Abstract：
:Renal involvement in von Hippel-Lindau (VHL) disease has emerged as the most prevalent cause of death in this hereditary disorder. In a group of 43 VHL patients (23 unrelated families) with renal lesions we examined whether severity of renal disease is affected by parental inheritance and VHL subtype (1, without pheochromocytoma; 2, with pheochromocytoma). We also tested whether and how nephron-sparing surgery could be applied. Renal involvement comprised multiple cysts and bilateral and multifocal carcinomas (RCC) which were detected by screening in 38 patients, at 30.5 (14 to 62) years of age. The severity of the renal disease was similar in VHL type 1 (79% of the pedigrees) and 2 (21%). It was not influenced by the sex of the carrier. Twenty-nine patients were operated on at a mean age of 33.6 years: 21 patients (28 kidneys or 61% of all operated kidneys) underwent nephron-sparing surgery, 4 had complete ablation of involved kidneys and thus required dialysis, 3 had uninephrectomy and 1 had cyst fenestration. Vascular thrombosis was the most severe early complication. It occurred in 4 of 9 kidneys treated by ex vivo surgery. During a median follow-up of 29 months, local recurrence occurred in 5 of 21 (24%) patients treated by nephron-sparing surgery, whereas 2 developed metastasis. Chronic renal failure (creatinine > 120 mumol/liter) affected 11 patients; in 9 of them, it was due to sequelae of surgery. In conclusion, screening of RCC and nephron-sparing surgery are of value in VHL patients. However, indications of ex vivo surgery should be drastically restricted and renal sequelae are not uncommon. Renal followup is required because of the risk of recurrence.
journal_name
Kidney Intjournal_title
Kidney internationalauthors
Chauveau D,Duvic C,Chrétien Y,Paraf F,Droz D,Melki P,Hélénon O,Richard S,Grünfeld JPdoi
10.1038/ki.1996.395subject
Has Abstractpub_date
1996-09-01 00:00:00pages
944-51issue
3eissn
0085-2538issn
1523-1755pii
S0085-2538(15)59689-0journal_volume
50pub_type
杂志文章abstract：:Parathyroid suppression by intraperitoneal calcitriol (1,25(OH)2D3) during peritoneal dialysis. The purpose of this study was to determine if parathyroid hormone (PTH) suppression could be achieved by increasing calcium mass transfer (Ca MT) with high dialysate Ca (4 mEq/liter) or via intraperitoneal (i.p.) 1,25(OH)2D...
journal_title：Kidney international
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1038/ki.1987.68
更新日期：1987-03-01 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
abstract：BACKGROUND:Patients undergoing myeloablative allogeneic hematopoietic cell transplantation (HCT) have a high incidence of acute renal failure (ARF). However, it is unclear if ARF is independently associated with mortality after this procedure. METHODS:We performed meta-analysis of published reports on ARF after myeloa...
journal_title：Kidney international
pub_type： 杂志文章,meta分析
doi：10.1111/j.1523-1755.2005.00301.x
更新日期：2005-05-01 00:00:00
abstract：:We have developed an immunological procedure for measuring advanced glycosylation end-products (AGEs) in serum. Using this method, we measured AGEs in healthy volunteers, patients with diabetes, renal failure without treatment and in patients with renal failure, treated with hemodialysis (HD) or continuous ambulatory ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1994.262
更新日期：1994-07-01 00:00:00
abstract：:Because the comparison of survival in patients with renal failure treated by dialysis and transplantation may be biased by pretreatment prognostic differences in the patients who receive these two therapies, we quantified the pretreatment prognosis of all 430 dialysis and transplant patients who began therapy for end-...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1984.132
更新日期：1984-07-01 00:00:00
abstract：:Intrarenal artery infusions of hypertonic saline can activate tubuloglomerular feedback (TGF), decreasing renal blood flow (RBF) and glomerular filtration rate (GFR). The response to infusion of hypertonic saline is enhanced by salt depletion and attenuated by salt loading, but has not previously been investigated in ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1987.236
更新日期：1987-10-01 00:00:00
abstract：:Ambulatory blood pressure monitoring (ABPM) remains a reference standard, but the minimal number of ABPM readings required to diagnose hypertension has not been empirically validated. Among 360 patients with chronic kidney disease and 38 healthy controls, 24-hour blood pressure was recorded 2 times per hour during the...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1016/j.kint.2018.08.021
更新日期：2018-12-01 00:00:00
abstract：:Copy number variations associate with different developmental phenotypes and represent a major cause of congenital anomalies of the kidney and urinary tract (CAKUT). Because rare pathogenic copy number variations are often large and contain multiple genes, identification of the underlying genetic drivers has proven to...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.2015.239
更新日期：2015-12-01 00:00:00
abstract：:We present a new hypothesis to explain the development of salt-dependent hypertension in humans. We propose that hypertension has two phases: an early phase in which elevations in blood pressure (BP) are mainly episodic and are mediated by a hyperactive sympathetic nervous or renin-angiotensin system, and a second pha...
