Purpose : The mucopolysaccharidoses (MPSs) are a heterogeneous group of lysosomal storage disorders. They are caused by a deficiency of the enzymes involved in the degradation of glycosaminoglycans. Early recognition is important because recombinant enzyme replacement therapy is now available for MPS. We studied the clinical characteristics of 80 MPS children with the object of determining the epidemiological, clinical and radiological features in Korean MPS children. Methods : Diagnosis of MPS was confirmed by skin fibroblast enzyme analysis in 80 patients between February 1995 and December 2004. Charts were retrospectively reviewed for clinical and radiological findings, as well as for intelligence and speech evaluations. Results : Hunter syndrome (MPS type II) was the most prevalent type, appearing in 51/80 cases (64 %), followed by Sanfilippo syndrome (MPS III-18%), Hurler syndrome (MPS I-15%), and Morquio syndrome (MPS IV-4%). The average age at diagnosis was 5.5 years (range 1 to 20), and the male-to-female ratio was 4.7 : 1. Typical radiographic changes were observed in 45/54 cases (83%). Mitral regurgitation was the most common cardiac defect. Moderate to profound mental retardation and hearing loss were present in 14/35 cases (56%) and 33/38 cases (82%), respectively. Four MPS II patients had bone marrow transplantation, with mixed outcomes. Five MPS I patients are currently on enzyme replacement therapy. Conclusion : Our study showed a high proportion of MPS II cases (64%), which may represent population variability. By studying the clinical features of these patients, we hope to alert pediatricians of the warning signs of MPS.
Kang, Sang Yul;Kim, Hyoung Jun;Kim, Tschung Il;Park, Hyun Ju;Na, Choon Ki;Han, Moo Young
Journal of Korean Society of Environmental Engineers
/
제38권8호
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pp.420-427
/
2016
This study was conducted on the sediment remediation using micro-bubble to remove fine particles. For this study, characteristics of contamination and release in sediment were analyzed. And then, the characteristics of bubbles on removal efficiency was investigated at various operation conditions. In particle size distribution of the sediment used for the study, the proportion of clay and silt (<0.075 mm) was about 7.7%, sand (0.075~4.75 mm) was about 67.8%, and gravel (${\geq}4.75$) was 24.5%. Total nitrogen (TN) and total phosphorus (TP) of the sediment were 2,790~3,260, 261~311 mg/kg respectively. Ignition loss and water content were 4.1~9.6, 32.9~53.2% respectively. In analysis of removal efficiency according to operation conditions of micro-bubble, it was the highest when operation condition is pressure 6 atm, pressurized water ratio 30%, and coagulant dosage 15 ppm. At the time, the sediment's removal efficiency was 19.9%. Accordingly removal efficiency of TN and TP were 21.4, 22.6% respectively. Finally a research was found that fine particles in sediment were almost removed by micro-bubble, which led to decrease nutrients' release at about 20.1~64.3% in comparison to sediment including lots of fine particles.
This study was conducted in the sandy loam soil to find out the effects of fly ash and zeolite applications on saving of irrigation water which was required great quantities during the cultivation days of dry seeded rice. Total water loss was reduced so rapidly in the early growth stage that n was the least in the 18th day after irrigation. After that day it was increasd due to the growth development and the rising temperature. In the daily mean of decreasing water level during the whole irrigation days, control was 47.7mm but the treatments of fly ash and zeolite were 31.8mm and 35.8mm respectively Amounts of the presumed irrigation water of fly ash and zeolite treatments were lower about 29.1% and 20.9%. respectively than that of control during the whole irrigation days These gaps between control and amendment treatments were greater in the early growth stage than in the late stage Hardness, bulk density and the ratio of solid phase in the soil after experiment were the highest in the fly ash treatment and those of control and zeolite treatments were showed the same trends each other. Amounts of water percoration of control and zeolite treatments in the soil were increased 8.2% and 2.6% respectively in comparision with those in the soil after amendment application, but that of fly ash treatment was decreased 42.5%, In contrast to the water percoration of control in the soil after experiment, those of fly ash and zeolite treatments were decreased 76% and 32%.
