A1 Segment Hypoplasia / aplasia Detected by Magnetic Resonance Angiography in Neuropediatric Patients

한윤기(Yoon Ki Han),김승(Seung Kim),윤춘식(Choon Sik Yoon),이영목(Young Mock Lee),강훈철(Hoon Chul Kang),이준수(Joon Soo Lee),김흥동(Heung Dong Kim) 대한소아신경학회 2011 대한소아신경학회지 Vol.19 No.3

목적 : 대뇌동맥륜의 변이는 흔하지 않지만, 그 중 가장 많이 관찰되는 유형은 전대뇌동맥 첫번째 분지(A1 segment)의 형성저하/무형성이다. 저자들은 소아신경 환아에서 전대뇌동맥 첫번째 분지의 형성저하/무형성의 발생빈도와 임상적 의의에 대해 연구하였다. 방법 : 뇌혈관 자기공명 조영술을 시행한 환아들 중 전대뇌동맥 첫번째 분지의 형성저하/무형성을 보인 환아들을 대상으로 후향적 연구를 하였으며 단독으로 전대뇌동맥 첫번째 분지의 형성저하/무형성의 변이를 보인 환아들과 다른 대뇌동맥륜의 변이를 동반한 환아들의 임상 및 영상의학적 양상을 비교 분석하였다. 결과 : 뇌혈관 자기공명 조영술을 시행한 301명의 환아들 중 34명(11.3%)이 전대뇌동맥 첫번째 분지의 형성저하/무형성의 소견을 보였다. 환아들은 만성 두통, 어지러움, 시각장애 같은 신경학적 증상을 보였는데, 34명 중 7명(20.6%)은 신경학적 질환의 가족력을 가지고 있었다. 27명(79.4%)은 전대뇌동맥 첫번째 분지의 형성저하/무형성의 단독 변이를 보였고, 7명(20.6%)은 다른 혈관 이상을 동반하고 있었다. 또한 7명(20.6%)에서는 뇌 자기공명 영상에서 이상 소견을 보였고, 뇌위축 소견이 5명(14.7%)으로 가장 많았다. 단독 혈관 변이를 보인 환아중 뇌 자기공명 영상의 이상 소견을 보인 빈도는 11.1% (3/27)이었고 다른 혈관 이상을 동반한 환아에서의 빈도는 57.1% (4/7)로 통계학적으로 유의하였다. 결론 : 소아에서 뇌혈관의 구조적 변이는 병태생 리학적으로 또한 임상적으로 중요한 영향을 미칠 수있으므로 뇌혈관 자기공명 조영술을 이용하여 대뇌동맥륜의 변이들, 특히 전대뇌동맥 첫번째 분지의 형성저하/무형성에 대한 적극적인 평가가 필요할 것으로 판단된다. Purpose : A variation in the circle of Willis is not so common, but the most frequenttype is hypoplasia/aplasia of the precommunicating anterior cerebral arteries (A1 segment). We aimed to examine the incidence and the clinical significance of A1 segment hypoplasia/aplasia in neuropediatric patients. Methods : We retrospectively studied children with A1 segment hypoplasia/aplasia in brain magnetic resonance angiography (MRA) and compared the clinical and radiological aspects between children with A1 segment hypoplasia/aplasia alone and with other variations in the circle of Willis. Results : Among 301 patients, 34 patients (11.3%) had A1 segment hypoplasia/aplasia. They presented neurological symptoms such as chronic headache, dizziness and visual disturbance. Seven (20.6%) had family history of neurological illness. Twenty seven (79.4%) had A1 segment hypoplasia/aplasia only, and seven (20.6%) had another vascular abnormality. Seven (20.6%) showed abnormal brain magnetic resonance angiography (MRI) results, cerebral atrophy being the most frequent (n=5, 14.7%). The incidence of abnormal brain MRI was 11.1% (n=3) in single vascular abnormality and 57.1% (n=4), significantly higher (p-value 0.02) in combined abnormality group. Conclusion : Structural alterations in the cerebral vasculature in children have important pathophysiological and clinical implications. Evaluation of variations in the circle of Willis, especially of A1 segment hypoplasia/aplasia using MRA is recommended.

