• 제목/요약/키워드: AGE-R

검색결과 3,624건 처리시간 0.038초

Not All Children with Cystic Fibrosis Have Abnormal Esophageal Neutralization during Chemical Clearance of Acid Reflux

  • Woodley, Frederick W.;Moore-Clingenpeel, Melissa;Machado, Rodrigo Strehl;Nemastil, Christopher J.;Jadcherla, Sudarshan R.;Hayes, Don Jr;Kopp, Benjamin T.;Kaul, Ajay;Di Lorenzo, Carlo;Mousa, Hayat
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제20권3호
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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.

Clinical and genetic characteristics of Korean patients with IARS2-related disorders

  • Lee, Jin Sook;Kim, Man Jin;Kim, Soo Yeon;Lim, Byung Chan;Kim, Ki Joong;Choi, Murim;Seong, Moon-Woo;Chae, Jong-Hee
    • Journal of Genetic Medicine
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    • 제16권2호
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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.

18개월 남아에서 간비장비대, 성장 부진을 동반한 3형 고셔병 증례: 효소 대체 요법 후 임상 경과 (A Case of an 18-month-old Boy with Type 3 Gaucher Disease Presenting with Hepatosplenomegaly and Growth Retardation: The Clinical Course after Enzyme Replacement Therapy)

  • 임영신;황정윤;김진섭;양아람;박형두;전태연;조성윤;진동규
    • 대한유전성대사질환학회지
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    • 제17권2호
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    • pp.55-62
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    • 2017
  • 고셔병은 리소좀축적병으로 lysosomal hydrolase glucocerebrosidase 결여로 간비장비대, 골격계 증상, 빈혈, 혈소판 감소증의 증상을 나타내는 드문 상염색체 유전 질환이다. 본 증례에서는 18개월 남아에서 간비장비대, 성장 부진이 관찰되었으며 안구 운동 장애 및 발달 지연이 동반되어 제 3형 고셔병을 의심하였고 효소 분석 및 유전자 검사를 통해 확진하였다. 환아에서 한국인 신경형 고셔병에서 흔하게 관찰되는 c.754T>A(F213I)와 c.887G>A (R257Q)가 이형 접합체 돌연변이로 확인되었고 17개월 간의 효소 대체 요법을 통해 성장, 혈액학적 지표, 간비장비대 및 골증상은 호전되었지만 신경학적 증상의 호전은 없었고, 샤프론 중암브록솔에 유의한 반응이 있다고 알려져 있는 c.754T>A이 확인됨에 따라 환아에서 3개월간 암브록솔 치료를 시도하였지만 뚜렷한 임상적 치료 효과를 확인할 수 없었기에 본 증례를 보고하는 바이다.

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Distribution of High Risk Human Papillomavirus Types in Western Kazakhstan - Retrospective Analysis of PCR Data

  • Bekmukhambetov, YZ;Balmagambetova, SK;Jarkenov, TA;Nurtayeva, SM;Mukashev, TZ;Koyshybaev, AK
    • Asian Pacific Journal of Cancer Prevention
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    • 제17권5호
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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.

경북 성주지역 장수 노인의 식행동 특성 (Characteristics of Eating Behaviors of the Long-lived Elderly People in Kyungpook Sung-Ju)

  • 이혜성;김자현;구보경;김규종;백지원;이연경;이성국
    • 대한지역사회영양학회지
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    • 제4권2호
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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.

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체중 잔차를 이용한 12세 아동의 정상 폐기능 예측식 (Prediction Equations for FVC and FEV1 among Korean Children Aged 12 Years)

