Abstract:
BACKGROUND/AIMS:Current status of primary hyperoxaluria (PH) has not been surveyed in Japan. METHODS:Japanese patients with PH were reviewed in the published literature. RESULTS:Fifty-nine patients were diagnosed as PH from 1962 to 2003. The median ages both at diagnosis and at the onset of initial symptoms were 17 (range: 0.02-63) and 13 (range: 0-58) years, respectively. Twenty-nine (49%) patients were older than 20 years at diagnosis, among whom 26 (90%) already presented end-stage renal failure (ESRF) or soon evolved into ESRF. Among 30 (51%) diagnosed as PH under 20 years old, only 13 (43%) were already in a terminal stage of renal insufficiency. Ten patients were diagnosed as PH1 by liver biopsy. We identified two types of enzymatic phenotypes in 3 of those patients examined. In 1 case, immunoreactive SPT/AGT protein level was very low due to accelerated proteolysis, while in other 2 cases, the immunoreactivity was detected on mitochondria due to mistargeting. Of 9 cases having been subjected to kidney transplantation at a median age of 20 years (range 7.3-40.0), it was only 2 cases that were reported to be successful, while the median survival time of the kidney grafts being 1.4 years (range 0-7). Of 4 patients having undergone combined liver/kidney transplantations (at the ages of 1.3, 1.4, 9 and 41 years, respectively), the surgery was successful in 3 cases; in the remaining one case, however, rejection required removal of the transplanted kidney was observed. The overall survival ratio of all the 59 PH cases accounted for 77, 71 and 55% at 5, 10 and 20 years, respectively. CONCLUSION:Assuming that the majority of the 59 patients with PH reported was classified as PH1, it is postulated that morbidity of violent infantile PH1 in Japan might be less than those in the USA and Europe, and symptoms of elderly Japanese PH1 patients seem to be milder than those of Western patients. Establishment of an early detection system of PH1 and more popular application of combined liver/kidney transplantation deserve further study.
journal_name
Am J Nephroljournal_title
American journal of nephrologyauthors
Takayama T,Nagata M,Ichiyama A,Ozono Sdoi
10.1159/000086361subject
Has Abstractpub_date
2005-05-01 00:00:00pages
297-302issue
3eissn
0250-8095issn
1421-9670pii
86361journal_volume
25pub_type
杂志文章abstract:BACKGROUND:Serum protein profiling patterns can reflect the pathological state of a patient and therefore may be useful for clinical diagnostics. Here, we present results from a pilot study of proteomic expression patterns in hemodialysis patients designed to evaluate the range of serum proteomic alterations in this po...
journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000077409
更新日期:2004-03-01 00:00:00
abstract:BACKGROUND/AIM:While the familial nature of chronic kidney disease (CKD) has been recognized, it has primarily been defined from studies of first-degree relatives of selected sets of cases. The goal of this study is an evaluation of the familial clustering of end-stage renal disease (ESRD) and CKD mortality using a pop...
journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000092280
更新日期:2006-01-01 00:00:00
abstract:BACKGROUND/AIMS:HFE protein controls iron absorption and cycling, and HFE mutations influence iron status. The aim was to evaluate the effect of the HFE genotype on the need for iron and erythropoietin in Italian hemodialysis patients. METHODS:Ninety-six prevalent patients were evaluated at the time of enrolment and p...
journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000111387
更新日期:2008-01-01 00:00:00
abstract::We have compared the renal effects of ioxitalamate, ioxaglate, and iopamidol in patients with chronic renal failure. Sixty consecutive patients with an estimated creatinine clearance (ECRCl) less than 60 ml/min were randomly assigned to receive either ioxitalamate, iopamidol, or ioxaglate. All patients received 500 cm...
journal_title:American journal of nephrology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1159/000168328
更新日期:1991-01-01 00:00:00
abstract:BACKGROUND:Patients with chronic kidney disease (CKD) show increased cardiovascular morbidity. We hypothesized that vascular properties which can be routinely evaluated noninvasively are related to different stages of CKD and their clinical and biochemical characteristics. METHODS:Arterial vascular properties were qua...
journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000110091
更新日期:2008-01-01 00:00:00
abstract:BACKGROUND/AIM:Slc26a6 (PAT1, CFEX) is a major chloride/base exchanger located on the apical membrane of the kidney proximal tubule. The purpose of the present study was to examine the effect of Slc26a6 deletion on the apical Na+/H+ exchanger 3 (NHE3) in the straight segment (S3) of the proximal tubule, which is the ma...
journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000111826
更新日期:2008-01-01 00:00:00
abstract::The prejunctional beta-adrenoceptors present in the isolated perfused kidney of normotensive rats (NR) and spontaneously hypertensive rats (SHR) were compared and the effects of tertatolol on these receptors were investigated. Kidneys of NR (Wistar + Wistar-Kyoto [WKY]) and SHR, were continuously perfused with Tyrode ...
journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000167330
更新日期:1986-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000168284
更新日期:1991-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000046671
更新日期:2002-01-01 00:00:00
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journal_title:American journal of nephrology
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doi:10.1159/000166914
更新日期:1985-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000166576
更新日期:1982-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000013611
更新日期:2000-07-01 00:00:00
abstract:BACKGROUND:Conditionally immortalized podocytes are valuable research tools but are difficult to efficiently transfect and do not provide graded transgene expression. METHODS:Conditionally immortalized mouse podocyte cell lines were established employing a tetracycline-inducible system. Glomerular cells, isolated from...
journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000151770
更新日期:2009-01-01 00:00:00
abstract:BACKGROUND/AIMS:Cisplatin (CIS) induces nephrotoxicity partly through renal vasoconstriction and decreased glomerular filtration effects thought to involve adenosine acting on adenosine A(1) receptors (A1Rs). We studied the effect of the orally active, A1R antagonist tonapofylline (BG9928) on biochemical measures of re...
journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000248762
更新日期:2009-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000168348
更新日期:1991-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000080464
更新日期:2004-07-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000119742
更新日期:2008-01-01 00:00:00
abstract::A multicentre trial (11 nephrology centres) was carried out to test the effects of ibopamine, an orally active dopamine-like drug, on the progression of chronic renal failure. For a 2-year period 189 chronic renal failure patients (serum creatinine level 1.5-4.0 mg/dl) were observed. They were homogeneous for basic ne...
journal_title:American journal of nephrology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1159/000169049
更新日期:1996-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1159/000168491
更新日期:1992-01-01 00:00:00
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更新日期:2014-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章
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更新日期:2017-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章,评审
doi:10.1159/000084141
更新日期:2005-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000168839
更新日期:1995-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章,随机对照试验
doi:10.1159/000258688
更新日期:2010-01-01 00:00:00
abstract:BACKGROUND/AIMS:Although HIV-infected persons are at higher risk for acute kidney injury (AKI) during hospitalization compared with their uninfected counterparts, risk factors for AKI are not well-defined. We aimed to describe the evolving incidence of AKI among HIV-infected individuals and to identify important AKI ri...
journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000337151
更新日期:2012-01-01 00:00:00
abstract::A noninvasive method for the diagnosis of cardiac calcinosis, a life-threatening complication in hemodialysis patients with end-stage renal disease (ESRD), has not, as yet, been firmly established. We tested whether whole body scanning with 99m-technetium methylene diphosphonate (MDP) might visualize cardiac calcinosi...
journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000013601
更新日期:2000-07-01 00:00:00
abstract:BACKGROUND:Tacrolimus is mainly metabolized by cytochrome P450 3A5 (CYP3A5), which is expressed in the liver. However, CYP3A5 is also expressed in the kidney tissue and may contribute to local tacrolimus clearance in the kidney allograft. We aimed to evaluate the association between the allograft CYP3A5 genotype and tr...
journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000487857
更新日期:2018-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000484903
更新日期:2017-01-01 00:00:00
abstract:BACKGROUND:Dietary sodium may influence cognitive function through its effects on cerebrovascular function and cerebral blood flow. METHODS:The aim of this study was to evaluate the association of dietary sodium intake with cognitive decline in community-dwelling older adults. We also evaluated the associations of die...
journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000486580
更新日期:2018-01-01 00:00:00
abstract::This study describes 10 cases of IgM nephropathy in whom the main morphological findings consisted of diffuse mesangial deposition of IgM and varying degrees of mesangial cell proliferation. In addition, focal segmental sclerosis was present in 1 patient and global sclerosis in another. An ill-defined electron-dense d...
journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000167485
更新日期:1987-01-01 00:00:00