Abstract:
:Tuberous sclerosis complex is a genetic disorder characterized by hamartomatous lesions in multiple organs, frequently involving the kidney. We conducted a retrospective review of the clinical and radiographic records of 167 patients with tuberous sclerosis to determine the frequency of renal disease, the likelihood of significant renal morbidity, and the effects of genotype (TSC1 vs TSC2) and gender on renal phenotype. Renal lesions were seen in 57.5% of patients. Of these, angiomyolipoma (AML) occurred in 85.4%, cysts in 44.8%, and renal cell carcinoma in 4.2%. Both AML and cysts were significantly more common and more numerous in TSC2 than in TSC1. AML was significantly more common in female than in male patients, but cysts showed no correlation with gender. Eleven patients developed renal abnormalities during their care in this practice at an average age of onset of 11.3 years (range 3.8-23 years). The frequency and number of renal lesions were positively correlated with age. Interventions, including arterial embolization and nephrectomy, were performed in 11 (6.6%) patients. Among female patients with lymphangioleiomyomatosis, renal AML was universally present. Our findings confirm a high rate of renal involvement; a low rate of serious complications; significant associations between renal involvement, genotype, and gender; and a significant association between renal and pulmonary involvement in female patients.
journal_name
Kidney Intjournal_title
Kidney internationalauthors
Rakowski SK,Winterkorn EB,Paul E,Steele DJ,Halpern EF,Thiele EAdoi
10.1038/sj.ki.5001853subject
Has Abstractpub_date
2006-11-01 00:00:00pages
1777-82issue
10eissn
0085-2538issn
1523-1755pii
S0085-2538(15)51794-8journal_volume
70pub_type
杂志文章abstract:BACKGROUND:Optimal nutrient intake is important in the maintenance of a positive nitrogen balance in hemodialysis (HD) patients. The objectives of this study were (1) to assess the influence of two levels of protein intakes on nitrogen balance in stable adult HD patients, and (2) to identify a minimum level of protein ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.2000.00171.x
更新日期:2000-07-01 00:00:00
abstract::Extracellular fluid volume expansion is known to produce exaggerated natriuresis in essential hypertension. In order to assess the role of hemodynamic and intrarenal physical factors upon natriuretic response to central volume expansion, two hour water immersion (WI) experiments were made in six uncomplicated essentia...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1988.79
更新日期:1988-04-01 00:00:00
abstract::Congenital chloride diarrhea is due to mutations in the intestinal Cl(-)/HCO(3)(-) exchange (SLC26A3) which results in sodium chloride and fluid depletion leading to hypochloremic and hypokalemic metabolic alkalosis. Although treatment with sodium and potassium chloride offers protection from renal involvement in chil...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2008.401
更新日期:2008-10-01 00:00:00
abstract::We examined the circulating levels of the proinflammatory cytokines tumor necrosis factor-alpha (TNF-alpha), interleukin (IL)-1 alpha, IL-6, granulocyte macrophage-colony stimulating factor (GM-CSF), and the anti-inflammatory cytokine IL-10, and their expression in kidneys acutely infected with murine malaria parasite...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1111/j.1523-1755.1998.00848.x
更新日期:1998-04-01 00:00:00
abstract::The impact of high-flux hemodialysis on clinical outcomes remains controversial. We have previously shown that in vivo transmigrated leukocytes from patients with low-flux bioincompatible hemodialysis have an impaired capacity to upregulate CD11b at the site of interstitial inflammation. In the present study, we inves...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5002090
更新日期:2007-03-01 00:00:00
abstract::A study of dialysis costs in five major dialysis centers throughout the United States was conducted in 1973. When available, home, home training, limited care, satellite and in-hospital-full-care dialysis were included. The study's objective was to determine the cost per dialysis for each separate dialysis modality. A...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1976.31
更新日期:1976-03-01 00:00:00
abstract:BACKGROUND:Increasing proteinuria in kidney disease is associated with an increased risk of renal failure. Urinary proteins such as albumin induce inflammatory signaling and gene expression in tubular epithelial cells (TECs). Fibronectin is an extracellular matrix protein that can exist in soluble form and is excreted ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1111/j.1523-1755.2005.00667.x
更新日期:2005-11-01 00:00:00
