• Title/Summary/Keyword: 속도양상

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The Clinical and Genetic Characteristics of Three Korean Patients with Glycogen Storage Disease Type V (McArdle Disease) (세 명의 대한민국 제 V형 당원축적근육병(McArdle 병) 환자들의 유전학적 및 임상적 특성 보고)

  • Lee, Sunghee;Kang, Eungu;Kim, Yoonmyung;Lee, Beom Hee;Kim, Gu Hwan;Yoo, Han Wook
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.16 no.2
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    • pp.93-101
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    • 2016
  • Purpose: McArdle disease, glycogen storage disease type V (GSD V), is one of the most common adolescent-onset glycogen storage diseases. It is caused by recessive mutations in PYGM encoding myophosphorylase, which is critical to glycogen metabolism. Since only a few korean patients have been reported, we will observe the clinical and genetic features of three korean patients with McArdle disease. Methods: We retrospectively reviewed the medical records of three patients with genetically confirmed McArdle disease, including the results of forearm ischemic exercise test, electromyogram, nerve conduction velocity, muscle biopsy, and PYGM analysis in peripheral leukocytes. Results: All three cases were males and their age of symptom onset was 12, 5, 14 years old, respectively. A high basal level of serum creatine kinase was noted in all three patients. They experienced the recurrent episodes of rhabdomyolysis, but second wind phenomenon was not definite. In muscle biopsy, subsarcolemmal space vacuoles including periodic acid schiff stained materials were found in two patients, while no evidence of glycogen storage disease was found in the other. A total of five different mutations, $p.Arg50^*$, p.Trp798Arg, $p.Arg50^*$, p.Glu779del, $p.Asp511Thrfs^*28$ and p.Phe710del, were found in three patients. Avoidance of isometric exercise, aerobic exercise and glucose intake before each exercise were recommended for all patients. Conclusion: The three Korean patients with McArdle disease showed the typical manifestations of the condition. The most mutations were private. Therefore, identification of more cases with long-term follow-up will be required to understand the clinical and genetic features of this disease among Korean population.

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The expression of MMP-1, -8, and -13 mRNA in the periodontal ligament of rats during tooth movement with cortical punching (백서의 치아이동 시 피질골 천공이 치주조직의 MMP-1, -8, -13 mRNA의 발현에 미치는 영향)

  • Gwack, Choon;Kim, Seong-Sik;Park, Soo-Byung;Son, Woo-Sung;Kim, Yong-Deok;Jun, Eun-Sook;Park, Mi-Hwa
    • The korean journal of orthodontics
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    • v.38 no.3
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    • pp.187-201
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    • 2008
  • Objective: The aim of this study was to determine whether cortical punching stimulates the expression of matrix metalloproteinase-1, -8, and -13 in orthodontic tooth movement in rats. Methods: A total of 32 male sprague-dawley rats at 15 weeks old were divided into two groups of 16 rats each, to form the tooth movement with cortical punching (TMC) group and tooth movement only (TM) group. A total of 20 gm of orthodontic force was applied to rat incisors to cause experimental tooth movement. Cortical punching was performed on the palatal side near the central incisor with a 1.0 mm width microscrew in the TMC group. The duration of tooth movement was 1, 4, 7, and 14 days. Results: Measurements of the mRNA expression were selected as the means to determine the identification of expression of MMP-1, -8, and -13. In the TMC group, the expression of collagen type I was greater than that of the TM group from day 4 to day 14. Expression of TIMP-1 in the TM group was greater than that of the TMC group in the pressure side of PDL and alveolar bone cell at day 4. In the TMC group, TIMP-1 was expressed at the osteoclast, but not at the tooth surface of the TM group at day 14, Maximum induction of the mRNA of MMP-1 was observed on day 4 in the TMC group, but it was observed on day 7 in the TM group. MMP-8 mRNA of the TMC group was twice greater than that of the TM group at f days. In the TMC group, maximum induction of MMP-13 mRNA was observed on day 1. Conclusions: These findings suggested that cortical punching can stimulate remodeling of PDL and alveolar bone connective tissues during experimental orthodontic tooth movement in rats.

