Abstract:
:Distal renal tubular acidosis (dRTA) results from impaired urinary acidification by the renal collecting duct. Acquired dRTA can be secondary to diverse pathological processes, including diabetic, ischemic, fibrosing, or immunological processes; less frequently it presents as a familial disorder with either an autosomal recessive or dominant pattern of transmission. Mutations in the SLC4A1/AE1/band 3 Cl(-)/HCO(3)(-) exchanger gene have been identified as causes for both dominant and recessive forms of dRTA. These mutations comprise a group almost entirely distinct from the SLC4A1 mutations that underlie the familial hemolytic anemia of hereditary spherocytosis. Why does one group of mutations express almost exclusively an isolated erythroid phenotype, whereas the second group of mutations expresses almost exclusively a phenotype explicable entirely by defective function of renal collecting duct type A intercalated cells? This review summarizes current research addressing this central question in the pathobiology of inherited dRTA associated with mutations in the SLC4A1 gene. Studying dRTA-associated mutant AE1 polypeptides can provide novel insights into the biology of the intercalated cell and the collecting duct as well as more generally into mechanisms by which epithelial cells generate and maintain functional polarity.
journal_name
Clin Exp Nephroljournal_title
Clinical and experimental nephrologyauthors
Shayakul C,Alper SLdoi
10.1007/s10157-003-0271-xsubject
Has Abstractpub_date
2004-03-01 00:00:00pages
1-11issue
1eissn
1342-1751issn
1437-7799journal_volume
8pub_type
杂志文章,评审abstract:BACKGROUND:The current (2012) histological classification of immunoglobulin A nephropathy was established using a case-control study of 287 patients. However, the risk of progression to end-stage renal disease (ESRD) has not been validated for the previous (2002) classification. This study aimed to determine whether th...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-013-0848-y
更新日期:2014-06-01 00:00:00
abstract:BACKGROUND:Hematuria is the first manifestation of urinary abnormality in immunoglobulin A nephropathy (IgAN). Hematuria has recently been reported as a risk factor for deterioration of renal function; however, its cause remains unknown. METHODS:We analyzed the surface marker of peripheral blood mononuclear cells befo...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-014-0968-z
更新日期:2015-04-01 00:00:00
abstract::A case of nephrotic syndrome (NS) and acute renal failure (ARF) associated with embryonal rhabdomyosarcoma (RMS) in a 10-year-old boy is reported. Ultrasound revealed irregular, echogenic, circumferential urinary bladder base mass, bilateral hydroureter and hydronephrosis. Histopathology of percutaneous renal and uret...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-007-0015-4
更新日期:2008-04-01 00:00:00
abstract:BACKGROUND:Proteinuria caused by glomerular disease is characterized by podocyte injury. Vasopressin V2 receptor antagonists are effective in reducing albuminuria, although their actions on glomerular podocytes have not been explored. The objective of this study was to evaluate the effects of tolvaptan, a selective ora...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-009-0196-0
更新日期:2009-10-01 00:00:00
abstract:BACKGROUND:The renoprotection achieved by angiotensin II blockade in the treatment of diabetic nephropathy is well established in both the clinical and the experimental settings. In contrast, the therapeutic efficacy of calcium channel blockers (CCBs) in the treatment of diabetic nephropathy still remains controversial...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-004-0303-1
更新日期:2004-09-01 00:00:00
abstract:BACKGROUND:Serum anion gap (AG) has recently been proven to represent a biomarker for predicting prognosis in patients with end-stage renal disease (ESRD). However, whether change in AG (ΔAG) at the time of starting hemodialysis predicts mortality after starting hemodialysis in elderly patients with ESRD remains unknow...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-019-01844-0
更新日期:2020-05-01 00:00:00
abstract:BACKGROUND:Adrenomedullin (AM) has antiproliferative and proapoptotic effects on mesangial cells in culture, but the regulation of AM and its receptors in glomeruli with glomerulonephritis have not been clarified. METHODS:We examined sequential changes in the mRNA expression of AM and its receptors (receptor-activity-...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-004-0309-8
更新日期:2004-12-01 00:00:00
abstract:BACKGROUND:Dotinurad is a novel selective urate reabsorption inhibitor (SURI) that selectively inhibits the reabsorption of uric acid in renal tubules and promotes the excretion of uric acid into urine. In this study, the effects of age and gender on the pharmacokinetics (PK), pharmacodynamics (PD), and safety of dotin...
