소아 중환자실에 입원한 혈액-종양 환아에서 발생한 폐 합병증의 원인과 예후에 대한 분석

정진영,홍수종,안영준,김자형,서종진,문형남,김태형,Jung, Jin Young,Hong, Soo-Jong,An, Young Jun,Kim, Ja Hyung,Seo, Jong Jin,Moon, Hyung Nam,Ghim, Thad 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.8

Purpose : In the course of treatment, patients with hematological or oncological disorders often develop pulmonary complication. The patients who develop a severe pulmonary complication have a poor outlook. The causes of pulmonary complication are either infectious or non-infectious in origin. We have analyzed the etiology and outcome of these patients admitted to the pediatric intensive care unit of Asan Medical Center. Methods : Medical records of 95 patients on Pediatric oncology service who were admitted to pediatric intensive care unit(PICU) of Asan Medical Center from Jan 1997 to May 2000 were retrospectively reviewed. Results : The mean age of the patients was 8.5 years(2 months-18 years). The underlying malignancies of these 95 patients were as following; acute lymphoblastic leukemia(31 cases), lymphoma (11 cases), acute myeloid leukemia(nine cases), brain tumor(eight cases) and other solid tumors(25 cases). Pulmonary complications included pneumonia, acute respiratory failure, pneumothorax and pleural effusion. The most common cause of pulmonary complication was infection(88%) in etiology. The overall mortality rate was 56.8%. Pulmonary complications in these patients carried high rates of mortality regardless of whether they were immune compromised(76%) or not(69%). Even without pulmonary complications, the hematological or oncological patients admitted to PICU had high mortality rates of 43%. Conclusion : Pulmonary complications are frequent finding in the hematological or oncological patients admitted to Intensive Care Unit. The main etiology of these pulmonary complications was infection, which carried a high mortality rate regardless of their immune status at the time when they were admitted to PICU. 목 적 : 혈액-종양 환자에서 항암화학요법으로 치료하는 과정 중에서 중증 폐 질환 합병증이 발생할 수 있다. 그 원인으로는 감염성 또는 비 감염성의 원인들이 있고 특히 이들 환자에서 폐 질환 합병증 또는 호흡부전으로 인한 인공환기 치료시에는 예후가 나쁘다고 알려져 있다. 저자들은 본원 소아 중환자실에 입원한 혈액-종양 환아에서 면역 저하상태(절대 호중구수 < $1,000/mm^3$)의 지표의 하나인 호중구 감소증과 폐합병증의 유무에 따른 원인과 예후를 분석하였다. 방 법: 1997년 1월부터 2001년 5월까지 서울중앙병원 소아 중환자실에 입원한 혈액-종양 환아 95명의 의무기록을 후향적으로 분석하여 폐 합병증의 원인과 진단 방법, 호중구 감소상태와 폐 합병증의 유무에 따른 사망률을 분석하였다. 결 과 : 연령 분포는 생후 2개월에서 18세까지 평균 8.5세였고, 원인 혈액-종양 질환으로는 급성 림프성 백혈병 31명, 급성 골수성 백혈병 9명, 림프선 종양 11명, 뇌종양 8명, 고형종양 25명, 그 외 혈액질환이 11명이었다. 95명의 환아 중 중환자실 입원당시의 원인으로는 패혈증 23명(24%), 패혈증을 동반한 폐렴 22명(23%), 급성 호흡부전 12명(12.5%), 의식소실 16명(17%), 급성 신부전, 심부전의 기타 질환이 15명(16%)이었다. 이들 중에서 폐 합병증을 동반한 환아는 42명(44%), 동반하지 않은 환아는 53명(56%)이었다. 폐 합병증으로는 폐렴, 늑막삼출, 급성호흡부전, 기흉이 있었다. 폐 합병증이 있는 환아 중 호중구 감소상태의 환아는 29명(69%), 호중구 감소상태가 아닌 환아는 13명(31%)이었다. 원인으로는 감염성인 경우가 37명(88%), 비 감염성인 경우가 2명(5%)이었고, 구분이 어려운 경우가 3명(7%)이었다. 시행된 각각의 검체에서 균이 검출된 빈도를 비교하면 혈액배양검사는 42명중 15명(36%), 객담검사는 42명 중 7명(16%), 소변 배양검사는 42명 중 3명(7%), 인공환기요법 중인 환아에서 시행한 기도내 분비물 배양검사는 28명 중 5명(18%)이었다. 주요 원인균으로는 Candida species 12명(33%), MRSA 6명(17%), Pseudomonas aeruginosa 4명(11%), Klebsiella pneumoniae 4명(11%)이었다. 골수이식술을 받은 환자는 모두 10명으로 이중 7명에서 폐 합병증이 있었고, 원인균이 동정된 경우는 모두 3명으로 각각 S.maltophilia, K.pneumoniae, Parainfluenza virus이었다. 10명의 환아 중 8명이 사망하였고 사망 원인으로는 폐혈증이 5명, 급성호흡부전 증후군이 2명, 뇌출혈이 1명이었다. 전체 사망률은 56.8%였고, 사망률을 보면 이중 폐 합병증이 있고 호중구 감소상태의 환아는 76%(22명), 폐 합병증이 있으나 호중구 감소상태가 아닌 환아는 69% (9명), 폐 합병증이 없으나 호중구 감소상태의 환아는 46%(7명), 폐 합병증이 없으며 호중구 감소상태가 아닌 환아는 43%(16명)이었다. 결 론 : 중환자실에 입원한 혈액-종양 환아에서 폐 합병증의 원인으로 감염성인 경우가 많았다. 사망률은 폐 합병증이 있는 경우가 없는 경우에 비해 높았으며, 특히 폐 합병증이 있는 환아 중 호중구 감소상태의 환아에서 사망률이 높았다. 이 결과로 미루어 폐 합병증이 있는 혈액-종양 환자에서 더 적극적인 원인 규명과 치료를 시도해야 할 것으로 생각된다.

