효소 보충 치료로 호전을 보인 Pompe병 1례

전유훈,은백린,이동환,Jeon, You Hoon,Eun, Baik Lin,Lee, Dong Hwan 대한유전성대사질환학회 2005 대한유전성대사질환학회지 Vol.5 No.1

저자들은 Pompe 병으로 진단된 3세 남아에 recombinant human GAA 정주를 통한 효소 보충 치료를 시행하여 운동 능력과 심기능이 호전되며 간비대도 호전된 1례를 경험하였기에 보고하는 바이다. Pompe disease is a genetic disorder caused by a deficiency of acid ${\alpha}$-glucosidase (GAA). This enzyme defect results in lysosomal glycogen accumulation in multiple tissues and cell types, with cardiac, skeletal, and smooth muscle cells the most seriously affected. Infantile-onset Pompe disease is uniformly lethal. Affected infants present in the first few months of life with hypotonia, generalized muscle weakness, and a hypertrophic cardiomyopathy, followed by death from cardiorespiratory failure or respiratory infection, usually by 1 year of age. Late-onset forms is characterized by a lack of severe cardiac involvement and a less severe short-term prognosis. Enzyme replacement therapy for Pompe disease is intended to address directly the underlying metabolic defect via intravenous infusions of recombinant human GAA to provide the missing enzyme. We experienced one case of Pompe disease in 3-years old boy that has improved his exercise ability and cardiac function after GAA enzyme replacement therapy.

소아 아토피피부염의 최신 치료

전유훈 ( You Hoon Jeon ),김정희 ( Jeong Hee Kim ) 대한천식알레르기학회(구 대한알레르기학회) 2021 Allergy Asthma & Respiratory Disease Vol.9 No.2

Atopic dermatitis is the most common chronic inflammatory skin disease in children. It usually develops in infancy and early childhood and is an entry point of ‘atopic march’, which may progress to other allergic diseases, including allergic rhinitis and asthma. Although there is no disagreement on the importance of early treatment of atopic dermatitis, it is difficult to treat children with moderate to severe atopic dermatitis due to concerns about treatment-related side effects and age restrictions for effective drugs in real world. As recent researches have extend our understanding of pathogenesis, new agents targeted key pathologic mechanisms have been developed. Therefore, it is time to establish a new paradigm of treatment for children with atopic dermatitis since new biologics have been demonstrated to be effective and safe in children. The objective of this article is to review the clinical characteristics, severity classification, pathogenesis, and treatment, in particular, biologics already approved/undergoing clinical trials for children with atopic dermatitis. (Allergy Asthma Respir Dis 2021;9:59-68)

소아 천식환아에서 흡입용 fluticasone 및 tulobuterol의 병합치료와 두 배 용량 흡입용 fluticasone 치료의 효과 및 안전성 비교에 대한 다기관 연구

전유훈 ( You Hoon Jeon ),양현종 ( Hyeon Jong Yang ),유영 ( Young Yoo ),김영호 ( Young Ho Kim ),정지태 ( Ji Tae Jeong ),이혜란 ( Hae Ran Lee ),편복양 ( Bok Yang Pyun ) 대한소아알레르기호흡기학회(구 대한소아알레르기 및 호흡기학회) 2009 소아알레르기 및 호흡기학회지 Vol.19 No.2

