• Journal of mucopolysaccharidosis and rare diseases
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• v.5 no.1
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• pp.39-41
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• 2021

Prader-Willi syndrome (PWS) is a rare genetic neurodevelopmental disorder of hyperphagia leading to severe obesity, intellectual deficits, compulsivity, and other behavioral problems. PWS is caused by the inactivation of contiguous genes on chromosome 15q11-q13, which complicates the development of targeted, effective therapeutics. Various preclinical studies have been conducted by developing mouse models that exhibit phenotypes similar to PWS. Oxytocin deficiency in PWS is associated with hyperphagia with impaired satiety and, food-seeking and behavior disorders. Here, we summarize the oxytocin study of ingestion behavior tested in the PWS mouse model and published data from clinical trials that have evaluated treatment effectiveness on ingestion behavior and social dysfunction in patients with PWS.

• Membrane and Water Treatment
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• v.11 no.1
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• pp.41-48
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• 2020

Lead is a highly toxic heavy metal that causes serious health problems. Nonetheless, it is increasingly being used for industrial applications and is often discharged into the environment without adequate purification. In this study, Pb(II) was removed by powdered waste sludge (PWS) based on the biosorption mechanism. Different PWSs were collected from a submerged moving media intermittent aeration reactor (SMMIAR) and modified Ludzack-Ettinger (MLE) processes. The contents of extracellular polymeric substances were similar, but the surface area of MLE-PWS (2.07 ㎡/g) was higher than that of SMMIAR-PWS (0.82 ㎡/g); this is expected to be the main parameter determining Pb(II) biosorption capacity. The Bacillaceae family was dominant in both PWSs and may serve as the major responsible bacterial group for Pb(II) biosorption. Pb(II) biosorption using PWS was evaluated for reaction time, salinity effect, and isotherm equilibrium. For all experiments, MLE-PWS showed higher removal efficiency. At a fixed initial Pb(II) concentration of 20 mg/L and a reaction time of 180 minutes, the biosorption capacities (q_e) for SMMIAR- and MLE-PWSs were 2.86 and 3.07 mg/g, respectively. Pb(II) biosorption using PWS was rapid; over 80% of the maximum biosorption capacity was achieved within 10 minutes. Interestingly, MLE-PWS showed enhanced Pb(II) biosorption with salinity values of up to 30 g NaCl/L. Linear regression of the Freundlich isotherm revealed high regression coefficients (R² > 0.968). The fundamental Pb(II) biosorption capacity, represented by the K_F value, was consistently higher for MLE-PWS than SMMIAR-PWS.

• Journal of mucopolysaccharidosis and rare diseases
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• v.4 no.2
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• pp.42-44
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• 2018

Prader-Willi syndrome (PWS) is a multisystemic complex disorder characterized by hyperphagia and impaired satiety which lead to severe and early obesity. In infancy, hypotonia and poor suck are main problems, and a child goes through Failure-to-thrive. During childhood, clinical manifestations change to food seeking as well as excessive weight gain, short stature, developmental delay, cognitive disability and behavioral problems. Also, growth hormone insufficiency is frequent. Most patients receive the recombinant growth hormone (rGH) therapy that provides improvement in growth, body composition, and physical attributes. The clinical care guideline for rGH therapy in PWS had been noticed in 2013. The rGH therapy helps in body fat, lean body mass, height SDS and head circumference. Also, the rGH therapy helps motor function, psychomotor development and cognition and behavioral issues.In Samsung medical center, there are clinical care guidelines for rGH therapy in PWS and an useful application for the patients. 'Living with PWS', the name of an moblie application for PWS patients, was introduced in the lecture. The application revised to version 2. It was made more convenient to users than in version 1. It helps caregivers to schedule the rGH therapy and to monitor height and weight.

