• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.20 no.3
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• pp.153-159
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• 2017

Purpose: Acid neutralization during chemical clearance is significantly prolonged in children with cystic fibrosis, compared to symptomatic children without cystic fibrosis. The absence of available reference values impeded identification of abnormal findings within individual patients with and without cystic fibrosis. The present study aimed to test the hypothesis that significantly more children with cystic fibrosis have acid neutralization durations during chemical clearance that fall outside the physiological range. Methods: Published reference value for acid neutralization duration during chemical clearance (determined using combined impedance/pH monitoring) was used to assess esophageal acid neutralization efficiency during chemical clearance in 16 children with cystic fibrosis (3 to < 18 years) and 16 age-matched children without cystic fibrosis. Results: Duration of acid neutralization during chemical clearance exceeded the upper end of the physiological range in 9 of 16 (56.3%) children with and in 3 of 16 (18.8%) children without cystic fibrosis (p=0.0412). The likelihood ratio for duration indicated that children with cystic fibrosis are 2.1-times more likely to have abnormal acid neutralization during chemical clearance, and children with abnormal acid neutralization during chemical clearance are 1.5-times more likely to have cystic fibrosis. Conclusion: Significantly more (but not all) children with cystic fibrosis have abnormally prolonged esophageal clearance of acid. Children with cystic fibrosis are more likely to have abnormal acid neutralization during chemical clearance. Additional studies involving larger sample sizes are needed to address the importance of genotype, esophageal motility, composition and volume of saliva, and gastric acidity on acid neutralization efficiency in cystic fibrosis children.

• Journal of Genetic Medicine
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• v.16 no.2
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• pp.55-61
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• 2019

Purpose: Genetic defects in the nuclear-encoded mitochondrial aminoacyl-tRNA synthetases were first identified as causes of various disorders in 2007. Variants in IARS2, which encodes a mitochondrial isoleucyl-tRNA synthetase, were first reported in 2014. These variants are associated with diverse phenotypes ranging from CAGSSS (CAtaracts, Growth hormone deficiency, Sensory neuropathy, Sensorineural hearing loss, and Skeletal dysplasia) and Leigh syndrome to isolated nonsyndromic cataracts. Here, we describe the phenotypic and genetic spectrum of Korean patients with IARS2-related disorders. Materials and Methods: Using whole-exome sequencing followed by Sanger sequencing, we identified five patients with IARS2 mutations. Their medical records and brain magnetic resonance images were reviewed retrospectively. Results: All five patients presented with developmental delay or regression before 18 months of age. Three patients had bilateral cataracts, but none had hearing loss or sensory neuropathy. No evidence of skeletal dysplasia was noted, but two had short stature. One patient had cardiomyopathy and another exhibited renal tubulopathy and hypoparathyroidism. Their brain imaging findings were consistent with Leigh syndrome. Interestingly, we found the recurrent mutations p.R817H and p.V105Dfs*7 in IARS2. Conclusion: To our knowledge, this is the first report of Korean patients with IARS2-related disorders. Our findings broaden the phenotypic and genotypic spectrum of IARS2-related disorders in Korea and will help to increase clinical awareness of IARS2-related neurodegenerative diseases.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.2
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• pp.55-62
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• 2017

Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by beta-glucosidase deficiency. An 18 month-old male with hepatosplenomegaly, anemia, thrombocytopenia, and growth retardation referred to our hospital. The patient showed neurological symptoms, such as supranuclear gaze palsy and developmental delay. Bone marrow biopsy performed to rule out malignancy and the results revealed no malignant cell; however, abnormal histiocytes suggesting storage disease was noted. Based on hepatosplenomegaly, bicytopenia and unexplained neurologic manifestations, enzyme activity and genetic analysis were conducted emergently with a strong suspicion of GD. Beta-glucosidase activity in leukocyte was decreased. GBA sequencing to confirm the diagnosis revealed compound heterozygous pathogenic variants (i.e., c.754T>A, c.887G>A), both previously reported as the cause of neuronopathic GD. Under the diagnosis of type 3 GD, the patient immediately received enzyme replacement therapy (ERT). After 17 months of ERT, the size of spleen decreased, and hemoglobin and platelet count returned to normal. In addition, the activity of chitotriosidase and angiotensin converting enzyme decreased. However, myoclonic movement and generalized seizure occurred at the age of 19 months and antiepileptic drug was started. Other neurological deterioration including supranuclear gaze palsy and developmental delay also persisted. A new therapy to overcome neurologic problems should be developed for patients with type 3 GD.

