• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.17 no.3
• /
• pp.85-91
• /
• 2017

Purpose: The aim of this study was to describe the clinical and biochemical features as well as the molecular analysis of a newly diagnosed illustrative case with ML II and to analyze the clinical features of 11 Korean patients with ML II/III. Method: Including a newly diagnosed patient, total 11 patients in 10 families were diagnosed as ML II (n=7) or ML III (n=4) were enrolled in the study. A diagnosis of ML II or III was made by demonstrating increased lysosomal enzyme activities in the plasma and sequence analysis of GNPTAB with characteristic clinical features. Result: A illustrative case of ML II patient was a 17 month-old boy showing characteristic facial appearance, multiple joint contractures with cardiac involvements. The enzyme assay showed increased lysosomal enzyme activities in the plasma. We identified compound heterozygous mutations in GNPTAB sequence analysis, including a frameshift (c.3428dupA [pAsn1143Lysfs*3]) and a nonsense variant c.673C>T (p.Gln225*). In total 11 patients with ML II/III, the patients with ML II showed severe growth retardation (height standard deviation score -3.2 [${\pm}1.5$]), compare to patients with ML III. Furthermore, patients with ML II patients had serious cardiac problem (n=4), hepatomegaly (n=3) and underwent tracheostomy (n=3) with further respiratory support due to respiratory distress. To improve osteoporosis and bone pain, all patients with ML III and four of 7 patients with ML II treated with intravenous pamidronate. Conclusion: Here we showed a newly diagnosed case of ML II and clinical features of 11 Korean patients with ML II or III. These data could be helpful for further diagnosis of mucolipidosis, a rare inherited metabolic disease, in Korea.

• Korean Journal of Ecology and Environment
• /
• v.45 no.1
• /
• pp.72-81
• /
• 2012

We investigated the effects of acid mine drainage (AMD) from abandoned coal mines on benthic macroinvertebrate communities in the upper reaches of the Nakdong River from May to October in 2009. Qualitative and quantitative sampling (Surber sampler: $50{\times}50$ cm; mesh size, 0.2 mm) was conducted at 7 study sites and 3 control sites in the study area. We thus sampled 117 species belonging to 53 families, 15 orders, 6 classes, and 5 phyla; the Ephemeroptera, Plecoptera, and Trichoptera group (EPT-group) represented the majority of the benthic macroinvertebrate community (71 species; 64.5%). In the quantitative sampling, a total of 11,575 individuals belonging to 58 species of benthic maroinvertebrates were sampled from the study sites (Sites 1-7), whereas 2,844 individuals belonging to 79 species were sampled from the control sites (Sites A-C). Tolerant species such as oligochaetes, $Epeorus$ $pellucidus$, $Baetis$ $fuscatus$, Hydropsychidae species, and Chironomidae species were predominant in the study sites. The community indices for the study sites, such as MacNaughton's dominance index (DI) (mean${\pm}$SD, $0.52{\pm}0.21$; range, 0.33-0.85) and the Shannon diversity index ($H^{\prime}$) ($2.06{\pm}0.60$; 1.06-2.57), were different from those for the control sites (DI: $0.29{\pm}0.07$, 0.22-0.35; $H^{\prime}$: $3.13{\pm}0.14$; 3.03-3.30). In the study sites, shredders and scrapers were scarce, whereas gathering-collectors (mainly Chironomidae species) were relatively abundant, as were clingers and burrowers. The detrended correspondence analysis (DCA) and similarity analyses showed that benthic macroinvertebrate communities in the study sites were clearly separated from those of the control sites, with the greatest dissimilarity being noted at the uppermost study site (Site 1), which is located close to an abandoned coal mine. The Korean saprobic index (KSI) and the ecological score using benthic macroinvertebrates (ESB) showed that the uppermost study site (Site 1) was ${\alpha}$-mesosaprobic or heavily polluted, whereas other study sites were in a fair or relatively good condition.

