Abstract：
BACKGROUND:Hyperoxaluria may be idiopathic, secondary, or due to primary hyperoxaluria (PH). Hepatic alanine:glyoxylate aminotransferase (AGT) or glyoxylate/hydroxypyruvate reductase (GR/HPR) deficiency causes PHI or PHII, respectively. Hepatic glycolate oxidase (GO) is a candidate enzyme for a third form of inherited hyperoxaluria. METHODS:Six children were identified with marked hyperoxaluria, urolithiasis, and normal hepatic AGT (N = 5) and GR/HPR (N = 4). HPR was below normal and GR not measured in one. Of an affected sibling pair, only one underwent biopsy. GO mutation screening was performed, and dietary oxalate (Diet(ox)), enteric oxalate absorption (EOA) measured using [13C2] oxalate, renal clearance (GFR), fractional oxalate excretion (FE(ox)) in the children, and urine oxalate in first-degree relatives (FDR) to understand the etiology of the hyperoxaluria. RESULTS:Mean presenting age was 19.2 months and urine oxalate 1.3 +/- 0.5 mmol/1.73 m2/24 h (mean +/- SD). Two GO sequence changes (T754C, IVS3 - 49 C>G) were detected which were not linked to the hyperoxaluria. Diet(ox) was 42 +/- 31 mg/day. EOA was 9.4 +/- 3.6%, compared with 7.6 +/- 1.2% in age-matched controls (P = 0.33). GFR was 90 +/- 19 mL/min/1.73 m2 and FE(ox) 4.2 +/- 1.4. Aside from the two brothers, hyperoxaluria was not found in FDR. CONCLUSIONS:These patients illustrate a novel form of hyperoxaluria and urolithiasis, without excess Diet(ox), enteric hyper-absorption, or hepatic AGT, GR/HPR deficiency. Alterations in pathways of oxalate synthesis, in liver or kidney, or in renal tubular oxalate handling are possible explanations. The affected sibling pair suggests an inherited basis.
journal_name
Kidney Intjournal_title
Kidney internationalauthors
Monico CG,Persson M,Ford GC,Rumsby G,Milliner DSdoi
10.1046/j.1523-1755.2002.00468.xsubject
Has Abstractpub_date
2002-08-01 00:00:00pages
392-400issue
2eissn
0085-2538issn
1523-1755pii
S0085-2538(15)48566-7journal_volume
62pub_type
杂志文章abstract：:Plasma newly-synthesized cholesteryl ester transfer (NCET) rate and concentrations of lipids, lipoproteins and apolipoproteins A1 and B were measured in chronic renal failure patients (dialysis independent and dialysis dependent), patients with a functioning renal transplant and in healthy control subjects with compar...
journal_title：Kidney international
pub_type： 临床试验,杂志文章
doi：10.1038/ki.1994.378
更新日期：1994-10-01 00:00:00
abstract：:While it is known that risk of death from sepsis is higher in patients with pre-existing chronic kidney disease its mechanism is unknown. To study this we established a two-stage mouse model where renal disease was first induced by folic acid injection followed by sub-lethal cecal ligation and puncture to induce sepsi...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.2008.346
更新日期：2008-10-01 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：:Incompatible donor/recipient pairs with broadly sensitized recipients have difficulty finding a crossmatch-compatible match, despite a large kidney paired donation pool. One approach to this problem is to combine kidney paired donation with lower-risk crossmatch-incompatible transplantation with intravenous immunoglob...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.2013.206
更新日期：2013-11-01 00:00:00
abstract：:Platelet-derived growth factor (PDGF)-D, a specific PDGF receptor β (PDGFR-β) ligand, mediates mesangial proliferation in vitro and in vivo. However, its role in renal development, physiology, and fibrosis is relatively unknown. In healthy murine kidneys, PDGF-D was found to be expressed on renal mesenchymal cells (me...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1016/j.kint.2015.12.037
更新日期：2016-04-01 00:00:00
abstract：:In organ transplantation, ischemia/reperfusion (I/R) results in damage that may affect cell viability and lead to organ failure. I/R injury involves a complex cascade of events, including loss of energy, derangement of the ionic hemostasis, production of reactive oxygen species, and cell death. In this context, mitoch...