목적: 소아 천식 치료에서 흡입용 스테로이드의 용량을 증가시키는 것과 기존 용량의 흡입용 스테로이드에 기관지 확장제인 tulobuterol을 추가하는 것을 효과와 안전성 면에서 비교해 보고자 하였다. 방법: GINA 가이드라인에 따라 2단계(경증지속성) 천식으로 진단받고 하루 50-100 μg의 저용량 흡입용 스테로이드(fluticasone)로 유지치료를 받고 있는 4-8세의 소아천식 환자 중에서 step-up 치료를 고려하는 53명을 대상으로 하였다. 이들을 무작위로 기존의 흡입용 fluticasone용량(50-100 μg/일)에 tulobuterol (Hokunalin(R) patch 1 mg)을 추가한 군 (Flt+Hk군)과 흡입용 fluticasone을 두 배 용량(100-200 μg/일)으로 늘린 군 (Flt군)으로 나누었다. 이들을 각각 4주간 치료하였으며 이후 치료의 효과와 부작용을 보기 위해 추적관찰하였다. 두 군에서 치료시작전과 치료 4주 후의 천식 증상 점수의 변화, 일중 최대호기유속의 변화, 야간 증상으로 잠을 깬 횟수, 증상완화제의 사용 횟수를 비교하였고 치료에 대한 보호자의 전체적인 만족도, 이상 반응의 횟수를 조사하였다. 결과: 추적관찰이 된 환자는 Flt+Hk 군, Flt군 각각 24명이었다. 치료시작전과 치료 4주 후 두 군간의 전체적인 천식증상점수 변화는 Flt+Hk 군에서 -0.01±0.24 , Flt 군에서 -0.05±0.17로 두 군 간에 통계적으로 유의한 차이는 없었다.(P=0.54) 아침 최대호기유속의 평균변화와 저녁 최대호기유속의 평균 변화는 통계적으로 큰 차이를 보이지 않았다.(P=0.83, P=0.83) 야간 증상으로 잠을 깬 횟수나 증상완화제의 사용 횟수, 치료에 대한 보호자의 전체적인 만족도도 두 군 간에 차이가 없었다.(P=0.17, P=0.32, P=0.63) 치료 중의 이상증상의 경험 빈도 역시 통계학적으로 유의한 차이를 보이지 않았다.(P=1.00) 결론: 소아천식환아에서 흡입용 fluticasone propionate및 tulobuterol의 병합치료는 두 배 용량의 흡입용 fluticasone propionate 단독치료만큼 효과가 있으며 안전성도 비슷한 것으로 나타났다. 스테로이드 치료에 대한 거부감이나 잠재적 부작용에 대한 두려움을 가지고 있는 환자에서 흡입용 스테로이드의 용량을 늘리는 치료에 대한 대체치료로 tulobuterol의 병합치료를 고려할 수 있겠다. Purpose: We aim to compare the effectiveness and safety of fluticasone propionate (Flt) plus tulobuterol (Hk) versus high-dose Flt alone in controlling asthma in children. Methods: Fifty three children aged 4 to 8 years, who were diagnosed with mild persistent asthma and underwent maintenance therapy with a low dose of inhaled corticosteroid (Flt) of 50-100 μg/day were randomized to receive Flt plus Hk (Hokunalin(R) patch 1 mg, Abbott Japan, Tokyo, Japan), or Flt alone at twice the dosage. Patients underwent new treatment for 4 weeks. Asthma symptom scores, mean changes in morning and evening peak expiratory flow (PEF), the frequency of night awakenings, the use of reliever medication, caregiver`s overall satisfaction and safety were evaluated and compared in each group. And they were followed-up again 4 week after treatment course for the evaluation of treatment-emergent adverse event (TEAE). Results: No significant difference was found between the groups in terms of mean changes in the morning and evening PEF, the frequency of night awakening, the use of rescue medication and caregiver`s overall satisfaction (P=0.83, P=0.83, P=0.17, P=0.32 and P=0.63). Furthermore, no statistically significant difference was observed between 2 groups in the incidence of any TEAE (P=1.00). Conclusion: This study demonstrated that a combination of Flt and Hk was as effective as a high-dose Flt therapy in the management of mild persistent asthma in children. The results of this study suggest that tulobuterol add-on therapy can be considered as a reasonable substitute to an increase in the dosage of steroid in the patients with steroid-phobia and it might be used to reduce the risk of high dose steroid therapy. [Pediatr Allergy Respir Dis(Korea) 2009;19:125-136]

영아기 아토피피부염 환자의 천명발생 위험인자에 대한 연구

전유훈 ( You Hoon Jeon ),양현종 ( Hyeon Jong Yang ),편복양 ( Bok Yang Pyun ) 대한소아알레르기 및 호흡기학회 2007 소아알레르기 및 호흡기학회지 Vol.17 No.2