소아 천식 환아의 호기농축시료에서 염증매개물질 측정의 의의

한윤기 ( Yoon Ki Han ),박준영 ( Jun Young Park ),김미나 ( Mi Na Kim ),김윤희 ( Yoon Hee Kim ),박현빈 ( Hyun Bin Park ),김민정 ( Min Jung Kim ),이경은 ( Kyung Eun Lee ),이희선 ( Hee Seon Lee ),허원일 ( Won Il Heo ),홍정연 ( Jeong Y 대한천식알레르기학회 2014 Allergy Asthma & Respiratory Disease Vol.2 No.5

Purpose: There has recently been increasing interest in the use of exhaled breath condensate (EBC) as a simple noninvasive means for understanding the physiology of asthma. The aim of this study was to evaluate the levels of leukotriene B4 (LTB4) and eosinophil cationic protein (ECP) in the EBC of asthmatic children. Methods: We measured LTB4 and ECP levels in EBC from children aged 6-14 years, including healthy children (n=25) and asthmatic children (n=25). We also measured serum LTB4 and serum ECP. Pulmonary function tests and methacholine challenge tests were performed on all subjects. Results: Exhaled LTB4 levels were increased significantly in patients with asthma compared to normal subjects (7.1±3.7 pg/mL vs. 2.2±1.7 pg/mL, P<0.05). Serum LTB4 levels were not significantly different in patients with asthma compared to normal subjects (674.7±484.1 pg/mL vs. 487.1±272.0 pg/mL, P=0.156,) and no significant correlations were found between exhaled and serum LTB4 concentrations in children with asthma (r=0.052, P=0.758). Exhaled ECP levels were not significantly different in patients with asthma compared to normal subjects (P=0.419). Serum ECP levels were significantly increased in patients with asthma compared to normal subjects (44.37±32.14 μg/L vs. 16.40±13.23 μg/L, P=0.001). Conclusion: We found significantly elevated LTB4 levels in the EBC of asthmatic children. Our results suggest that EBC may be one of the supportive tools to measure airway inflammation in children with asthma.(Allergy Asthma Respir Dis 2014;2:332-336)

일산화질소 흡입요법에 반응하지 않는 중증 신생아 지속성 폐동맥 고혈압증 환자에서 분무식 iloprost (Ventavis(R))의 치료효과

한윤기 ( Yoon Ki Han ),이순민 ( Soon Min Lee ),은호선 ( Ho Seon Eun ),김정은 ( Jeong Eun Kim ),남궁란 ( Ran Namgung ),박민수 ( Min Soo Park ),박국인 ( Kook In Park ),이철 ( Chul Lee ) 대한주산의학회 2011 Perinatology Vol.22 No.1

Inhaled iloprost, a stable carbacyline derivative of prostacyclin, has been used recently for the treatment of adults with pulmonary hypertension but only few reports are available about its use in neonatal critical care. We report therapeutic trial of inhaled iloprost in newborn infants with persistent pulmonary hypertension of the newborn (PPHN) who did not respond to inhaled nitric oxide (iNO). Inhaled iloprost (Ventavis(R), Bayer Shering Pharma, Germany) was effective in neonates with severe PPHN who showed inadequate response to iNO. We suggest that inhaled iloprost could be considered as an additional therapeutic option in PPHN refractory to iNO.

고해상도 흉부 단층 촬영 결과를 통한 감염 후 폐색성 세기관지염의 임상적 접근

김종덕 ( Jong Deok Kim ),한윤기 ( Yoon Ki Han ),김경원 ( Kyung Won Kim ),이미정 ( Mi Jung Lee ),손명현 ( Myung Hyun Sohn ),김규언 ( Kyu Earn Kim ) 대한소아알레르기호흡기학회(구 대한소아알레르기 및 호흡기학회) 2012 소아알레르기 및 호흡기학회지 Vol.22 No.4

