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Clinical Findings of Phenylketonuria Patients in Korea (페닐케톤뇨증의 임상적 고찰)

  • Shin, Ik Soon;Lee, Dong Hwan
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.12 no.1
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    • pp.14-22
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    • 2012
  • Objectives: This study was performed to review clinical manifestations of hyperphenylalaninemia patients in Korean. Methods: 178 cases of hyperphenyalaninemia were diagnosed at department of pediatrics, Soonchunhyang University Hospital from January, 1983 to August. We reviewed DNA analysis, MR imaging, EEG, radiography of the left hand and wrist, bone densitometry (BMD), IQ test of hyperphenyalaninemia patients. Results: Out of 178 cases, 161 cases were diagnosed classic phenylketonuria and 17 cases were diagnosed BH4 deficiency. 122 cases performed DNA analysis. R243Q (10.3%), Y204C (9.9%), and IVS4-1G>A (8.1%) mutations were predominant. 22 cases underwent MR imaging. Varying degrees of symmetrical high signal intensity were noted on T2-weighted sequences in the periventricular deep white matter of 15 cases. 23 cases were performed EEG. 12 cases (52.3%) showed abnormal pattern. EEG abnormalities showed in 11 cases. On lumbar BMD four of 11 cases (36%) showed reduced bone density of more than 1 S.D. in four of 11 cases, bone age was less than chronological age by at least one year. 18 cases were performed IQ test. Mean IQ scores was $84{\pm}21.6$. Among older than 15 years (9 cases), Mean IQ scores was $72{\pm}21.2$. PTPS deficiency was 14 cases, DHPR deficiency was 2 cases, and GTPCH deficiency was 1 case. Conclusion: We confirmed there were varieties of DNA mutations. And MR imaging and EEG were nonspecific in PKU patients. Older children showed lower IQ score. Low phenylalanine diet prevents brain damage in PKU patient. Not only first few years of life but also lifetime, Keeping low phenylalanine diet is important.

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Epidemiologic Study of Zoophilic Dermatophytoses between 2010 and 2016 (2010~2016년 동안 동물친화성 피부 사상균 감염의 역학적 연구)

  • Kim, Su Jung
    • Korean Journal of Clinical Laboratory Science
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    • v.49 no.4
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    • pp.439-445
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    • 2017
  • In recent years, change in life patterns gave rise to an increase in the number of families with companion animals, and as a result, frequent dermatophytes infections have been reported. Microsporum canis, Trichophyton mentagrophytes, and Trichophyton verrucosum, are among these species of zoophilic dermatophytes. Trichophyton mentagrophytes are transmitted to humans by contact with wild animals. Infection from it causes strong inflammation in humans. Conversely, Trichophyton verrucosum is transmitted by contact with cattles. Microsporum canis will become latent carriers in cats or dogs, causing infectious diseases when it comes in contact with humans. We investigated zoophilic dermatophytes isolated according to annual, sex, age, season, body sites, and clinical types between 2010 and 2016. According to our results, the isolation rate of zoophilic dermatophytes was 0.37%, among which, 88 T. mentagrophytes, 228 Microsporum canis, and 18 Trichophyton verrucosum were isolated in human. It is interesting to note that Microsporum canis has been on the rise since 2014. Microsporum canis and Trichophyton verrucosum were highly isolated in females, but T. mentagrophytes was isolated similarly in both sexes. According to an age-based survey, the isolation rate was higher in children younger than 10 years. Our results is a valuable data for predicting and studying the isolation of zoophilic dermatophytes in the future.

Quality Changes in Oyster Mushrooms during Modified Atmosphere Storage as Affected by Temperatures and Packaging Materials (저장 온도와 포장재에 따른 느타리버섯의 MA 저장 중 품질변화)

