Journal of mucopolysaccharidosis and rare diseases
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v.2
no.1
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pp.5-7
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2016
Mucolipidoses II and III alpha/beta (ML II and ML III) are lysosomal disorders in which the essential mannose-6-phosphate recognition marker is not synthesized onto lysosomal hydrolases and other glycoproteins. The disorders are caused by mutations in GNPTAB, which encodes two of three subunits of the heterohexameric enzyme, N-acetylglucosamine-1-phosphotransferase ML II, recognizable at birth, often causes intrauterine growth impairment and sometimes the prenatal "Pacman" dysplasia. The main postnatal manifestations of ML II include gradual coarsening of neonatally evident craniofacial features, early cessation of statural growth and neuromotor development, dysostosis multiplex and major morbidity by hardening of soft connective tissue about the joints and in the cardiac valves. Fatal outcome occurs often before or in early childhood. ML III with clinical onset rarely detectable before three years of age, progresses slowly with gradual coarsening of the facial features, growth deficiency, dysostosis multiplex, restriction of movement in all joints before or from adolescence, painful gait impairment by prominent hip disease. Cognitive handicap remains minor or absent even in the adult, often wheelchair-bound patient with variable though significantly reduced life expectancy. As yet, there is no cure for individuals affected by these diseases. So, clinical manifestations and conservative treatment is important. This review aimed to highlight the extra-skeletal clinical problems in ML II and III.
Park, Hyun-Bin;Lee, Soon-Min;Lee, Jin-Sung;Park, Min-Soo;Park, Kook-In;NamGung, Ran;Lee, Chul
Clinical and Experimental Pediatrics
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v.53
no.11
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pp.965-970
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2010
Purpose: The life expectancy of patients with spinal muscular atrophy (SMA) type I is generally considered to be less than 2 years. Recently, with the introduction of proactive treatments, a longer survival and an improved survival rate have been reported. In this study, we analyzed the natural courses and survival statistics of SMA type I patients and compared the clinical characteristics of the patients based on their survival periods. Methods: We reviewed the medical records of 14 pediatric patients diagnosed with SMA type I during a 9-year period. We examined the demographic and clinical characteristics of these patients, calculated their survival probabilities, and plotted survival curves as on the censoring date, January 1, 2010. We also compared the characteristics of the patients who died before the age of 24 months (early-death, ED group) and those who survived for 24 months or longer (long-survival, LS group). Results: The mean survival time was $22.8{\pm}2.0$ months. The survival probabilities at 6 months, 12 months, 18 months, 24 months, and 30 months were 92.9%, 92.9%, 76.0%, 76.0%, and 65.1%, respectively. Birth weight was the only factor that showed a statistically significant difference between the ED and LS groups ($P$=0.048). Conclusion: In this study, the survival probabilities at 2 years were far greater than expected. Because of the limited number of patients and information in this study, the contribution of improved supportive care on longer survival could not be clarified; this may be elucidated in larger cohort studies.
Journal of rehabilitation welfare engineering & assistive technology
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v.9
no.3
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pp.223-230
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2015
There is an increasing trend of medical devices and products moving out of hospitals and clinics into community and residual homes for use by the general public due to both the technological developments and demographic changes resulted from the increased life expectancy and decreased birth rate. In Korea, however, the definition of "home medical device" is rather ambiguous and we thus compared the definition of the term used in the global market with that in Korea. FDA definition of "home medical device" includes the devices intended for use in both professional healthcare facilities and home. The KFDA, does not provide the definition for the "home medical device"and the definition has only been inferred from the results of consumer surveys. With a paradigm shift in advent of u-healthcare era, the definition of "home medical device" in Korea should include the medical devices that could be used both at hospitals and at home.