• The Korean Journal of Malacology
• /
• v.24 no.2
• /
• pp.121-130
• /
• 2008

In other aquaculture species, large improvements in growth have been achieved through selective breeding. Ezo abalone(Haliotis discus hannai) and disk abalone(H. discus discus) are major aquatic animals cultured in Asia, but selective breeding for the promotion of growth with these abalones has not been actively pursued. Recently significant efforts are being made to promote production of these species through selective breeding in Korea. The aims of this work were to estimate the general genetic parameters, heritabilities, and genetic and phenotypic correlations on growth-related traits at 1-year old in two Korean abalone subspecies, H. discus hannai and H. discus discus, by using multiple trait animal model. The data were collected from the records of 1,504 individuals produced from 22 sires and 26 dams in H. discus hannai and 297 individuals produced from 5 sires and 6 dams in H. discus discus, which evaluated by the Genetics and Breeding Research Center, National Fisheries Research & Development Institute(NFRDI). Genetic parameters were estimated for these abalone subspecies raised in Bukjeju branch, NFRDI, from May 20, 2004 to May 16, 2005, respectively. The heritability estimates obtained from restricted maximum likelihood(REML) were higher than expected, ranging from 0.40 to 0.43 for growth traits shell length, shell width and body weight in H. discus hannai and from 0.26 to 0.51 in H. discus discus, respectively. The heritabilities for shell shape and condition factor were lower than others of growth traits such as ranging from 0.09 to 0.19 in H. discus hannai and from 0.10 to 0.23 in H. discus discus, respectively. Genetic and phenotypic were > 0.93 between shell parameters and weight in two abalone species, respectively, indicating that breeding for weight gains could be successfully achieved by selecting for shell length.

• Journal of dental hygiene science
• /
• v.10 no.3
• /
• pp.147-153
• /
• 2010

The purpose of this study was to investigate the correlation between food intake pattern body component bone mineral density(BMD) and dental caries experience of college women and we wanted to determine the correlation of these factors with dental caries experience. Research was conducted to offer basic data to develope a nutritional program for the future prevention from oral disease. The subjects in this study were 132 college women who participated in the food intake survey and who's body component and BMD was measured. The statistical analysis was done by using the SPSS 15.0 program. The results of this study were as follows; 1. The food intake pattern was not directly correlated with DMFT index. 2. The body component was not directly correlated with DMFT index. 3. The SOS, BQI was directly correlated with DMFT index(p<0.05). 4. T-score of BMD had the greatest influence on DMFT index(p<0.05). Above results indicated that bone mineral density are related with the incidence of dental caries experience and further research is necessary to develop a nutritional and health promotional program in order to prevent oral illness.

• Korean Journal of Food Science and Technology
• /
• v.45 no.2
• /
• pp.241-247
• /
• 2013

AOAC Mouse Bioassay Analysis (MBA) has been the gold standard for the analysis of paralytic shellfish poisoning toxin (PSP toxin) for more than 50 years. However, this method has inaccurate limit of quantification and cannot be used to determine toxic profiles. An HPLC method (PCOX) was optimized for Korean shellfish to establish an alternative or supplementary method for PSP analysis and was intended to be used for the official monitoring and regulation of food. The recovery rate of the PCOX method was 83.5-112.1% and the limit of quantification for total toxin was about $8.6{\mu}g$/100 g. A long-term comparison study showed a good correlation of the PCOX results with the AOAC MBA results: the correlation factors were 0.9534 and 0.9109 for oyster and mussel matrices, respectively. The PCOX method may be used as an alternative or supplementary method for AOAC MBA to monitor the occurrence of PSP and to analyze PSP toxin profile in oysters and mussels.

• Progress in Medical Physics
• /
• v.21 no.2
• /
• pp.153-164
• /
• 2010

To determine the clinical target volumes considering vascularity and cellularity of tumors, the software was developed for mapping of the analyzed biological clinical target volumes on anatomical images using regional cerebral blood volume (rCBV) maps and apparent diffusion coefficient (ADC) maps. The program provides the functions for integrated registrations using mutual information, affine transform and non-rigid registration. The registration accuracy is evaluated by the calculation of the overlapped ratio of segmented bone regions and average distance difference of contours between reference and registered images. The performance of the developed software was tested using multimodal images of a patient who has the residual tumor of high grade gliomas. Registration accuracy of about 74% and average 2.3 mm distance difference were calculated by the evaluation method of bone segmentation and contour extraction. The registration accuracy can be improved as higher as 4% by the manual adjustment functions. Advanced MR images are analyzed using color maps for rCBV maps and quantitative calculation based on region of interest (ROI) for ADC maps. Then, multi-parameters on the same voxels are plotted on plane and constitute the multi-functional parametric maps of which x and y axis representing rCBV and ADC values. According to the distributions of functional parameters, tumor regions showing the higher vascularity and cellularity are categorized according to the criteria corresponding malignant gliomas. Determined volumes reflecting pathological and physiological characteristics of tumors are marked on anatomical images. By applying the multi-functional images, errors arising from using one type of image would be reduced and local regions representing higher probability as tumor cells would be determined for radiation treatment plan. Biological tumor characteristics can be expressed using image registration and multi-functional parametric maps in the developed software. The software can be considered to delineate clinical target volumes using advanced MR images with anatomical images.