집먼지진드기 항원을 이용한 고식적인 면역요법이 말초혈액 호염기구 히스타민 유리능에 미치는 영향

심정연(Jung Yeon Shim),김봉성(Bong Seong Kim),조상헌(Sang Heon Cho),민경업(Kyung Up Min),홍수종(Soo Jong Hong) 대한천식알레르기학회 2000 천식 및 알레르기 Vol.20 No.5

N/A Background: Allergen-specific immunotherapy has been shown to be clinically effective in the treatment of patients with atopic asthma, but the mechanisms are still unclear. Some of the immunologic changes include increase of an allergen-specific IgG antibody, decrease of allergen-specific IgE after transient increase, allergen-specific T-cell shift in cytokine expression from Th2 to Th1 cytokines, and decrease of basophil histamine releasability. Objectives : To investigate the influence of immunotherapy on basophil releasability, we examined the changes of IgE-mediated and non-IgE-mediated basophil histamine releasability during immunotherapy. Methods : Fourteen Dermatophagoides farinae(D.f) sensitive asthmatic children with conventional immunotherapy were investigated. Basophil histamine releasability was measured prior to immunotherapy and 4 and 9 months after immunotherapy. Basophils were stimulated with D.f and goat antihuman IgE antibody as IgE-mediated stimuli which act on IgE-receptor, and formyl-Met-Leu-Phe(fMLP) as non-IgE-mediated stimuli which acts on non-IgE receptor, and calcium ionophore A23187 as non-IgE-mediated stimuli which does not act on cell surface receptors, Histamine was measured by automated fluorometric technique. Results : Before immunotherapy. there were significant correlations between histamine release by D.f and histamine release by fMLP, and between histamine release by D.f and histamine release by anti-IgE antibody, but no correlation between histamine release by D.f and histamine release by calcium ionophore. Histamine release by D.f and by anti-IgE antibody decreased at 4 and 9 months after immunotherapy compared to those before immunotherapy. Histamine release by fMLP and by calcium ionophore showed no significant changes after immunotherapy. There was no significant change of total histamine release after immunotherapy. Conclusion : Conventional immunotherapy has influenced only the IgE-mediated basophil releasability. IgE-mediated and non-IgE-receptor-mediated basophil releasability was correlated before immunotherapy, but only IgE-receptor-mediated basophil releasability decreased after immunotherapy. These findings suggest that a kind of physicochemical change may happen on the IgE receptors of basophil, which may induce decrease of IgE-mediated basophil histamine releasability after immunotherpy. (J Asthma Allergy Clin Immunol 20: 731-740, 2000)