BOND STRENGTH OF BONDED AMALGAM USING DENIAL ADHESIVES (치과용 접착제를 사용한 접착 아말감의 결합 강도)

  • Kam, Dong-Hoon;Lee, Sang-Dae;Hahn, Se-Hyun
    • Journal of the korean academy of Pediatric Dentistry
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    • v.26 no.2
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    • pp.284-295
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    • 1999
  • The purpose of this study was to measure and analyze the bond strength of bonded amalgam using dental adhesives and to compare this with light-curing composite resin. Sections 8mm in diameter were punched out from the labial surface of bovine anterior teeth. These were embedded in clear acrylic resin blocks with labial surface facing out. 55 specimens were made for enamel and dentin each. After dividing these into 5 groups, group 1: Superbond C&B, group 2: Panavia 21, group 3: All-Bond 2, group 4: Fuji I Glass Ionomer Luting Cement, group 5: Scotchbond Multi-Purpose(Restorative Z-100), molds with holes of 6.3mm in diameter and 1.5mm in depth were placed over the specimens. The exposed tooth surfaces were treated with adhesives and the molds were filled with amalgam. In group 5, the mold was filled with composite resin and light-cured for 40 seconds. The author measured all specimens for bond strength 24 hours after amalgam filing and analyzed fracture surfaces. The following results were obtained: 1. Among the dentin groups, groups 1, 2 and 4 showed significantly lower bond strength compared with group 5(P<0.05). 2. Among the enamel groups, group 4 showed significantly lower bond strength compared with group 5(P<0.05). 3. In group 2, 2D showed significantly lower bond strength compared with group 2E(P<0.05). Other adhesives showed no such differences in bond strength between dentin and enamel(P>0.05). 4. Cohesive failure was observed in groups 1E and 5D, while mixed failure was seen in groups 1 and 5. Only adhesive failures were noted in groups 2, 3, 4.

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Bud Development and Bud Break Characteristics in Water Cuttings of 'Campbell Early' Grapevine during Dormancy ('캠벨얼리' 포도의 휴면기 눈 발달 및 수삽을 통한 발아 특성 조사)

  • Lee, ByulHaNa;Park, YoSup;Kwon, YongHee;Han, Jeom-Hwa;Park, Hee-Seung
    • Horticultural Science & Technology
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    • v.33 no.2
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    • pp.202-209
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    • 2015
  • In this study, we investigated the cumulative effect of low temperature on bud dormancy release and bud break characteristics in 'Campbell Early' grapevine (Vitis labruscana B.) cuttings grown in water culture. Additionally, we observed the development of buds while exposed to low temperatures in an attempt to improve our understanding of dormancy and bud break. The shoots were collected 120 days after full bloom (DAFB; leaf abscission period), and the accumulated chill unit (CU) value was calculated by reducing the temperature to $7.2^{\circ}C$ at 125 DAFB. The rate of bud break was 100% in shoots collected at 150 DAFB, The period until the first bud break was two times longer than in the shoots collected 165 DAFB, and bud break speed was significantly reduced. These results indicate that buds are released from endodormancy after 165 DAFB, because at this point the bud break was complete (bud break rate 100%) and it occurred in a very short time period. During this period, when the low-temperature accumulated value was 321h and 442CU according to the CH and Utah models, respectively. Furthermore, the survival rate of main buds decreased rapidly after 165 DAFB, and survival rate of accessory buds was maintained at more than 90% without seasonal differences. The rate of flower bud formation of main buds was much higher than in accessory buds (1:0.23) before the release from endodormancy at 150 DAFB. The final ratio of accessory buds to main buds was high, 1:1.54, at 255 DAFB. Correlation analysis of each investigated factor revealed that bud survival rate and bud formation rate were related only for the main buds, and there was a close relationship between the survival rate of main bud and time. In addition, the survival rate of main buds was positively correlated to the rate of flower bud formation.

Clinical Observations on 12 Children with Alport Syndrome (Alport 증후군 환아 12명의 임상적 고찰)

  • Bae Young-Min;Kim Seoung-Do;Kang Hyeon-Ho;Cho Byoung-Soo
    • Childhood Kidney Diseases
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    • v.4 no.1
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    • pp.48-56
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    • 2000
  • Purpose: Alport SD., the most common herectitary rephriris, is a renal disease with rapid progression. Deafness, ocular abnormalities and a specific EM finding may be associated in addition to a family history. We have aralyged retrospectively. Methods: We observed 12 children with Alport syndrome who were diagnosed at Dept. of pediatrics in Kyunghee Univ., College of Medicine, from Apr. 1991 until Jun. 1999. We used four criteria for diagnosis: renal disease, family history, deafness or eye abnormalities, and a specific finding in electron microscopy Results: 2 of 12 patients had all features of the four diagnostic criteria. We could not trace an exact family history in 3 patients, and 6 patients did not exhibit deafness or eye abnormality. One could not have renal biopsy because offer chronic renal failure. Other three criteria were observed in her. The ratio of male to female observed was 1:2 respectively and the mean age of initial renal symptom was 5.6 years. 9 of 12 patients had a family history of renal disease. In the audiogram and ocular examination for 11 of 12 cases, sensorineural hearing loss was observed in 6 and ocular abnormality in 2 cases. In electron microscopic finding, irregular thickness of the capillary basement membranes with lamination of lamina densa and foot process obliteration was noted in 9 of 11 and thin basement membrane with splitting and foot process obliteration was noted in the other 2. The mean period of follow-up was 3 6/12 years. And one patient developed the chronic renal failure until now and had kidney transplantation. Conclusion: For the diagnosis of Alport syndrome, the following four diagnostic criteria are very important : renal disease, family history, deafness or eye abnormalities, and a specific finding on electron microscopy. We expect that more patients can be detected through the analysis of these characteristics.