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.5
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• pp.2667-2672
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• 2016

Background: Virtually all cases of cervical cancer are caused by persistent infections with a restricted set of human papillomaviruses (HPV). Cancer of the cervix is the third or even the second most common cancer in women worldwide, more than 85% of the cases occurring in developing countries, such as China and India, including the Republic of Kazakhstan. The purpose was to determine the HPV type distribution to evaluate efficacy of vaccination and adjust cancer prevention strategy in Western Kazakhstan in the future. Materials and Methods: A retrospective analysis was conducted of data obtained from PCR laboratories in 4 regional centers for the time period covering 12 months, 2013-2014, using AmpliSens$^{(R)}$ Real-Time PCR kits for HPV testing of 12 genotypes (16, 18, 31, 33, 35, 39, 45, 51, 52, 56, 58, and 59). Results: A total of 1,661 persons were HPV tested within 2013-14, but a proprotion examined for 16 and 18 genotypes only (563) was not been included for statistic analysis of distribution and ratio of the most common genotypes. Males accounted for only a small number (N=90 in total). Conclusions: Total number of the HPV-positive appeared to be 26.0%, or 286 of N=1098. Types distribution was as follows: type 16 (10.7%), 39 (5.83%), 51 (5.27%), 31 (4.85%), 56 (4.58%), 18 (3.61%), 59 (2.64%), 58 (2.22%), 35 (1.94%), 33 (1.25%). Overall the HPV infection was highest in 16-29 years old (62.4%) and decreased with age. Total prevalence of the HR-HPVs amongst male population was 21.4% with top five types 16, 18, 39, 51, 31. Trends forcorrelations between Aktau site and type 33 (Cramer's V 0.2029), between Caucasian ethnicity and type 33 (Cramer's V .1716), and between European ethnicities in Uralsk and type 45 (Cramer's V .1752) were found. Of N 563 tested separately for 16 or 18 types, 13.6% were positive. As a whole, the distribution of 16/18 types had a ratio of 3.53:1. Given the vaccine-targeted type 16 is widely spread amongst this regional population, HPV immunization program of adolescent girls 10-13 years should be implemented appropriately.

• Korean Journal of Community Nutrition
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• v.4 no.2
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• pp.219-230
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• 1999

The purpose of this study was to find out the common characteristics of eating behaviors of the long-lived elderly in order to offer the basic data for establishing dietary guidelines for a long and healthy life for the general population. The subjects were three-hundred elderly people over age 85 living in Kyungpook Sung-ju who have no problems in daily living. The general characteristics, nutrition knowledge and attitudes, meal patterns, and food preferences of the subjects were surveyed by individual intervies. The levels of the nutrition knowledge of the subjects were very low(average score ; 4.3) and the correlation coefficient between their nutrition knowledge and attitude score was also low(r=0.323, p=0.000). Most of the subjects(93%) had a regular meal pattern consuming three meals a day. The major staple food was rice mixed with other grains(75%) and the number of side dishes was mostly under four(99%). A majority(73%) had no habit of overeating and 51% of the subjects were taking snacks besides regular meals. Thirty one percent of the subjects had drinking habits and 80% of the drinking subjects had over 40 years of drinking history. The most common frequencies for intakes of various food groups were ; more than once a day(95%) for vegetables ; 2∼3 times a week(74%) for green and orange color vegetables ; 2∼3 times a week(72%) for fruits ; once a day(70%) for fish, eggs and legumes ; 2∼3 times a week(49%) for milk ; 2∼3 times a week(72%) for fruits ; once a day(70%) for fish, eggs and legumes ; 2-3 times a week(49%) for milk ; 2-3 times a week(85%) for seaweeds and 2-3 times a week(81%) for foods cooked with oil. The most preferred foods by the subjects were white rice(staple foods), soybean paste soup(soups), beef and eggs(meats and eggs), yellow croaker and hair tail(fish and shellfish), yoghurt(milk and milk products), all vegetables except carrot(vegetables), and watermelon(fruits). The popular food preparation methods included mixing with seasonings and the most preferred taste was sweet. The results showed that the eating pattern of long-lived elderly was characterized by regularity, simplicity, and no overeating.

