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- 저자 : 홍수종 ( Soo Jo) (검색결과 3 건)
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난치성 아토피피부염 Work Group 보고서 난치성 아토피피부염의 치료에 관한 전문가 의견서
박준수 ( Joon Soo Park ),김범준 ( Beom Joon Kim ),박양 ( Yang Park ),이소연 ( So Yeon Lee ),김우경 ( Woo Kyung Kim ),김정은 ( Jeong Eun Kim ),염혜영 ( Hye Yung Yum ),남동호 ( Dong Ho Nahm ),김현희 ( Hyun Hee Kim ),홍수종 ( Soo Jo 대한천식알레르기학회 2010 천식 및 알레르기 Vol.30 No.4
Background: Management of severe/recalcitrant atopic dermatitis (SRAD) is a difficult task for both patients and clinicians. Method: We developed this report to provide experts` opinion on the treatment of SRAD by a literature review and repeated group discussions made at the Work Group on Severe Recalcitrant Atopic Dermatitis in the Korean Academy of Asthma, Allergy and Clinical Immunology. Result: Specific aggravating factors, including inhalant allergens or food allergens, should be identified by laboratory tests in patients with SRAD. Patients should be educated about avoidance measures for specific aggravating factors, moisturization of the skin, and control of skin inflammation using low-potency topical corticosteroids and/or topical calcineurin inhibitors. For patients with SRAD who cannot be properly controlled by the above treatments, systemic immunoregulatory treatment such as cyclosporin could be effective. Allergen-specific immunotherapy has been attempted as a useful therapeutic option in selected patients with SRAD sensitized to aeroallergens. Psychological counseling and educational programs should be recommended for the management of patients with SRAD. Conclusion: Therapeutic approaches aimed at personalized medicine including stepwise treatment according to clinical severity, combination therapy, proactive therapy and multi-disciplinary approach should be tried to achieve the best outcome for SRAD. (Korean J Asthma Allergy Clin Immunol 2010;30:255-270)
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국내 X-관련성 범저감마글로불린혈증 세가족에 대한 Bruton's Tyrosine Kinase 단백질 발현 및 유전자 변이 분석
송창화,조은경,박정규,김정수,홍수종,이재호,Song, Chang-Hwa,Jo, Eun-Kyeong,Park, Jeong-Kyu,Kim, Jung-Soo,Hong, Soo-Jong,Lee, Jae-Ho 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.3
목 적: 본 연구에서는 임상적으로 XLA로 진단받고 현재 치료 중인 국내 환아 세가족의 네명의 환아와 모친 등을 대상으로 말초혈액 단핵구의 Btk 단백질 발현과 Btk 유전자 변이를 분석하고자 하였다. 방 법: 말초혈액 단핵구의 Btk 발현도를 항 Btk 항체를 이용한 유세포측정을 통해 분석하고 직접 염기서열 분석에 의해 Btk 유전자 변이를 분석하였다. 결 과 : 환아들의 B 림프구의 발현은 2.1% 미만으로 정상인에 비해 매우 저하되어 있었으며 유세포 측정에 의한 환아 단핵구 유래 Btk 단백질 발현도 1.0% 이하로 정상인에 비해 매우 감소되었다. 유전자 변이 분석 결과 XLA 가족 1과 3에서는 각각 intron 18과 intron 1의 짜집기 공여 위치 부위에서 1개의 점 돌연변이가 발견되었으며 cDNA상 짜집기 오류 현상을 야기하였다. 그리고 XLA 가족 2의 경우 exon 10을 포함하는 980 bp의 유전자 결손(intron 9+191T부터 intron 10-215C까지)이 발견되었으며 세계적으로 처음 보고되는 새로운 유전자 변이였다. 또한 가족 2의 경우 가족 중 환아에서 만 Btk 단백질 결핍 및 유전자 변이를 진단하여 국내 XLA의 환아 중 최초의 산발적인 발병 양상을 확인하였다. 결 론: 본 연구 결과를 종합해 볼 때 임상적으로 XLA로 진단된 환아와 가족에 대한 항 Btk 항체를 이용한 유세포측정 방법은 XLA 환아 및 보인자의 진단에 매우 유용한 방법으로 생각된다. 