Abstract:
:Rare autosomal dominant tubulointerstitial kidney disease is caused by mutations in the genes encoding uromodulin (UMOD), hepatocyte nuclear factor-1β (HNF1B), renin (REN), and mucin-1 (MUC1). Multiple names have been proposed for these disorders, including 'Medullary Cystic Kidney Disease (MCKD) type 2', 'Familial Juvenile Hyperuricemic Nephropathy (FJHN)', or 'Uromodulin-Associated Kidney Disease (UAKD)' for UMOD-related diseases and 'MCKD type 1' for the disease caused by MUC1 mutations. The multiplicity of these terms, and the fact that cysts are not pathognomonic, creates confusion. Kidney Disease: Improving Global Outcomes (KDIGO) proposes adoption of a new terminology for this group of diseases using the term 'Autosomal Dominant Tubulointerstitial Kidney Disease' (ADTKD) appended by a gene-based subclassification, and suggests diagnostic criteria. Implementation of these recommendations is anticipated to facilitate recognition and characterization of these monogenic diseases. A better understanding of these rare disorders may be relevant for the tubulointerstitial fibrosis component in many forms of chronic kidney disease.
journal_name
Kidney Intjournal_title
Kidney internationalauthors
Eckardt KU,Alper SL,Antignac C,Bleyer AJ,Chauveau D,Dahan K,Deltas C,Hosking A,Kmoch S,Rampoldi L,Wiesener M,Wolf MT,Devuyst O,Kidney Disease: Improving Global Outcomes.doi
10.1038/ki.2015.28subject
Has Abstractpub_date
2015-10-01 00:00:00pages
676-83issue
4eissn
0085-2538issn
1523-1755pii
S2157-1716(15)32277-2journal_volume
88pub_type
共识发展会议,杂志文章,实务指引abstract::Hyperlipidemia can induce or aggravate renal tubulointerstitial injury. Experiments in a complex rat model with chronic glomerulonephritis and long-standing, coexisting hyperlipidemia suggested that induction of xanthine oxidase (XO), with increased oxygen radical generation, is involved in aggravation of tubulointers...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5000121
更新日期:2006-02-01 00:00:00
abstract::Current characterization of the immune risk in renal transplant patients is only focused on the assessment of preformed circulating alloantibodies; however, alloreactive memory T cells are key players in mediating allograft rejection. Immune monitoring of antidonor alloreactive memory/effector T cells using an IFN-γ E...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2013.236
更新日期:2013-12-01 00:00:00
abstract::Healthy male rats were exposed to a chronic ingesta of an isotonic NaCl solution during a period of four months. Two different preparations were utilized to study the effect of this treatment on the active mechanisms of Na+ reabsorption of kidney proximal tubular cells: outermost kidney cortex slices (rich in proximal...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1986.216
更新日期:1986-10-01 00:00:00
abstract::Diabetic nephropathy shows a higher incidence in male subjects, which may in part be owing to genetic factors. The angiotensin II type 2 receptor (AT2), present in the renal glomerulus, may oppose the deleterious effects of the type I receptor (AT1) through vasodilatation and growth inhibition. We determined whether t...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5000348
更新日期:2006-05-01 00:00:00
abstract::Quartan malarial infection causes an immune complex nephritis in some individuals, which, once established, is sustained by mechanisms not yet fully explained, but which probably involve autoimmune processes. The presenting clinical and biochemical findings of the quartan malarial nephrotic syndrome are similar to tho...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1979.103
更新日期:1979-07-01 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::Acute kidney injury (AKI) severe enough to require dialysis is increasing and associated with high mortality, yet robust information about temporal epidemiology of AKI requiring dialysis in England is lacking. In this retrospective observational study of the Hospital Episode Statistics (HES) data set covering the enti...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2015.234
更新日期:2015-11-01 00:00:00
abstract::Vascular calcification is recognized as a major contributor to cardiovascular disease (CVD) in end stage renal disease (ESRD) patients. Susceptibility to vascular calcification is genetically determined and actively regulated by diverse inducers and inhibitors. One of these inducers, hyperphosphatemia, promotes vascul...
