• 제목/요약/키워드: CD19+

검색결과 750건 처리시간 0.032초

A novel BTK gene mutation, c.82delC (p.Arg28 Alafs*5), in a Korean family with X-linked agammaglobulinemia

  • Lee, Jeongeun;Rhee, Minhee;Min, Taek Ki;Bang, Hae In;Jang, Mi-Ae;Kang, Eun-Suk;Kim, Hee-Jin;Yang, Hyeon-Jong;Pyu, Bok Yang
    • Clinical and Experimental Pediatrics
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    • 제59권sup1호
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    • pp.49-52
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    • 2016
  • X-linked agammaglobulinemia (XLA) is a hereditary humoral immunodeficiency that results from Bruton's tyrosine kinase (BTK ) gene mutations. These mutations cause defects in B-cell development, resulting in the virtual absence of these lymphocytes from the peripheral circulation. Consequently, this absence leads to a profound deficiency of lg all isotypes, and an increased susceptibility to encapsulated bacterial infections. A 15-month-old Korean boy presented with recurrent sinusitis and otitis media after 6 months of age, and had a family history of 2 maternal uncles with XLA. Laboratory tests revealed a profound deficiency of Ig isotypes, and a decreased count of $CD19^+$ B cells in the peripheral circulation. Based on his family history and our laboratory test results, he was diagnosed with XLA. We performed BTK gene analysis of peripheral blood samples obtained from family members to confirm the diagnosis. Mutational analysis revealed a novel hemizygous frameshift mutation (c.82delC, p.Arg28Alafs*5), in the BTK gene. His mother and maternal grandmother were heterozygous carriers of this mutation and his two maternal uncles were hemizygous at the same position. After XLA diagnosis, intravenous immunoglobulin (400 mg/kg, monthly) treatment was initiated; recurrent sinusitis and otitis media were subsequently brought under control. To our knowledge, this is the first reported case of a Korean pedigree with a novel mutation in the BTK gene.

성염색체 열성 범저감마글로불린혈증 환자의 임상 소견 및 치료 (Clinical Manifestations and Treatment in Korean Patients with X-Linked Agammaglobulinemia)

  • 조한나;김중곤
    • Pediatric Infection and Vaccine
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    • 제24권3호
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    • pp.152-159
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    • 2017
  • 목적: 본 연구에서는 서울대학교 어린이병원에서 범저감마글로불린혈증(X-linked agammaglobulinemia [XLA])로 진단된 환자들의 임상적 증상 및 치료에 대해서 보고하고자 하였다. 방법: 14명의 남자 환자가 XLA로 진단되었으며 CD19+ B 세포가 2% 미만이며 Bruton's tyrosine kinase (Btk) 유전자 돌연변이가 있는 경우를 기준으로 하였다. 결과: 모든 XLA 환자에서 반복적인 세균성 감염이 있었고 환자의 20%에서 호중구 감소증이 있었으며 환자 어머니의 75%가 보인자였다. Btk gene study에서는 돌연변이가 다양한 부위에서 나타났으며 특정부위에서 집중적으로 발생하지는 않았다. 면역글로불린 정맥주사 후 반복적인 세균성 감염은 줄어들었으나 합병증으로 기관지 확장증이 14명 중 3명, 지속적인 부비동염은 4명에서 발생하였다. 결론: 4세 이전 세균 감염이 반복되는 경우 XLA와 같은 체액성 면역결핍을 의심하여 글로불린 검사를 하는 것이 조기 진단에 중요하며 Btk 유전자변이 검사를 시행하여 확진한 후 면역글로불린 정맥주사 및 예방적 항생제 등 적절한 치료를 통해 합병증을 줄이고 무 질병 상태를 유지할 수 있다.

음식폐기물의 양돈사료 자원화를 위한 처리공정 및 사료가치 평가 (Processing Method and Feed Value of Food Wastes as Swine Feed Resources)