• Journal of Preventive Medicine and Public Health
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• v.32 no.1
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• pp.60-64
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• 1999

Objectives. Changes in lung function are frequently used as biological markers to assess the health effects of criteria air pollutants. We tried to formulate the prediction models of pulmonary functions based on height, weight, age and gender, especially for children aged 12 years who are commonly selected for the study of health effects of the air pollution. Methods. The target pulmonary function parameters were forced vital capacity(FVC) and forced expiratory volume in one second(FEV1). Two hundreds and fifity-eight male and 301 female 12-year old children were included in the analysis after excluding unsatisfactory tests to the criteria recommended by American Thoracic Sosiety and excluding more or less than 20% predicted value by previous prediction equations. The weight prediction equation using height as a independent variable was calculated, and then the difference of observed weight and predicted weight (i.e. residual) was used as the independent variable of pulmonary function prediction equations with height. Results. The prediction equations of FVC and FEV1 for male are FVC(ml) = $50.84{\times}height(cm)+7.06{\times}weight$ residual 4838.86, FEV1(ml) = $43.57{\times}height(cm)+3.16{\times}weight$ residual - 4156.66, respectively. The prediction equations of FVC and FEV1 for female are FVC(ml) = $42.57{\times}height(cm)+12.50{\times}weight$ residual - 3862.39, FEV1(ml) = $36.29{\times}height(cm)+7.74{\times}weight$ residual - 3200.94, respectively.

• Research in Community and Public Health Nursing
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• v.9 no.2
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• pp.533-549
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• 1998

This descriptive study was designed to measure the degrees of work stress and job satisfaction and use interpersonal conflict resolution strategy as an attempt to provide clinical nurses basic data to develop strategies releasing work stress and improving job satisfaction. These data were collected from 367 clinical nurses working in 9 hospitals nation-wide from May 1, to June 30, 1998. A structured questionaire was used for data collection. The questionnaire included Kim and Koo's work stress scale, Lee's interpersonal conflict resolution strategy scale and a Minesota satisfaction questionnaire. Data analysis was done by the use of the SAS computer program with descriptive statistics, t -test, ANOVA, Scheffe test, Pearson Correlation Coefficient and Cronbach - ${\alpha}$. The results were as follows 1. The mean score of work stress for the subjects was 4.34(SD=0.64). The highest scored reasons for work stress were 'inadequate com pensation' 4.93(SD=0.86), 'lack of professional knowledge and skill' 4.70(SD=0.92) and 'unsatisfactory relationship with superior authorities' 4.63(SD=0.97). The lowest scored reasons were 'unsatisfactory relationship with inferior staff' 3.72(SD=1.02), 'inadequate mechanical environment' 3.72(SD=1.11) and 'interpersonal problems' 3.85 (SD=1.06), The work stress in clinical nurses differed significantly depending on the hospital type (F=4.00, p=0.0082). 2. The clinical nurses used compromise(45.1%) as the best interpersonal conflict resolution strategy. The second was collaboration(32.8%), the third accomodation(14.2%), the forth avoidance(4.2%) and the fifth competition(3.6%). 3. The item score of job satisfaction of the subject was 3.00(SD=0.43), The job satisfaction in clinical nurses differed significantly depending on age(F=5.67, p=0.0000), marital status(t= -1.89, p=0.0511), academic career(F=5.84, 0.0001), official position (F=7.38, p=0.0001), and work career(F=6.33, p=0.0001). 4. The result of the analysis of relationship between work stress and job satisfaction was significant(r= -0.34, p=0.0000). In conclusion, it was found that work stress was very high in clinical nurses. They used interpersonal conflict resolution strategies to release their interpersonal problems and to increase their job satisfaction. Therefore, adequate stress management for clinical nurses will provide them with higher job satisfaction and hence lead to more qualitified nursing care.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.4
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• pp.526-531
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• 2010

Early childhood caries which mainly affect maxillary anterior area, defined as 'the presence of 1 or more decayed, missing, or filled tooth surfaces in any primary tooth in a child 71 months of age or younger'. Extraction of teeth when early childhood caries affected in maxillary primary incisors often develops progressively, result in severe destruction of crowns, acute or chronic pulpitis, and periapical abscess formation. Maxillary primary incisors are need to preserve as possible, because the early loss of maxillary primary incisors may lead to various functional, esthetical, and psychological problems. It is necessary to the availability of an easy to perform technique capable of providing efficient, durable, functional, and esthetic restorative methods. Polyethylene fiber-reinforced post can be used in strengthen of composite resins that is esthetic and good physical and mechanical properties. $Ribbond^{(R)}$ is made from an polyethylene fiber, has numerous usages, its surface is treated to enhance adhesion to resins, ease of manipulation, relatively cost effective. We report this case, had restored of maxillary primary incisors with severe coronal destruction due to affecting severe early childhood caries, using polyethylene fiber-reinforced posts, composite resin cores, and celluloid crowns. We could obtain good result of treatment.

