• Journal of the Korean Vacuum Society
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• v.6 no.4
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• pp.348-353
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• 1997

Hydrogenated amorphous carbon(a-C:H) films were deposited on p-type Si(100) by DC saddle-field plasma enhanced CVD to investigate the effect of substrate bias on optical properties and structural changes. They were deposited using pure methane gas at a wide range of substrate bias at room temperature and 90 mtorr. The substrate bias voltage ($V_s$) was employed from $V_s=0 V$ to $V_s=400 V$. The information of optical properties was investigated by photoluminescence and transmitance. Chemical bondings of a-C:H have been explored from FT-IR and Raman spectroscopy. The thickness and relative hydrogen content of the films were measured by Rutherford backscattering spectroscopy (RBS) and elastic recoil detection (ERD) technigue. The growth rate of a-C:H film was decreased with the increase of $V_s$, but the hydrogen content of the film was increased with the increase of $V_s$. The a-C:H films deposited at the lowest $V_s$ contain the smallest amount of hydrogen with most of C-H bonds in the of $CH_2$ configuration, whereas the films produced at higher $V_s$ reveal dominant the $CH_3$ bonding structure. The emission of white photoluminescence from the films were observed even with naked eyes at room temperature and the PL intensity of the film has the maximum value at $V_s$=200 V. With $V_s$ lower than 200 V, the PL intensity of the film increased with V, but for V, higher than 200 V, the PL intensity decreased with the increase of $V_s$. The peak energy of the PL spectra slightly shifted to the higher energy with the increase of $V_s$. The optical bandgap of the film, determined by optical transmittance, was increased from 1.5 eV at $V_s$=0V to 2.3 eV at $V_s$=400 V. But there were no obvious relations between the PL peak and the optical gap which were measured by Tauc process.

• The Journal of Industrial Distribution & Business
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• v.1 no.1
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• pp.5-12
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• 2010

The retail business of drugstore was introduced to Korea for the first time 10 years ago. Since Olive Young introduced a retail store in the name of drugstore in 1999 for the first time in Korea, new distribution channel combining drugstore, cosmetic products and dairy products, etc has been made. At initial stage, the new distribution channel grew up slowly because of low specialty and economic stagnation. However, the three big distribution channels, that is to say, Olive Young (CJ), Watsons (GS) and W Store (Kolon Well Care), etc, were established to produce new distribution system following large-scaled discount stores as well as convenience stores. The purpose of the study is to investigate ways making Korean style drugstore be new retail business in addition to traditional markets, department stores, E-Mart and other general super markets and to examine problems preventing the drugstore from being promoted and to find out solutions. The speciality retailers that is called a category killer attacking department stores as well as marts is expanding market quickly. New consumption trend that gives priority to wellbeing is being expanded in accordance with high level of standards of living life: The drugstore is thought to be new alternative of distribution because it keeps special products. Young ladies who are main customers of drugstores respond to the trend sensitively to have more buying power that is thought to be promising. And, consumers' desire has become concrete and special. This is because consumers want not only convenient shopping but also special shopping system that is current trend. These days, so called Multi-shop and Total shop and other special shops have been recently opened. Special multi-shop has been concentrated on fashion product and miscellaneous goods so far: Health total wellbeing shop shall be popular in accordance with wellbeing trends. Drugstores can play an important role. Drugstores were opened for the first time ten years ago. In particular, Olive Young succeeded in going into the black after making efforts for a long time by many persons. Drugstores could succeed in the business owing to many persons in the past as well as customers who liked drugstores. However, drugstores once lost ways and recorded poor business results. The three drugstores, that is to say, Olive Young, Watsons making efforts to go into the black and W-Store pursuing traditional drugstore shall compete each other and make effort to satisfy customers' desire. In that way, the three drugstores can be assured of present business as well as future business. The consumers' demand trend has become special at sub-division so that drugstores that can satisfy the demand can succeed in the business. Large businesses may be more interested in the 4th generation retail business to produce good income and to have bright future. Drugstore business and market are likely to expand and develop owing to large business' participation in drugstore business. Drugstores expanded shop at Seoul and Gyeonggi-do until middle of 2000. Drugstore business at station sphere in Seoul and Gyeonggi-do that have high ratio of temporary population has low customer loyalty to have limitation on continuous growth. Since 2009, drugstores have opened new shops at local towns: From the year of 2010, drugstores need to establish multiple shop strategy by accelerating business speed and to allow customers to drop in the shop anywhere in the nation and to enter consumers' life deeply, so that they can strengthen business base definitely. Drugstores need to have price competitiveness to have multiple shop opening strategy and to satisfy consumers and to supply high quality services that is future subject to solve. And, Olive Young and Watsons that are Korean style drugstore need to keep system in order and to strengthen substance as Korean style drugstore and to expand marketing, so that they can get business outcome within 5 years that was done 10 years before and they become the 4th generation retail business. The study had difficulties at collecting material from the three drugstore because of poor cooperation. And, the author had great difficulty at collecting statistical material that was made in disorder. Further effort is needed considering such problems.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.103-108
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• 2017