  • 정우진;이정채;김태환;임계택
    • 한국환경농학회지
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    • 제19권1호
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    • pp.44-50
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    • 2000
  • MS균을 이용한 음식폐기물의 효율적인 처리 방법의 모색과 양돈사료 자원화를 위한 기초자료를 확보하고자 처리방법에 따른 사료성상, 조성분, 광물질 및 유해 중금속을 분석한 결과 다음과 같은 결론을 얻었다. MS균을 처리하기전 수거된 음식폐기물은 그 배출원과 수집일에 따라 성상 및 조성분 성분이 다양하였으며, 음식폐기물 집하장에서 건조처리전 수거된 시료의 수분 함량은 계절적 요인에 크게 영향을 받았다. MS균에 의한 발효 공정을 거친 후 음식폐기물의 악취가 거의 소멸되었고, 사료로서의 좋은 성상을 보여주었다. 특히, 조지방 및 조단백질 함량이 높은 사료자원으로 평가되었다. MS균 처리 후 음식폐기물의 광물질 함량 분석결과 다량원소 (특히 칼슘)의 함량이 곡류나 농산부산물보다 높았다. 전반적으로 다른 식물성 및 동물성 단미사료원에 비해 균형있는 광물질 구성 및 함량을 가지는 것으로 분석되었다. MS균 처리 전과 후 처리과정 중 음식폐기물내의 중금속 분석결과 수은, 비소, 불소 및 크롬은 미검출되었고 납과 카드뮴은 미량존재하나 배합사료내 허용범위에 훨씬 낮은 수준을 함유하고 있어 사료자원으로의 재활용함에 있어 안전한 것으로 나타났다. 이상의 결과로부터 음식폐기물의 성상 및 사료성분을 고려할 때, 적절한 처리 및 발효공정을 통해 양돈사료자원으로서 재활용될 수 있는 충분한 가치가 있음을 제시한다.

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황사발원지 (몽골) 토양에 대한 특성 분석 (A Study on the Characteristics of Soil in the Asian Dust Source Regions of Mongolia)

  • 김덕래;김정수;반수진
    • 한국대기환경학회지
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    • 제26권6호
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    • pp.606-615
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    • 2010
  • This study aims to identify the characteristics of soil in Mongolia, one of the major Asian dust sources that influence the Korean Peninsula. Soil particle size was analyzed and the result shows that sand (57.5~97.3%) was identified prominently in most regions, followed by silt (2.5~34.7%) and clay (0.0~7.8%). Soil pH of the covered regions were in the range 7.1~10.1, either weak alkaline or strong alkaline. Analysis of ion species in the soil samples exhibited that $Na^+$ ($91.9\;mg\;kg^{-1}$), $Cl^-$ ($65.9\;mg\;kg^{-1}$), and $Ca^{2+}$ ($53.5\;mg\;kg^{-1}$) were detected more in the soil than other species such as ${SO_4}^{2-}$ ($19.2\;mg\;kg^{-1}$), ${NO_3}^-$ ($46.6\;mg\;kg^{-1}$), ${NH_4}^+$ ($3.9\;mg\;kg^{-1}$), $K^+$ ($22.0\;mg\;kg^{-1}$), and $Mg^{2+}$ ($10.2\;mg\;kg^{-1}$). As for heavy metal content in the soil, concentrations of soil-borne metals including Fe, Al, Ca, Mg, and K tended to be high, while metals that come from manmade sources Pb, Cd, Cr, V, and Ni were remarkably low. The concentration of organic carbon (OC) was relatively high at $15.9\;{\mu}g\;mg^{-1}$, while elemental carbon (EC), directly released in the process of fossil fuel combustion, was not detected at all or found in very small amounts. The result indicates that pollution from manmade sources scarcely occurred. The analysis results from this study may contribute to improving modeling accuracy by providing input data for Asian dust prediction models, and be used as base data for determining the process of physiochemical transformation of Asian dust during long-range transport.

Transfection of Mesenchymal Stem Cells with the FGF-2 Gene Improves Their Survival Under Hypoxic Conditions