  • Choi, Mi-Hee;Kim, Gun-Hee
    • Korean Journal of Food Science and Technology
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    • v.35 no.6
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    • pp.1079-1085
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    • 2003
  • Modified atmosphere packaging was applied to oyster mushrooms (Pleurotus ostreatus) to study the effect of storage temperatures and packaging materialso. Whole mushrooms (200g) were package with polyethylene film $(PE,\;60{\mu}m\;thickness)$, ethylene vinyl acetate (EVA), or ceramic film (containing 5% zeolite) and stored at 0, 5, 10 and $20^{\circ}C$. Weight loss, color, firmness, gas composition $(O_2,\;CO_2)$ inside the film package and ethanol content in the tissue of MA packaged mushrooms were examined. Mushroom that were packed unwrapped in a conventional hardboard box (2 kg) lost marketability at a very early stage of storage due to weight loss, shrinkage, browning, and spore formation. During storage, film packaging prevented or retarded the deterioration of the mushrooms in the aspects of appearance, texture, and discoloration. Firmness slightly decreased with storage time. Total color difference was much higher in the control than in the film-packaged mushroom and rapidly increased at the early of storage. Correlation analysis showed a high correlation between total color difference and b values. These results were characterized by the reduced respiration rate resulting from elevated carbon dioxide and reduced oxygen levels in the package. At all storage temperatures, ethanol content in the tissue increased slightly at the early part of storage and rose considerably towards the end of the storage period. Ethanol content in the oyster mushrooms was higher in the stipe than in pileus tissues. The shelf life of the oyster mushrooms was about $8{\sim}11$ days at $0^{\circ}C$, about $4{\sim}6$ day at $5^{\circ}C$, about $2{\sim}3$ days at $10^{\circ}C$, and about $1{\sim}2$ days at $20^{\circ}C$.

Changes in Quality of Fried Rice with Red Snow Crab Meat Depending on the Storage Period and Temperature (저장기간 및 저장온도에 따른 붉은대게풍미 볶음밥의 품질변화)

  • Jung, Ji Hee;Lim, Ji Hoon;Jeong, Min Jeong;Jeong, In Hack;Kim, Byoung Mok
    • Korean journal of food and cookery science
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    • v.31 no.4
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    • pp.387-394
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    • 2015
  • The red snow crab lives at a depth 200-2,000 m in the east coast. It has a smooth taste with a rich texture. However mostly red snow crab are only utilized materials. For seafood development, research is needed on using red snow crab in various products. In this study, quality changes in fried rice prepared with red snow crab meat, red snow crab emulsion sauce and red snow crab effluent were investigated. Physicochemical, microbiological and sensory characteristics were determined during storage at -20, 4 and $25^{\circ}C$ for 5 weeks to assess changes in the quality of the fried rice. The pH and acidity values did not show any significant differences at $-20^{\circ}C$. The VBN and TBA values of fried rice stored at 4 and $25^{\circ}C$ were significantly higher than those of fried rice stored at $-20^{\circ}C$ during the same storage period. The viable cell count of the fried rice stored at $-20^{\circ}C$ changed little during the storage period. During storage at $25^{\circ}C$, the overall quality was initially 8.0, but rapidly decreased to 1.0 after 5 weeks. In conclusion, the best storage temperature for both quality and safety was $-20^{\circ}C$. The storage condition for instant rice containing red snow crab affects the quality and we confirmed the applicability of using materials from red snow crab.

The Effect of working Noise Exposure and Military Background on the Hearing Threshold (특수병과의 과거 군 소음 노출이 소음 노출 작업자의 청력에 미치는 영향)

  • Chung, Ho-Keun;Kim, Kyoo-Sang
    • Journal of Preventive Medicine and Public Health
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    • v.36 no.2
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    • pp.137-146
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    • 2003
  • Objectives : Impaired hearing is a prevalent occupational hazard, not only in industry, but also in the armed forces. In military life, noise has unusual characteristics, and constitutes a serious hazard to hearing. The aim of this study was to analyze the hearing threshold data in order to compare the hearing loss among shipyard workers, representing different workers, and a military service background. Methods : A cross-sectional audiological survey, combined with a questionnaire study, was conducted on a stratified random sample of 440 shipyard workers, with long-term exposure to noise. The employees were divided into four groups, according to their working and military service backgrounds, in relation to their exposure to noise. Results : As expected, the working and military noise exposure group (Group I) had significantly poorer hearing than the other groups. The high frequencies (2-8 kHz) showed the greatest difference in terms of poorer fearing in both ears. The prevalence of noise-induced hearing loss (NIHL) was highest in Group I. A logistic regression analysis was applied to determine the dependence of the NIHL in relation to age, smoking, drinking, working duration, ear protection, past history of ear diseases, and working and military sonics backgrounds, on the noise exposure The important factors found to be related to the NIHL, in relation to noise exposure were: age, work duration, and working and military service backgrounds. The adjusted odds ratio estimates for NIML in the right ear were 4.5 times greater (95% CI 1.7-11.6) for the military noise exposed group, and 7.9 times greater (95% CI 2.0-31.3) for the working noise exposed group than in the controls. The hearing thresholds at the pure-tone average and 4 kHz were significantly increased with age and work duration with both the working and military service backgrounds. Conclusions : From these results, specific preventive programs were planned, which should be assessed by epidemiological surveillance of the military noise exposed population.