abstract::Innate lymphoid cells are a recently recognized group of immune cells with critical roles in tissue homeostasis and inflammation. Regulatory innate lymphoid cells are a newly identified subset of innate lymphoid cells, which play a suppressive role in the innate immune response, favoring the resolution of intestinal i...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1016/j.kint.2019.07.019
更新日期:2020-01-01 00:00:00
abstract::Experimental unilateral ureteral obstruction (UUO) is widely used to study renal fibrosis; however, renal injury can only be scored semiobjectively by histology. We sought to improve the UUO model by reimplanting the obstructed ureter followed by removal of the contralateral kidney, thus allowing longitudinal measurem...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5002797
更新日期:2008-04-01 00:00:00
abstract::Freeze-fracture and thin-section electron microscopy and morphometry were used to characterize further the response of the rat medullary collecting duct to potassium depletion. In freeze-fracture replicas, principal cells and intercalated cells were identified based on the assumption that intercalated cells possess a ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1980.6
更新日期:1980-01-01 00:00:00
abstract:BACKGROUND:Proteomic techniques have recently become available for large-scale protein analysis. The utility of these techniques in identification of urinary proteins is poorly defined. We constructed a proteome map of normal human urine as a reference protein database by using two differential fractionated techniques ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1111/j.1523-1755.2002.kid565.x
更新日期:2002-10-01 00:00:00
abstract::Acute kidney injury (AKI) following cardiac surgery is closely interrelated with hemodynamic instability and sympathetic activity, and adversely influences prognosis. Here, we investigated in a randomized placebo-controlled trial whether dexmedetomidine, an α2 adrenoreceptor agonist, could prevent AKI after valvular h...
journal_title:Kidney international
pub_type: 杂志文章,随机对照试验
doi:10.1038/ki.2015.306
更新日期:2016-03-01 00:00:00
abstract::These studies were undertaken to examine effects of elevated glucose levels on glycolysis, sorbitol pathway activity, and the cytosolic redox state of NADH/NAD+ in isolated glomeruli. Blood-free glomeruli were isolated from kidneys of male, Sprague-Dawley rats using standard sieving techniques, then incubated for one ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1992.121
更新日期:1992-04-01 00:00:00
abstract::Recent insights have defined the central role of podocytes both in rare genetic diseases and as a general determinant of the progression of human glomerular diseases. In a recent issue of Nature, Weavers et al. described a podocyte-like cell type in Drosophila, the nephrocyte, that closely resembles mammalian podocyte...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2008.653
更新日期:2009-03-01 00:00:00
abstract::Expression of the polycystin-1 C-terminal cytoplasmic tail increases Cl(-) channel activity in Xenopus oocytes. Background. Cyst expansion in autosomal-dominant polycystic kidney disease (ADPKD) is characterized by active Cl(-) secretion in excess of solute reabsorption. However, the connections between elevated epith...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1111/j.1523-1755.2005.00441.x
更新日期:2005-08-01 00:00:00
abstract::We studied the glomerular architecture in renal biopsies of 95 patients undergoing bariatric surgery for extreme obesity but whose renal function was normal. The comparison group was 40 control patients having protocol biopsies. These latter patients had normal weight and renal function, were non-diabetic, non-hyperte...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5002796
更新日期:2008-04-01 00:00:00
abstract::In this study, we have evaluated 50 children (30 girls and 20 boys; mean age, 10.1 years) with a variety of renal diseases in whom renal biopsy specimens showed crescents in greater than or equal to 50% of glomeruli. Initial clinical features included edema in 61%; hypertension in 51%; gross hematuria in 73%; 3 to 4+ ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1985.30
更新日期:1985-02-01 00:00:00
abstract::Podocyte injury and loss contribute to the progression of glomerular diseases, including diabetic kidney disease. We previously found that the glomerular expression of Sirtuin-1 (SIRT1) is reduced in human diabetic glomeruli and that the podocyte-specific loss of SIRT1 aggravated albuminuria and worsened kidney diseas...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1016/j.kint.2017.12.008
更新日期:2018-06-01 00:00:00
abstract::Diagnosis and treatment of atherosclerotic renal artery stenosis (ARAS) in the elderly is a never-ending challenge for every nephrologist. Kalra et al. report that adjusted hazard ratios for ARAS increased threefold from 1992 to 2004 in 16 million United States Renal Data System participants aged 66 years or older. Ho...