  • Song, Heesang;Kwon, Kihwan;Lim, Soyeon;Kang, Seok-Min;Ko, Young-Guk;Xu, ZhengZhe;Chung, Ji Hyung;Kim, Byung-Soo;Lee, Hakbae;Joung, Boyoung;Park, Sungha;Choi, Donghoon;Jang, Yangsoo;Chung, Nam-Sik;Yoo, Kyung-Jong;Hwang, Ki-Chul
    • Molecules and Cells
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    • 제19권3호
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    • pp.402-407
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    • 2005
  • Bone marrow mesenchymal stem cells (MSCs) have shown potential for cardiac repair following myocardial injury, but this approach is limited by their poor viability after transplantation. To reduce cell loss after transplantation, we introduced the fibroblast growth factor-2 (FGF-2) gene ex vivo before transplantation. The isolated MSCs produced colonies with a fibroblast-like morphology in 2 weeks; over 95% expressed CD71, and 28% expressed the cardiomyocyte-specific transcription factor, Nkx2.5, as well as ${\alpha}$-skeletal actin, Nkx2.5, and GATA4. In hypoxic culture, the FGF-2-transfected MSCs (FGF-2-MSCs) secreted increased levels of FGF-2 and displayed a threefold increase in viability, as well as increased expression of the anti-apoptotic gene, Bcl2, and reduced DNA laddering. They had functional adrenergic receptors, like cardiomyocytes, and exposure to norepinephrine led to phosphorylation of ERK1/2. Viable cells persisted 4 weeks after implantation of $5.0{\times}10^5$ FGF-2-MSCs into infarcted myocardia. Expression of cardiac troponin T (CTn T) and a voltage-gated $Ca^{2+}$ channel (CaV2.1) increased, and new blood vessels formed. These data suggest that genetic modification of MSCs before transplantation could be useful for treating myocardial infarction and end-stage cardiac failure.

2000~2002년 우리나라에서 관측된 황사의 화학 조성 및 특성 (Chemical Composition and Features of Asian Dust Observed in Korea (2000~2002))

  • 신선아;한진석;홍유덕;안준영;문광주;이석조;김신도
    • 한국대기환경학회지
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    • 제21권1호
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    • pp.119-129
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    • 2005
  • The ambient TSP data measured at Seoul, Incheon. Taean, Daegu, Busan in Korea were used to explain the chemical composition and general features of Asian Dust (AD) observed in Korea. 9 episodes out of 19 were sampled from 2000 through May 2002, and measurements were conducted covering ionic and metal components with mass concentration. The results showed that daily averaged mass concentration (TSP) during the AD episodes was 458 $\mu\textrm{g}$/㎥, and ionic and metal concentrations were 27.93 $\mu\textrm{g}$/㎥ and 71.7 $\mu\textrm{g}$/㎥, respectively, accounting for 6.1 % and 15.5% of the total aerosol mass. TSP concentrations during episodes were varied from 120 to 1742 $\mu\textrm{g}$/㎥ according to the impact of Asian Dusts and had a tendency of showing higher values at sites in the west side of Korea, which can be explained by the effect of diffusion and deposition. In this study, ionic components like Ca (NO$_3$)$_2$, CaSO$_4$, NaNO$_3$, Na$_2$SO$_4$ were prominent types in secondary aerosol during AD periods and also indicated that V, Co as well as soil elements such as Ca, Fe, Mg, Mn, K correlated well with Al, while Cu, Cd, Pb, Zn didn't agree well with it. In addition, enrichment factors (EFs) for each metal component were obtained to provide simple information about source contribution of Asian Dust, and the results were compared with those from other AD studies. In this study, the results showed that aerosol properties in Korea during the Asian Dust were considerably different from those of general atmospheric condition and specially varied from case to case rather than site to site, which implies that there are certain variations in the soil of source region, pathways of air mass, and meteorological condition. For the enhanced study, those factors should be combined with the features of Asian Dust resolved from this study.

Synthesis and Spectroscopic Characterization of Manganese(II), Iron(III) and Cobalt(III) Complexes of Macrocyclic Ligand. Potential of Cobalt(III) Complex in Biological Activity

  • El-Tabl, Abdou S.;Shakdofa, Mohamad M.E.;El-Seidy, Ahmed M.A.
    • 대한화학회지
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    • 제55권6호
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    • pp.919-925
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    • 2011
  • A new series of manganese(II), iron(III) and cobalt(III) complexes of 14-membered macrocyclic ligand, (3,6,10,13,16,19-hexaazabicyclo[6.6.6]icosane-1,8-diamine) have been prepared and characterized by elemental analyses, IR, UV-VIS, $^1H$- and $^{13}C$- NMR spectra, magnetic susceptibilities, conductivities, and ESR measurements. Molar conductance measurements in DMF solution indicate that the complexes are electrolytes. The ESR spectrum for cobalt(III) complex in $CD_3OD+10%D_2O$ after exposure to $^{60}Co-{\gamma}$-rays at 77 K using a 0.2217 M rad $h^{-1}$ vicrad source showed $g_{\perp}$ > $g_{\parallel}$ > $g_e$, indicating that, the unpaired electron site is mainly present in the $d_z2$ orbital with covalent bond character. In this case, the ligand hyperfine tensors are nearly collinear with ${\gamma}$-tensors, so there is no major tendency to bend. Therefore, little extra delocalization via the ring lobe of the $dz^2$ orbital occurs. However, the ESR spectrum in solid state after exposure to $^{60}Co-{\gamma}$-rays at 77 K showed $g_{\parallel}$ > $g_{\perp}$ > $g_e$, indicating that, the unpaired electron site is mainly present in the $d_x2_{-y}2$ ground state as the resulting spectrum contains a large number of randomly oriented molecules provided that, the principle directions of g and A tensors. Manganese (II) complex 2, $[H_{12}LMn]Cl_4.2H_2O$, showed six isotropic lines characteristic to an unpaired electron interacting with a nucleus of spin 5/2, however, iron(III) complex 3, $[H_{12}LFe]Cl_5.H_2O$, showed spectrum of a high spin $^{57}Fe$ (I=1/2), $d^5$ configuration. The geometry of these complexes was supported by elemental analyses, IR, electronic and ESR spectral studies. Complex 1 showed exploitation in reducing the amount of electron adducts formed in DNA during irradiation with low radiation products.