목 적 : 폐색성 세기관지염은 만성 폐쇄성 폐 질환의 한 형태로 소아에서는 여러 원인에 의한 폐 감염 후 발생하는 경우가 가장 많은 것으로 보고되고 있다. 본 연구는 폐색성세기관지염 진단에 유용하게 사용되는 고해상도 흉부 단층 촬영 결과를 토대로 임상 양상을 조사하여 감염 후 폐색성세기관지염을 진단하고 이들의 임상적 특징과 원인, 방사선 소견 등을 조사하고 이를 대조군과 비교하여 향후 진단과 치료적 접근에 도움을 주고자 시행되었다. 방 법: 2007년 1월부터 2011년 3월까지 연세대학교 세브란스병원 소아청소년과에서 시행한 모든 고해상도 흉부 단층 촬영의 결과들을 확인하여 폐색성 세기관지염에 특징적인 소견을 동반한 결과를 보인 환아들의 영상 소견 및 임상 증상을 재확인하였고 이를 통해 감염 후 폐색성 세기관지염으로 진단된 52명을 대상으로 후향적 조사를 시행하였다. 또한 급성 호흡기 증상을 주소로 내원하여 고해상도 흉부 단층 촬영을 시행한 환아에서 단순 하기도 감염으로 진단된 65명을 대조군으로 설정하여 내원 당시의 임상적 특징들을 감염 후 폐색성 세기관지염군과 비교하였다. 결 과 : 감염 후 폐색성 세기관지염 진단 당시의 평균 연령은 41.3±5.9개월이었고, 초기 감염 시 평균 연령은 14.6±2.8개월이었다. 이에 비해 대조군의 진단 당시의 평균 연령은 57.1±5.1개월이었고, 초기 감염 시 평균 연령은 50.9±5.0개월로 통계적으로 유의한 차이가 있음을 확인하였다. 또한 초감염 후 진단 당시까지 급성 하기도 감염으로 입원 치료 받은 빈도수에서 폐색성 세기관지군이 대조군에 비해 상대적으로 빈번하게 노출되었음을 확인하였다. 결 론 : 본 연구에서는 지속되는 호흡기 증상을 주소로 내원한 환아에서 반복적인 호흡기 감염의 입원력이 있고, 2세미만의 어린 연령에서 하기도 감염의 과거력이 있는 경우, 고해상도 흉부 단층 촬영을 통한 폐색성 세기관지염의 진단적 접근을 시행할 필요가 있으며 진단 후 지속적인 차후 관리가 이루어져야 한다고 생각한다. Purpose : Bronchiolitis obliterans (BO) is a type of chronic obstructive lung disease. In children, the most common presentation is a post infectious origin. The aim of this study was to determine the clinical features, etiology, and radiologic findings of post infectious BO, which was diagnosed by high resolution computed tomography (HRCT). The results were then compared to those of the control group. Methods : We diagnosed 52 children with BO by a review of all performed HRCT findings at Severance Children`s Hospital between January 2007 and March 2011. A retrospective study was conducted to define the clinical features, etiology, symptoms, and radiologic findings of post infectious BO. Sixty-five children who were diagnosed with lower respiratory infection confirmed by HRCT were defined as the control group for comparison with post infectious BO patients. Results : The mean age of the patients at the time of diagnosis with BO was 41.3±5.9 months, and that of the control group was 57.1±5.1 months. The initial insult age of BO patients was 14.6±2.8 months, and that of the control group was 50.9±5.0 months. The frequency of previous admissions due to lower respiratory infection was relatively higher in BO patients than the control group. Conclusion : The results suggest that the development of post infectious BO should be early suspected and HRCT should be performed in children with a recurrent admission history of lower respiratory infection, especially in those younger than 2 years of age. Furthermore, continued management of prevent additional infection should be conducted. [Pediatr Allergy Respir Dis(Korea) 2012;22:397-403]

소아 천식 환아에서 호기 온도 측정의 임상적 의의

김수연 ( Soo Yeon Kim ),김정윤 ( Jung Yoon Kim ),한윤기 ( Yoon Ki Han ),김종덕 ( Jong Deok Kim ),설인숙 ( In Suk Sol ),박영아 ( Young A Park ),김윤희 ( Yoon Hee Kim ),김경원 ( Kyung Won Kim ),손명현 ( Myung Hyun Sohn ),김규언 ( Ky 대한소아알레르기호흡기학회 2017 Allergy Asthma & Respiratory Disease Vol.5 No.3

