• Title/Summary/Keyword: 경화부

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A case of Hyper-IgE syndrome with a mutation of the STAT3 gene (STAT3유전자 돌연변이 검사로 확진된 고면역글로불린E 증후군 1례)

  • Kang, Ji-Man;Suh, Jung-Min;Kim, Ji-Hyun;Kim, Hee-Jin;Kim, Yae-Jean;Lee, Hun-Seok;Shin, Young-Kee;Ahn, Kang-Mo;Lee, Sang-Il
    • Clinical and Experimental Pediatrics
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    • v.53 no.4
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    • pp.592-597
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    • 2010
  • Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency disease which is characterized by high serum IgE levels, eczema, and recurrent infections. Herein we present the case of a patient with HIES associated with STAT3 gene ($stat3$) mutation. A 16 year-old girl was admitted to our hospital due to hemoptysis caused by pneumonia with bronchiectasis. She had a history of recurrent skin and respiratory tract infections, such as pneumonia caused by MRSA (methicillin-resistant $Staphylococcus$ $aureus$) and $Pseudomonas$ $aeruginosa$. On physical examination, a broad round shaped nose, oral thrush, and chronic eczematous skin rash over her whole body were found. Laboratory data showed an elevated eosinophil count ($750/{\mu}L$) and total IgE level (5,001 U/mL). The patient's National Institutes of Health (NIH) score for HIES was 44. Direct sequencing of the STAT3 gene revealed that the patient was heterozygous for a missense mutation in the DNA binding domain of the STAT3 protein (c.1144C>T, p. Arg382Trp). HIES should be suspected in patients with recurrent infections and can be confirmed by clinical scoring and genetic analysis.

Physiological Characteristics Related to Cold Injury in Rice (수도 냉해에 관련된 생리적 특성 고찰)

  • 석순종;허일봉;임정남
    • KOREAN JOURNAL OF CROP SCIENCE
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    • v.36 no.5
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    • pp.429-444
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    • 1991
  • Cold stress influence plant growth through a wide range of growth characters. Adverse effects of low temperature to plant growth come from results of colligative and complex physiological responses to cold stress. To evaluate more exactly cold tolerance of crop plant, it is needed to observe physiological changes induced by cold stress and to analyze relationships between intraspecific variations in physiological factors related to cold tolerance and the extent of cold tolerance in the field. Therefore, the composition and unsaturation ratio of fatty acids in phospholipid, a constituent of membrane, the transition-temperature in respiratory activity of mitochodria, the chlorophyll fluorescence as a factor related to photosynthesis were investigated in rice plant and data on these factors were compared with the degree of cold tolerance obtained in the field experiment. Also, effects of hardening and Mn++ treatment were evaluated as a method to reduce chilling injuries. The unsaturation ratio of fatty acids, whether rice plants were grown in a natural condition or under the chilling stress, was higher in the cold- tolerant varieties and was significantly correlated with the degree of cold tolerance (1-9) observed in the field experiment. And it was also increased by chilling treatment or hardening treatment, due to a reduction in palmitic acid content and an increase in linolenic acid content. The transition-temperature of respiratory activity of mitochodria isolated from etiolated rice seedlings ($25^{\circ}C$, two week-grown in the dark), was correlated with the degree of cold tolerance in the field, cold -tolerant varieties showing a lower transition-temperature. It was not influenced by growth stages. The intensity of chlorophyll fluorescence was highly correlated with the degree of cold tolerance, cold-tolerant varieties having a higher fluorescence intensity. By foliar application of Mn, the transition-temperature of respiratory activity was lowered as much as 0-2$^{\circ}C$ in all tested varieties. Soil application of Mn induced more significant effect in cold-susceptible varieties with a possibility of reducing chilling injuries. On the whole, there were high correlationships among the degree of cold tolerance, the unsaturation ratio of fatty acids in phospholipid, the transition- temperature of respiratory activity and chlorophyll fluorescence except for a few varieties. The transition- temperature of respiratory activity appeared to be negatively correlated with the unsaturation ratio of fatty acids. and the chlorophyll fluorescence to be positively correlated with the unsaturation ratio. This implies that these physical and physiological factors were very closely related to cold tolerance and can be used as an effective index of the evaluation of cold tolerance of crop plant. But other factors as well as three factors discussed above are needed to be considered colligatively and altogether with a systematic analysis for the more exact evaluation of cold tolerance. in rice cultivars. in rice cultivars.