Background: Breast cancer is the most common malignancy in women worldwide and its incidence is generally increasing. In 2012, it was the second most common cancer in the world. It is necessary to obtain information on incidence and mortality for health planning. This study aimed to investigate the relationship between the human development index (HDI), and the incidence and mortality rates of breast cancer in the world in 2012. Materials and Methods: This ecologic study concerns incidence rate and standardized mortality rates of the cancer from GLOBOCAN in 2012, and HDI and its components extracted from the global bank site. Data were analyzed using correlation tests and regression with SPSS software (version 15). Results: Among the six regions of WHO, the highest breast cancer incidence rate (67.6) was observed in the PAHO, and the lowest incidence rate was 27.8 for SEARO. There was a direct, strong, and meaningful correlation between the standardized incidence rate and HDI (r=0.725, $p{\leq}0.001$). Pearson correlation test showed that there was a significant correlation between age-specific incidence rate (ASIR) and components of the HDI (life expectancy at birth, mean years of schooling, and GNP). On the other, a non-significant relationship was observed between ASIR and HDI overall (r=0.091, p=0.241). In total, a significant relationship was not found between age-specific mortality rate (ASMR) and components of HDI. Conclusions: Significant positive correlations exist between ASIR and components of the HDI. Socioeconomic status is directly related to the stage of the cancer and patient's survival. With increasing the incidence rate of the cancer, mortality rate from the cancer does not necessariloy increase. This may be due to more early detection and treatment in developed that developing countries. It is necessary to increase awareness of risk factors and early detection in the latter.
Background: The aim of this study was to evaluate the incidence and mortality of stomach cancer, and its relationship with the Human Development Index (HDI) and its components in Asia in 2012. Materials and Methods: This ecological study wa conducted based on GLOBOCAN project of WHO for Asian countries. We assessed the correlations between standardized incidence rates (SIR) and standardized mortality rates (SMR) of stomach Cancer with HDI and its components using SPSS18. Results: A total of 696,231 cases (68.7% in males and 31.3% in females, ratio of 2.19:1) and 524,465 deaths (67.1% in men and 33.0% in women, ratio 2.03:1) were included in 2012. Five countries with the highest SIR of stomach cancer were Republic Korea, Mongolia, Japan, China and Tajikistan. Five countries with the highest SMR of stomach cancer were Mongolia, Tajikistan, Kyrgyzstan, Kazakhstan and China. Correlation between HDI and SIR was 0.241 (p = 0.106), in men 0.236 (p = 0.114) and in women -0.250 (p = 0.094). Also between HDI and SMR -0.250 (p = 0.871) in men -0.018 (p = 0.903) and in women -0.014 (p = 0.927). Conclusions: No significant correlation was observed between the SIR of stomach cancer, and the HDI and its dimensions, such as life expectancy at birth, mean years of schooling, and income level of the population.
Objectives: Busan had the highest mortality and the shortest life expectancy at birth among 16 provinces in Korea in 2008 and there were considerable health inequalities within the region. This study was performed to build up a priority setting framework in Healthy City Busan project. Methods: Analytic hierarchy process was used to determine the relative priority weight for different strategic and program dimensions along with the consistency of response. An on-site workshop-based meeting (calculating importance) and online survey (calculating risk) were conducted to obtain data from 8 experts. Results: The results showed that in strategic criteria "active health promotion & diseases prevention" and "building infrastructure for the Health City project" were two most important factors. In program criteria, considering both importance and risk scores, "making a healthy community" and "building community health centers" in disadvantaged areas were a top priority group. In addition, "enacting an ordinance for the Healthy City", "building the infrastructure for health impact assessment" and "making health care safety net for vulnerable population" were also higher priorities group. Conclusions: Our findings suggest that the Healthy City project in Busan should be focused on strengthening health equity and building infrastructure for sustainability of the project.
Purpose: This study aimed to investigate the incidence and mortality of breast cancer, and its relationship with human development index (HDI) and its components in Asia in 2012. Materials and Methods: This study was an ecologic study in Asia for assessment of the correlation between age-specific incidence rate (ASIR) and age-specific mortality rate (ASMR) with HDI and its details that include: life expectancy at birth, mean years of schooling and gross national income (GNI) per capita. Data about SIR and SMR for every Asian country for the year 2012 were obtained from the global cancer project. We used a bivariate method for assessment of the correlation between SIR and SMR and HDI and its individual components. Statistical significance was assumed if P<0.05. All reported P-values are two-sided. Statistical analyses were performed using SPSS (Version 15.0, SPSS Inc.). Results: In 2012, 639,824 cases of breast cancer were recorded in Asian countries. Countries with the highest standardized incidence rate (ASIR) (per 100,000) were Israel (80.5), Lebanon (78.7), Armenia (74.1) and the highest standard mortality rate (ASMR) was observed in Pakistan (25.2), Armenia (24.2), and Lebanon (24). There was a positive correlation between the ASIR of breast cancer and HDI (r = 0.556, p <0.001), whereas there was a negative correlation between the ASMR of breast cancer and HDI (r = -0.051). Conclusions: Breast cancer incidence in countries with higher development is greater, while mortality is greatest in countries with less development. There was a positive and significant relationship between the ASIR of breast cancer and HDI and its components. Also there was a negative but non significant relationship between the ASMR of breast cancer and HDI.