Purpose: Exhaled breath temperature (EBT) has been suggested as a noninvasive marker of airway inflammation in asthma. The aim of this study was to determine its clinical implication in children with asthma. Methods: A total of 233 children were enrolled in this study. Among them, 116 were asthmatic children and 117 were healthy children. Spirometry, bronchodilator response (BDR) test, methacholine challenge test, and skin prick test were performed. EBT, frac-tional exhaled nitric oxide (FeNO), blood eosinophils, and total IgE levels were measured. EBT was measured by using X-halo. Results: EBT was significantly higher in the asthma group than in the control group (median [interquartile range], 32.1°C [30.0°C- 33.9°C] vs. 29.7°C [29.0°C-31.3°C], P<0.001). EBT was significantly higher in poorly or partly controlled asthmatic children than well-controlled asthmatic children (33.5°C [31.0°C-34.4°C] vs. 30.3°C [29.3°C-32.9°C], P<0.0001). Among total subjects, EBT was significantly higher in the atopic group than in the nonatopic group (32.4°C [30.3°C-34.0°C] vs. 29.8°C [29.0°C-30.3°C], P<0.001). There were neither significant associations between EBT and BDR (r=0.109, P=0.241) nor between EBT and PC20 (provocation concentra-tion causing a 20% fall in FEV1) in total subjects (r=0.127, P=0.316). EBT did not show any association with FeNO (r=0.353, P=0.071). Conclusion: Our study suggests that EBT might play a role as an ancillary marker for allergic airway inflammation and the degree of control in pediatric asthma patients. Additional studies are required to explore the value of EBT in detail. (Allergy Asthma Respir Dis 2017;5:147-152)

소아 천식 환자에서 전신 염증 유형에 따른 천식의 특성

설인숙 ( In Suk Sol ),김윤희 ( Yoon Hee Kim ),이희선 ( Hee Seon Lee ),김민정 ( Min Jung Kim ),한윤기 ( Yoon Ki Han ),박영아 ( Young A Park ),김경원 ( Kyung Won Kim ),손명현 ( Myung Hyun Sohn ),김규언 ( Kyu Earn Kim ) 대한소아알레르기호흡기학회 1992 소아알레르기 및 호흡기학회지 Vol.2 No.3

Purpose: Asthma is a chronic airway inflammation. We evaluated whether systemic inflammatory patterns could reflect the nature of airway inflammation. We assessed characteristics of asthma according to systemic inflammatory patterns. Methods: A total of 413 children with asthma were enrolled in the study. Four systemic inflammatory patterns were classified according to eosinophil and neutrophil counts in peripheral blood. Children with neutrophil count≥5,000/μL were defined as the NEUhi group, those with neutrophil count <2,720/μL as the NEUlo group. The intermediate group with neutrophil count between 2,720/μL and 5,000/uL was excluded from the study. Children with eosinophil≥650/μL were defined as the EOShi group, those with eosinophil count<240/μL as the EOSlo group. The remaining patients were excluded from the study. The characteristics of asthma include pulmonary function test results, bronchodilator response, airway hyperresponsiveness, and atopy. Results: The EOShi group had a lower PC20 (provocative concentration of methacholine causing a 20% fall in forced expiratory volume in 1 second [FEV1]), a lower FEV1, and a higher immunoglobulin E level rather than the EOSlo groups, although there were no significant differences between the NEUhi and NEUlo groups. The eosinophil percentages of the induced sputum samples were higher in the EOShi group than the EOSlo group and correlated with blood eosinophil counts. Conclusion: Eosinophilic inflammation was related to characteristics of asthma and sputum eosinophils. However, neutrophilic in- flammation reflected neither asthma features, sputum neutrophils, nor eosinophilic inflammation. Further studies on blood neutrophils involving asthma phenotypes in terms of more specific characteristics of asthma should be needed in children. (Allergy Asthma Respir Dis 2014;2:165-170)