The Mechanism of Iron Transport after Intratracheal Instillation of Iron in Rats (랏트의 기관내 Fe 노출후 Fe 이동에 대한 연구)

  • Kwon, Min;Choi, Byung-Sun;Park, Eon-Sub;Chung, Nam-Hyun;Park, Sung-Jo;Lim, Young;Park, Jung-Duck
    • Journal of Preventive Medicine and Public Health
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    • v.37 no.4
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    • pp.329-336
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    • 2004
  • Objectives : Iron (Fe) is an essential element in biological processes; however excessive Fe is harmful to human health. Some air pollutants contain a high level of Fe, and the human lung could therefore be over-exposed to Fe through inhaled air pollutants. This study was performed to investigate the role of metal transporters (divalent metal transporter 1, DMT1, and metal transporter protein 1, MTP1) in the lung under the environments of Fe deficiency in the body and Fe over-exposure in the lung. Methods : Rats were fed Fe deficient (FeD, 2-6 mg Fe/kg) or Fe supplemented (FeS, 120 mg Fe/kg) diet for 4 weeks, followed by a single intratracheal instillation of ferrous sulfate at low (10 mg/kg) or high (20 mg/kg) dose. Fe concentration was analyzed in the serum, lung and liver, and histopathological findings were observed in the lung at 24 hours after Fe administration. The level of DMT1 and MTP1 expression in the lung was analyzed by RT-PCR. Also, the effect of Fe deficiency in the body was evaluated on the level of Fe concentration and metal transporters compared to FeS-diet fed rats at the end of 4-week FeD or FeS diet. Results : The 4-week FeD diet in rats induced an Fe deficiency anemia with decreased serum total Fe, increased unsaturated Fe binding capacity and hypochromic microcytic red blood cells. The concentration of Fe in the lung and liver was lower in the FeD-diet fed rats than in the FeS-diet fed rats. The level of metal transporters mRNA expression was higher in the FeD-diet fed rats than in the FeS-diet. The concentration of Fe in the lung was increased in a dose-dependent pattern after intratracheal instillation of Fe into the rats, while the level of Fe in the serum and liver was not increased in the low-dose Fe administered rats. Therefore, DMT1 and MTP1 mRNA was highly expressed in both FeD-diet and FeS-diet fed rats, after intratracheal instillation of Fe. Conclusions : DMT1 and MTP1 mRNA were more highly expressed in FeD-diet fed rats than in FeS-diet fed rats. The over-exposure of Fe intratracheally induced high expression of metal transporters and increased Fe deposition in the lung in both FeD-diet and FeS-diet fed rats, but did not increase the Fe level of the serum and liver in low-dose Fe administered rats. These results suggest that the role of metal transporters in the lung might be different in a part from the duodenum under the environment of over-exposure to Fe.

Initial $^{99m}Technetium-dimercaptosuccinic$ Acid (DMSA) Renal Scan Finding and Vesicoureteral Reflux as Predicting Factor of Renal Scarring (신반흔 예측인자로서 초기 $^{99m}Technetium-dimercaptosuccinic\;Acid$ 신주사 소견과 방광요관 역류의 유무 및 정도)

  • Lee Soo-Yeon;Lim So-Hee;Lee Dae-Yeol
    • Childhood Kidney Diseases
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    • v.7 no.1
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    • pp.44-51
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    • 2003
  • Purpose : Acute pyelonephritis in children may result in permanant renal damage which later in life may lead to hypertension and renal failure. The purpose of this study was to evaluate the factors that might be useful for predicting the development of renal scar in children with urinary tract infection(UTI). Methods : We retrospectively reviewed 442 patients with UTI who were admitted to the Department of Pediatrics of Chonbuk National University Hospital, during the period from April 1992 to March 2002. The patients were divided into two groups according to the presence of renal scar on the follow-up DMSA renal scan, and we compared the factors associated with renal scarring between the two groups. Results : There were no significant differences in sex, causative organism and acute phase reactants between the groups with and without renal scar. The age at diagnosis was significantly higher in the renal scar group compared to that without scar. Of the 60 patients with renal scar, 78% had vesicoureteral reflux(VUR), but 13% of patients without scar had VUR. Furthermore, the severity of VUR was significantly correlated with renal scar formation. 53% showed multiple cortical defects on the initial DMSA renal scan, compared to 32% in the non-scar group. In addition, 76% of patients showing multiple cortical defects on the initial DMSA renal scan with VUR had renal scar. Conclusion : The presence and grade of VUR, and findings on the initial DMSA renal scan would contribute to predict risk of renal scar formation in children with UTI.