• Journal of Periodontal and Implant Science
• /
• v.30 no.2
• /
• pp.213-231
• /
• 2000

이 연구의 목적은 다공성의 CPP 내부에 쥐의 장골의 골수에서 유래된 세포를 접종하고 3차 원적으로 배양하여 CPP가 골 형성을 위한 조직공학의 지지체로 적용가능한가를 연구하는 것과 Calcium PolyPhosphate(CPP)의 돌연변이 유발성을 검사하는 것이다. 무수 ($Ca(H_2PO_4)$)를 condensation하여 무결정의 ($Ca(PO_3)$)를 얻고 이를 용융하고 냉각시킨 후 분쇄하여 Calcium polyphosphate(CPP) powder를 얻었다. 다공성의 CPP는 5% $SiO_2$를 첨가하여 sponge 형태로 $450-550{\mu}m$ 소공의 크기를 가지는 것과(CPP-45ppi) $200-300{\mu}m$의 소공의 크기를 가지는 것(CCP-60ppi) 2가지로 제작하였다. 각각의 CPP matrices는 $5mm{\times}5mm{\times}1mm$의 블록 형태로 만들었다. 체중 100g 내외의 백서에서 장골(femur, tibia)을 채취하여 백서의 장골 골수 세포를 분리하여 배양한 후 24well에 CPP block을 넣고 CPP block 당 $10^5$개의 배양한 세포를 접종하였다. 배양 1, 7, 14, 및 21 일째에 각 well에서 trypsin EDTA를 이용하여 2회 반복하여 cell을 분리하였고, 원심분리한 후 hemacytometer로 측정하였다. 또, 45ppi와 60ppi, 그 리 고 Tissue Culture Polystyrene(control group)에 접종, 배양된 세포들의 염기성 인산분해효소활성도를 배양 7, 14, 및 21 일째에 각각 측정하였다. 각 기간별로 배양된 세포-CPP 혼합체내에서 세포의 부착 및 증식과 형성된 조직의 3차원적 형태를 관찰하기 위하여 주사전자현미경하에서의 관찰하였다. CPP의 돌연변이 유발성 검사 (mutagenicity test)를 위해 hypoxanthine-guanine phosphoribosyl transferase(HPRT) assay를 하였다. NIH3T3 cell line과 CHO-K1 cell line으로 각각 $1000{\mu}g/m{\ell}$, $100{\mu}g/m{\ell}$, $10{\mu}g/m{\ell}$ 그리고 $1{\mu}g/m{\ell}$의 CPP 농도에서 측정하였다. 통계적 분석을 위해서 모든 측정은 각군당 4개체 이상 시험하였고, 각 측정값은 평균값${\pm}$표준편차로 나타내었다. 각 군간의 통계적 유의성 검정을 위해서 Analysis of variance(ANOVA)를 이용하였고 Tukey의 방법으로 사후분석을 실시하였다. 제작된 CPP matrices 소공들이 서로간에 연결이 잘 되어있는 형태였다. 두 가지로 제조된 CPP(45ppi와 60ppi) 모두에서 세포의 부착이 잘 일어났고, 부착된 세포의 분열도 잘 일어났다. 2 가지의 CPP 모두에서 7, 14, 21일째의 세포 수는 1일째에 비해 유의성 있게 증가하였다(P<0.01). 3차원적 구조인 Calcium PolyPhosphate에서 배양한 세포는 24well dish(tissue culture polystyrene)에서 평면적으로 배양한 대조군의 세포에서 보다 염기성 인산분해효소 (Alkaline Phosphatase)를 유의성 있게 높게 나타냈다. 주사전자현미경에서 세포-CPP 혼합체를 관찰한 결과, CPP block에 세포들이 잘부착되어 있었고, 시간이 지남에 따라 세포가 여러 층을 형성하면서 뭉치는 현상을 보였다. 또, HPRT assay 결과 , Calcium PolyPhosphate는 돌연변이 유발성을 보이지 않았다. 이상의 결과로 볼 때 CPP에는 세포부착이 잘 일어나고, 지지체 상에서 세포의 분열도 활발하게 일어나므로 골조직을 위한 조직공학의 우수한 지지체가 될 수 있을 것으로 사료된다.