마그네트론 스퍼터링에 의해 phosphorous 도핑된 ZnO 박막의 전기적, 광학적, 구조적 특성의 연관성 (Correlationship of the electrical, optical and structural properties of P-doped ZnO films grown by magnetron sputtering)

  • 안철현;김영이;강시우;공보현;한윈석;조형균
    • 한국전기전자재료학회:학술대회논문집
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    • 한국전기전자재료학회 2007년도 하계학술대회 논문집 Vol.8
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    • pp.177-177
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    • 2007
  • ZnO는 3.36eV의 넓은 밴드캡을 가지는 II-IV족 반도체로써 태양전지, LED와 같은 광학적 소자로 이용이 기대가 되는 물질이다. 더욱이, 상온에서의 60meV에 해당하는 큰 엑시톤 에너지와 밴드캡 에지니어링이 가능하다는 장점 때문에 광학적 소자로 널리 이용되고 있는 GaN을 대체할 수 있는 물질로 주목을 받고 있다. 하지만, p-type ZnO는 형성이 어렵고 낮은 이동도와 케리어 농도의 특성을 보이고, 대기 중에 장시간 노출할 경우 n-type ZnO의 특성으로 돌아가는 불안정성을 보이고 있다. 최근에 몇몇의 연구자들에 의해 V족의 원소인 P(phosphorous), N(nitrogen), As(arsenic))를 도핑하여 p-type ZnO의 형성에 대한 논문이 발표되고 있다. 또한, V족 원소 중에 P는 p-type ZnO 형성에 효과적인 도핑 물질로 보고되 고 있다. 본 연구는 마그네트론 스퍼터링을 이용하여 다양한 온도에서 성장된 P도핑 ZnO 박막의 특성에 대해 연구하였다. P도핑된 ZnO 박막은 사파이어 기판에 buffer층을 사용한 Insulator 특성의 ZnO박막위에 400, 500, 600, $700^{\circ}C$에서 성장되 었다. 박막의 특성 분석에는 325nm의 파장을 가지는 He-Cd의 레이져 광원을 사용하여 10K의 저온 PL과 0.5T의 자기장을 사용한 van der Pauw configuration에 의한 Hall effect측정, 그리고 결정성 분석에는 XRD와 TEM을 이용하였다. 상온 Hall-effect 측정 결과, $400{\sim}600^{\circ}C$ 에서 성장된 박막은 n-type의 특성을 보였고, $700^{\circ}C$에서 성장된 Phosphorous 도핑 ZnO박막은 $1.19{\times}10^{17}$의 캐리어 농도를 가지는 p-type의 특성을 보였다. 그리고 XRD분석과 TEM분석을 통하여 박막의 성장온도가 증가 할수록 P도핑된 ZnO박막의 결정성이 향상되는 것을 알 수 있었다. 또한 10K의 저온 PL분석을 통해 p도핑에 의한 액셉터에 관련된 피크들을 관찰할 수 있었다.