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Evaluation of Microbial PCE Reductive Dechlorination Activity and Microbial Community Structure using PCE-Contaminated Groundwater in Korea (사염화에틸렌(PCE)으로 오염된 국내 4개 지역 지하수 내 생물학적 PCE 탈염소화 활성 및 미생물 군집의 비교)

  • Kim Young;Kim Jin-Wook;Ha Chul-Yoon;Kwon Soo-Yeol;Kim Jung-Kwan;Lee Han-Woong;Ha Joon-Soo;Park Hoo-Won;Ahn Young-Ho;Lee Jin-Woo
    • Journal of Soil and Groundwater Environment
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    • v.10 no.2
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    • pp.52-58
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    • 2005
  • In Korea, little attention has been paid to microbial perchloroethylene (PCE) and/or trichloroethylene (TCE) dechlorination activity and identification of microorganisms involved in PCE reductive dechlorination at a PCE-contaminated aquifer. We performed microcosm tests using the groundwater samples from 4 different contaminated sites (i.e. Changwon A, Changwon B, Bucheon and Yangsan) to assess PCE reductive dechlorination activity. We also adapted molecular techniques to screen what types of known reductive dechlorinators are present at the PCE-contaminated aquifers. In the Changwon A and Changwon B active microcosms where potential electron donors such as sodium propionate, sodium lactate, sodium butyrate, and sodium fumarate, were added, ethylene, an end-product of complete reductive dechlorination of PCE, was detected after a period of 90 days of incubation. In the Bucheon and Yangsan active microcosms, cis-1,2-dichloroethylene (c-DCE) was accumulated without the production of vinyl chloride (VC) and ethylene. Molecular techniques were used to evaluate the microbial community structures in the Changwon B and Yangsan aquifer. We found two sequence types that were closely related to a known PCE to ethylene dechlorinator, named uncultured bacterium clone DCE47, in the Changwon B site clone library. However, in the Yangsan site clone library, no sequence type was closely related to known PCE dechlorinators reported. It is plausible that microorganisms being capable of completely dechlorinating PCE to ethylene may be present in the Changwon B site aquifer. In this study we find that complete PCE reductive dechlorinators are present at some PCE-contaminated sites in Korea. In an engineering point of view this information makes it feasible to apply a biological reductive dechlorination process for remediating PCE- and/or TCE-contaminated aquifers in Korea.

Change of Nutrition Loss of Long-term Application with Different Organic Material Sources in Upland Soil (유기물원이 다른 퇴비연용 밭토양에서 양분유실량 변화)

  • Kim, Jong-Gu;Lee, Kyeong-Bo;Kim, Jae-Duk;Han, Sang-Su;Kim, Seong-Jo
    • Korean Journal of Soil Science and Fertilizer
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    • v.33 no.6
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    • pp.432-445
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    • 2000
  • The objective of this study was to determine the effects of various kinds of composts on the change of nutrition loss in upland soils. Field experiments were conducted in the loam and sandy loam soils, while the clay loam and sandy loam soils were used for laboratory experiments. Various kinds of composts such as poultry manure compost(PMC), cow manure compost(CMC), human excrement sludge(HES), and food industrial sludge compost(FISC) were applied annually at rates of 0, 40, and $80mg\;ha^{-1}$ to soils grown with soybean and maize plants for 4 years during 1994 to 1997. The results of this study were as follows : The loss of nutrients in the form of cation and anion by run-off water increased with the increase of compost application rate. Compared with bare soils, maize cultivation decreased the nutrient loss by run-off from soils by 43% in anionic form and 32% in cationic form. Amount of cation loss were ordered $K^+$ > $Ca^{2+}$ > $Na^+$ > $Mg^{2+}$ > $NH_4{^+} $ and that of anion loss were ordered $SO_4{^{2-}}$ > $NO_3{^-}$ > $Cl^-$ > $PO_4{^{3-}}$. Nutrient loss of sand loam soil in the cation and anion by percolation water increased 1.7 times compared with loam soil. $NO_3{^-}-N$ contents in percolated water were high at the initial stage after compost application, and the amounts were higher in sandy loam soil than loam soil. The maize cultivation also decreased the $NO_3{^-}-N$ contents in percolated water by 82% in loam soil, and 58% in sand loam soil. Soil pH of composts determined by laboratory incubation test increased pH 6.1~6.8 application with poultry and cow manure compost but application with human excrement sludge decreased pH 4.5~4.7. Soil EC were increased initially composts application and decreased up to 2 weeks, thereafter kept a certain level. Nitrogen mineralization rates of composts determined by laboratory incubation test at $25^{\circ}C$ were 39~76% in sandy loam soil, and 16~48% in clay loam soil.