소아천식에서 천식 조절 상태 평가 도구의 유용성

김환수 ( Hwan Soo Kim ),김윤희 ( Yoon Hee Kim ),이희선 ( Hee Seon Lee ),한윤기 ( Yoon Ki Han ),박영아 ( Young Ah Park ),김경원 ( Kyung Won Kim ),손명현 ( Myung Hyun Sohn ),김규언 ( Kyu Earn Kim ) 대한천식알레르기학회 2015 Allergy Asthma & Respiratory Disease Vol.3 No.4

Purpose: The goal of asthma control is to maintain well-controlled state. In this study, we investigated whether childhood asthma control test (C-ACT) may reflect lung function and whether fractional exhaled nitric oxide (FeNO) can be used to improve the accuracy of C-ACT in reflecting the asthma control level. Methods: We reviewed the medical records of 155 patients with asthma underwent lung function tests and C-ACT upon visiting our outpatient clinic. We compared lung function test results according to the C-ACT score stratified by atopy and also examined FeNO according to C-ACT and the Global Initiative for Asthma (GINA) guidelines. The diagnostic accuracy of well-controlled asthma by C-ACT, FeNO, and C-ACT+FeNO was examined. We also calculated the cutoff value of FeNO and C-ACT for well-controlled asthma. Results: Peak expiratory flow (PEF) showed a significant correlation with the C-ACT score. Stratified by atopy, PEF, and forced expiration in one second (FEV1) showed significant correlations with the C-ACT score in the atopic asthma group. There was no difference in FeNO between subjects with C-ACT≥20 and <20, but FeNO was significantly higher in the uncontrolled asthma according to the GINA guidelines. The diagnostic accuracy of well-controlled asthma was higher when FeNO was combined with the C-ACT score than C-ACT or FeNO. Our study showed that the cutoff values of C-ACT and FeNO 19 and 18.3 ppb (parts per billion), respectively, for well-controlled asthma. Conclusion: C-ACT showed a significant correlation with PEF, and atopic asthma group showed significant correlations with PEF and FEV1. A combination of C-ACT with FeNO might reflect asthma control status more accurately. (Allergy Asthma Respir Dis 2015;3:261-266)

소아 천식 환자에서 전신 염증 유형에 따른 천식의 특성

설인숙 ( In Suk Sol ),김윤희 ( Yoon Hee Kim ),이희선 ( Hee Seon Lee ),김민정 ( Min Jung Kim ),한윤기 ( Yoon Ki Han ),박영아 ( Young A Park ),김경원 ( Kyung Won Kim ),손명현 ( Myung Hyun Sohn ),김규언 ( Kyu Earn Kim ) 대한천식알레르기학회 2014 Allergy Asthma & Respiratory Disease Vol.2 No.3

Purpose: Asthma is a chronic airway inflammation. We evaluated whether systemic inflammatory patterns could reflect the nature of airway inflammation. We assessed characteristics of asthma according to systemic inflammatory patterns. Methods: A total of 413 children with asthma were enrolled in the study. Four systemic inflammatory patterns were classified according to eosinophil and neutrophil counts in peripheral blood. Children with neutrophil count≥5,000/μL were defined as the NEUhi group, those with neutrophil count <2,720/μL as the NEUlo group. The intermediate group with neutrophil count between 2,720/μL and 5,000/uL was excluded from the study. Children with eosinophil≥650/μL were defined as the EOShi group, those with eosinophil count<240/μL as the EOSlo group. The remaining patients were excluded from the study. The characteristics of asthma include pulmonary function test results, bronchodilator response, airway hyperresponsiveness, and atopy. Results: The EOShi group had a lower PC20 (provocative concentration of methacholine causing a 20% fall in forced expiratory volume in 1 second [FEV1]), a lower FEV1, and a higher immunoglobulin E level rather than the EOSlo groups, although there were no significant differences between the NEUhi and NEUlo groups. The eosinophil percentages of the induced sputum samples were higher in the EOShi group than the EOSlo group and correlated with blood eosinophil counts. Conclusion: Eosinophilic inflammation was related to characteristics of asthma and sputum eosinophils. However, neutrophilic inflammation reflected neither asthma features, sputum neutrophils, nor eosinophilic inflammation. Further studies on blood neutrophils involving asthma phenotypes in terms of more specific characteristics of asthma should be needed in children.(Respir Dis 2014;2:165-170)

