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11.
Rossella Siligato Valeria Cernaro Chiara Nardi Francesca De Gregorio Guido Gembillo Giuseppe Costantino 《Expert opinion on investigational drugs》2013,22(11):839-879
ABSTRACTIntroduction: Minimal change disease (MCD) and Focal and segmental glomerulosclerosis (FSGS) are two of the major causes of nephrotic syndrome (NS) in children and adults. According to KDIGO (Kidney Disease: Improving Global Outcomes) guidelines, the treatment of adult primary MCD and FSGS should be based on immunosuppressants and antiproteinuric drugs. Recently, Rituximab, a humanized monoclonal antibody (mAb) has emerged as a potential treatment for steroid or calcineurin inhibitor-dependent patients; it has however demonstrated lower efficacy in those with nephrotic syndrome that is resistant to the above indicated drugs.Area covered: Analysis of ongoing and already completed clinical trials, retrieved from clinicaltrials.gov, clinicaltrialsregister.eu and PubMed involving new therapies for nephrotic syndrome secondary to MCD and FSGS.Expert opinion: The most promising drugs under investigation for MCD and FSGS are mAbs. We are hopeful that new therapeutic options to treat multi-drug resistant MCD and FSGS will emerge from currently ongoing studies. What appears certain is the difficulty in enrolling patients affected by orphan renal diseases and the selection of valid endpoints in clinical trials, such as kidney failure. 相似文献
12.
Obesity-related focal and segmental glomerulosclerosis: normalization of proteinuria in an adolescent after bariatric surgery 总被引:1,自引:0,他引:1
Fowler SM Kon V Ma L Richards WO Fogo AB Hunley TE 《Pediatric nephrology (Berlin, Germany)》2009,24(4):851-855
Obesity-related glomerulopathy (ORG) is a secondary form of focal and segmental glomerulosclerosis (FSGS) occurring in severely
obese patients. A significant percentage of individuals with ORG will develop renal insufficiency or end stage renal disease.
We report here a 17-year-old girl with morbid obesity (body mass index 56.8 kg/m2) and ORG presenting with nephrotic range proteinuria, who failed to improve following treatment with diet, exercise and angiotensin-converting
enzyme inhibitor (ACEi)/angiotensin receptor blocker (ARB) therapy. Laparoscopic gastric bypass surgery was performed, and
within 2 weeks following the surgery, the patient had lost 5.7 kg body weight and showed a remarkable decrease in protein
excretion to one tenth of pre-surgery levels. More than 1 year after surgery, the patient’s urine protein and kidney function
have remained normal while off renin–angiotensin system inhibition therapy. This is the first report of successful use of
gastric bypass surgery for obesity-related glomerulopathy in an adolescent. We propose that gastric bypass surgery be considered
for patients with ORG. 相似文献
13.
Schwaderer P Knüppel T Konrad M Mehls O Schärer K Schaefer F Weber S 《Pediatric nephrology (Berlin, Germany)》2008,23(2):251-256
A small fraction of patients with initial steroid-sensitive nephrotic syndrome (SSNS) develops late steroid resistance, i.e.
a lack of remission after 4 weeks of relapse treatment despite previous response to steroids. The pathophysiological basis
of late resistance and the long-term prognosis remain obscure. Fourteen out of 360 patients with SSNS who were seen in our
department between 1954 and 2005 developed late resistance. Median age at onset of NS was 4 years and median duration of development
of late resistance 4.6 months. Histology showed minimal-change (MC) nephropathy in six patients and focal segmental glomerulosclerosis
(FSGS) in three patients on initial biopsy and four patients on repeat biopsies. Late resistance was treated with cyclophosphamide
in five patients, cyclosporine A in three, and both drugs in one. Eight of these nine patients went into remission. All 14
patients maintained a stable kidney function during their period of observation. NPHS2 mutation analysis in eight patients revealed no pathogenic mutations, suggesting that late resistance is not typically associated
with mutations in the NPHS2 gene. With respect to the clinical course, late resistance appears to resemble SSNS and is characterized by a favorable outcome. 相似文献
14.
Situs inversus totalis is a rare congenital anomaly that often occurs concomitantly with other disorders. A spectrum of renal abnormalities of patients with situs inversus has been reported. Developmental anomalies, including agenesis, dysplasia, hypoplasia, ectopia, polycystic kidney, and horseshoe kidney, have been reported. The association of situs inversus with nephrotic syndrome is very rare. We report the first known case of situs inversus totalis with nephrotic syndrome caused by primary focal segmental glomerulosclerosis, and the possible mechanism of this association. 相似文献
15.
