The endothelium is a single-layered structure that responds to physical and chemical signals with various factors it synthesizes. In the early days of its discovery, as the inner wall of the vessels, the endothelium was thought to be a simple barrier that lays on the surface. Over time it is discovered that endothelium maintains body homeostasis with the molecules it synthesizes, despite its simple single-layer structure. It has been accepted as an important organ that contributes to the maintenance of vascular tone, cell adhesion, inflammation, vascular permeability and coagulation. Any imbalance in these physiological and pathological events causes endothelial dysfunction. This can cause many diseases such as atherosclerosis, hypertension, diabetes, or it can occur because of these. Endothelial related disorders may also complicate hematopoietic stem cell transplantation (HSCT), which is used to treat various hematologic and neoplastic diseases. These life-threatening complications include graft-versus-host disease, hepatic veno-occlussive disease, transplant-associated thrombotic microangiopathy and diffuse alveolar hemorrhage. They share a similar pathophysiology involving endothelial cells with different clinical presentations. Therefore, current researche on the issue is putting the endothelium under the spotlight for novel markers and treatment options that should be used to monitor or treat at least some of these complications following HSCT. 相似文献
Background: Corneal Dystrophy and Perceptive Deafness (CDPD) or Harboyan syndrome is an autosomal recessive rare disorder, characterized by congenital corneal opacities and progressive sensorineural hearing loss, which usually begins after the second decades of life. This study reports the ophthalmic, audiological and genetic features, in five CDPD affected patients from three Chilean families.
Materials and Methods: Five individuals affected with CDPD from three unrelated Chilean families were clinically and genetically examined. To evaluate a putative founder mutation 7 SNPs were analyzed in the three families, an Argentinian patient (carrier of the same mutation previously reported) and 87 Chilean controls.
Results: The ophthalmic symptoms in the five patients were bilateral and symmetric, starting before one year of age, and visual acuity varied from 0.1 to 0.3. In all cases, hearing loss began over 8 years old. The sequence of the 19 exons of SLC4A11 gene of all the affected patients exhibited homozygous eight nucleotide sequence duplication (c.2233_2240dup TATGACAC, p.(Ile748Metfs*5)) at the end of exon 16. All the affected patients of the three families were homozygous for a haplotype composed of five SNPs and covering 4,1 Mb. The same haplotype was present in one allele of the heterozygous Argentinean patient and has a frequency of 2.76% in Chilean population.
Conclusions: The five CDPD patients were homozygous for the same mutation in the SLC4A11 gene. Haplotype analysis of all the affected, including the case reported from Argentina was in accordance with a founder mutation. 相似文献
ObjectiveTo investigate the safety and efficacy of femtosecond laser-assisted cataract surgery compared to conventional cataract surgery. MethodsEighty-seven patients (114 eyes) were enrolled in this prospective cohert study between December 2013 and May 2014 in Shanxi Eye Hospital. Forty-four patients (60 eyes) underwent femtosecond laser-assisted cataract surgery. Forty-three patients (54 eyes) underwent 2.2 mm coaxial microincision cataract surgery. Femtosecond laser-assisted cataract surgery involved anterior capsulotomy, corneal incision and lens fragmentation based on optical coherence tomography-guided treatment mapping. An aspheric intraocular lens (SN60WF) or a multifocal intraocular lens (SN6AD1) was implanted based on the patient′s preference. Intraoperative complication rates and phaco energy parameters were recorded. Visual acuity, central corneal thickness and corneal endothelial cell loss were analyzed and the measurements were compared between the two groups. ResultsThere were no statistically significant differences in visual acuity and best corrected visual acuity (BCVA). The reduction in the mean number of endothelial cells was lower in the femtosecond laser-assisted cataract surgery group, but the difference was not significant. U/S total time was reduced to 30% compared to the conventional cataract surgery group (t=-2.739, P<0.01). Cumulative dissipated energy (CDE) was reduced 49% (Z=-2.979, P<0.01). In the femtosecond laser-assisted cataract surgery group, an incision was easily opened in 78%; incisions for the remaining patients were more difficult to perform. 93% of the capsule buttons were free floating and 7% were incomplete and a manual capsulorrhexis was required to complete the capsulotomy. ConclusionFemtosecond laser capsulotomy and lens fragmentation significantly reduce ultrasound use compared to conventional surgery. The new surgery appears to be safer and more efficient than conventional cataract surgery in the short term postoperatively. 相似文献
The objective of the study was to determine the relationship between serum concentrations of selenium (SeS) and zinc (ZnS), total antioxidant status (TAS) and endothelial function assessed by ultrasonographic method of dilatation of the brachial artery in 141 hypertensive patients.Patients with SeS < median were characterized by a statistically significantly lower flow-mediated dilation (FMD) than patients with SeS ≥ median. Patients with TAS < median were characterized by a significantly lower FMD than patients with TAS ≥ median. Older age, higher BMI, male gender, higher blood total cholesterol, ischemic heart disease, smoking and lower SeS constitute independent predictors of inferior endothelial function, expressed in lower FMD values. Smoking is an independent predictor of lower TAS, and the use of β-blockers and higher serum selenium levels are independent predictors of higher TAS.In summary, a decrease in TAS should be considered as a mechanism of inferior endothelial function in hypertensive patients conditioned by a decrease in SeS. 相似文献
We have designed a laboratory extracorporeal normothermic blood perfusion system for whole organs (e.g., kidney) that achieves pulsatile flow, low levels of hemolysis, and a blood priming volume of 60 mL or less. Using this uniquely designed extracorporeal circuit, we have achieved perfusion of two isolated ex vivo constructs. In the first experiment, we successfully perfused a rabbit epigastric flap based on the femoral vessels. In the second experiment, we were able to perfuse the isolated rabbit kidney for 48 h (range for all kidneys was 12–48 h) with excellent urine output, normal arterial blood gasses at 24 h, and normal ex vivo kidney histology at the conclusion of the experiments. These parameters have not been achieved before with any known or previously published laboratory extracorporeal circuits. The study has implications for prolonged organ perfusion prior to transplantation and for tissue engineering of vascularized tissues, such as by the perfusion of decellularized organs. 相似文献
Obesity is associated with adverse metabolic diseases including cardiovascular disease (CVD) and chronic kidney disease (CKD). These obesity-related diseases are highly associated with excess fat accumulation in adipose tissue. However, emerging evidence indicates that visceral adiposity associates more with metabolic and cardiovascular risk factors. Perirenal adipose tissue, surrounding the kidney, is originally thought to provides only mechanical support for kidney. However, more studies demonstrated perirenal adipose tissue have a closer association with renal disease than other visceral fat deposits in obesity. Additionally, perirenal adipose tissue is also an independent risk factor for CKD and even associated more with CVD. Thus, perirenal adipose tissue may be a connection of CVD with CKD. Here, we will provide an overview of the perirenal adipose tissue, a neglected visceral adipose tissue, and the roles of perirenal adipose tissue linking with CVD and CKD and highlight the perirenal adipose tissue as a potential strategy for future therapeutics against obesity-related disease. 相似文献