• Asian-Australasian Journal of Animal Sciences
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• v.21 no.3
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• pp.420-425
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• 2008

The storage of rice polishing (RP) with and without addition of antioxidant for sixteen weeks and its effect on rancidity and metabolizable energy values during the summer season was determined. Fifteen Single Comb White Leghorn cockerels of approximately uniform age and weight were procured and kept in metabolic cages under standard feeding and management practices. Five force feeding trials were conducted. In the first trial, fresh RP with 0 weeks of storage (diet 1 and 2) was used followed by four feeding trials with 4 (diet 3, 4), 8 (diet 5, 6), 12 (diet 7, 8), and 16 (diet 9, 10) weeks of storage of RP. The same birds were used in all trails. The birds were fasted for a period of 21 h, followed by force feeding of 20 g of RP with and without antioxidant for all storage periods. The control/fasting group was also maintained to measure endogenous fecal losses. Excreta were collected after 48 h for the determination of AME and TME values of RP. Along with the biological trials, laboratory assay of the RP stored with and without antioxidant was conducted to measure the extent of rancidity in terms of Thiobarbituric acid value (TBA). The TBA values were affected (p<0.05) by storage period and the values increased when the storage period increased from 4 to 16 weeks. However, the TBA values were significantly reduced (p<0.05) when RP was stored after addition of antioxidant when compared with the values obtained from RP stored without antioxidant (diet 3 vs. 4, 5 vs. 6, 7 vs. 8, and 9 vs. 10). The AME MJ/kg and TME MJ/kg values of RP were neither affected by increase in storage period nor addition of antioxidant. The findings of this study revealed that there was no effect of rancidity and storage time on the nutritive value, AME or TME of RP in poultry. However, TBA values were increased with the increase in storage period.

• Asian-Australasian Journal of Animal Sciences
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• v.33 no.5
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• pp.788-801
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• 2020

Objective: This study was conducted to evaluate the effects of diets containing different wet rice distillers' by-product (RDP) levels on growth performance, nutrient digestibility, blood profiles and gut microbiome of weaned piglets. Methods: A total of 48 weaned castrated male crossbred pigs, initial body weight 7.54±0.97 kg, and age about 4 wks, were used in this experiment. The piglets were randomly allocated into three iso-nitrogenous diet groups that were fed either a control diet, a diet with 15% RDP, or a diet with 30% RDP for a total of 35 days. Chromium oxide was used for apparent digestibility measurements. On d 14 and d 35, half of the piglets were randomly selected for hemato-biochemical and gut microbiota evaluations. Results: Increasing inclusion levels of RDP tended to linearly increase (p≤0.07) average daily gain on d 14 and d 35, and decreased (p = 0.08) feed conversion ratio on d 35. Empty stomach weight increased (p = 0.03) on d 35 while digestibility of diet components decreased. Serum globulin concentration decreased on d 14 (p = 0.003) and red blood cell count tended to decrease (p = 0.06) on d 35, parallel to increase RDP levels. Gene amplicon profiling of 16S rRNA revealed that the colonic microbiota composition of weaned pigs changed by inclusion of RDP over the period. On d 14, decreased proportions of Lachnospiraceae_ge, Ruminococcaceae_ge, Ruminococcaceae_UCG-005, and Bacteroidales_ge, and increased proportions of Prevotellaceae_ge, Prevotella_2, and Prevotella_9 were found with inclusion of RDP, whereas opposite effect was found on d 35. Additionally, the proportion of Lachnospiraceae_ge, Ruminococcaceae_ge, Ruminococcaceae_UCG-005, and Bacteroidales_ge in RDP diets decreased over periods in control diet but increased largely in diet with 30% RDP. Conclusion: These results indicate that RDP in a favorable way modulate gastrointestinal microbiota composition and improve piglet performance despite a negative impact on digestibility of lipids and gross energy.