journal_title:Clinical and experimental nephrology
pub_type: 临床试验,杂志文章
doi:10.1007/s10157-019-01836-0
更新日期:2020-03-01 00:00:00
abstract:BACKGROUND:Genetic susceptibility to atypical hemolytic uremic syndrome (aHUS) may lie within genes regulating or activating the alternate complement and related pathways converging on endothelial cell activation. METHODS:We tested 32 Indian patients of aHUS negative for antibodies to complement factor H for genetic v...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-017-1478-6
更新日期:2018-06-01 00:00:00
abstract:BACKGROUND:The Japanese classification of diabetic nephropathy reflects the risks of mortality, cardiovascular events and kidney prognosis and is clinically useful. Furthermore, pathological findings of diabetic nephropathy are useful for predicting prognoses. In this study, we evaluated the characteristics of patholog...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章,多中心研究
doi:10.1007/s10157-017-1485-7
更新日期:2018-06-01 00:00:00
abstract:BACKGROUND:The post-marketing surveillance of edaravone has reported serious adverse reactions, including renal and hepatic disorders. Renal disorders were the most frequently reported serious/important adverse reactions, which led to the evaluation of their causation by edaravone, their severity, and the recovery of r...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-007-0495-2
更新日期:2007-12-01 00:00:00
abstract::A 69-year-old woman presented with unusual electron microscopic findings. The patient was admitted to ascertain the cause of her persistent proteinuria, and kidney biopsy was performed. While light microscopic findings and immunofluorescence study suggested membranous nephropathy, electron microscopic study showed mic...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-008-0089-7
更新日期:2008-12-01 00:00:00
abstract:BACKGROUND:Chronic kidney disease is a predictor of end-stage renal disease (ESRD) and cardiovascular disease (CVD). Therefore, the medical expenses are higher with the decrease in glomerular filtration rate (GFR). However, few studies have examined the medical expenses according to the baseline GFR. METHODS:We invest...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-012-0718-z
更新日期:2013-06-01 00:00:00
abstract:BACKGROUND:Tolvaptan slowed the rates of total kidney volume (TKV) growth and renal function decline over a 3-year period in patients with autosomal dominant polycystic kidney disease (ADPKD) enrolled in the Tolvaptan Efficacy and Safety in Management of Autosomal Dominant Polycystic Kidney Disease and Its Outcomes (TE...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-020-02009-0
更新日期:2021-01-20 00:00:00
abstract::Endothelial dysfunction due to the reduced bioavailability of nitric oxide (NO) is involved in the course of atherosclerotic cardiovascular disease as well as chronic kidney disease (CKD). NO is synthesized from L-arginine via the action of NO synthase, which is blocked by endogenous L-arginine analogues such as asymm...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章,评审
doi:10.1007/s10157-007-0471-x
更新日期:2007-06-01 00:00:00
abstract:BACKGROUND/AIMS:Fibroblast growth factor 23 (FGF23) and soluble α-Klotho are emerging potential biomarkers of phosphorus and vitamin D metabolism which change in concentration in early chronic kidney disease (CKD) in order to maintain normal phosphorus levels. Tubular reabsorption of phosphate (TRP) has been commonly u...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-014-0962-5
更新日期:2015-04-01 00:00:00
abstract:BACKGROUND:Depression is common in chronic kidney disease (CKD) patients and associated with significant increase in morbidity and mortality. In recent years, a relationship between vitamin D deficiency and depression has been shown. The aim of this study is to investigate the relationship between 25-hydroxy (OH) vitam...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-019-01794-7
更新日期:2020-01-01 00:00:00
abstract:BACKGROUND:Pulmonary-renal syndrome is characterized by pulmonary hemorrhage and rapidly progressive glomerulonephritis in various immunological states. Histopathological analysis of pulmonary-renal syndrome is not yet complete. METHODS:Wistar-Kyoto (WKY) rats were sensitized using the noncollagenous (NC1) domain of t...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-009-0260-9
更新日期:2010-04-01 00:00:00
abstract:BACKGROUND:In patients with IgA nephropathy (IgAN), recurrence after steroid pulse therapy is associated with reduced renal survival. However, the predictors of recurrence have not yet been clarified. METHODS:All patients who received 6-month steroid pulse therapy from 2004 to 2010 in our four affiliated hospitals and...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章,多中心研究