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Performance of Direct Seeded Rice in Ridged Dry Soil at Different Seeding Methods and Seeding Rates (벼 건답휴립직파재배에서 파종양식과 파종량에 따른 생육과 수량)

  • 이석순;백준호;김태주
    • KOREAN JOURNAL OF CROP SCIENCE
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    • v.37 no.6
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    • pp.514-520
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    • 1992
  • Performance of direct seeded paddy rice in ridged dry soil was evaluated at different seeding methods (broadcasting, drilling, and seeding in group) and seeding rates (4, 6, and 8kg /10a). The number of maximum tillers at broadcasting and drilling of seeds was higher than that at seeding in group. At broadcasing and drilling the number of maximum tillers at seeding rate of 6kg /10a was greater than that at 4 or 8kg /10a, but at seeding in group the number of maximum tillers increased with seeding rates. Among the seeding methods there were no differences in the number of seedlings per unit area, culm and panicle lengths, and productive tiller ratio, but heading date at seeding in group was delayed by a day compared with broadcasting or drilling. At heading stage leaf area index(LAI) and dry matter production at broadcasting of seeds were higher compared with drilling and seeding in group, but light transmission ratio at drilling and seeding in group was higher than that at broadcasting of seeds. Although the number of seedlings increased with seeding rates, LAI and dry matter production at heading stage, culm and panicle lengths, and productive tiller ratio were not different among the seeding rates. There were no differences in the number of panicles and spikelets per unit area, 1,000 grain weight, yield, and harvest index among the seeding methods. The number of spikelets per panicle at seeding in group was higher, but percent ripened grains was lower compared with broadcasting and drilling. There were no significant differences in the number of panicles and spikelets per panicle and unit area, percent ripened grains, and harvest index among the seeding rates, but yield at seeding rate of 6kg /10a was higher than at 4 or 8kg /10a. There were no significant differences in tiller length, bending moment, and fresh weight of tiller among the seeding methods. Breaking strength was lower in the order of seeding in group, drilling, and broadcasting of seeds. However, lodging index was similar among the seeding rates and lodging was not occurred in the field. There was a significant interaction in the cellulose, hemicellulose, and lignin contents of culm base between seeding methods and seeding rates.

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Surgical Treatment of Thoracoabdominal Aortic Aneurysm (흉복부 대동맥류의 외과적 치료)

  • Ahn, Hyuk;Kim, Jun-Seok
    • Journal of Chest Surgery
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    • v.29 no.2
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    • pp.177-184
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    • 1996
  • Between 1987 and 1994, 21 patients were treated surgically for aortic aneurysm involving the thoracoabdominal aorta. There were 11 males and 10 females, and their age ranged from 20 to 67 years old and mean age was 41.5 years. Many complained of back pain, chest pain or discomfort, and flank pain, but three patients were asymptomatic. 15 patients had chronic dissection (71.4%) and 6 had nondissecting fusiform or saccular aneurysm(28.5%), and of those 15 patients with chronic dissection, 6(28.5%) had atherosclerosis assniated with hypertension, 5 (23.8%) were Martian syndrome, and 2 (9.5%) were associated with pregnancy. The diameter of an aneurysm ranged from 6cm to 12cm, and their extent was classified as type I in 7(33.3%), type II in 8(38.1%), type III in 3(14.3%), and type IV in 3(14.3%) patients based on Crawford classification for TAA . Diseased aorta was replaced with artificial vascular graft in all but one patient. In whom the aortic tear site due to pseudoaneurysm was closed by primary suture. For the spinal cord protection during the operation, we used partial cardiopulmonary bypass (FV-FA or PA-FA bypass) in 12 patients (57.1%), Biopump (LA-FA bypass) in 4(19.0%), total circulartory arrest and CPB in 2 (9.5%), Gott's heparinized shunt in 1(4.7%), and simple aortic cross clamping in 2 (9.5%). The most common complication after the operation was hoarseness due to unilateral vocal cord palsy which onured in 5 patients (23.8%), and the next common complication was wound infection in 4 patients(19.0%), paraplegia in 2 patients (9.5%), chylothorax in 1 patient(4.7%). The hospital mortality rate was 9.5% (2deaths), and there was no late death. Our experience shows that the graft replacement of TAAA had reasonable rate of mortality, low rate of serious complication, and provided good post operative state of the pati nts, and since the thoracoabdominal aortic operation is not a high risky procedure anymore, we recommend a radical operation for the indicated patients.