2`7`-dichlorofluoroscein diacetate 를 사용하여 유세포분석법으로 진단된 반성유전 만성육아종성 질환 2 례

이정주(Jung Joo Lee),심정연(Jung Yeon Shim),홍수종(Soo Jong Hong),박찬정(Chan Jeoung Park),지현숙(Hyun Sook Chi) 대한소아알레르기호흡기학회 1997 소아알레르기 및 호흡기학회지 Vol.7 No.1

Chronic granulomatous disease is a group of rare X-linked or autosomal recessive genetic disorders due to the NADPH oxidase defect, which causes the defect in neutrophil respiratory burst function, the recurrent bacterial and fungal infection, and the granulomatous lesion. This disease can be diagnosed by measuring the decreased neutrophil respiratory burst activity in patient who shows X-linked recessive or autosomal recessive inheritance and the recurrent infection. To measure the neutrophil respiratory burst activity, NBT(nitroblue tetrazolium dye) test has been used. Recently, it has become possible to diagnose the chronic granulomatous disease patient as well as the carrier simply and rapidly with the flow cytometry which measures hydrogen peroxide within the neutrophil, using 2 7 dichlorofluorescein diacetate(DCF-DA). We diagnosed two boys with history of recurrent infections and their carrier mothers as X-linked chronic granulomatous disease by flow cytometry using DCF-DA, and could follow up neutrophil respiratory burst activity after the administration of interferon gamma(INF-γ) to these patients.

Audible Doppler Ultrasound(8 MHz)를 이용한 소아와 영아에서 중심정맥 도관 삽입술에 관한 연구

안영준,정진영,홍수종,Ahn, Young Joon,Jung, Jin Young,Hong, Soo-Jong 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.9

Purpose : Percutaneous cannulation of the central vein in children and infants may be technically difficult and can cause serious complications. There are many techniques to improve the success rate and to decrease the complications in central vein cannulation. This study was undertaken to determine whether audible Doppler guidance can help operators improve the results of central vein cannultion in pediatric ICU. Methods : A total of 46 central vein cannulations were performed using audible Doppler guidance technique. Using an 8-MHz Doppler ultrasound device(Pocket-Dop II. iMex. USA), the artery and the vein were identified by their characteristic Doppler sounds. Results : There was a total of 46 patients with 20 boys and 26 girls, respectively. The mean age was $3years{\pm}45months$(range : 1-156 months). The number of infants was 18 and the mean age was $6.3{\pm}3.8$ months(range : 1-12 months). The most common site of central vein cannulation was the right internal jugular vein(63%). The cannulation was successful in 44 out of 46 procedures(96%). The success rate at the first attempt was 59% with 48% in the patients who had the history of previous cannulation and 70% in the patients who did not, respectively. The success rate in infants was 94%(17 out of 18). The average access time was $5.2{\pm}8.3$ minutes in children contrary to $7.5{\pm}10.3$ minutes in infants. There were no serious complications, but six patients had complications including four hematoma, one arterial puncture, and one pneumothorax. Conclusion : This central vein cannulation using Doppler guidance may improve the success rate of central vein cannulation and decrease the development of serious complications in infants and children. 목 적: 소아에서 장기간의 정맥유지, 고단위 영양 수액투여, 혈액채취, 중심정맥압 측정 등을 위해 중심 정맥 도관 삽입술을 시행하고 있다. 그런데 소아는 경부의 해부학적 구조의 구별이 어려워 중심정맥에 도관 삽입이 상당히 어렵고, 여러 가지 심각한 합병증을 초래할 수가 있다. 저자들은 소아에서 audible Doppler ultrasound(8 MHz)를 이용하여 쉽게 중심정맥을 확인하여 카테타를 삽입할 수 있는지 알아보고자 하였다. 방 법: 2001년 4월에서 7월까지 서울아산병원 소아과에 입원한 환아 중 중심정맥 삽입술을 시행한 46명을 대상으로 하였다. 휴대용, 8 MHz Doppler ultrasound device(Pocket-Dop II, iMex, USA)를 이용하여 중심정맥 삽입술을 시행하였고, 이들 환아에서 삽입술의 성공률과 천자시간, 천자횟수, 합병증 등을 조사하였다. 결 과 : 1) 대상 환아는 46례로 남아 20례, 여아 26례였고 평균연령은 3세(범위 1-156개월)였으며, 특히 영아는 18명이었으며 평균연령은 6.3개월이었다. 2) 중심정맥에 도관을 삽입한 가장 흔한 부위는 우측내경정맥(63%)이며, 다음으로 좌측내경정맥, 대퇴정맥, 쇄골하정맥 순이었다. 3) 중심정맥 도관 삽입술의 성공률은 96%(46례 중 44례)였으며, 소아는 96%였고, 영아는 94%였다. 4) 평균 천자시간은 5.2분이었고, 소아는 3.7분, 영아는 7.5분 소요되었다. 5) 46례 중 6례에서 합병증이 발생했으며, 혈종 4례, 동맥천자 1례, 기흉 1례 등이었다. 6) 1회 시도 성공률은 전체 환아는 59%였으며, 소아는 71%, 영아는 39%였다. 중심정맥 삽입 과거력이 없을 경우 전체 환아의 1회 시도 성공률은 70%였고, 있을 경우는 48%였다. 결 론 : 소아나 영아에서 휴대용, 8 MHz Doppler ultrasound device를 이용하여 쉽게 중심정맥의 위치를 확인하여 중심정맥 도관 삽입술의 성공률을 높이고, 심각한 합병증을 줄일 수 있어서, audible Doppler ultrasound를 이용하여 중심정맥 도관 삽입술을 시행하는 것이 유용할 것으로 생각한다.