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Clinical Analysis of Patients with Abdomen or Neck-penetrating Trauma (복부와 경부 관통상 환자에 대한 임상적 고찰)

  • Noh, Ha-Ny;Kim, Kwang-Min;Park, Joon-Beom;Ryu, Hoon;Bae, Keum-Seok;Kang, Seong-Joon
    • Journal of Trauma and Injury
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    • v.23 no.2
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    • pp.107-112
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    • 2010
  • Purpose: Recently, the change to a more complex social structure has led to an increased frequency of traumas due to violence, accident and so on. In addition, the severity of the traumas and the frequency of penetrating injuries have also increased. Traumas to cervical and abdominal areas, what are commonly seen by general surgeons, can have mild to fatal consequences because in these areas, various organs that are vital to sustaining life are located. The exact location and characteristics of the injury are vital to treating patients with the trauma to these areas. Thus, with this background in mind, we studied, compared, and analyzed clinical manifestations of patients who were admitted to Wonju Christian hospital for penetrating injuries inflicted by themselves or others. Methods: We selected and performed a retrospective study of 64 patients who had been admitted to Wonju Christian Hospital from January 2005 to December 2009 and who had cervical or abdominal penetrating injuries clearly inflicted by themselves or others. Results: There were 51 male (79.7%) and 13 female (20.3%) patients, and the number of male patients was more dominant in this study, having a sex ratio of 3.9 to 1. The range of ages was between 20 and 86 years, and mean age was 43.2 years. There were 5 self-inflicted cervical injuries, and 19 self-inflicted abdominal injuries, making the total number of self-inflicted injury 24. Cervical and abdominal injuries caused by others were found in 11 and 29 patients, respectively. The most common area involved in self-inflicted injuries to the abdomen was the epigastric area, nine cases, and the right-side zone II was the most commonly involved area. On the other hand, in injuries inflicted by others, the left upper quadrant of the abdomen was the most common site of the injury, 14 cases. In the neck, the left-side zone II was the most injured site. In cases of self-inflicted neck injury, jugular vein damage and cervical muscle damage without deep organ injury were observed in two cases each, making them the most common. In cases with abdominal injuries, seven cases had limited abdominal wall injury, making it the most common injury. The most common deep organ injury was small bowel wounds, five cases. In patients with injuries caused by others, six had cervical muscle damage, making it the most common injury found in that area. In the abdomen, small bowel injury was found to be the most common injury, being evidenced in 13 cases. In self-inflicted injuries, a statistical analysis discovered that the total duration of admission and the number of patients admitted to the intensive care unit were significantly shorter and smaller, retrospectively, than in the patient group that had injuries caused by others. No statistically significant difference was found when the injury sequels were compared between the self-inflicted-injury and the injury-inflicted-by-others groups. Conclusion: This study revealed that, in self-inflicted abdominal injuries, injuries limited to the abdominal wall were found to be the most common, and in injuries to the cervical area inflicted by others, injuries restricted to the cervical muscle were found to be the most common. As a whole, the total duration of admission and the ICU admission time were significantly shorter in cases of self-inflicted injury. Especially, in cases of self inflicted injuries, abdominal injuries generally had a limited degree of injury. Thus, in our consideration, accurate injury assessment and an ideal treatment plan are necessary to treat these patients, and minimally invasive equipment, such as laparoscope, should be used. Also, further studies that persistently utilize aggressive surgical observations, such as abdominal ultrasound and computed tomography, for patients with penetrating injuries are needed.