Kabuki syndrome is a rare congenital disorder that causes multiple birth defects and mental retardation. Mutation of the lysine methyltransferase 2D (KMT2D) gene is the primary cause of Kabuki syndrome. We report a 4-year-old Korean girl diagnosed with Kabuki syndrome based on distinctive facial features (eversion of the lower lateral eyelid, arched eyebrows, depressed nasal tip, prominent ears), skeletal anomalies, short stature, and molecular analysis, which revealed a novel frameshift mutation in the KMT2D gene. A 4-year-old patient had a past history of congenital cardiac malformations (coarctation of the aorta, ventricular septal defect, atrial septal defect, patent ductus arteriosus), subclinical hypothyroidism and dysmorphic features at birth including webbed neck, short fingers, high arched palate, micrognathia and horseshoe kidney. She showed unique facial features such as a long palpebral fissure, long eyelashes, arched eyebrows with sparseness of the lateral third, broad nasal root, anteverted ears, and small mouth. Her facial features suggested Kabuki syndrome, and genetic analysis discovered a novel heterozygous frameshift mutation (c.4379dup, p.Leu1461Thrfs*30) in exon 15 of the KMT2D gene. The diagnosis of our 4-year-old patient was made through thorough physical examination and history taking, and genetic testing. It is challenging to diagnose patients with Kabuki syndrome at birth, since the characteristic facial features are expressed gradually during growth. Clinical suspicion aroused by regular follow-ups may lead to earlier diagnosis and interventions.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.1
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• pp.21-29
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• 1999

Purpose: The aims of this study are to examine clinical characteristics, patterns of medical care utilization, and factors which determine medical care utilization of elementary school children with recurrent abdominal pain (RAP), to find posssible factors influencing the onset and the course of the disorder. Method: We performed questionnaires in Kwangju on children from two primary schools from June,1 1998 to June 30 and carried out statistical analysis. Result: 1) Total number of questionnaires were 1417. 715 were male and 702 were female and the ratio of male to female was 1.02:1. Average age was 10.3 years. 2) 268 children had RAP (18.9%), boys 132 (18.4%), girls136 (19.2%). 3) The duration of the pain within 10 minutes was 68.5%. 178 children with RAP (66.3%) visited the doctor. The utilization pattern of medical facilities of the pupils with RAP; the most frequently utilized medical facility was pediatrics (35.2%) and the order ran as internal medicine (31.5%), and pharmacy (29.25). The utilization pattern of medical facilities for the older students; the utilization rate of pediatrics decreased, but internal medicine increased. The major factors affecting the selection of the medical facility were geographic accessibility, kindness of the personnel, good results and traffic convenience. 4) Symptoms which were accompanied with abdominal pain were headache (44.5%), chest pain (28.2%), dizziness (26.6%), vomiting (9%), and 119 children (44.5%) had no accompanied symptoms. 5) In 95 children (35.3%) abdominal pain, occured at postprandial time, in 55 children (20.5%) before meal and in 39 children (14.7%) at school. The highest incidence rate of RAP was observed on Monday (21.4%), and the lowest on Saturday (8.7%). 6) The most frequent involved part of the abdomen was periumbrical area (38%) and the order ran as epigastrium and suprapubic area. The most frequent characteristics of abdominal pain were burning pain (36.9%) and the order ran as dull, cramping and colicky pain. Conclusion: RAP is a frequent disease entitiy in children. Too many times children with RAP are treated by other departments instead of Pediatrics. A child has a peculiar growth and development which is different to those from an adult with advancing years. So, it is necessary to choose special medical care and an adequate medical facility.

