Abstract：
BACKGROUND:Fabry disease is an X-linked generalized progressive debilitating lysosomal storage disorder. The disease usually manifests in childhood, but the diagnosis is often made following the occurrence of late-stage complications such as progressive kidney failure. In order to identify key novel renal diagnostic imaging features of Fabry disease, we conducted a cross sectional case-control study of kidney involvement in patients with Fabry disease. METHODS:Twenty-four patients (mean age 36.1 +/- 8.1 years, median 37 years, range 20 to 48 years) with chronic neuropathic pain who were enrolled in an enzyme replacement trial underwent prospective renal imaging evaluation with magnetic resonance imaging (MRI) and computed tomography (CT) at the National Institutes of Health, Bethesda, Maryland. We concurrently enrolled 19 age-matched healthy controls (mean age 34.6 +/- 12.0 years, median 33 years, range 18 to 61 years). The presence and localization of kidney cysts as well as the ratio of the signal intensity between medulla and cortex were determined. RESULTS:Fifty percent of the Fabry disease patients had renal sinus cysts, compared to one individual (7%) in the control group. The ratio of the signal intensity between medulla and cortex on T2-weighted scans in Fabry disease patients with sinus cysts was elevated compared with Fabry disease patients without cysts (P= 0.0083), and elevated in Fabry disease patients without cysts compared with normal controls without cysts (P= 0.0173). CONCLUSION:The finding of multiple renal sinus cysts in the workup of a patient with kidney disease patient should strongly suggest the consideration of Fabry disease in the differential diagnosis. The cause of such cysts in Fabry disease remains to be elaborated.
journal_name
Kidney Intjournal_title
Kidney internationalauthors
Ries M,Bettis KE,Choyke P,Kopp JB,Austin HA 3rd,Brady RO,Schiffmann Rdoi
10.1111/j.1523-1755.2004.00846.xsubject
Has Abstractpub_date
2004-09-01 00:00:00pages
978-82issue
3eissn
0085-2538issn
1523-1755pii
S0085-2538(15)50146-4journal_volume
66pub_type
杂志文章abstract：:Mitochondria are critical in determining a cell's energy homeostasis and fate, and mitochondrial dysfunction has been implicated in the pathogenesis of chronic kidney disease (CKD). We sought to identify causative mitochondrial microRNAs. A microarray screen of kidney tissue from healthy mice identified 97 microRNAs t...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1016/j.kint.2018.12.028
更新日期：2019-06-01 00:00:00
abstract：:Acute kidney injury (AKI), previously termed acute renal failure, is associated with increased mortality, prolonged hospital stay, and accelerated chronic kidney disease (CKD). Over the past 2 decades, dramatic rises in the incidences of AKI have been reported, particularly within the United States. The question arise...
journal_title：Kidney international
pub_type： 杂志文章,评审
doi：10.1038/ki.2014.293
更新日期：2015-01-01 00:00:00
abstract：:Progression of diabetic nephropathy appears directly related to renal tubulointerstitial injury, but the involved genes are incompletely delineated. To identify such genes, DNA microarray analysis was performed with RNA from renal proximal tubules (RPTs) of streptozotocin-induced diabetic Wistar rats, spontaneously di...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/sj.ki.5000206
更新日期：2006-03-01 00:00:00
abstract：:Metabolic changes due to chronic renal failure (CRF) were studied in isolated liver cells. In 14 CRF and 14 sham-operated rats, liver cells were isolated by the Berry and Friend method and incubated with various substrates in order to study gluconeogenesis, ureagenesis, ketogenesis, oxygen consumption as well as cytos...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1995.215
更新日期：1995-06-01 00:00:00
abstract：BACKGROUND:We evaluated the effects of different concentrations of iron dextran administered through the intraperitoneal route, in iron-deficient rats, on hematocrit (Hct in percentage), serum iron (mg/dL), total iron binding capacity (TIBC in mg/dL), and the function and histology of the peritoneal membrane. METHODS:...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.2001.059002764.x
更新日期：2001-02-01 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：:Disturbed pulsatile LH secretion has been suggested to play a role in the etiology of delayed puberty and disturbed reproductive function in chronic renal failure (CRF), but interpretation of gonadotropin secretion from plasma concentration measurements is confounded by alterations in hormone metabolic clearance. To s...