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The Clinical Aspects of Acute Septic Arthritis and Acute Osteomyelitis in Children (소아기 급성 화농성 관절염과 급성 골수염의 임상적 고찰)

  • Kim, Jin-Man;Lee, So-Yeon;Kim, Young-Ho;Shin, Eon-Woo;Oh, Phil-Soo;Kim, Kwang-Nam
    • Pediatric Infection and Vaccine
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    • v.12 no.2
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    • pp.149-156
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    • 2005
  • Purpose : Acute septic arthritis and acute osteomyelitis are not rare diseases in pediatric population. But when the diagnosis is delayed or inappropriate treatments are given, permanent disabilities of joint or bone can be followed. We analysed clinical manifestations, laboratory findings, X-ray findings, causative microorganisms and antibiotic susceptibility results of the two diseases in children. Methods : During January 1992 and May 2002, we conducted a retrospective study of 103 children who were diagnosed as acute septic arthritis and acute osteomyelitis. We selected out 34 children who had positive culture results in the blood or involved sites. Results : 19 cases were diagnosed as acute septic arthritis and 15 cases were acute osteomyelitis. These diseases were most common in preschool children and next in neonates. Hip joints and tibia were the most common sites in each disease. X-ray findings showed abnormalities in 6 cases(36%) of acute septic arthritis and 7 cases(50%) of acute osteomyelitis on admission. The most common microorganism isolated from the involved sites was Staphylococcus aureus; 12 out of 14 cases in acute septic arthritis and 6 out of 13 cases in acute osteomyelitis. Conclusion : It is difficult to make a clear initial diagnosis of the two diseases. We could not find any differences between these two diseases on clinical manifestations such as fever, swelling, tenderness and limitation of movements in joint and bone. The most common microorganism was Staphylococcus aureus.

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Clinical manifestation of human bocavirus infection in children (소아 human bocavirus의 임상적 고찰)

  • Choi, Chang Sun;Pak, Chan Hee;Jung, Kwan;Lee, Gun;Sun, Kyu Keun;Kim, Eun Young;Kim, Kyoung Sim;Kim, Yong Wook;Seo, Jin-Jong;Chung, Yoon-Seok
    • Pediatric Infection and Vaccine
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    • v.14 no.2
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    • pp.136-144
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    • 2007
  • Purpose : Human bocavirus (HBoV) was recently identified world widely in clinical specimens from infants and children with respiratory tract illness, but the role of HBoV in respiratory tract illnesses is unknown. The aim of this study was to investigate the frequency and the clinical manifestation of HBoV in pediatric patients. Methods : We retrospectively investigated 1,777 throat swab obtained between 2005 and 2006 from pediatric in-patients with acute respiratory tract diseases at the Kwang-ju Christian Hospital. The medical records of patients with positive results were reviewed for demographic and clinical data of HBoV infections. Results : HBoV DNA was found in 84 (4.7%) of the 1,777 hospitalized children and the mean age was 19 months. The most common diagnosis were pneumonia (67.8%), bronchiolitis (35.7%). HBoV infections were found year-round, though most occurred in spring and winter months. Conclusion : HBoV is frequently found in hospitalized infants and children with acute respiratory tract diseases in Korea, but an association of HBoV with a distinct respiratory tract manifestation was not apparent. To clarify the clinical significance of HBoV, further evaluation of various age groups and clinical groups is needed.