Background: Liver cancer (LC) is the sixth world most common cancer and the second leading cause of cancer death. Due to the importance and necessity of awareness about the incidence and mortality of diseases to perform prevention programs, this study focused on data for LC and its relationship with the human development index (HDI) and its components in Asia in 2012. Materials and Methods: This ecological study was based on GLOBOCAN data for Asian countries. We assessed correlations between standardized incidence rates (SIR) and standardized mortality rates (SMR) of LC with HDI and its components using of SPSS18. Results: A total of 582,420 incident cases and 557,097 deaths were recorded in Asian countries in 2012. The five with the highest SIR were Mongolia, Lao PDR, Vietnam, Republic of Korea and Thailand and those with the highest SMR were Mongolia, Lao PDR, Vietnam, Cambodia and Thailand. A negative relation was observed between HDI and LC for SIR of 0.049 (P=0.748) and for SMR of 0.07 (P=0.645), with life expectancy at birth a positive relation for SIR of 0.061 (P=0.687) and a negative relation for SMR of 0.079 (P=0.603), with the average years of education a negative relation fo SIR of 0.476 (p=0.952) and for SMR of 0.032 (P=0.832), and with the country income level per person a negative relation for SMI of 0.11 (p=0.465) and for SMR of 0.113 (P=0.455). Conclusions: The incidence of LC is more in less developed and developing countries but statistically significant correlations were not found between standardized incidence and mortality rates of LC, and HDI and its dimensions.
Park, Jin Su;Jeong, Ji Seong;Yang, Chul Seung;Lee, Jeong Gi
The Journal of the Convergence on Culture Technology
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v.8
no.6
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pp.899-904
/
2022
As the world becomes an aging society due to a decrease in the birth rate and an increase in life expectancy, a system for health management of the elderly population is needed. Among them, various studies on occupancy and activity types are being conducted for smart home care services for indoor health management. In this paper, we propose a random forest model that classifies activity type as well as occupancy status through indoor temperature and humidity, CO2, fine dust values and UWB radar positioning for smart home care service. The experiment measures indoor environment and occupant positioning data at 2-second intervals using three sensors that measure indoor temperature and humidity, CO2, and fine dust and two UWB radars. The measured data is divided into 80% training set data and 20% test set data after correcting outliers and missing values, and the random forest model is applied to evaluate the list of important variables, accuracy, sensitivity, and specificity.
The aim of this paper is to describe a long-term trend toward earlier retirement and its reversal since 1985 in the United States, together with changes in socioeconomic conditions and social-policy programs which have contributed to this new development. The American people's recent propensity to retire at relatively younger ages was mainly a result of secular increase in individual wealth that had made it possible for them to enjoy higher standard of living without their participation in labor market activities at older ages. In addition to the introduction of compulsory retirement system, both social security retirement pension program and corporate pension system have also contributed significantly to the declining retirement age and its reversal around the mid-1980s. This paper pays full attention to the set of social policy programs which are currently being used to sustain the recent reversal in ages at retirement. The basic question to be raised here, however, is about whether or not the U. S. government will ave to continue to implement the social policies and programs used to discourage the elderly from retiring at relatively younger ages in the future. In this paper, it is argued that labor productivity growth and improvement in work attitude prior to retirement will help the elderly find little difficulties in having higher standard of living, despite their further lengthening of life expectancy at birth and post-retirement survival chances, the latter being often called the "third life". Most American people hope that the social-policy programs that have promoted early retirement will remain unchanged in the first part of the 21st century while they will put significant financial burden on their future descendants who have to work in the paid labor market. Taking this observation in consideration, this paper concludes that the U. S. government has to focus more on developing the programs that improve work propensity and labor productivity among the currently working-age population rather than continuing to implement the programs that sustain the recent reversal in retirement ages.ment ages.
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