한국 어린이의 혈중 납 노출 경로

김은정(Eun-Jung Kim),권호장(Ho-Jang Kwon),하미나(Mina Ha),홍윤철(Yun-Chul Hong),임종한(Jong-Han Leem),사공준(Joon Sakong),김수영(Su-Young Kim),이철갑(Chul-Gab Lee),강동묵(Dongmug Kang),홍수종(Soo-Jong Hong) 한국환경독성학회 2011 한국독성학회 심포지움 및 학술발표회 Vol.2011 No.10

정읍시골, 정읍시, 서울시에서의 실내 항원감작과 인구 통계학적 특징의 관련성

정현희 ( Hyun Hee Jung ),권지원 ( Ji Won Kwon ),이소연 ( So Yeon Lee ),서주희 ( Ju Hee Seo ),송영화 ( Young Hwa Song ),김병주 ( Byoung Ju Kim ),김효빈 ( Hyo Bin Kim ),유진호 ( Jin Ho Yu ),홍수종 ( Soo Jong Hong ),박강서 ( Kang Seo 대한천식알레르기학회 2010 천식 및 알레르기 Vol.30 No.4

Background: The prevalence of allergic disease is increasing across the world. The development of allergic disease is associated with allergen sensitization. Objective: We investigated regional differences of allergen sensitization and relations between demographic characteristics and allergen sensitization among Jeongeup Countyside, Jeongeup City and Seoul City in Korea. Method: We conducted a cross-sectional survey of 1,792 children aged 9∼12 years from Jeongeup Countyside, Jeongeup City and Seoul City in Korea. A questionnaire and skin prick test for 16 common allergens were performed. Result: Participants from Jeongeup Countryside and City had more pets or livestock, siblings and breast feeding than those from Seoul City. A higher body mass index and a more frequent history of antibiotic therapy during infancy and parental allergy were found in Seoul City. The rate of sensitization to more than one allergens was higher in Seoul City than Jeongeup Countyside and City. The sensitization rate to cockroach was higher in Jeongeup Countyside and City than in Seoul City. The sensitization rate to Alternaria had a tendency to decrease with urbanization. The sensitization rate to house dust mite and cat were higher in Seoul City than in Jeongeup Countyside and City. Conclusion: The rate and type of allergen sensitization may differ among regions, which may be due to environmental or habitual factors. Therefore, allergen sensitization can be reduced by controlling these factors. (Korean J Asthma Allergy Clin Immunol 2010;30:277-284)