또한 분자유전학 기법을 이용하여 Btk의 noncoding region을 포함한 광범위한 유전자 영역의 염기서열 분석을 시행한 결과 새로운 1개의 유전자 결손 및 2개의 점돌연변이에 의한 짜집기 오류를 확인하여 XLA를 최종 확진할 수 있었다. Purpose : X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells(PBMC) from three XLA families in Korea. Methods : Heparinized venous blood samples were collected from four XLA patients and additional family members in three unrelated XLA families. Mononuclear cells were separated from their blood and the intracellular Btk protein was characterized by a flow cytometry. The mutation analysis was performed using direct sequencing. Results : Cytoplasmic expression of Btk protein in monocytes was not detected in the patients with XLA. We observed a novel deletion and two point mutations within introns(intron 1 and intron 18) resulting in alternative splicings. In XLA family 2, a 980 bp deletion(from intron 9+191 T to intron 10-215 C) including exon 10 was found in patient P2. He was the only sporadic case in this study, because his mother and brother showed a normal Btk expression by flow cytometry. Conclusion : These identified genetic alterations support the molecular heterogeneity of Btk gene in XLA disease. Additionally, by means of flow cytometric analysis, we diagnosed three hypogammaglobulinemia patients as XLA. Advancements in diagnostic methods has facilitated a prompt and definite diagnosis of this disease.
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NC/Nga 마우스에서 Dermatophagoides farinae의 피부 적용과 기도 유발에 의해 발생한 기도 염증에 대한 CpG Oligodeoxynucleotide의 효과
김영준 ( Young Joon Kim ),장성옥 ( Seong Ok Jang ),최원아 ( Won Ah Choi ),강미진 ( Mi Jin Kang ),정현돈 ( Hyun Don Jung ),김하정 ( Ha Jung Kim ),김형영 ( Hyung Young Kim ),서주희 ( Ju Hee Seo ),유진호 ( Jin Ho Yu ),홍수종 ( Soo Jo 대한천식알레르기학회 2011 천식 및 알레르기 Vol.31 No.2
Background: Atopic dermatitis (AD) is the initial step of allergic march that progresses to airway allergic disease. There has been much interest in the interruption of the allergic march, but the results are not promising. CpG oligodeoxynucleotide (ODN) has been reported to have both preventive and therapeutic effects on the development of asthma in mouse models. Objective: To identify the development of airway inflammation after having atopic dermatitis-like skin lesions induced by topical application of Dermatophagoides farinae (Df), and to evaluate the preventive effect of CpG ODN on the development of airway inflammation. Method: We injected 5 μg of Df intradermally 8 times for 2 weeks into 8-week-old NC/Nga mice and then challenged them with 3 times of Df intranasally. CpG ODN with or without Df was injected subcutaneously before airway challenge. Airway hyperreactivity was determined by the change inenhanced pause (Penh) during methacholine provocation. Lung tissue and bronchoalveolar lavage specimens were evaluated. Result: Topical Df application induced AD-like skin lesion microscopically, but not macroscopically. NC/Nga mice with topical Df application developed more severe airway inflammation upon intranasal Df challenge than those with topical PBS application. However, the mice with CpG ODN injection before airway challenge showed reduced airway inflammation. The levels of Df-specific IgG1 were increased in the mice with both topical Df application and intranasal Df challenge but were decreased in the mice with CpG ODN injection. Conclusion: These results suggest that CpG may inhibit the development of airway inflammation from those with previous AD-like skin lesions induced by Df. (Korean J Asthma Allergy Clin Immunol 2011;31:131-139)
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