The effects of vacuum precooling(VP) on the shelf-life of polyethylene film(PE) packaged King oyster mushrooms(Pleurotus eryngii) during storage at $-1^{\circ}C$ were investigated. VP was conducted below $0^{\circ}C$ in a $-1^{\circ}C$ cold chamber of 40 minutes, and mushrooms were stored for 30 days in batches of 1kg. The weight loss of the VP-treated mushroom was slightly lower than that of control. The $O_2$ concentrations of VP-treated mushroos, within 4 days of storage, were 2.44-14.50 %/kg-package/hr, thus higher than control values(2.01-8.19%/kg-package/hr). $CO_2$ generation of VP-treated mushrooms, again within 4 days of storage, was 0.47%/kg-package/hr, thus lower than that of controls(0.58%/kg-package/hr). The $CO_2/O_2$ ratio peaked on day 4 of storage in the control group, tbut no such peak was observed in VP-treated mushrooms. In the VP-treated fungi, lightness was higher, and redness and yellowness lower, than in controls, at all storage times.. In VP-treated mushrooms, strength, hardness and chewiness were significantly higher than in controls, but there were no significant differemces in springiness or cohesiveness. Softening and breakdown of under-cap wrinkles were observed in control mushrooms stored for 30 days, but occurred to a lesser extent in VP-treated fungi. Stipe reticulum tissue vessels in the 30 day-stored VP-treated mushrooms were relatively well-defined and clear, but were softer and diffuse in the control fungi. The results thus confirmed that VP after harvest enhanced mushroom shelf-life and PE packaging prolonged storage time. The data will have industrial applications.
Journal of the Korean Society of Food Science and Nutrition
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제33권1호
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pp.83-90
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2004
This study was conducted to examine the effects of $\beta$3-adrenergic receptor polymorphism on the blood glucose level and obesity in 530 volunteers, who attended a weight loss program in a local obesity clinic. The age differences in total subjects and the distribution of male and female were 26.55$\pm$0.31 yr, 9.1% (n=48), 90.9% (n=492). The genotype distribution of $\beta$3-AR gene polymorphism were WW type 75%, WR type 22% and RR type 3%. Among many parameters, fasting blood glucose was significantly higher in WR+RR type (p=0.001) compared with WW type. When the subjects were divided into two groups by 6.105 mmol/L of the fasting blood glucose level, the frequency of hyperglycemia was 23.3% in WW type subjects, while there was a increase to 35.6% in WR+RR type subjects (p=0.011, $\chi$$^2$-analysis). When hyperglycemia group was compared with normoglycemia group, obesity index (p=0.044), %body fat (p=0.046) and TG (p=0.000) were significantly higher, and HDL (p=0.006) was significantly lower in the hyperglycemia. When all of the above factors were included in stepwise logistic regression analysis to find risk factors of hyperglycemia, the results were that the odds ratio for hyperglycemia were 2.015 (p=0.011) for WR+RR type of $\beta$3-AR gene, 2.165 (p=0.000) for TG and 0.419 (p=0.059) for HDL cholesterol. There was a significantly positive correlation between the blood glucose vs BMI, WHR, body fat in the WW type (r=0.099, 0.119, 0.082) However, in the WR and RR type there were no significance between the blood glucose vs BMI, WHR, body fat. These data suggest that the WR+RR genotype of $\beta$3-AR has a very strong association with increased blood glucose level and might be a significant risk factor for hyperglycemia among Korean subjects.