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Mucosal Immunity Related to FOXP3+ Regulatory T Cells, Th17 Cells and Cytokines in Pediatric Inflammatory Bowel Disease

  • Cho, Jinhee;Kim, Sorina;Yang, Da Hee;Lee, Juyeon;Park, Kyeong Won;Go, Junyong;Hyun, Chang-Lim;Jee, Youngheun;Kang, Ki Soo
    • Journal of Korean Medical Science
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    • 제33권52호
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    • pp.336.1-336.12
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    • 2018
  • Background: We aimed to investigate mucosal immunity related to forkhead box P3 ($FOXP3^+$) regulatory T (Treg) cells, T helper 17 (Th17) cells and cytokines in pediatric inflammatory bowel disease (IBD). Methods: Mucosal tissues from terminal ileum and colon and serum samples were collected from twelve children with IBD and seven control children. Immunohistochemical staining was done using anti-human FOXP3 and anti-$ROR{\gamma}t$ antibodies. Serum levels of cytokines were analyzed using a multiplex assay covering interleukin $(IL)-1{\beta}$, IL-4, IL-6, IL-10, IL-17A/F, IL-21, IL-22, IL-23, IL-25, IL-31, IL-33, interferon $(IFN)-{\gamma}$, soluble CD40L, and tumor necrosis factor-${\alpha}$. Results: $FOXP3^+$ Treg cells in the lamina propria (LP) of terminal ileum of patients with Crohn's disease were significantly (P < 0.05) higher than those in the healthy controls. $ROR{\gamma}t^+$ T cells of terminal ileum tended to be higher in Crohn's disease than those in the control. In the multiplex assay, serum concentrations (pg/mL) of IL-4 ($9.6{\pm}1.5$ vs. $12.7{\pm}3.0$), IL-21 ($14.9{\pm}1.5$ vs. $26.4{\pm}9.1$), IL-33 ($14.3{\pm}0.9$ vs. $19.1{\pm}5.3$), and $IFN-{\gamma}$ ($15.2{\pm}5.9$ vs. $50.2{\pm}42.4$) were significantly lower in Crohn's disease than those in the control group. However, serum concentration of IL-6 ($119.1{\pm}79.6$ vs. $52.9{\pm}39.1$) was higher in Crohn's disease than that in the control. Serum concentrations of IL-17A ($64.2{\pm}17.2$ vs. $28.3{\pm}10.0$) and IL-22 ($37.5{\pm}8.8$ vs. $27.2{\pm}3.7$) were significantly higher in ulcerative colitis than those in Crohn's disease. Conclusion: Mucosal immunity analysis showed increased $FOXP3^+$ T reg cells in the LP with Crohn's disease while Th17 cell polarizing and signature cytokines were decreased in the serum samples of Crohn's disease but increased in ulcerative colitis.

이식신 계획생검 및 재생검에서 Kidney Injury Molecule-1 표현과 이식신 기능 변화 (Changes of Kidney Injury Molecule-1 Expression and Renal Allograft Function in Protocol and for Cause Renal Allograft Biopsy)

  • 김연희;이아란;김명수;주동진;김범석;허규하;김순일;김유선;정현주
    • 대한이식학회지
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    • 제28권3호
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    • pp.135-143
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    • 2014
  • Background: Kidney injury molecule-1 (KIM-1) is known as a good ancillary marker of acute kidney injury (AKI) and its expression has also been observed in acute rejection and chronic graft dysfunction. We tested usefulness of KIM-1 as an indicator of acute and chronic renal graft injury by correlating KIM-1 expression with renal graft function and histology. Methods: A total of 133 zero-time biopsies and 42 follow-up biopsies obtained within 1 year posttransplantation were selected. Renal tubular KIM-1 staining was graded semiquantitatively from 0 to 3 and the extent of staining was expressed as the ratio of KIM-1 positive/CD10 positive proximal tubules using Image J program. Results: KIM-1 was positive in 39.8% of zero-time biopsies. KIM-1 positive cases were predominantly male and had received grafts from donors with older age, deceased donors, and poor renal function at the time of donation, compared with KIM-1 negative cases. KIM-1 expression showed correlation with delayed graft function and acute tubular necrosis. In comparison of KIM-1 expression between stable grafts (n=23) and grafts with dysfunction (n=19) at the time of repeated biopsy, the intensity/extent of KIM-1 staining and renal histology at zero-time did not differ significantly between the two groups. Histologically, KIM-1 expression was significantly increased with both acute and chronic changes of glomeruli, tubules and interstitium, peritubular capillaritis, and arteriolar hyalinosis. Conclusions: KIM-1 can be used as an ancillary marker of AKI and a nonspecific indicator of acute inflammation and tubulointerstitial fibrosis. However, KIM-1 expression at zero-time is not suitable for prediction of long-term graft dysfunction.