Tae-Hyun Yoo Christopher E. Pedigo Johanna Guzman Mayrin Correa-Medina Changli Wei Rodrigo Villarreal Alla Mitrofanova Farah Leclercq Christian Faul Jing Li Matthias Kretzler Robert G. Nelson Markku Lehto Carol Forsblom Per-Henrik Groop Jochen Reiser George William Burke Alessia Fornoni Sandra Merscher 《Journal of the American Society of Nephrology : JASN》2015,26(1):133-147
Diabetic kidney disease (DKD) is the most common cause of ESRD in the United States. Podocyte injury is an important feature of DKD that is likely to be caused by circulating factors other than glucose. Soluble urokinase plasminogen activator receptor (suPAR) is a circulating factor found to be elevated in the serum of patients with FSGS and causes podocyte αVβ3 integrin-dependent migration in vitro. Furthermore, αVβ3 integrin activation occurs in association with decreased podocyte-specific expression of acid sphingomyelinase-like phosphodiesterase 3b (SMPDL3b) in kidney biopsy specimens from patients with FSGS. However, whether suPAR-dependent αVβ3 integrin activation occurs in diseases other than FSGS and whether there is a direct link between circulating suPAR levels and SMPDL3b expression in podocytes remain to be established. Our data indicate that serum suPAR levels are also elevated in patients with DKD. However, unlike in FSGS, SMPDL3b expression was increased in glomeruli from patients with DKD and DKD sera-treated human podocytes, where it prevented αVβ3 integrin activation by its interaction with suPAR and led to increased RhoA activity, rendering podocytes more susceptible to apoptosis. In vivo, inhibition of acid sphingomyelinase reduced proteinuria in experimental DKD but not FSGS, indicating that SMPDL3b expression levels determined the podocyte injury phenotype. These observations suggest that SMPDL3b may be an important modulator of podocyte function by shifting suPAR-mediated podocyte injury from a migratory phenotype to an apoptotic phenotype and that it represents a novel therapeutic glomerular disease target. 相似文献
16.
John J. Sim Simran K. Bhandari Michael Batech Aviv Hever Teresa N. Harrison Yu-Hsiang Shu Dean A. Kujubu Tracy Y. Jonelis Michael H. Kanter Steven J. Jacobsen 《Mayo Clinic proceedings. Mayo Clinic》2018,93(2):167-178
Objective
To compare renal function decline, incident end-stage renal disease (ESRD), and mortality among patients with 5 common glomerular diseases in a large diverse population.Patients and Methods
A retrospective cohort study (between January 1, 2000, and December 31, 2011) of patients with glomerulonephropathy using the electronic health record of an integrated health system was performed. Estimated glomerular filtration rate (eGFR) change, incident ESRD, and mortality were compared among patients with biopsy-proven focal segmental glomerulosclerosis (FSGS), membranous glomerulonephritis (MN), minimal change disease (MCD), immunoglobulin A nephropathy (IgAN), and lupus nephritis (LN). Competing risk models were used to estimate hazard ratios for different glomerulonephropathies for incident ESRD, with mortality as a competing outcome after adjusting for potential confounders.Results
Of the 2350 patients with glomerulonephropathy (208 patients [9%] younger than 18 years) with a mean follow-up of 4.5±3.6 years, 497 (21%) progressed to ESRD and 195 (8%) died before ESRD. The median eGFR decline was 1.0 mL/min per 1.73 m2 per year but varied across different glomerulonephropathies (P<.001). The highest ESRD incidence (per 100 person-years) was observed in FSGS 8.72 (95% CI, 3.93-16.72) followed by IgAN (4.54; 95% CI, 1.37-11.02), LN (2.38; 95% CI, 0.37-7.82), MN (2.15; 95% CI, 0.29-7.46), and MCD (1.67; 95% CI, 0.15-6.69). Compared with MCD, hazard ratios (95% CIs) for incident ESRD were 3.43 (2.32-5.08) and 2.35 (1.46-3.81), 1.28 (0.79-2.07), and 1.02 (0.62-1.68) for FSGS, IgAN, LN, and MN, respectively. No significant association between glomerulonephropathy types and mortality was detected (P=.24).Conclusion
Our findings from a real-world clinical environment revealed significant differences in eGFR decline and ESRD risk among patients with 5 glomerulonephropathies. These variations in presentation and outcomes warrant different management strategies and expectations. 相似文献17.