• Journal of mucopolysaccharidosis and rare diseases
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• v.5 no.1
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• pp.29-33
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• 2021

Prader-Willi Syndrome (PWS) is a neurodevelopmental genomic imprinting disorder involving a lack of gene expression from the paternal chromosome 15q11-q13 region. This is typically due to paternal 15q11-q13 deletions (in approximately 60% of cases), maternal uniparental disomy 15, or when both 15s are from the mother (about 35% of cases). An imprinting center controls the expression of imprinted genes in the chromosome 15q11-q13 region. PWS is a neurodevelopmental disorder characterized by mental retardation and distinct physical, behavioral, and psychiatric features. Characteristic behavioral disturbances in PWS include excessive interest in food, skin picking, difficulty with a change in routine, temper tantrums, obsessive and compulsive behaviors, and mood fluctuations. Individuals with PWS typically have intellectual disabilities (borderline to mild/moderate mental retardation) and exhibit a higher overall level of behavior disturbances compared to individuals with similar intellectual disabilities. This condition severely limits social adaptations and quality of life. Different factors have been linked to the intensity and form of these behavioral disturbances, but there is no consensus regarding the cause. Consequently, there is still controversy surrounding management strategies and there is a need for new data. PWS is a multisystem disorder. Family members, caregivers, physicians, dieticians, and speech-language pathologists all play an important role in the management and treatment of symptoms in an individual with PWS. Here we analyze behavioral problems in children and adults with PWS by age and review appropriate management and treatment strategies for these symptoms.

• The Journal of Information Systems
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• v.32 no.3
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• pp.183-203
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• 2023

Purpose The purpose of this study is to examine the current operational status and problems of the Public Warning System (PWS) in China, and to propose feasible solutions to improve the performance and efficiency of the PWS through a comparative analysis with the Cell Broadcast Service (CBS)-based disaster SMS system adopted by other developed countries in the world. Design/methodology/approach In this study, the characteristics of PWS using SMS, applications, and CBS, respectively, are analyzed in detail, and compared and analyzed in terms of convenience, standardization, data security, speed, and location accuracy. In addition, CBS-based PWS in developed countries, such as U.S., E.U., Korea and Japan, were studied and their performance on key criteria was evaluated. Findings Based on the results of the study, the problems of China's PWS are summarized and recommendations are made to improve the PWS through the introduction of CBS technology. To this end, specific improvement measures are proposed in terms of the application of CBS technology, system construction and operation, and improvement of data security. In addition, the comparative analysis of PWSs in other developed countries is conducted to provide reference for the direction of PWS's improvement.

• BMB Reports
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• v.37 no.5
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• pp.522-526
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• 2004

Prader-Willi (PWS) and Angelman (AS) are syndromes of developmental impairment that result from the loss of expression of imprinted genes in the paternal (PWS) or maternal (AS) 15q11-q13 chromosome. Diagnosis on a clinical basis is difficult in newborns and young infants; thus, a suitable molecular test capable of revealing chromosomal abnormalities is required. We used a variety of cytogenetic and molecular approaches, such as, chromosome G banding, fluorescent in situ hybridization, a DNA methylation test, and a set of chromosome 15 DNA polymorphisms to characterize a cohort of 27 PWS patients and 24 suspected AS patients. Molecular analysis enabled the reliable diagnosis of 14 PWS and 7 AS patients, and their classification into four groups: (A) 6 of these 14 PWS subjects (44%) had deletions of paternal 15q11-q13; (B) 4 of the 7 AS patients had deletions of maternal 15q11-q13; (C) one PWS patient (8%) had a maternal uniparental disomy (UPD) of chromosome 15; (D) the remaining reliably diagnoses of 7 PWS and 3 AS cases showed abnormal methylation patterns of 15q11-q13 chromosome, but none of the alterations shown by the above groups, although they may have harbored deletions undetected by the markers used. This study highlights the importance of using a combination of cytogenetic and molecular tests for a reliable diagnosis of PWS or AS, and for the identification of genetic alterations.