• Clinical and Experimental Pediatrics
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• v.45 no.10
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• pp.1263-1272
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• 2002

Purpose : Rett syndrome(RTT) is an X-linked dominant neurodevelopmental disorder affecting 1 per 10,000-15,000 female births worldwide. It was initially described by Andreas Rett in 1966. RTT involves developmental regression characterized stereotypic hand movements, tremors, gait apraxia, seizures, deceleration of head growth after the age of 6-18 months. The disease-causing gene was identified as MECP2 on chromosome Xq28. We carried out mutational analysis of MECP2 genes in RTT patients. Methods : Whole blood(5 cc) of 34 sporadic RTT patients was collected in EDTA-anticoagulated tubes. Genomic DNA was extracted from peripheral blood using the E.Z.N.A. blood DNA kit. Four exons of the MECP2 gene were amplified by PCR in 34 Korean with RTT. We carried out PCR divided the exon three into two parts and the exon four into five parts. Primer sequences designed by Amir et al. in 1999 were almost used(AF030876). Sequencing primers used were the same as PCR. DNA sequencing reactions were performed using an ABI 377 DNA sequencer and ABI PRISM dye terminator cycle sequencing reaction kit(Perkin-elmer). The results were compared with the normal DNA sequence(X99686). To confirm the change of sequence on novel mutations, RFLP analysis was performed. Results : The MECP2 mutations were detected in 23(67.6%) of the 34 patients. The mutations consisted of 12 different types including nine missense and three nonsense mutations. Of these, three (L100V, G161E and T311M) mutations were newly identified. Most of the mutations discovered are located within MBD(39.1%) and TRD(39.1%). In this study, three(T158M, R270X, R306C) mutations were identified high frequency. Conclusion : MECP2 gene was also an important cause of Korean RTT patients. MECP2 gene study is an important tool for diagnosis of Korean RTT patients.

• Journal of Genetic Medicine
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• v.4 no.1
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• pp.72-79
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• 2007

Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.1
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• pp.25-30
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• 2023

Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder with clinical and genetic heterogeneity. Recently, balletic IARS2 variants have been identified in a number of patients presenting broad clinical phenotypes from Leigh and West syndrome to a rare syndrome CAGSSS characterized by cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia syndrome (OMIM#616007). We describe a child with Korean Leigh syndrome with urologic manifestations resulting from a compound heterozygote mutation in IARS2. A 5-year-old girl visited the emergency room with a complaint of abdominal pain accompanied by abdominal distension. Abdominal-pelvic CT showed a markedly distended urinary bladder without definite obstructive lesions. She was diagnosed with neurogenic bladder dysfunction based on a urodynamic study. She had global delayed development due to neurologic regression after 6 months of age and a history of bilateral cataract surgery at the age of 2 years. Her brain magnetic resonance imaging showed symmetrically increased signal intensities in the bilateral putamen and caudate nuclei with diffuse cerebral atrophy. No gene variants were identified through whole-mitochondrial genome analysis. Whole exome sequencing was performed for diagnosis, and compound heterozygous pathogenic variants were identified in IARS2: c.2446C>T (p. Arg816Ter) and c.2450G>A (p. Arg817His). To the best of our knowledge, this is the first case report of bladder dysfunction manifestation in a patient with IARS2-related Leigh syndrome. Thus, it broadens the clinical and genetic spectrum of IARS2-associated diseases.

• Journal of Bio-Environment Control
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• v.26 no.4
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• pp.378-385
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• 2017