De novo CMV‐associated collapsing focal segmental glomerulosclerosis in a kidney transplant recipient 下载免费PDF全文
Collapsing focal segmental glomerulosclerosis (FSGS) is a variant of FSGS and is associated with severe nephrotic syndrome and acute kidney injury and can occur after kidney transplantation. The exact mechanism of collapsing FSGS after kidney transplantation is unknown, but potential causes include autoimmune diseases, certain malignancies, bisphosphonates, m‐TOR inhibitors, interferon‐alpha, HIV infection, and other viruses. We describe a case of de novo Cytomegalovirus (CMV)‐associated collapsing FSGS in a kidney transplant recipient with a UL97 phosphotransferase mutation that was successfully treated with intravenous ganciclovir, intravenous immunoglobulin, and steroids. 相似文献
18.
M. Hattori Y. Akioka H. Chikamoto N. Kobayashi K. Tsuchiya M. Shimizu S. Kagami H. Tsukaguchi 《American journal of transplantation》2008,8(7):1550-1556
Recurrent focal segmental glomerulosclerosis (FSGS) is a major challenge in the field of transplantation. Integrin-linked kinase (ILK) has emerged as a key mediator of podocyte–glomerular basement membrane (GBM) interactions. To clarify the involvement of plasma factors in FSGS recurrence, we examined the effects of plasma from FSGS patients with or without posttransplant recurrence on cultured podocytes, focusing particularly on ILK activity. Podocytes from a conditionally immortalized mouse podocyte cell line were treated with plasma from 11 FSGS patients, and ILK activity was determined using an immune complex kinase assay. Treatment with plasma from three patients with recurrence induced an increase in ILK activity. In contrast, no increase in ILK activity was observed in cultured podocytes treated with plasma from the remaining three patients with recurrence and five patients without recurrence. Cultured podocytes treated with plasma that induced ILK activity showed alterations of focal contact and detachment from the laminin matrix. In conclusion, this preliminary study provides experimental evidence suggesting the possible presence of circulating toxic factors in the plasma of some patients with recurrent FSGS, which induce an increase in podocyte ILK activity that may lead to the detachment of podocytes from the GBM. 相似文献
19.
20.
Berdeli A Mir S Yavascan O Serdaroglu E Bak M Aksu N Oner A Anarat A Donmez O Yildiz N Sever L Tabel Y Dusunsel R Sonmez F Cakar N 《Pediatric nephrology (Berlin, Germany)》2007,22(12):2031-2040
The podocin (NPHS2) gene encodes podocin protein, which has an important role in glomerular ultrafiltration and controlling slit membrane permeability.
The detection of an NPHS2 mutation affects the treatment plan for children with nephritic syndrome (NS). The frequency and spectrum of podocin mutations
in the Turkish population have remained largely unknown. The aim of this study was to screen for podocin mutations in Turkish
patients with steroid-resistant NS (SRNS) and to compare it with other published series. There were 295 children with SRNS,
originating from Turkey, included in this study. Forty-one patients (13.8%) had familial NS and 254 patients (86.2%) had sporadic
NS. Mutation analysis was performed in all eight exons of the NPHS2 gene with the direct DNA sequencing method. There were 53 different pathogenetic NPHS2 mutations detected, including 37 novel mutations. The mutation detection rate was 24.7% for all patients, 29.2% for familial,
and 24% for sporadic SRNS. The most common mutated exon was exon 5 (52 allele). The presence of mutations in exon 4 was found
to increase the risk of end-stage renal disease (ESRD). Among patients with mutations, the rates of renal failure and/or ESRD
(26%) were significantly higher than in those without mutations (12.6%). The mean time of progression to renal failure and
ESRD in patients with mutations (1.8 ± 2.5 years) was significantly shorter than in patients without mutations (3.7 ± 4.0 years).
Additionally, in patients with heterozygote mutations, fewer cases (13.6%) progressed to renal failure and/or ESRD than in
with patients who had homozygote/compound heterozygote mutations (31.3%). In conclusion, podocin mutations are responsible
for some of both familial and sporadic SRNS cases in Turkey. The mutations in this gene should be searched for in every child
after presentation with the first episode of NS. 相似文献