반복적인 객혈을 보인 영아에서 진단된 선천성 폐정맥 협착 1례

정창우 ( Chang Woo Jung ),이소연 ( So Yeon Lee ),유진호 ( Jin Ho Yu ),김병주 ( Byoung Joo Kim ),윤태진 ( Tae Jin Yun ),고재곤 ( Jae Kon Ko ),홍수종 ( Soo Jong Hong ) 대한소아알레르기 및 호흡기학회 2007 소아알레르기 및 호흡기학회지 Vol.17 No.4

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.

아토피 천식 환아 말초혈액 오염기구의 히스타민 유리능

심정연,변원철,홍정수,문희범,홍수종 ( Jung Yeon Shim,Won Chul Byun,Jeong Soo Hong,Hee Bom Moon,Soo Jong Hong ) 대한천식알레르기학회 1998 천식 및 알레르기 Vol.18 No.3

Objective .' To evaluate the basophil histamine releasability in response to IgE- and non- IgE-mediated stimuli in children with atopic asthma. Met: Basophil histamine releasability was measured in Dermatophagoides farinae (D. farinae) -sensitive atopic asthmatics, D.farinae -sensitive healthy atopics, non-atopic asthmatics, and healthy non-atopics. Basophils were stimulated with D.farinae, goat antihuman IgE antibody, formyl-Met-Leu-Phe(fMLP), and Calcium ionophore A23187. Histamine was measured by automated fluorometric technique. Results '. Sponianeous histamine release was higher in atopic asthmatics compared to healthy non-atopics. Histamine release by D.farinae and by anti-IgE antibody was higher in atopic asthmatics compared to the other groups. There was no difference in histamine release by fMLP among all groups. Histamine release by Calcium ionophore was higher in healthy atopics and non-atopic asthmatics compared to healthy non-atopics. The atopics showed correlation between histamine release by D.farinae, by anti-IgE antibody and total serum IgE levels. Conclusions '. Spontaneous and IgE-mediated histamine release were related to the presence of both atopy and asthma, whereas non-IgE mediated histamine release was different depending on the stimuli.

결핵성 흉막염 치료 중 발생한 역설적 반응

정명기 ( Myung Ki Jung ),이혜진 ( Hye Jeen Lee ),김미진 ( Me Jin Kim ),김영호 ( Young Ho Kim ),양승 ( Seung Yang ),황일태 ( Il Tae Whang ),김병주 ( Byoung Ju Kim ),홍수종 ( Soo Jong Hong ),이혜란 ( Hae Ran Lee ) 대한소아알레르기호흡기학회(구 대한소아알레르기 및 호흡기학회) 2009 소아알레르기 및 호흡기학회지 Vol.19 No.1

적절한 항결핵제로 치료하여 호전을 보이던 결핵 환자에서 치료 도중, 이전에 존재하던 결핵병변이 악화되거나 또는 새로운 병변이 발생하는 경우가 있는데 이를 항결핵제에 대한 `역설적 반응`이라 한다. 14세 여자 환자의 결핵성 흉막염 치료 중 역설적 반응이 발생하여 6주간의 스테로이드 투여와 지속적인 항결핵제 투여로 호전을 보였기에 관련문헌 고찰과 함께 보고하는 바이다. It is defined as the paradoxical response when the clinical or radiologic worsening of old lesions or the development of new lesion occur in spite of appropriate antituberculous therapy. The paradoxical response can occur as an intracranial tuberculoma, pleurisy, pericarditis and contralateral new parenchymal lesions. However, poor compliance with therapy, drug resistance, non-tuberculous mycobacterium, or another underlying condition as lung cancer should be ruled out before concluding that the treatment is the cause of the exacerbation. The case reports of paradoxical response have been mainly reported in adults, but extremely rare in children. We report a case of paradoxical response in which a new parenchymal lung lesion developed during antituberculous therapy in a 14-year-old female patient with tuberculous pleurisy. She experienced clinical improvement with steroid therapy in addition to antituberculous therapy. [Pediatr Allergy Respir Dis(Korea) 2009;19:71-77]