Pacheco Dowling (canadatable07)

Platelets play a major role in hemostasis as ppwell as in many other physiological and pathological processes. Accordingly, production of about 1011 platelet per day as well as appropriate survival and functions are life essential events. Inherited platelet disorders (IPDs), affecting either platelet count or platelet functions, comprise a heterogenous group of about sixty rare diseases caused by molecular anomalies in many culprit genes. Their clinical relevance is highly variable according to the specific disease and even within the same type, ranging from almost negligible to life-threatening. Mucocutaneous bleeding diathesis (epistaxis, gum bleeding, purpura, menorrhagia), but also multisystemic disorders and/or malignancy comprise the clinical spectrum of IPDs. The early and accurate diagnosis of IPDs and a close patient medical follow-up is of great importance. A genotype-phenotype relationship in many IPDs makes a molecular diagnosis especially relevant to proper clinical management. Genetic diagnosis of IPDs has been greatly facilitated by the introduction of high throughput sequencing (HTS) techniques into mainstream investigation practice in these diseases. However, there are still unsolved ethical concerns on general genetic investigations. Patients should be informed and comprehend the potential implications of their genetic analysis. Unlike the progress in diagnosis, there have been no major advances in the clinical management of IPDs. Educational and preventive measures, few hemostatic drugs, platelet transfusions, thrombopoietin receptor agonists, and in life-threatening IPDs, allogeneic hematopoietic stem cell transplantation are therapeutic possibilities. Gene therapy may be a future option. Regular follow-up by a specialized hematology service with multidisciplinary support especially for syndromic IPDs is mandatory.We synthesized a series of polystyrene derivatives modified with precursors of liquid crystal (LC) molecules, including 4-(trans-4-ethylcyclohexyl)phenol (homopolymer PECH and copolymer PECH#; # = 5, 10, 15, 20, 40, 60, and 80, where # indicates the molar fraction of 4-(trans-4-ethylcyclohexyl)phenoxymethyl in the side chain), 4-(trans-4-propylcyclohexyl)phenol (PPCH), 4-(trans-4-butylcyclohexyl)phenol (PBCH), and 4-(trans-4-amylcyclohexyl)phenol (PAmCH) via polymer modification reactions in order to investigate the orientation of LC molecules on polymer films exhibiting part of the LC molecular structure. A stable and uniform vertical orientation of LC molecules was observed in LC cells fabricated with PECH#, having 15 mol% or more of 4-(trans-4-ethylcyclohexyl)phenoxymethyl side groups. In addition, the vertical orientation of LC molecules was observed in LC cells fabricated with homopolymers of PECH, PPCH, PBCH, and PAmCH. The water contact angle on the polymer films could be associated with the vertical orientation of the LC molecules in the LC cells fabricated with polymer films. For example, a vertical LC orientation was observed when the water contact angle of the polymer films was higher than ~81°. Good orientation stability was observed at 200 °C and 15 mW/cm2 of UV irradiation for LC cells fabricated with PECH films.This study describes the development of a landslide monitoring system for the purpose of reducing damages caused by landslides in natural terrain. The system was developed to analyze the effects of landslide-inducing rainfall and the behavior of slopes through 12 monitoring stations that are distributed across eight national parks in Korea. Several sensors and a data acquisition equipment to monitor landslide were installed in each station. The composition of the system and its operating program were designed to efficiently manage the sizeable amounts of real-time monitoring data that are collected from the various stations. To test the potential of the developed system for reliable landslide hazard evaluations, data measured over a five-year period by the two monitoring st