doi:10.1007/s10157-015-1170-7
更新日期:2016-06-01 00:00:00
abstract:BACKGROUND:Intravascular administration of iodinated contrast media continues to be a common cause of hospital-acquired acute kidney injury. Accumulating evidence suggests that radiocontrast agent-induced nephrotoxicity is associated with increased oxidative stress, which leads to renal tissue damage with DNA fragmenta...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-013-0774-z
更新日期:2013-12-01 00:00:00
abstract:BACKGROUND:It has been recently reported that peroxisome proliferator-activated receptors (PPARs)gamma exist in various tissues and that they exibit anti-inflammatory effects. METHODS:We investigated the effects of PPARgamma activators on the development of crescentic glomerulonephritis. Crescentic glomerulonephritis ...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s101570300003
更新日期:2003-03-01 00:00:00
abstract:BACKGROUND:Albuminuria is a biomarker for chronic kidney disease and an independent predictor of cardiovascular and all-cause mortality. A recent meta-analysis concluded that these risks increase with urinary albumin concentration, even when below the microalbuminuria threshold. Thus, minimizing urinary albumin may be ...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-015-1103-5
更新日期:2015-12-01 00:00:00
abstract:BACKGROUND:Dotinurad, a novel selective urate reabsorption inhibitor (SURI), reduces serum uric acid levels by selectively inhibiting urate transporter 1 (URAT1) for the treatment of hyperuricemia with or without gout. We confirmed the serum uric acid lowering effect and safety of dotinurad. METHODS:This was a confirm...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1007/s10157-019-01818-2
更新日期:2020-03-01 00:00:00
abstract::A 69-year-old Caucasian man was admitted to our hospital because of myocardial infarction. A central venous catheter (CVC) for infusive therapy was inserted. After two weeks he developed fever, purpura, and knee arthralgia. Hemoculture yielded methicillin-sensitive Staphylococcus aureus. Subsequently, oliguric renal f...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-007-0024-3
更新日期:2008-06-01 00:00:00
abstract:BACKGROUND:Cinacalcet markedly reduces the serum intact parathyroid hormone (PTH) level of hemodialysis (HD) patients with secondary hyperparathyroidism. Parathyroidectomy also reduces the serum intact PTH level of HD patients and it increases their bone mineral density (BMD). However, there is little information about...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-012-0665-8
更新日期:2013-02-01 00:00:00
abstract:BACKGROUND:Several comorbidity scoring systems have been developed and validated, mostly in western hemodialysis patients with a high risk of cardiovascular disease. The performance of comorbidity scoring, however, depends on the patient population. In this study, we determine the optimal comorbidity scoring system for...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-017-1418-5
更新日期:2018-02-01 00:00:00
abstract:BACKGROUND:Sirt1, a mammalian homolog of silent information regulator 2 (Sir2), is the founding member of class III histone deacetylase (HDAC). METHODS:In this study, we examined whether Sirt1 is involved in the modification of acetylated histone H3, acetylated p53 and Werner syndrome protein (WRN), which is stabilize...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-011-0421-5
更新日期:2011-06-01 00:00:00
abstract:BACKGROUND:The Stewart model for analyzing acid-base disturbances emphasizes serum albumin levels, which are ignored in the traditional Boston model. We compared data derived using the Stewart model to those using the Boston model in patients with nephrotic syndrome. METHODS:Twenty-nine patients with nephrotic syndrom...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-017-1387-8
更新日期:2017-10-01 00:00:00
abstract:BACKGROUND:Two randomized, double-blind, placebo-controlled trials (EPPIC-1 and EPPIC-2) investigated the efficacy and safety of AST-120, an oral spherical carbon adsorbent, in adults with chronic kidney disease (CKD). While the benefit of adding AST-120 to standard therapy was not supported by these trials, we perform...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1007/s10157-017-1447-0
更新日期:2018-04-01 00:00:00
abstract:BACKGROUND:Equations for the creatinine-based estimated glomerular filtration rate (eGFR) were recently established for Japanese adults (>18 years old) and children (2-11 years old), respectively, but it is unclear whether eGFR can be as useful as 24-h creatinine clearance (CCr) for assessing renal function in patients...
journal_title:Clinical and experimental nephrology
pub_type: 杂志文章
doi:10.1007/s10157-014-0976-z
更新日期:2015-04-01 00:00:00