journal_title：Kidney international
pub_type： 杂志文章,评审
doi：10.1111/j.1523-1755.2004.761_9.x
更新日期：2004-08-01 00:00:00
abstract：:About 75% of urinary stones contain oxalate. As Oxalobacter formigenes is a Gram-negative anaerobic bacterium that degrades oxalate in the intestinal tract, we assessed the role of O. formigenes in oxalate metabolism by evaluating its intestinal absorption, plasma concentration, and urinary excretion. Of 37 calcium ox...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.2013.104
更新日期：2013-06-01 00:00:00
abstract：BACKGROUND:Regular access monitoring is recommended to detect and treat access stenosis in order to prevent access thrombosis and failure. METHODS:In 1999, we instituted monthly access blood flow monitoring using the ultrasound dilution technique (UDT). In a sequential observational trial, 222 patients were studied fo...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1111/j.1523-1755.2005.00697.x
更新日期：2005-11-01 00:00:00
abstract：:In the course of studying the genetics of chlorambucil mutagenesis, we have uncovered a new model for autosomal polycystic kidney disease (PKD). In the homozygous condition, the gene, jcpk, causes a very severe disease characterized by cysts in all segments of the nephron. Death usually occurs before 10 days of age. E...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1995.69
更新日期：1995-02-01 00:00:00
abstract：:Levels of components of the classical and alternative complement pathways and the activity of the C3 nephritic factor (C3NeF) were measured in serum specimens from patients with type I (subendothelial deposits) and type II (intramembranous dense deposits) membranoproliferative glomerulonephritis (MPGN) and the results...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1976.6
更新日期：1976-01-01 00:00:00
abstract：:The in vitro heparin sensitivity of 18 nephrotic children was compared with that of 10 normal children and 13 children with other renal diseases. The influence of age on the heparin sensitivity of 52 normal subjects (aged 12 to 85 years) was also studied. The heparin sensitivity was calculated from the dose-response c...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1987.155
更新日期：1987-06-01 00:00:00
abstract：:Transgenic rats [TGR; strain name TGR(mRen2)27] harboring the mouse Ren-2 renin gene have been recently generated as a model for the study of primary hypertension that offers the advantage of a clearly-defined genetic alteration. Expression of the mouse Ren-2 gene causes severe hypertension (200 to 260 mm Hg) which is...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1992.4
更新日期：1992-01-01 00:00:00
abstract：BACKGROUND:Mice overexpressing thymic stromal lymphopoietin (TSLP) develop mixed cryoglobulinemia with renal disease closely resembling human cryoglobulin-associated membranoproliferative glomerulonephritis (MPGN), including glomerular deposits of immunoglobulins and complement. We assessed the effect of complement inh...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1111/j.1523-1755.2004.00495.x
更新日期：2004-04-01 00:00:00
abstract：:Acute kidney injury (AKI) activates pathways of cell death and cell proliferation. Although seemingly discrete and unrelated mechanisms, these pathways can now be shown to be connected and even to be controlled by similar pathways. The dependence of the severity of renal-cell injury on cell cycle pathways can be used ...
journal_title：Kidney international
pub_type： 杂志文章,评审
doi：10.1038/ki.2009.224
更新日期：2009-09-01 00:00:00
abstract：:To investigate whether alpha (alpha)-adrenoceptor agonists have a stimulatory effect on the expression of the angiotensinogen (Ang) gene in opossum kidney (OK) cells, we used OK 27 cells with a fusion gene containing the 5'-flanking regulatory sequence of the rat angiotensinogen gene fused with a human growth hormone ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1995.277
更新日期：1995-07-01 00:00:00
abstract：BACKGROUND:While the association of complement activation and glomerulonephritis has been recognized for decades, the pathogenic mechanisms of complement-mediated glomerular damage are incompletely understood. Expression of the C5a receptor in the kidney suggests that C5a could play a direct role in initiating or promo...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.1998.00155.x
更新日期：1998-11-01 00:00:00
abstract：:We examined the effect of daily cyclosporin A administration on the development and extent of tubulointerstitial nephritis produced in rats immunized with tubular basement membranes in adjuvant. Six mg/kg/day of cyclosporin A, given from the time of immunization, completely blocked the development of interstitial lesi...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1988.119
更新日期：1988-06-01 00:00:00
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...