In this study, we evaluated the changes of fruit quality indices during fruit development and ripening in Korean new pear cultivar 'Changjo', developed from a cross between 'Tama' and '81-1-27' ('Danbae' ${\times}$ 'Okusankichi') in 1995 and named in 2009, to determine appropriate harvest time and to enhance the market quality and broaden the cultivation area. The fruits of 'Changjo' pears harvested from 132 days after full bloom (DAFB) to 160 DAFB. Fruit growth and quality indices were monitored at 1 week interval by measuring fruit weight, length, diameter, firmness, and taste related quality indices. The calculated fruit fresh weight increased continuously with fruit development and reached to an average of 594g on Sep. 20 (160 DAFB). The ratio of length to diameter declines as fruit maturation progress, resulting in 0.898 for ripe fruit stage as a round oblate shape. Flesh firmness of 'Changjo' pears showed over 30N until 153 DAFB and then decreased abruptly with fruit ripening, reaching a final level of about 26.44N on 160 DAFB. Starch content of fruit sap was also decreased abruptly after 146 DAFB which decreased almost half of the fruits harvested at 139 DAFB. In parallel with the decrease of flesh firmness, ethanol insoluble solids (EIS) content decreased sharply with fruit ripens, only 50% of EIS was detected on the fruits harvested on 160 DAFB when compared to that of the fruits harvested on 139 DAFB (Aug. 30). The maximum value of soluble solids contents was observed in the fruits harvested on 153 DAFB, resulting in $14.2^{\circ}Brix$. The changes of skin color difference $a^*$ which means loss of green color occurred only after 139 DAFB, coincide with the decrease of SPAD value of the fruit skin. The sugars of the 80% ethanol soluble fraction consisted mainly of fructose, sorbitol, glucose and sucrose, also increased during maturation and ripening. Fructose and sucrose contents were larger than those of glucose and sorbitol in flesh tissues. These results were explained that stored starch is converted to soluble sugars during fruit maturation, mainly in fructose and sucrose increasing the sweetness of this cultivar. Total polyphenols were increased up to middle of fruit maturation (146 DAFB) and then decreased continuously until the end of fruit maturation. Consequently, our results suggested that the commercial harvest time of 'Changjo' pears should not be passed 153 DAFB and late harvest of this cultivar would not good for quality maintenance during shelf-life. As a result of the post-harvest low-temperature acclimation experiment during the short-term transportation period, fruits harvested at 146 DAFB tended to maintain higher firmness after 14 days of simulated marketing at $25^{\circ}C$ compared to fruits harvested at 153 DAFB regardless of temperature set. And, the slower the rate of decrease to the final transport temperature of $5^{\circ}C$, the higher the incidence of internal browning and ethylene production. Therefore, in order to suppress the physiological disorder and to maintain the fruit quality when exporting to Southeast Asia in the 'Chanjo' pears, it is desirable to lower the temperature of the fruits within a short time after harvest and to set the harvest time before 146 days after full bloom.

• Applied Biological Chemistry
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• v.16 no.1
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• pp.18-30
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• 1973

The flag and lower leaves (4th or 5th) of rice plant from the field of NPK simple trial and from three low productive area were analyzed in order to find out certain diagnostic criteria of nutritional status at harvest. 1. Nutrient contents in the leaves from no fertilizer, minus nutrient and fertilizer plots revealed each criterion for induced deficiency (severe deficient case induced by other nutrients), deficiency (below the critical concentration), insufficiency (hidden hunger region), sufficiency (luxuary consumption stage) and excess (harmful or toxic level). 2. Nitrogen contents for the above five status was less than 1.0%, 1.0 to 1.2, 1.2 to 1.6, 1.6 to 1.9 and greater than 1.9, respectively. 3. It was less than 0.3%, 0.3 to 0.4, 0.4 to 0.55 and greater than 0.55 for phosphorus $(P_2O_5)$ but excess level was not clear. 4. It was below 0.5%, 0.5 to 0.9, 0.9 to 1.2, 1.2 to 1.4 and above 1.4 for potassium. 5. It was below 4%, 4 to 6, 6 to 11 and above 11 for silicate $(SiO_2)$ and no excess was appeared. 6. Potassium in flag leaf seemed to crow out nitrogen to ear resulting better growth of ear by the inhibition of overgrowth of flag leaf. 7. Phosphorus accelerated the transport of Mg, Si, Mn and K in this order from lower leaf to flag, and retarded that of Ca and N in this order at flowering while potassium accelerated in the order of Mn, and Ca, and retarded in the order of Mg, Si, P and N at milky stage. 8. Transport acceleration index (TAI) expressed as (F_2L_1-F_1L_2)\;100/F_1L_1$ where F and L stand for other nutrient cotents in flag and lower leaf and subscripts indicate the rate of a nutrient applied, appears to be suitable for the effect of the nutrient on the translocation of others. 9. The content of silicate $(SiO_2)$ in the flag was lower than that of lower leaf in the early season cultivation indicating hinderance in translocation or absorption. It was reverse in the normal season cultivation. 10. The infection rate of Helminthosporium frequently occurred in the potassium deficient field seemed to be related more to silicate and nitrogen content than potassium in the flag leaf. 11. Deficiency of a nutrient occured simultaniously with deficiency of a few other ones. 12. Nutritional disorder under the field condition seems mainly to be attributed to macronutrients and the role of micronutrient appears to be none or secondary.