journal_title：Kidney international
pub_type： 杂志文章,评审
doi：10.1038/ki.1997.442
更新日期：1997-11-01 00:00:00
abstract：:Kidney disease is associated with increased cardiovascular morbidity, but underlying mechanisms are poorly understood. We tested the hypothesis that chronic renal insufficiency impairs angioadaptation in a rat model of hindlimb ischemia. Twenty male Sprague-Dawley rats (8 weeks old) underwent subtotal nephrectomy (5/6...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/sj.ki.5000448
更新日期：2006-06-01 00:00:00
abstract：:Gene expression differs substantially among individual cells of the same type. We speculate that genes that are expressed in all but a portion of cells of a given cell type would be likely essential and required for either the cell survival (housekeeping) or for the cell type's unique structure and function, enabling ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1016/j.kint.2017.04.022
更新日期：2017-11-01 00:00:00
abstract：:The inner medullary collecting duct (IMCD) is a major target site of atrial natriuretic peptide (ANP) for diuresis and natriuresis, and it is in a hypertonic condition made by the renal countercurrent multiplication system. We investigated the effects of hyperosmolality on ANP-stimulated cGMP generation in IMCD and gl...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1994.178
更新日期：1994-05-01 00:00:00
abstract：BACKGROUND:Increased venous pressure (VP) and decreased access flow (Qa) are predictors of dialysis access graft thrombosis. VP is easily obtainable. Qa assessment requires a special device and takes more time. The aims of our randomized multicenter studies were to compare outcome in patients with grafts monitored by V...
journal_title：Kidney international
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1046/j.1523-1755.2001.0590041551.x
更新日期：2001-04-01 00:00:00
abstract：:Severe segmental glomerulonephritis (Seg GN) (greater than or equal to 50% involvement) in systemic lupus erythematosus (SLE) is classified as diffuse GN (DPGN) in the WHO classification. We tested the validity of the assumption that severe Seg GN and DPGN have the same prognosis by determining the proportion of glome...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1987.203
更新日期：1987-08-01 00:00:00
abstract：:Early graft loss (EGL) is a feared outcome of kidney transplantation. Consequently, kidneys with an anticipated risk of EGL are declined for transplantation. In the most favorable scenario, with optimal use of available donor kidneys, the donor pool size is balanced by the risk of EGL, with a tradeoff dictated by the ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1016/j.kint.2020.01.043
更新日期：2020-06-01 00:00:00
abstract：:The rate of myofibrillar protein degradation was measured by 3-methylhistidine (3MH) release in moderately uremic and sham-operated control rats. The rats were studied in the fed state or after 24 and 48 hours of fasting. When fed, both uremic and control rats gained weight at the same rate. During 48 hours of fasting...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1981.141
更新日期：1981-09-01 00:00:00
abstract：:We have described a simple technique for the measurement of picomoles or nanomoles of ammonium. Samples can be easily processed on the day of an experiment. analysis of 14C-glutamate can be done at a later date by an accurate sensitive and reliable method. We can readily process 50 samples per day. The sensitivity can...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1981.10
更新日期：1981-01-01 00:00:00
abstract：:The plasminogen activators (PA), which are regulated by their specific inhibitor, PAI-1, convert the zymogen plasminogen to plasmin, a protease involved in fibrinolysis and extracellular matrix turnover. Interleukin 1 beta (IL-1) is a key cytokine released from infiltrating monocytes/macrophages during the initial sta...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1996.159
更新日期：1996-04-01 00:00:00
abstract：:The inactivation of physiological glucocorticoids by 11 beta-hydroxysteroid dehydrogenase (11 beta-HSD) confers mineralocorticoid specificity to certain aldosterone target tissues. Both NADP, and NAD-dependent isoforms of 11 beta-HSD have been described. An NAD-dependent isoform of 11 beta-HSD (11 beta-HSD2) was recen...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1996.39