계란 알레르기 환아에서 인플루엔자 백신 접종의 안정성에 대한 연구

장수진 ( Soo Jin Chang ),설인숙 ( In Suk Sol ),김윤희 ( Yoon Hee Kim ),이희선 ( Hee Seon Lee ),한윤기 ( Yoon Ki Han ),박현빈 ( Hyun Bin Park ),김민정 ( Min Jung Kim ),김경원 ( Kyung Won Kim ),손명현 ( Myung Hyun Sohn ),김규언 ( Ky 대한소아알레르기호흡기학회 1991 소아알레르기 및 호흡기학회지 Vol.1 No.4

Purpose: Although influenza vaccine contains some residual egg protein (ovalbumin), recent studies have been reported that the influenza vaccine is even safe for patients with egg allergy. The object of this study was to assess the safety of influenza vaccination and estimate the risk factors of allergic reactions to influenza vaccination in children with egg allergy. Methods: The medical records of 108 children were reviewed retrospectively, those were diagnosed as egg allergy at Department of Pediatrics in Severance Children’s Hospital between January 2006 and December 2011. All of them were vaccinated with very low ovalbumin concentration (≤0.12 μg/mL). Patients were vaccinated in graded doses by the international guideline. Subjects without allergic reactions to influenza vaccine among egg allergy patients were recruited as control subjects. Results: Only 12 subjects of patients had adverse reactions to influenza vaccination. There were no significant relationships between adverse reactions to influenza vaccine and their own history of other allergy, history of breast feeding or graded vaccination. The egg allergy symptoms or egg-specific IgE levels were not associated with adverse reactions to influenza vaccination. Conclusion: These results show that patients with egg allergy may have adverse reactions with influenza vaccine, but severe adverse reactions are rare as general population. Therefore, the patients with egg allergy can be safe vaccinated with influenza vaccine, regardless of severity of allergic reaction after egg ingestion or methods of vaccination. (Allergy Asthma Respir Dis 2013;1:333-338)

계란 알레르기 환아에서 인플루엔자 백신 접종의 안정성에 대한 연구

장수진 ( Soo Jin Chang ),설인숙 ( In Suk Sol ),김윤희 ( Yoon Hee Kim ),이희선 ( Hee Seon Lee ),한윤기 ( Yoon Ki Han ),박현빈 ( Hyun Bin Park ),김민정 ( Min Jung Kim ),김경원 ( Kyung Won Kim ),손명현 ( Myung Hyun Sohn ),김규언 ( Ky 대한천식알레르기학회 2013 Allergy Asthma & Respiratory Disease Vol.1 No.4

Purpose: Although influenza vaccine contains some residual egg protein (ovalbumin), recent studies have been reported that the influenza vaccine is even safe for patients with egg allergy. The object of this study was to assess the safety of influenza vaccination and estimate the risk factors of allergic reactions to influenza vaccination in children with egg allergy. Methods: The medical records of 108 children were reviewed retrospectively, those were diagnosed as egg allergy at Department of Pediatrics in Severance Children`s Hospital between January 2006 and December 2011. All of them were vaccinated with very low ovalbumin concentration (≤0.12 μg/mL). Patients were vaccinated in graded doses by the international guideline. Subjects without allergic reactions to influenza vaccine among egg allergy patients were recruited as control subjects. Results: Only 12 subjects of patients had adverse reactions to influenza vaccination. There were no significant relationships between adverse reactions to influenza vaccine and their own history of other allergy, history of breast feeding or graded vaccination. The egg allergy symptoms or egg-specific IgE levels were not associated with adverse reactions to influenza vaccination. Conclusion: These results show that patients with egg allergy may have adverse reactions with influenza vaccine, but severe adverse reactions are rare as general population. Therefore, the patients with egg allergy can be safe vaccinated with influenza vaccine, regardless of severity of allergic reaction after egg ingestion or methods of vaccination.