journal_title：Kidney international
pub_type： 杂志文章,多中心研究
doi：10.1038/ki.1994.191
更新日期：1994-05-01 00:00:00
abstract：:Viral peritonitis is an exceptionally rare occurrence in peritoneal dialysis. In fact, up to now, only one case report has been documented in the literature. In a prospective study, peritoneal dialysis effluent (PDE) was specifically cultured for the following viruses: the herpes group of viruses, including herpes sim...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1989.191
更新日期：1989-08-01 00:00:00
abstract：BACKGROUND:Segmental glomerular necrosis has been described in the biopsy material in a minority of patients with idiopathic IgA nephropathy in the oldest studies on this disease, but this marker of active capillaritis has received little attention in the subsequent literature, and its significance and relevance for th...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.2001.059002682.x
更新日期：2001-02-01 00:00:00
abstract：:Antioxidants have been used as therapies to decrease oxidative stress and improve CVD risk in hemodialysis (HD) patients. A systematic search of the Medline database (search date 30 April 2011) found 56 studies investigating the effects of antioxidant therapies on biomarkers of oxidative stress (53 studies) or clinica...
journal_title：Kidney international
pub_type： 杂志文章,评审
doi：10.1038/ki.2011.341
更新日期：2012-02-01 00:00:00
abstract：:Patients with chronic renal failure frequently have hypogonadism. To elucidate the molecular mechanisms involved, we tested the ability of serum from these patients to inhibit recombinant human luteinizing hormone receptors. Using a cell line expressing functional human luteinizing hormone receptors, we found that ade...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1997.109
更新日期：1997-03-01 00:00:00
abstract：BACKGROUND:Unilateral ureteral obstruction (UUO) leads to interstitial fibrosis of the obstructed kidney, and TGF-beta is considered to play an important role in this fibrotic process. Smad7 has been recently identified as an antagonist of TGF-beta signaling. To investigate whether this novel molecule can be exploited ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.2002.0610s1094.x
更新日期：2002-01-01 00:00:00
abstract：:Metabolic acidosis is common in patients with chronic kidney disease, which is known to affect bone metabolism. We examined the effect of metabolic acidosis on the development of vascular and other soft-tissue calcifications in uremic rats treated with calcitriol. Extraskeletal calcification was measured in vivo, in c...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/sj.ki.5002646
更新日期：2008-02-01 00:00:00
abstract：:Kidney fibrosis is the common consequence of chronic kidney diseases that inexorably progresses to end-stage kidney disease with organ failure treatable only with replacement therapy. Since transforming growth factor-β1 is the main player in the pathogenesis of kidney fibrosis, we posed the hypothesis that recombinant...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1016/j.kint.2020.05.041
更新日期：2020-11-01 00:00:00
abstract：:We have studied the effect of neutralizing the surface charge of rat glomerular epithelial cells (GEC) in culture on prostanoid production. Incubation of rat GEC with polycations poly-L-lysine (PL) resulted in a dose dependent increase of 6-keto-PGF1 alpha (up to 8 to 10-fold) and PGE2 (up to 7 to 8-fold) production. ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1987.171
更新日期：1987-07-01 00:00:00
abstract：BACKGROUND:Among the members of the small leucine-rich proteoglycan family, decorin, biglycan, and fibromodulin have been proposed to be potent modulators of transforming growth factor-beta (TGF-beta) activity, thereby playing an important role in the pathogenesis of fibrotic kidney diseases. Furthermore, decorin expre...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.2000.00317.x
更新日期：2000-10-01 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：BACKGROUND:Antineutrophil cytoplasm antibodies (ANCAs) are implicated in the pathogenesis of systemic vasculitis. We asked whether ANCA could induce nitric oxide (NO) release from human neutrophils and, if so, whether this NO production was dependent on NO synthase (NOS) activity. METHODS:Neutrophil NO production was ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.2001.059002593.x
更新日期：2001-02-01 00:00:00