  • 강종원;성주헌;조수헌;주영수
    • Journal of Preventive Medicine and Public Health
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    • 제32권1호
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    • pp.60-64
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    • 1999
  • 환경오염과 관련된 건강효과 연구에서 흡연, 직업 등 교란변수의 영향을 효과적으로 배제할 수 있으면서 폐기능검사가 제대로 시행될 수 있는 연구대상으로 선호되고 있는 특정 연령층인 12세 학동의 보다 정확한 FVC와 FEV1 예측식을 만 들고자 전국 11개 중학교의 학생들(남자 256명, 여자 301명)을 대상으로 측정된 신장, 체중, 그리고 폐기능검사 값으로 신장-체중의 회귀식을 유도하였고, 이를 통해 12세 인구의 신장별 표준체중을 산출하였다. 이 표준체중과 실측체중의 차이인 잔차를 독립변수로 하여 폐기능 예측 식을 남녀별로 만들었는데, 남자의 경우는, FVC(ml) = 50.84 $\times$ 신장(cm) + 7.06 $\times$ 체중 잔차 - 4838.86, FEV1(m1) = 43.57 $\times$ 신장(cm) + 3.16 $\times$ 체증 잔차 4156.66 이었다. 여자에서는 FVC(ml) = 42.57 $\times$ 신장(cm) + 12.50 $\times$ 체중 잔차 - 3862.39, 그리고 FEV1(ml) = 36.29 $\times$ 신장(cm) + 7.74 $\times$ 체중 잔차 - 3200.94 이었다. 이렇게 얻어진 예측값들의 설명력(R2)은 남자에서 FVC, FEV1가 각각 0.708, 0.670이었고, 여자에서는 FVC, FEV1가 각각 0.580, 0.513이었다.

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임상 간호사의 직무 스트레스, 대인갈등 해결 전략 및 직무 만족도 (A Survey on the Work Stress, Interpersonal Conflict Resolution Strategy and Job Satisfaction in Clinical Nurse)

  • 양혜주;박정숙
    • 지역사회간호학회지
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    • 제9권2호
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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.

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폴리에틸렌 섬유 강화형 포스트를 이용한 상악 유전치의 수복 (RESTORATION OF MAXILLARY PRIMARY INCISORS USING POLYETHYLENE FIBER-REINFORCED POST)

  • 윤효진;박호원;이주현;서현우
    • 대한소아치과학회지
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    • 제37권4호
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    • pp.526-531
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    • 2010
  • 유아기 우식증은 '71개월 이하의 어린이의 유치에서, 1개 이상의, 우식, 상실, 충전 치면의 존재'로 정의 되며, 이는 생후 1~2세의 나이에 수유와 연관되어 상악 전치부에 나타나는 독특한 형태가 특징적이다. 상악 유전치에 발생한 유아기 우식증은 종종 급속하게 진행되어 심각한 치관의 파괴, 급성 또는 만성 치수염, 치근단 농양을 형성하여 발치를 하기도 한다. 조기에 상악 유전치를 상실한 경우 여러 가지 기능적, 심미적, 심리적 문제를 야기하므로 상악 유전치는 되도록 보존하는 것이 추천되며, 시술이 간단하고, 효율적이며, 견고하고, 기능적이며, 심미적인 수복방법이 필요하다. 폴리에틸렌 섬유 강화형 포스트는 우수한 물리적, 기계적 성질 및 심미적인 색상으로 전치부의 복합레진을 강화하기 위해 사용될 수 있다. 폴리에틸렌 섬유로 구성된 Ribbond..는 특수한 표면처리로 인해 레진과 결합할 수 있는 성질이 있으며, 다루기가 쉽고, 비교적 저렴하여 다양하게 사용되고 있다. 본 증례는 중증 유아기 우식증에 이환되어 치관이 심각하게 손상된 상악 유전치부에 폴리에틸렌 섬유 강화형 포스트와 복합레진 코어, 셀룰로이드 크라운을 이용한 수복으로 양호한 치료결과를 얻었기에 이를 보고하는 바이다.

Effect of Storage Time on the Rancidity and Metabolizable Energy of Rice Polishing in Poultry

  • Pasha, T.N.;Khattak, F.M.;Khan, D.R.;Jabbar, M.A.
    • Asian-Australasian Journal of Animal Sciences
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    • 제21권3호
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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.

Effect of increasing levels of rice distillers' by-product on growth performance, nutrient digestibility, blood profile and colonic microbiota of weaned piglets

  • Cong, Oanh Nguyen;Taminiau, Bernard;Kim, Dang Pham;Daube, Georges;Van, Giap Nguyen;Bindelle, Jerome;Fall, Papa Abdulaye;Dinh, Ton Vu;Hornick, Jean-Luc
    • Asian-Australasian Journal of Animal Sciences
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    • 제33권5호
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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.