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The effects of enamel matrix derivatives on the proliferation and gene expression of PDL fibroblast, $SaOs_2$ cells and Cementum derived cells (법랑기질유도체가 치주인대세포, 불멸화 조골세포, 백악질 유래세포의 증식과 유전자 발현에 미치는 영향)

  • Jeong, Yoo-Jee;Kim, Kyoung-Hwa;Kim, Tae-Il;Seol, Yang-Jo;Ku, Young;Lee, Hae-Jun;Rhyu, In-Chul;Chung, Chong-Pyoung;Han, Soo-Boo;Lee, Yong-Moo
    • Journal of Periodontal and Implant Science
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    • v.35 no.2
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    • pp.321-333
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    • 2005
  • 1. 목적 in vitro 상에서 법랑기질유도체가 치주인대섬유아세포, 불멸화 조골세포와 백악질 유래세포의 증식과 유전자 발현에 미치는 영향을 알아보고자 하였다. 2. 연구방법 및 재료 <세포증식 연구> 교정을 목적으로 발거한 치아에서 분리, 배양한 치주인대섬유아세포와 백악질유래세포, 그리고 $SaOs_2$ 세포를 이용하였다. 법랑기질유도체가 세포 증식에 미치는 영향을 알아보기 위해, 35 mm Petri dish에 dish 당 $5{\times}10^3$ 개의 세포를 접종하였다. 대조군은 1% 항생제와 10% FBS를 포함한 DMEM 배지를 이용했고, 5mM 초산을 첨가한 군과 첨가하지 않은 두 개의 대조군이 이용되었다. 실험군은 100 ${\mu}g/ml$의 법랑기질유도제를 첨가한 군과 100 ${\mu}g/ml$의 법랑기질유도체와 5 mM의 초산을 첨가한 2개의 실험군이 이용되었다. 각 군은 세 개의 배양접시에 행해졌고, 1, 3, 8일에 세포의 수를 각각 측정하였다. 결과는 repeated measures ANOVA로 통계 처리하였다. <유전자 발현 연구> 각 세포의 형질 특성을 알아보기 위해 RT-PCR을 실시하여 조골세포 분화 표식자와 연관된 Human collagen type I(COL I), human osteopontin(OP), human osteocalcin(OC), human alkaline phosphatase(ALP)와 human bone sialoprotein(BSP)의 mRNA 발현을 실험 1, 3, 8일에 걸쳐, 세 군의 차이를 비교 관찰하였다. 3. 결과 <세포증식 연구> 치주인대세포와 백악질유래세포, 그리고 $SaOs_2$ 세포의 증식은 법랑기질유도체에 의해 영향을 받지 않았다. 대조군과 초산이 포함된 대조군 그리고 법랑기질유도체와 초산이 포함된 실험군에서 유의할 만한 세포 수의 차이가 실험 기간 1, 3, 8일에 걸쳐 나타나지 않았다(p<0.05). <유전자 발현 연구> ALP와 COL I은 세 군의 세포에서 모두 발현되었고, 발현 정도는 EMD에 영향을 받지 않았다. OC은 세 군에서 모두 비교적 약하게 발현되었고, 특히 $SaOs_2$ cell과 백악질유래세포에서 약하게 발현되었다. EMD는 OC의 발현정도를 약하게 하였다. OP은 백악질유래세포에서 1, 3, 8일에 걸쳐 EMD 유무에 관련 없이 발현되지 않았다. 그러나 치주인대세포와 $SaOs_2$ cell에서는 강하게 발현되었다. BSP는 치주인대세포와 $SaOs_2$ cell에서 1, 3, 8일에 걸쳐 비교적 고르게 발현되었다. EMD 배지에서 배양된 백악질유래세포는 8일에는 BSP가 발현되지 않았다. 4 결론 이번 실험 결과에 의하면 법랑기질유도체는 치주인대세포, 불멸화 조골세포와 백악질 유래세포의 증식에 있어 유의성 있는 효과를 나타내지 않았다. 그러나, 유전자 발현에 있어서는, 치주인대세포와 백악질유래세포, 그리고 $SaOs_2$ 세포 모두에서 OC mRNA의 발현을 억제하는 효과를 나타내었다. EMD는 세포의 증식에는 영향을 미치지 않지만, 유전자 발현에 있어 일부 영향을 미치는 것으로 보인다. 법랑기질유도체가 세포의 증식과 유전자 발현에 미치는 영향은 배양된 세포의 형질특성, 배양환경, 배양일수 등에 따라 달라질 수 있다. 그러므로 법랑기질유도체가 in vitro 상에서 세포에 미치는 영향은 보다 정량화된 연구가 필요하다.