journal_title:Kidney international
pub_type: 信件
doi:10.1038/ki.2009.326
更新日期:2010-01-01 00:00:00
abstract::Stimulation of proximal tubule cell (PTC) growth in a variety of physiological and pathological renal conditions is preceded by increased renal production of transforming growth factor-beta 1 (TGF-beta 1) and by augmented tubular sodium transport via activated sodium hydrogen exchange (NHE). Since TGF-beta 1 has been ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.1998.00916.x
更新日期:1998-06-01 00:00:00
abstract::The transition between the glomerulus and the proximal tubule was studied by light and electron microscopy in perfusion-fixed rabbit kidneys. In most nephrons a transitional segment of tubule occurred between the Bowman's capsule and the proximal tubule proper. This initial part of the tubule was lined by flattened ce...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1975.22
更新日期:1975-03-01 00:00:00
abstract::The parathyroid hormone (PTH) response to hypocalcemia was studied in 18 hemodialysis patients with osteitis fibrosa, and the relationship with PTH and bone histology in 26 hemodialysis patients. Hypocalcemia was produced during hemodialysis by the use of a dialysate devoid of calcium. Both amino (N) (P less than 0.05...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1984.37
更新日期:1984-02-01 00:00:00
abstract::The development of effective therapies for acute kidney injury (AKI) has been hindered by delayed diagnosis in the clinical setting, varying definitions of AKI, and limited prognostic information. In a study by Portilla et al., elevations in liver fatty acid-binding protein (L-FABP) were found to predict AKI in childr...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5002739
更新日期:2008-02-01 00:00:00
abstract:BACKGROUND:Vascular access failure is the main cause of morbidity in hemodialysis. Venous stenosis and subsequent thrombosis, as the result of intimal hyperplasia, is the major cause of vascular access failure. Intimal hyperplasia of the arteriovenous fistula (AVF) closely resembles the main histopathologic feature of ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.2003.00120.x
更新日期:2003-08-01 00:00:00
abstract::The cannabinoid receptor type 2 (CB2) is a G protein-coupled seven transmembrane receptor that transmits endogenous cannabinoid signaling. The role of CB2 in the pathogenesis of kidney injury and fibrosis remains poorly understood. Here we demonstrate that CB2 was induced, predominantly in kidney tubular epithelium, i...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1016/j.kint.2018.05.023
更新日期:2018-10-01 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::Residual kidney function (RKF) may confer a variety of benefits to patients on maintenance dialysis. RKF provides continuous clearance of middle molecules and protein-bound solutes. Whereas the definition of RKF varies across studies, interdialytic urine volume may emerge as a pragmatic alternative to more cumbersome ...
journal_title:Kidney international
pub_type: 杂志文章,评审
doi:10.1016/j.kint.2016.02.037
更新日期:2016-08-01 00:00:00
abstract::Recent studies suggest the presence of local angiotensin generating system in the kidney. By using in situ hybridization technique, mRNA for angiotensinogen has been shown to be present in the proximal tubule. In the present study, we have attempted to examine the production of angiotensinogen and renin-like activity ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1991.117
更新日期:1991-05-01 00:00:00
abstract::Primary hyperoxaluria type I (PH I) is a congenital error of metabolism that can be manifested by an increased oxalate production, and ultimately result in kidney failure. After a combined liver/kidney transplantation, children with PH I have persistent excretion of oxalate that causes crystal formation in the urinary...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.1998.00876.x
更新日期:1998-05-01 00:00:00
abstract:BACKGROUND:Patients initiating dialysis after a failed kidney transplant are a subgroup of chronic kidney disease (CKD) patients who have not been characterized. Exposure to immunosuppressive medications differentiates these patients from the general incident end-stage renal disease (ESRD) population. METHODS:Data fro...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.2002.00373.x
更新日期:2002-06-01 00:00:00