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Differential Weed Competition of Two Rice Cultivars under Various Cropping Patterns (수도(水稻) 이품종(異品種)의 재배양식(栽培樣式)에 따른 잡초(雜草) 경합구조(競合構造) 해석(解析))

  • Guh, J.O.;Kwon, S.L.;Heu, S.M.
    • Korean Journal of Weed Science
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    • v.3 no.1
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    • pp.57-68
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    • 1983
  • To estimate the possible amount of weed-crop competitions under the various cropping patterns of two rice cultivars of different type, comparisons between weedy check and weed-free plots in plant height, tillering number, and plant weight were investigated. Fluctuations in Importance Value, competition value, and net productivities were used to discuss the crop-weed competition, respectively. As a result, differences in dominant weed species, competitive crop variety, most effective cropping pattern, and crop yielding characteristics under the different weed competitions were investigated, respectively.

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Identification and Fermentation Characteristics of Lactic Acid Bacteria Isolated from Dongchimi as Starter for Radish Juice (무 쥬스 제조를 위한 starter로써 동치미에서 분리한 유산균의 동정 및 발효 특성)

  • Kim, Jung-Hee;Kim, Jong-Il
    • Korean Journal of Microbiology
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    • v.35 no.4
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    • pp.307-314
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    • 1999
  • This study was aimed at the investigation of the possibility of the addition of lactic acid bacteria as "starter"for the preparation of radish juice. Forty strains of lactic acid bacteria were isolated from dongchimi that was fermented by a traditional method. The isolates were assorted into 5groups, Leuconostoc mesenteroides subsp. mesenteroides (J-9), Lacobacillus brevis (J-12), Lactobacillus fermentum (J-7), Lactobacillus sake (J-20), and Lactobacillus plantarum (J-39). Leuconostoc mesenteroides was predominated in the sample of dongchimi with frequency of 52.5%. Each of the strain, which exhibited the beat growth in the species, was selected in the 5species, and investigation of the fermentation characteristcis was carried out. The fermentation were performed for 9 days at 25${\circ}C$ after the inoculation of 0.3% ($10^{6}$ cfu/㎖) to each ultra-filtrated radish juice. The pH, total acidity, content of non-volatile organic acids were examined during the fermentation period. Lactobacillus plantarum showed the highest growth rate and the growth rate of Lactobacillus sake was the lowest. The pH (6.3-6.36) and total acidity (0.09-1.0 %) fo the ultrafiltrated radish juice before fermentation were changed to 3.2-4.3 and 0.65-1.2% after 9days, respectively. The changes of the pH and total acidity were related with the growth of the lactic acid bacteria; the better growth of lactic acid bacteria, the more rapid decrease of pH and increase of the total acidity. when the amount of non-volatile organic acids were estimated during fermentation, citric acid, malic acid, malonic acid, and succinic acid were decreased in all cases. However, the content of lactic acid increased with the progression of fermentation. L. mesenteroides (J-9), L. brevis (J-12) and L. fermentum (J-7) were chosen for the candidates of the starter for the lactic fermentation of radish juice based on the biochemical analysis and sensory evaluation.

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Comparison of hypertonic saline treatment in meningitis with cerebral edema (뇌수막염에서 발생한 뇌부종 치료에서 고장성 식염수 비교)

  • Kim, Hyung Su;Kim, Hee Ra
    • Clinical and Experimental Pediatrics
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    • v.49 no.12
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    • pp.1275-1281
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    • 2006
  • Purpose : Cerebral edema in meningitis is a potentially complication. Hypertonic saline of various concentrations are frequently used to treat cerebral edemas in meningitis. We analyzed the safety and efficacy of osmotic therapy in cerebral edema by comparison of various hypertonic saline concentrations and mannitol. Methods : The medical records of 42 patients, who were followed up in the Department of Pediatrics, Busan Medical Center, from Jan. 2002 to Oct. 2005, were analyzed retrospectively. We measured intracranial pressure, mean flow velocity, and various laboratory parameters. Results : In cerebral edema developed in meningitis, intracranial pressure and symptoms were improved in treatment of hypertonic saline and mannitol. Serial bolus infusion of 3 percent hypertonic saline resulted in the best outcome. There was not a statistically considerable difference on the mean values of the intracranial pressure gap. On transcranial doppler, mean flow velocity was increased and pulsatilty index was decreased. Laboratory findings (osmolarity, Na, Cl, pH, lactic acid, Ca) were diffenent during the treatment period as opposed to K, Hb, bicarbonate, base excess. There was not a specific form of hypertonic saline used in meningitis treatment with cerebral edema. Conclusion : The therapy for cerebral edema in meningitis remains largely empirical. Serial bolus infusion of 3 percent hypertonic saline is better than other hypertonic salines. Various concentrations and different infusion methods of hypertonic saline statistically does not influence the result of treatment. More research aimed at improving cerebral edema treatment is needed to identify new, effective forms of treatment.