This study identified the impact of childhood cancer on the Korean family. The purpose was to contribute knowledge for family nursing and pediatric hospice care practice with sick children and their families. This descriptive study was conducted during a 6 month period with children who were being treated for cancer at six university hospitals in Seoul. The data were gathered from members of 68 families ; 24(Group A), with a child newly diagnosed with cancer : 27(Group B), with a child under treatment and without complications, and 17 (Group C), with a child in relapse. Medical records, structured questionnaires and interviews were used for data collection. The questionnaires and interview schedules had been used previously in Martinson's research in the USA and China. The findings, conclusions, and suggestions are as follows. 1. The impact of childhood cancer on the family. Members of the family experienced fear, helplessness, guilty feelings, and anger at the time of the initial diagnosis and at relapse. Mothers complained of headache, anorexia and poor appetite, weight loss, sleep disturbance, and bad dreams. Many of the fathers either lost or changed jobs, and all working mothers stopped working. Half the parents reported changes in their marital relationships such as frequent quarrels but also stronger unity. Family members perceived cancer as the most frightening disease. Change in their world view was expressed as living on faith understanding suffering, determining to live a better life, wanting to live an upright life and valuing health as the most important. Religious activities are found most helpful through this difficult experience. Financial debt due to the treatment and care of the sick child, burdened 22 families. The above mentioned impact was most evidant in Group B(those presently undergoing treatment) and Group C(those in relapse). Findings indicate that nursing care should embrace the family of a child who is being treated for cancer. 2. Characteristics of the child with cancer The majority of the children in this sample had a diagnosis of leukemia. Their mean age was 6.8 and the ratio of boys to girls was 1.12 ; 1. The mean hospitalization frequency was 13.5 times and the mean duration of illness was 16.8 months. Most of 1.he children perceived cancer as the most frightening disease ; 32.7% of the children described their sickness as serious. Children in Group C were hospitalized more frequently, stayed in hospital for longer periods, and expressed their sickness as quite serious more often than the other two groups. These findings indicate how much comprehensive pediatric hospice nursing care services are needed along with relevant research and nursing education. 3. Characteristics of the families. The mean age of the father was 39.5 and the mother, 36,6 ; they are in their most productive life period. Mothers especially expressed feelings of financial uneasiness and powerlessness about giving up their jobs, and guilty feelings for not providing enough care and concern to other children due to taking care of the sick one. The burden of caring for the sick child can bring negative changes in family dynamics which they think provoke potential health problems in members of the family These findings suggest a need for nursing support and counselling resources. Findings also suggest the need for ethical inquiry about such questions as who should give information to the child in regard to diagnosis and prognosis, when, and how. Other suggestions included : 1) Quality health care for childhood cancer such as home care and pediatric hospice programs should be established. 2) Special and practical consideration for long-term patients should be made in the present insurance coverage. The reimbursement period for long-term patients should be lengthened. 3) Further in-depth qualitative studies are needed. 4) Education programs including guided practice experience for pediatric hospice care practitioners are needed.
Amine can undergo irreversible reactions by $O_{2}$ and high temperature in amine scrubbing process and these phenomena are called "degradation". Degradation causes not only a loss of valuable amine, but also operational problems such as foaming, corrosion and fouling. In this study, using various chemical absorbents(MEA; monoethanolamine, AMP; 2-amino-2-methyl-1-propanol, DAM; 1,8-diamino-p-menthane), we examined the following variable. I) loading ratio of $CO_{2}$ at $50^{\circ}C$ and $120^{\circ}C$, ii) concentration variation and initial degradation rate constant of absorbent in $CO_{2}$ and $CO_{2}/O_{2}$ system, and iii) effect of degradation by $O_{2}$. The $CO_{2}$ loading of 20 wt% DAM was 400% and 270% higher than that of 20 wt% MEA and AMP at 50, respectively and was the largest the difference of $CO_{2}$ loading between absorption $(50^{\circ}C)$ and regeneration $(120^{\circ}C)$ condition. The initial degradation rate constant of 20 wt% DAM was $2.254{\times}10^{-4}cycle^{-1}$ which was slower than that of MEA $(2.761{\times}10^{-4}cycle^{-1})$ and AMP $(2.461{\times}10^{-4}cycle^{-1})$ in $CO_{2}$ system. Also, it was increased 30% by $O_{2}$ that effects on the degradation by $O_{2}$ was less than 100% increased. these degradation reactions was able to identify by formation of new peak in GC and FT-IR spectrum analysis.
Background : To evaluate the clinical characteristics of lung cancer patients in Korea, where there is a higher number of smokers than in Western countries. Methods : A retrospective study was performed on 1655 lung cancer patients, who were diagnosed at a university hospital between September 1996 and August 2005. Age, gender, cell types and clinical stage were analysed. Of 941 patients, who responded to a questionnaire at the time of diagnosis, the smoking habits, occupational history, family history of lung cancer in the first-degree relatives, coexisting diseases (diabetes mellitus and cardiovascular disease), body weight loss, ECOG performance status and presenting symptoms, were examined prospectively. In addition, coexisting diseases including chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis and active pulmonary tuberculosis were evaluated. Results : Of the 1655 patients, the male to females ratio was 3.6. Squamous cell carcinoma was the most common cancer whereas adenocarcinoma was more common in lifetime nonsmokers or women. 19.9% of the patients were non smokers and 80.1% ever smokers. Since 2000, there was an increase in the incidence of adenocarcinoma with a corresponding decrease in the incidence of squamous cell carcinoma. 6.2% of patients were asymptomatic. A coincident diagnosis of chronic obstructive pulmonary disease, cardiovascular disease, diabetes mellitus, active pulmonary tuberculosis, and idiopathic pulmonary fibrosis was made in: 44.1%, 22.2%, 10.7%, 3.9%, and 1.6% of patients, respectively. A positive family history of lung cancer in the first-degree relatives was identified in 4.4% of patients. An occupational history relevant to lung cancer was identified in 12.2% of patients. Conclusion : There is a high proportion of cigarette smokers in Korean lung cancer patients. The most common cell type was squamous cell carcinoma. However, a more detailed, prospective study of the clinical characteristics will be needed to better characterize lung cancer in Korea.