journal_title:Kidney international
pub_type: 杂志文章,评审
doi:10.1038/ki.2008.644
更新日期:2009-05-01 00:00:00
abstract::The contractile properties of recombinant human erythropoietin (rHuEPO) on isolated resistance vessels of renal and mesenteric vascular beds were studied in an in vitro model using a small vessel myograph. Under isometric conditions, rHuEPO caused a contraction of this vasculature in a concentration range between 10 U...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1991.31
更新日期:1991-02-01 00:00:00
abstract::We used the perfused hemicorpus preparation to measure individual rates of protein synthesis and degradation. Using fed animals, perfused either with or without insulin, muscle protein synthesis and hemicorpus protein degradation rates were similar, but myofibrillar protein degradation was clearly increased in the ure...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1986.119
更新日期:1986-06-01 00:00:00
abstract:BACKGROUND:Familial nephrotic syndrome (NS) has both autosomal dominant and recessive forms of inheritance. Recent studies in families with an autosomal dominant form of focal segmental glomerulosclerosis (FSGS) have been at odds concerning linkage to chromosome 19q13 (Mathis et al, Kidney Int 53:282-286, 1998; Winn et...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.2000.057003875.x
更新日期:2000-03-01 00:00:00
abstract::The Dietary Approaches to Stop Hypertension (DASH) diet lowers blood pressure, an important risk factor for chronic kidney disease (CKD) and end-stage renal disease (ESRD). However, it is unclear whether adherence to a DASH diet confers protection against future ESRD, especially among those with pre-existing CKD and h...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1016/j.kint.2018.12.027
更新日期:2019-06-01 00:00:00
abstract::In a rat model of glomerular immune injury induced by administration of anti-glomerular basement membrane antibody and resembling human rapidly progressive glomerulonephritis, we explored whether activation of inducible nitric oxide synthase (iNOS) regulates synthesis of eicosanoids originating from cyclooxygenation o...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.1998.00791.x
更新日期:1998-03-01 00:00:00
abstract::There is a paucity of studies describing malignancy-related mortality after kidney transplantation. To help quantify this, we extracted data for all kidney-alone transplant procedures performed in England between April 2001 and March 2012. Data linkage analysis was performed between Hospital Episode Statistics and the...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2013.458
更新日期:2014-06-01 00:00:00
abstract::Regardless of the source of injury, an imbalance in the control of mesangial cell proliferation appears to play a direct role in the degree of progressive renal injury and glomerulosclerosis. Some of the regulatory mechanisms include specific soluble or non-soluble extracellular factors and a complex array of receptor...
journal_title:Kidney international
pub_type: 杂志文章,评审
doi:10.1046/j.1523-1755.1999.00710.x
更新日期:1999-10-01 00:00:00
abstract:BACKGROUND:Transforming growth factor-beta (TGF-beta) is known to play an important role in the pathophysiology of renal tubular disease. Researchers have recently identified a novel mitogen-activated protein kinase kinase kinase (MAPKKK), TAK (TGF-beta activated kinase)1, which stimulates the MKK3/6-p38K pathway. The ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.1999.00665.x
更新日期:1999-10-01 00:00:00
abstract::Activation of cAMP signaling pathway was shown to inhibit some pathobiologic processes in mesangial cells (MC). We investigated whether adrenomedullin (ADM), a potent agonist of adenylate cyclase, is synthesized in MC and whether it can, via cAMP, suppress the generation of reactive oxygen metabolites (ROM) and prolif...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1997.413
更新日期:1997-10-01 00:00:00
abstract::Tubulointerstitial nephritis (TIN) is a disease pattern with heterogeneous causes. Recently a specific subtype of autoimmune TIN, IgG4-related TIN, has been identified that is part of systemic IgG4-related disease/ autoimmune pancreatitis. On biopsy, this TIN shows an IgG4+ plasma cell-rich infiltrate, akin to the pan...