更新日期：1996-01-01 00:00:00
abstract：:The effect of intravenous infusion of angiotensin II on microvascular parameters of the renal microcirculation of rats was studied. With the aid of fluorescence microscopy and a high sensitivity video system we observed the passage of fluorescence-labeled erythrocytes through single glomerular capillaries on the surfa...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1985.4
更新日期：1985-01-01 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：:Adiponectin levels are increased in patients with type I diabetes especially in the presence of microangiopathy. Here we determined the predictive value of serum adiponectin levels and 8 adiponectin gene polymorphisms for mortality, cardiovascular events and end-stage renal disease in type I diabetic patients. This pr...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.2008.201
更新日期：2008-09-01 00:00:00
abstract：:Monocyte chemoattractant protein 1 (MCP-1) is a C-C chemokine with potent monocyte chemotactic and activating properties that may contribute to glomerular macrophage infiltration in anti-GBM Ab GN. We have previously reported increased glomerular steady state expression of MCP-1 mRNA relative to GAPDH mRNA in the hete...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1996.363
更新日期：1996-08-01 00:00:00
abstract：:Pathogenesis of vascular calcification in chronic kidney disease. Background. Hyperphosphatemia and hypercalcemia are independent risk factors for higher incidence of cardiovascular events in patients with chronic kidney disease. In addition to increased calcium-phosphate product, hyperphosphatemia accelerates the pro...
journal_title：Kidney international
pub_type： 杂志文章,评审
doi：10.1111/j.1523-1755.2005.00421.x
更新日期：2005-08-01 00:00:00
abstract：:Angiotensin converting enzyme (ACE) inhibitors are known to lower urinary protein excretion in human renal disease. This proteinuria lowering effect of ACE inhibition has been hypothesized to be a result of renal hemodynamic changes due to the inhibition of angiotensin II (Ang II) production. To test this hypothesis w...
journal_title：Kidney international
pub_type： 临床试验,杂志文章
doi：10.1038/ki.1991.268
更新日期：1991-10-01 00:00:00
abstract：:Fibroblast growth factor-23 (FGF23), a regulator of mineral metabolism, has been linked to cardiovascular disease in chronic kidney disease. As community-based data of the longitudinal association between FGF23 and cardiovascular events are lacking, we investigated a possible relationship in 727 men of the Uppsala Lon...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.2012.327
更新日期：2013-01-01 00:00:00
abstract：:Glucocorticoid induced leucine zipper protein (GILZ) is an aldosterone-regulated protein that controls sodium transport in cultured kidney epithelial cells. Mice lacking GILZ have been reported previously to have electrolyte abnormalities. However, the mechanistic basis has not been explored. Here we provide evidence ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1016/j.kint.2016.10.038
更新日期：2017-05-01 00:00:00
abstract：:Recent studies of timing of dialysis initiation have challenged the recent trend to earlier initiation of therapy. The observed outcomes though are a consequence of the balance between the risks of advanced uremia versus the inherent dangers relating to dialysis therapy itself. Many of these risks are inherent in how ...
journal_title：Kidney international
pub_type： 杂志文章,评审
doi：10.1038/ki.2012.133
更新日期：2012-08-01 00:00:00
abstract：BACKGROUND:Intrauterine growth retardation (IUGR) is associated with higher morbidity and mortality not only in perinatal life but also in later life. The purpose of our study was to determine whether IUGR has any effect on the course of minimal change nephrotic syndrome (MCNS) in children. METHODS:Forty children who ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.1998.00121.x
更新日期：1998-10-01 00:00:00
abstract：:Ravulizumab is a long-acting C5 inhibitor engineered from eculizumab with increased elimination half-life, allowing an extended dosing interval from two to eight weeks. Here we evaluate the efficacy and safety of ravulizumab in adults with atypical hemolytic uremic syndrome presenting with thrombotic microangiopathy. ...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1016/j.kint.2020.01.035
更新日期：2020-06-01 00:00:00