As an abiotic stress, chilling stress is one of the major factors limiting plant growth and increasing susceptibility to pathogens. Therefore, enhancing stress tolerance in plants is an important strategy for their survival under unfavorable environmental conditions. The objective of this study was to determine the effects of the exogenous application of salicylic acid (SA) or nitric oxide (NO) on chilling tolerance in pepper seedlings. Pepper (Capsicum annuum L. 'kidaemanbal') seedlings were grown under normal growing conditions ($20/25^{\circ}C$, 15 hours photoperiod, $145{\pm}5{\mu}mol{\cdot}m^{-2}{\cdot}s^{-1}$, fluorescence lamps) for 23 days after transplanting. The solution (3 mL) of 1 mM SA and 0.3 mM NO with surfactant triton 0.1% were sprayed two times a week, respectively. Right after the completion of chemical application, seedlings were subjected to chilling condition at $4^{\circ}C$ for 6 hours under dark condition and then the seedlings were recovered at the normal growing conditions for 2 days. In order to assess plant tolerance against chilling stress, growth characteristics, chlorophyll fluorescence (Fv/Fm), and membrane permeability were determined after chilling stress imposition. Total phenolic concentration and antioxidant capacity were measured during the whole experimental period. Disease incidence for pepper bacterial spot and wilt was also analyzed. Pepper seedlings treated with SA or NO were maintained similar dry mass ratio, while the value in control increased caused by chilling stress suggesting relatively more water loss in control plants. Electrolyte leakage of pepper seedlings treated with SA or NO was lower than that of control 2 days after chilling treatment. Fv/Fm rapidly decreased after chilling stress in control while the value of SA or NO was maintained about 0.8. SA increased higher total phenolic concentration and antioxidant capacity than NO and control during chemical treatment. In addition, increase in total phenolic concentration was observed after chilling stress in control and NO treatment. SA had an effect on the reduction of bacterial wilt in pepper seedlings. The results from this study revealed that pre-treatment with SA or NO using foliar spray was effective in chilling tolerance and the reduction of disease incidence in pepper seedlings.
Purpose: Aneuploidy is the cause of diseases such as Down syndrome or Edward syndrome and, more generally, is a major cause of mental retardation and fetal loss. The purpose of this study was to evaluate the association between MTHFR (C677T) or MTRR (A66G) polymorphisms and fetal aneuploidy. Materials and Methods: Data was collected from 37 women who had a fetus with aneuploidy (cases) and 78 women who had previously delivered at least two healthy children without aneuploidy and did not have a history of miscarriage or abnormal pregnancy (controls). The MTHFR (C677T) or MTRR (A66G) polymorphisms were analyzed by PCR-restriction fragment length polymorphism assay. Results: The frequencies of the MTHFR 677 CC, CT, and TT genotypes were 30.7%, 48.7%, and 20.6% in the control group and 37.8%, 48.6%, and 13.5% in the case group, respectively. There were no significant differences in genotype frequencies between the two groups. For the MTRR A66G polymorphism, the frequencies of the AA, AG and GG genotypes were 50%, 46.1%, and 3.9% in the control group and 13.5%, 81.1%, and 5.4% in case group, respectively. The frequency of the MTRR AG mutant was significantly increased in the case group, with an odds ratio of 6.5 (95% CI: 2.3-18.6, P<0.05). Conclusion: The results of this study suggest that mother carriers with the MTRR G allele have an increased risk of fetal aneuploidy, while the MTHFR T allele is not associated with increased risk of fetal aneuploidy. The MTRR A66G polymorphism may be a risk factor for producing a child with chromosomal aneuploidy.
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