abstract：BACKGROUND:Cellular and molecular mechanisms responsible for cisplatin-induced nephrotoxicity to renal tubular epithelial cells are not well understood. Although caspases play a critical role in the execution of the cell death pathway, their specific role in toxic injury to renal tubular epithelial cells has not been e...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.2001.00026.x
更新日期：2001-11-01 00:00:00
abstract：:Tenofovir, a widely prescribed antiretroviral medication for treatment of HIV-1 infection, is infrequently associated with renal dysfunction and biopsy findings of acute tubular necrosis. We examined the clinical and pathological findings in 13 cases of tenofovir nephrotoxicity (7 men and 6 women, mean age of 51.1±9.6...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.2010.318
更新日期：2010-12-01 00:00:00
abstract：:Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pneumonia emerged in Wuhan, China in December 2019. Unfortunately, there is a lack of evidence about the optimal management of novel coronavirus disease 2019 (COVID-19), and even less is available in patients on maintenance hemodialysis therapy than in the g...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1016/j.kint.2020.04.031
更新日期：2020-07-01 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
abstract：:Apoptosis mediated by verotoxins (VTs) has been identified in a renal carcinoma cell line, ACHN cells, which are an in vitro model of renal tubular epithelial cells. ACHN cells express the renal tubular marker CD24 as well as globotriaosyl ceramide/CD77, the receptor for VTs. VT binding to the ACHN cell surface was co...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.1998.00939.x
更新日期：1998-06-01 00:00:00
abstract：:The importance of the endothelial isoform of nitric oxide synthase (eNOS) has been well established. Endothelium-derived nitric oxide has been shown to be essential for vascular homeostasis and modulation of eNOS has thus become a target in prevention of cardiovascular disease. The role of the inducible form of nitric...
journal_title：Kidney international
pub_type： 杂志文章,评审
doi：10.1046/j.1523-1755.2002.00235.x
更新日期：2002-03-01 00:00:00
abstract：:The intracellular distribution of carbonic anhydrase was studied in nine human donor kidneys by the histochemical method of Hansson and by an immunofluorescence technique using antisera specific against the cytoplasmic isoenzymes HCA B and HCA C. Only HCA C was found in the renal tubules. Convoluted proximal tubules s...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1984.106
更新日期：1984-06-01 00:00:00
abstract：:One hundred six patients with terminal renal insufficiency and 29 medical personnel were given three doses of hepatitis B vaccine at an interval of 0, 1, and 6 months (Merck, Sharp and Dohme, West Point, Pennsylvania, part of a joint study no. 649). Chronic hemodialysis patients (N = 99) received 40 micrograms vaccine...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1984.18
更新日期：1984-01-01 00:00:00
abstract：:Glomerular podocytes are terminally differentiated cells, and podocyte loss is a common feature of progressive renal pathologies. In this issue, Romoli et al. focus on podocyte progenitors that were proposed to reside in the Bowman's capsule. They demonstrate that suppression of CXCL12/CXCR4 signaling activates pariet...
journal_title：Kidney international
pub_type： 评论,杂志文章
doi：10.1016/j.kint.2018.09.011
更新日期：2018-12-01 00:00:00
abstract：:The nature of the light chains in mesangial IgA deposits and serum IgA was studied in patients with IgA nephropathy. Immunofluorescence (IF) studies using murine monoclonal antibodies, rabbit and goat anti-human monospecific antisera were performed in kidney sections from 15 IgA nephritic patients with only IgA isotyp...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1988.37
更新日期：1988-02-01 00:00:00
abstract：UNLABELLED:Hereditary systemic amyloidosis associated with a new apolipoprotein AII stop codon mutation Stop78Arg. BACKGROUND:Mutations in the gene for apolipoprotein AII (apoAII) have recently been found to cause hereditary renal amyloidosis. In each case amyloid deposition has been associated with a peptide extensio...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.2003.00047.x
更新日期：2003-07-01 00:00:00
abstract：BACKGROUND:Recently, the in vivo pathogenic role of anti-neutrophil cytoplasm autoantibodies (ANCA) in ANCA-associated vasculitis has been challenged by Abdel-Salam et al. In their report, they observed that ANCA directed against proteinase 3 (PR3) cannot bind to their target autoantigen PR3 on circulating neutrophils ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1111/j.1523-1755.2005.00431.x
更新日期：2005-08-01 00:00:00
