microscopy. The luminal diameters of the arcuate and interlobular arteries as well as the afferent and efferent arterioles were measured. Glomerular blood flow was determined by the dual slit tech...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1989.104
更新日期:1989-05-01 00:00:00
abstract::Lysyl oxidase like-2 (LOXL2) is an amine oxidase with both intracellular and extracellular functions. Extracellularly, LOXL2 promotes collagen and elastin crosslinking, whereas intracellularly, LOXL2 has been reported to modify histone H3, stabilize SNAIL, and reduce cell polarity. Although LOXL2 promotes liver and lu...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1016/j.kint.2018.02.024
更新日期:2018-08-01 00:00:00
abstract:BACKGROUND:We have recently found evidence for increased reactive oxygen species (ROS) in rats with lead-induced hypertension. We hypothesized that increased ROS activity may contribute to hypertension by enhancing inactivation of nitric oxide (NO) in this model. METHODS:Rats were treated for 12 weeks with either lead...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.1999.00670.x
更新日期:1999-10-01 00:00:00
abstract:BACKGROUND:The "intact" parathyroid hormone (PTH) assay recognizes PTH-(1-84) as well as amino terminally truncated PTH fragments, that is, large carboxyterminal PTH fragments (C-PTH fragments). The present study investigated whether the use of the plasma PTH-(1-84)/C-PTH fragment ratio enhances the noninvasive assessm...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.2001.00949.x
更新日期:2001-10-01 00:00:00
abstract::Transepithelial fluid secretion promotes the progressive enlargement of cysts in autosomal dominant polycystic kidney disease (ADPKD). Recent indirect evidence indicated that active chloride transport may drive net fluid secretion in cultures of epithelia derived from ADPKD cysts. We now report that forskolin, which s...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1996.304
更新日期:1996-07-01 00:00:00
abstract::Innate lymphoid cells are a recently recognized group of immune cells with critical roles in tissue homeostasis and inflammation. Regulatory innate lymphoid cells are a newly identified subset of innate lymphoid cells, which play a suppressive role in the innate immune response, favoring the resolution of intestinal i...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1016/j.kint.2019.07.019
更新日期:2020-01-01 00:00:00
abstract::Acid (H+) retention appears to contribute to progressive decline in glomerular filtration rate (GFR) in patients with chronic kidney disease (CKD), including some patients without metabolic acidosis. Identification of patients with H+ retention but without metabolic acidosis could facilitate targeted alkali therapy; h...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1016/j.kint.2018.11.033
更新日期:2019-05-01 00:00:00
abstract:BACKGROUND:Various gene transfer vectors as well as delivery systems have been developed; however, many problems remain to be solved. We already achieved a technique to introduce genes into glomerular mesangial cells by hemagglutinating virus of Japan (HVJ) liposome-mediated gene transfer via renal artery. The main lim...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.2001.0590041390.x
更新日期:2001-04-01 00:00:00
abstract::The defect in urinary acidification induced by amphotericin B (AMB) was further characterized in turtle bladder. Since AMB has been shown to increase the hydrogen ion (H+) permeability of this epithelium in the absence of exogenous bicarbonate ions (HCO3-), we explored the permeability characteristics in the presence ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1977.39
更新日期:1977-04-01 00:00:00
abstract::IgG4-related disease is a recently recognized multi-organ disorder characterized by high levels of serum IgG4 and dense infiltration of IgG4-positive cells into several organs. Although the pancreas was the first organ recognized to be affected by IgG4-related disorder in the syndrome of autoimmune pancreatitis, we pr...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2010.271
更新日期:2010-11-01 00:00:00
abstract::Intestinal regulation of oxalate absorption is a complex mechanism, not exclusively reliant on the oxalate-degrading anaerobe Oxalobacter formigenes. Using metagenomics, Miller et al. were able to describe a network of bacterial taxa co-occurring with Oxalobacter formigenes in fecal samples from non-stone forming cont...
journal_title:Kidney international
pub_type: 评论,杂志文章
doi:10.1016/j.kint.2019.03.020
更新日期:2019-07-01 00:00:00
abstract::The survival of patients with diabetes mellitus in the general population has improved in recent years. Here we assessed whether similar trends have occurred in 1688 kidney recipients, including 413 with diabetes prior to transplant between 1996 and 2007. Compared to patients without diabetes, the 5-year mortality was...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2014.70
更新日期:2014-09-01 00:00:00
abstract::Glutamine accumulation in rat kidney was investigated by giving large loads of gluta, mate and aspartate and measuring glutamine changes in seven completely defined portions of the nephron and in small patches from the thin limb area and from the papilla. Both glutamate and aspartate (which is partly coverted to gluta...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1978.145
更新日期:1978-11-01 00:00:00
abstract::The vasopressin inhibitor tolvaptan is clinically effective in slowing growth of renal cysts and reduction in estimated glomerular filtration rate (eGFR) in autosomal dominant polycystic kidney disease (ADPKD), but these effects are mitigated by the associated polyuria. Changes of total kidney volume, eGFR, and sympto...
journal_title:Kidney international
pub_type: 评论,杂志文章
doi:10.1038/ki.2015.143
更新日期:2015-07-01 00:00:00
abstract::Our genetic hypercalciuric (GH) rats have been selected and inbred for 29 generations to maximize urine calcium (UCa) excretion compared to identical gender controls (Ctl). To determine the effect of the increased UCa on urinary supersaturation and stone formation, we pair fed 15 GH and 15 Ctl rats a standard 1.2% cal...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1995.468
更新日期:1995-12-01 00:00:00
abstract::It has been shown that somatostatin inhibits the antidiuretic action of vasopressin in toad urinary bladder in vitro and in dogs and rats in vivo. The presence of somatostatin-like immunoreactivity (SLI) in the urinary bladder and kidney of the toad has suggested the possibility that somatostatin may act as a local pa...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1983.223
更新日期:1983-12-01 00:00:00