journal_title：Kidney international
pub_type： 评论,杂志文章
doi：10.1038/ki.2011.301
更新日期：2012-01-01 00:00:00
abstract：:Autosomal dominant polycystic kidney disease (ADPKD) is an inherited, progressive nephropathy accounting for 4-10% of end stage renal disease worldwide. PKD1 and PKD2 are the most common disease loci, but even accounting for other genetic causes, about 7% of families remain unresolved. Typically, these unsolved cases ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1016/j.kint.2019.08.038
更新日期：2020-02-01 00:00:00
abstract：:Urinary proteomics is a promising tool for biomarker investigation, particularly in complex kidney diseases. Fabris and colleagues report that urinary laminin subunit alpha-2 is a potential diagnostic marker of medullary sponge kidney (MSK) disease by using a label-free quantitative proteomics platform and a clinicall...
journal_title：Kidney international
pub_type： 评论,杂志文章
doi：10.1016/j.kint.2016.10.032
更新日期：2017-02-01 00:00:00
abstract：:Colony stimulating factor-1 (CSF-1) is a chemoattractant and growth factor for macrophages. In autoimmune MRL-lpr mice, CSF-1 is detected in the circulation and there is an increase in CSF-1 transcripts and macrophages in the kidney. The purpose of this study was to establish whether the MRL-lpr kidney is responsible ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1996.9
更新日期：1996-01-01 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:Recent advances enable the direct measurement of small molecule clearance, Kecn, during each dialysis. Average Kecn and treatment length, t, are multiplied giving total clearance, Kt. The body surface area (BSA) is a fixed transformation of height and weight and is a well recognized measure of body size. Thi...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1111/j.1523-1755.2005.00533.x
更新日期：2005-09-01 00:00:00
abstract：BACKGROUND:Glucocorticoids (GC) represent the mainstay of treatment of idiopathic nephrotic syndrome (INS) and might be involved in the pathogenesis of the disease. We evaluated basal secretion of cortisol, number and affinity of glucocorticoid receptors, dexamethasone (Dex)-mediated inhibition of concanavalin-A (Con-A...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1111/j.1523-1755.2004.00392.x
更新日期：2004-02-01 00:00:00
abstract：BACKGROUND:Home nocturnal hemodialysis is an intensive form of hemodialysis, where patients perform their treatments at home for about 7 hours approximately 6 nights a week. Compared with in-center conventional hemodialysis, home nocturnal hemodialysis has been shown to improve physiologic parameters and reduce health ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.2003.00157.x
更新日期：2003-09-01 00:00:00
abstract：:Polycystic renal disease was induced in rats by feeding 2-amino-4,5-diphenylthiazole. Tubular (TBM) and glomerular basement membranes (GBM) were purified and analyzed for possible structural changes that may be a factor in the development of the tubular dilations and cysts. Changes in the relative quantities of TBM po...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1985.193
更新日期：1985-11-01 00:00:00
abstract：:Diabetes mellitus is associated with natriuresis, whereas estrogen has been shown to be renoprotective in diabetic nephropathy and may independently regulate renal sodium reabsorption. The aim of this study was to determine the effects of 17-beta estradiol (E(2)) replacement to diabetic, ovariectomized (OVX) female ra...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/sj.ki.5000140
更新日期：2006-02-01 00:00:00
abstract：:Water and small solute fluxes through cell membranes are ensured in many tissues by selective pores that belong to the major intrinsic protein family (MIP). This family includes the water channels or aquaporins (AQP) and the neutral solute facilitators such as the glycerol facilitator (GlpF). We have compared the char...
journal_title：Kidney international
pub_type： 杂志文章,评审
doi：10.1046/j.1523-1755.2001.060002422.x
更新日期：2001-08-01 00:00:00
abstract：:Tamm-Horsfall protein (THP), a monomeric glycoprotein (M(r) 80 to 100 kDa), is produced by the mammalian kidney's thick ascending limb of Henle cells and excreted into the urine. The function of THP is uncertain. Here we report that a high molecular weight contaminant in sheep THP (sTHP) preparations was identified as...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1993.343
更新日期：1993-11-01 00:00:00
abstract：:Systemic AA type amyloidosis with renal involvement is the major cause of morbidity and mortality in patients with familial Mediterranean fever (FMF). A histopathological examination is essential to achieve a definite diagnosis in systemic amyloidosis. The diagnostic yield of the procedure varies according to the biop...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1993.318
更新日期：1993-10-01 00:00:00