• Korean Journal of Environmental Agriculture
• /
• v.13 no.1
• /
• pp.39-46
• /
• 1994

Two types of sunshine recorders, Jordan and bimetal, were used for measuring the duration of sunshine and percentage of sunshine in Weather Forecast Offices(WFO) and Weather Observation Stations(WOS) in Korea, respectively.These two gauges showed different values in each element observation. To evaluate the solar energy resources by duration and percentage of sunshine, relevant parameter should be adapted to use the two kinds of data for zoning of agricultural climatic area and comparison of regional solar energy distributions. In this respect, the correlation and distribution pattern were found by analyzing data from the two types of sunshine recorders. The results were as follows. The monthly duration of sunshine by the Jordan type was $50{\sim}60$ hours lower than the bimetal type and its value in May was the highest in a year. The percentage of sunshine by the Jordan type was $5{\sim}10%$ lower than the bimetal type. The seasonal difference of sunshine hour data by two types of sunshine recorder became small in winter but large in summer. Standard deviation of monthly duration of sunshine of WFO and WOS was $11{\sim}32$ and $17{\sim}25$ hours and percentage of sunshine was $3{\sim}11$ and $4{\sim}9$ % respectively. The range of deviation in WOS data was smaller than WFO. The highest distribution of duration and percentage of sunshine was in the Southern Coastal Area, whereas the lowest in the Central North Western Area.

• Analytical Science and Technology
• /
• v.23 no.5
• /
• pp.485-491
• /
• 2010

A certified reference material (CRM) of KRISS 109-03-SSD was developed for the analysis of hazardous elements in soil. The target elements were As, Cd, Cr, Cu, Hg, Ni, Pb, Zn being regulated by the Soil Environment Conservation Act. Starting material was collected from tailing dump of an unworked tungsten mine at Sangdong (Gangwon-do, Korea). The starting material under-went through a series of fabricating process steps of screening, drying, grinding, sieving, blending, bottling, sterilization and was certified according to the ISO Guide 35. Isotope dilution-inductively coupled mass spectrometry (ID-ICP/MS) and instrumental neutron activation analysis (INAA) were used for the measurement. Homogeneity was tested according to ISO 13528 annex B. The certified values were determined using the results from two different methods or from two independent measurements using a method. Finally, certified values of seven elements of arsenic, cadmium, chromium, copper, lead, nickel and zinc were determined. Mercury did not satisfied the criteria of homogeneity and the result would be provided for information only, together with iron and tungsten. It was also studied, the extractable fraction of elements by aqua regia according to the ISO 11466 protocol being frequently studied for the purpose of environmental monitoring. It was performed as a inter-laboratory study by 6 laboratories of a public institute and universities. Standard deviation among the laboratories was much bigger than the uncertainty of the certified value. The result of inter-laboratory study on the extractable fraction will be provided for information, only.

• Journal of Chest Surgery
• /
• v.37 no.8
• /
• pp.660-664
• /
• 2004

Background: Dilution of blood cardioplegia is not needed in IAWBC as it is in cold blood cardioplegia because it does not aggregate red blood cells on normal body temperature and does not compromise micro coronary circulation. This study was designed to evaluate the safety and efficacy of undiluted potassium solution in IAWBC. Material and Method: Thirty patients who underwent CABG with IAWBC were grouped into dilutedplegia (n=14) and microplegia (n=16). Potassium was delivered conventionally with 4 : 1 delivery kit in the dilutedplegia group. The undiluted potassium was directly connected on the blood of oxygenator in the microplegia group. Result: There were no differences in sex, age, left ventricular ejection fraction, number of grafts, aortic cross clamping time, and the value of perioperative myocardial enzyme between the two groups. There were no perioperative myocardial infarction and hospital mortality. The amount of crystalloid cardioplegia was 1346$\pm$597 mL in dilutedplegia (mean$\pm$standard deviation, and 28$\pm$9 mL in microplegia (p<0.0001). The hematocrit during cardiopulmonary bypass was 21$\pm$4% in dilutedplegia and 24$\pm$3% in microplegia (p>0.05). 11 patients in dilultedplegia received blood transfusion, but 4 patients in microplegia received blood transfusion (p<0.05). The amount of urine and hemofiltration during the operation were more in dilutedplegia (1250$\pm$810 mL, 1689$\pm$548 mL) than in microplegia (959$\pm$410 mL, 1461$\pm$784 mL; p<0.05). Conclusion: The undiluted potassium of IAWBC in CABG operation is a safe, effective technique for myocardial protection to prevent fluid overload, and blood transfusion. There is no need to use the delivery kit.