• Herbal Formula Science
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• v.23 no.2
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• pp.225-233
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• 2015

Objectives : The purpose of this study was to investigate the interpretation of the Pyeongwi-san(PWS) prescription in order to obtain the evidence for clinical applications. Methods and Result : We interpreted on the PWS-related contents based on 15 classic books, analyzed it according to followed categories ; origin, indication and precaution, usage, meaning of name, physiological and pathological situation of PWS, explain about each herbs. Conclusion : 1. PWS was first mentioned in the Bakjebang, its application was enhancing appetite. Application of PWS not only expanded digestive disease, infectious disease, and pain in articular, but also used to toniyfing drug. 2. Generous application method of PWS was ‘Sujunbok(水煎服)’. Pill preparation(丸劑) and decoction with salt(鹽湯劑) were also used 3. "Pyeongwi(平胃)“ in PWS means "flatten the stomach". There are some theory about methodology to "Pyeongwi (平胃)“, one is "drain the pathogen in stomach(敦阜說)”, other is "tonifying stomach's healthy qi(卑監說)“, the other is compromise theory. 4. Most medical literature related application situation of PWS to dampness(濕邪) in stomach. In Gangsulwongobangsunju, there are more detailed explain focused on metal qi(金氣), and fire qi(火氣) 5. Atractylodes japonica Koidz(蒼朮) drys dampness and fortifys the spleen, Magnolia officinalis Rehder(厚朴) eliminates dampness and treats fullness, Citrus reticulata Blanco(陳皮) moves and drains qi, Glycyrrhiza uralensis Fisch(甘草) tonify and harmonize in herbal formula

• Journal of Convergence for Information Technology
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• v.12 no.1
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• pp.180-188
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• 2022

Goal of this study is to identify effect of Oral Motor Facilitation Technique(OMFT) on feeding development, tube weaning of PWS baby. Subject was 8months old PWS girl. 2 times of OT, 1 times of OMFT per weeks were provided from July, 2020 to June, 2021. Feeding development and skills was increased and could eat food by mouth after OMFT treatment. Especially, she could eat food by her mouth after 3mts of OMFT and age-appropriated food intake, chewing function, drinking by straw was increased. Through this case study, early detecting and treatment for feeding development of PWS is very important. OMFT is good treatment protocol for increasing feeding development and oral motor skills of PWS.

• Journal of Physiology & Pathology in Korean Medicine
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• v.21 no.1
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• pp.165-170
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• 2007

Pyungwi-san(PWS) have been using as a basic prescription of digestive disorder in Korean traditional medicine. This study was performed to examine the in vitro antioxidant activity of the extract using different antioxidant tests including by 1,1-diphenyl-2-picryl-hydrazil (DPPH) radical scavenging, superoxide anion radical scavenging, metal chelating hydrogen peroxide scavenging, lipid peroxydation protective effect and scavenging effect of nitric oxide and peroxynitrite. Herbal-acupuncture solution of PWS(PWS-HS) exhibited a concentration-dependent inhibition of DPPH radical adduct formation and it showed dose-dependent free radical scavenging activity onto superoxide anions. In addition, the result of metal chelating hydrogen peroxide scavenging and ammonium thiocyanate experiments showed that PWS-HS was an active scavenger of hydroxyl radicals. Furthermore, it was also found to be effective in scavenging nitric oxide and peroxynitrite, well-known cytotoxic species that can oxidize several cellular components such as proteins, lipids and DNA.

• Clinical and Experimental Pediatrics
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• v.54 no.2
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• pp.55-63
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• 2011

Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder that is caused by the lack of expression of paternally inherited imprinted genes on chromosome 15q11-q13. This syndrome has a characteristic phenotype including severe neonatal hypotonia, early-onset hyperphagia, development of morbid obesity, short stature, hypogonadism, learning disabilities, behavioral problems, and psychiatric problems. PWS is an example of a genetic condition caused by genomic imprinting. It can occur via 3 main mechanisms that lead to the absence of expression of paternally inherited genes in the 15q11.2-q13 region: paternal microdeletion, maternal uniparental disomy, and an imprinting defect. Over 99% of PWS cases can be diagnosed using DNA methylation analysis. Early diagnosis of PWS is important for effective long-term management. Growth hormone (GH) treatment improves the growth, physical phenotype, and body composition of patients with PWS. In recent years, GH treatment in infants has been shown to have beneficial effects on the growth and neurological development of patients diagnosed during infancy. There is a clear need for an integrated multidisciplinary approach to facilitate early diagnosis and optimize management to improve quality of life, prevent complications, and prolong life expectancy in patients with PWS.