journal_title:Kidney international
pub_type: 评论,杂志文章
doi:10.1038/ki.2010.342
更新日期:2010-11-01 00:00:00
abstract:BACKGROUND:The detailed mechanisms of glucocorticoid action in idiopathic nephrotic syndrome and progressive glomerulonephritides have not been clearly elucidated. The pharmacological actions of glucocorticoids are mediated by their binding to an intracellular protein, the glucocorticoid receptor (GR). The determinatio...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.1999.00503.x
更新日期:1999-07-01 00:00:00
abstract:BACKGROUND:Assessment of mRNA levels has the potential to predict renal outcome. The objective of this study was to optimize several steps in the protocol for obtaining cDNA from routine clinical kidney biopsy material. METHODS:RNA integrity was compared between different methods for extraction of RNA, synthesis of cD...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1111/j.1523-1755.2004.00366.x
更新日期:2004-01-01 00:00:00
abstract::The hypoxia-inducible factor-1 (HIF-1) was first described as a DNA binding activity that specifically recognizes an 8 bp hypoxia response element (HRE) known to be essential for oxygen-regulated erythropoietin gene expression. In electrophoretic mobility shift assays (EMSAs) HIF-1 DNA binding activity is only detecta...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1997.80
更新日期:1997-02-01 00:00:00
abstract::Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive enlargement of kidney cysts leading to chronic kidney disease (CKD) and end-stage renal disease (ESRD). Identification of an early biomarker that can predict progression of CKD is urgently needed. In an earlier Consortium for Radiolog...
journal_title:Kidney international
pub_type: 杂志文章,多中心研究
doi:10.1016/j.kint.2017.09.027
更新日期:2018-03-01 00:00:00
abstract::The natural course of idiopathic membranous nephropathy is variable, with some patients slowly progressing to renal failure while others maintain normal renal function over the entire time. Whether to treat this disease or not is controversial due to the lack of controlled data about the long-term effects of treatment...
journal_title:Kidney international
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1038/ki.1995.453
更新日期:1995-11-01 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::Antibody responses to antigens are impaired in humans and animals with chronic renal failure (CRF), and parathyroid hormone (PTH) inhibits Staphylococcus aureus Cowan I (SAC) or pokeweed mitogen (PWM)-induced antibody production by B cells from normal subjects. Since CRF is associated with secondary hyperparathyroidis...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1991.286
更新日期:1991-11-01 00:00:00
abstract:BACKGROUND:Altered mineral metabolism contributes to bone disease, cardiovascular disease, and other clinical problems in patients with end-stage renal disease. METHODS:This study describes the recent status, significant predictors, and potential consequences of abnormal mineral metabolism in representative groups of ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1111/j.1523-1755.2005.00185.x
更新日期:2005-03-01 00:00:00
abstract::It is known that in rodents recombinant human growth hormone (rhGH) and recombinant human insulin-like growth factor (rhIGF-1) increase renal mass. It is uncertain, however, whether renal mass increases in proportion to body growth, or whether renal growth is stimulated selectively. In 120 to 150 g female Sprague-Dawl...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1993.376
更新日期:1993-12-01 00:00:00
abstract::Antibodies against purified rat-kidney-cortex pyruvate carboxylase were raised in rabbits. These polyspecific antibodies recognize pyruvate carboxylase enzymes alone without cross reactivity with other carboxylases as detected by immunoblotting. The abundance of pyruvate carboxylase in the various renal fractions was ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1991.147
更新日期:1991-06-01 00:00:00
abstract::Aboriginal dialysis patients have reduced access to kidney transplantation. The reasons for this disparity are unknown. Tonelli et al. show that in Canada, residence location does not significantly impact on an Aboriginal dialysis patient's likelihood of receiving kidney transplantation. This Commentary explores the i...
journal_title:Kidney international
pub_type: 评论,杂志文章
doi:10.1038/sj.ki.5001762
更新日期:2006-09-01 00:00:00
abstract::Intrauterine growth retardation (IUGR) aggravates the course of acute mesangioproliferative glomerulonephritis (GN) in the rat. Observational studies in children suggest that IUGR may be associated with a severe course of kidney diseases such as IgA nephropathy. We tested the hypothesis that IUGR leads to aggravation ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5001966
更新日期:2006-12-01 00:00:00