Anemia is a common complication and contributes to increased morbidity and mortality in chronic kidney disease (CKD) patients. Whereas there has been a significant improvement of understanding the underlying mechanism of erythropoiesis, the treatment of renal anemia is still restricted to erythropoietin (EPO)-stimulating agents. The purpose of this article is to review the physiology of erythropoiesis, functional role of EPO and underlying molecular and cellular basis that regulate EPO production. Regulation of EPO production is at mRNA level. When anemia or hypoxia occurs, transcriptional factor, hypoxia-inducible factor (HIF), binds to EPO 5' hypoxic response element and EPO gene transcription increases. The renal EPO is mainly produced by pericytes. In CKD, pericytes transdifferentiate to myofibroblasts, and subsequently the ability of EPO production decreases, leading to renal anemia. Recent experimental and clinical studies show the promising efficacy of prolyl hydroxylase inhibitors in renal anemia through increasing EPO production by stabilizing HIF. Recent advances on epigenetics create a new field to study EPO gene expression at chromatin level. We will discuss the role of demethylating agent on restoring EPO expression, providing a novel approach to the treatment of renal anemia.
Background/Aims: Fabry disease (FD), a rare x-lined genetic disorder is a cause of renal deterioration. The phenotype of FD is highly variable and nonspecific, and correct diagnosis has always been delayed. We aimed to explore the prevalence and clinical presentation of FD in this high-risk male population in a Northern Taiwan medical center. Methods: This is the first study to survey the incidence of FD in this high-risk population through the platform of a chronic kidney disease (CKD) education program in Asia. A total of 1,012 male patients with unknown CKD causes were screened using an assay of alpha-galactosidase A activity (α-Gal A) by dried blood spots (DBS). A final GLA gene analysis was also done for those with low enzyme activity. Results: We identified two new patients with classic FD and four patients with late-onset FD. One novel GLA mutation with c.413 G>A was found in one classic FD patient (index 5). The prevalence of FD is about 0.59 % (6 in 1,012) in the high-risk population group with CKD. The clinical symptoms of FD patients are nonspecific except in those with various degrees of renal failure. Those patients’ correct diagnosis was delayed, taking years and even decades. Three patients received enzyme replacement therapy and one started regular hemodialysis due to persistent renal function deterioration. Another two patients were found from family screening through a new index. In addition, a false negative result occurred in one patient who was proved to have FD by his kidney pathology as determined by this screening. Conclusion: FD is not such as rare a disease and its prevalence is greater in this high-risk male population. Clinicians need to be aware that FD should be included in the differential diagnosis in CKD with unknown etiology.
Surface plasmon in nanoscaled materials has recently attracted a great deal of attention due to its possibility in a wide range of application. From a practical standpoint, it is desirable for the devices having a tunability of surface plasmon frequency. To achieve this goal, in this study, a composite consisting of two-dimensional gold nanoparticles array embedded in elastic polydimethylsiloxane (PDMS) membrane has been synthesized. Because the elastic PDMS membrane has a high malleability, with an external stress, it is very easy to regulate the interparticle distance in the gold nanoparticle array. The change in the distance between each nanoparticle will alter the surface plasmon interaction, and hence surface plasmon frequency can be manipulated. It is found that when the interparticle distance increases, the enhanced surface plasma mutual coupling will cause the blueshift of surface plasmon resonance frequency. The observed result satisfies the forecast based on electromagnetic theory.
The use of this validated intelligent system can identify patients who are at risk of IDH in advance, which may facilitate well-timed personalized management and intervention.
In order to examine the chronologic changes in genetic variance and heritability of anthropometric characteristics of Chinese infants in Taiwan, a total of 521 pairs of same-sexed twin neonates given birth in four major general teaching hospitals in Taipei City were studied. Based on the placental pattern and 12 red blood cell antigens, 428 MZ and 93 DZ twin pairs were identified and followed up to the age of one year. There was no significant genetic variance for all anthropometric characteristics adjusted for sex and gestational week before the age of six months. After adjusting for sex and gestational week, a significant genetic variance was observed at the age of six months, with heritability values of 0.51 (weight), 0.63 (head circumference), 0.77 (chest circumference), and 0.53 (arm circumference), as well as at one year, although with considerably lower heritability values. This implies that growth is dynamically determined by both genetic and environmental factors during infancy.
A conventional piezoelectric energy harvester suffers from narrow bandwidth and uni-directional harvesting. This paper presents a U-shaped multi-modal bi-directional piezoelectric energy harvester to overcome the above issues. The U-shaped structure, composed of a main beam and two identical side beams, can be fabricated by bending a beam into a U shape with both ends clamped. The theoretical model of the U-shaped piezoelectric energy harvester is developed and validated with experimental results. This U-shaped piezoelectric energy harvester demonstrates the capability of harvesting vibration energy in both the vertical and horizontal directions, which are the transverse direction of the main beam and that of the side beams, respectively. Parametric studies are conducted to investigate the influence of the aspect ratio of the U-shaped structure on the modal frequencies and voltage output. It is shown that the first two horizontal modal frequencies can be tuned close to each other by shortening the side beams or extending the main beam. The ratio between the horizontal and vertical modal frequencies can also be adjusted to fit the vibration sources by altering the side beams or the main beam.
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