국내 소아 지역획득폐렴의 치료

전유훈 ( You Hoon Jeon ),김정희 ( Jeong Hee Kim ) 대한천식알레르기학회(구 대한알레르기학회) 2017 Allergy Asthma & Respiratory Disease Vol.5 No.4

Community-acquired pneumonia is the leading cause of pediatric morbidity and mortality. However, there is a lack of data on the epidemiology of pneumonia in Korean children. In this review, we aimed to summarize pneumonia studies in Korea and suggest diagnostic methods and treatment for Korean children who were referred based on the foreign guidelines for pediatric community-acquired pneumonia. A Korean guideline for pediatric pneumonia in tune with domestic circumstances is needed. (Allergy Asthma Respir Dis 2017;5:177-184)

소아 아토피피부염에서 아토피성과 비아토피성의 중증도와 검사소견의 비교

신정은 ( Jung Eun Shin ),전유훈 ( You Hoon Jeon ),양현종 ( Hyeon Jong Yang ),편복양 ( Bok Yang Pyun ) 대한소아알레르기호흡기학회(구 대한소아알레르기 및 호흡기학회) 2008 소아알레르기 및 호흡기학회지 Vol.18 No.3

목적: 아토피피부염의 발생기전에 있어 IgE와 호산구 및 ECP가 아토피피부염의 발생기전에 중요한 역할을 하리라 생각되고 있으나 이러한 검사결과와 임상적 중증도와의 상관관계에 대해서는 논란이 있다. 본 저자들은 아토피피부염 소아에서 아토피성과 비아토피성의 임상양상을 비교해보고 총 혈청 IgE, 혈중 호산구수, ECP 농도 등의 실험실 검사소견과 아토피피부염의 임상적 중증도의 상관관계를 밝혀 이의 임상적 유용성을 알아보고자 하였다. 방법: 2005년 10월부터 2008년 3월까지 순천향대학교병원 소아알레르기 호흡기센터에서 아토피피부염으로 진단받은 환아 271명을 대상으로 하였고 대상 환아에서 혈청 총 IgE치와 원인 항원 특이 IgE치, 말초 혈액 내 총 호산구수, ECP를 측정하였으며, 피부단자 시험을 시행하였다. SCORAD 점수를 통해 임상적 중증도를 반영하는 지표로 사용하였으며 천식과 알레르기비염의 동반여부와 알레르기 질환에 대한 가족력을 조사했다. 전체 환아는 특이 IgE가 한 가지 이상 양성이거나 피부단자시험에서 양성을 보인 아토피군과 특이 IgE가 모두 음성이며 피부 단자시험에서도 음성을 보인 비아토피군으로 분류하여 두 군에서 임상적 중증도를 반영하는 SCORAD 점수와 혈청 총 IgE치, 말초 혈액 내 호산구수, ECP의 상관관계를 조사하였다. 결과: 총 271명의 대상 환아중 아토피군은 162명, 비아토피군은 109명이었다. 총 IgE치, 말초 혈액내 호산구수, ECP, 아토피피부염의 가족력이 아토피군에서 비아토피군보다 더 높은 수치를 보였으며, 아토피군에서 SCORAD 점수가 더 높아 임상적 중증도가 더 높은 것으로 나타났다. 아토피군에서는 혈청 총 IgE치, 말초 혈액 내 호산구수, ECP 모두 SCORAD 점수와 통계적으로 유의한 상관관계를 보였으며, 이중 말초 혈액 내 호산구수의 상관계수가 가장 높았으며, 비아토피군에서는 말초 혈액내 호산구수만 SCORAD 점수와 통계적으로 유의한 상관관계를 보였으며 혈청 총 IgE치와 ECP는 상관관계를 보이지 않았다. 결론: 혈청 총 IgE치, 말초혈액 내 호산구수, ECP농도를 아토피성 아토피피부염 환아에서 중증도의 평가 지표로 사용할 수 있으며 호산구수는 비아토피성 환아에서도 중증도 판정에 역할을 할 수 있겠다. Purpose: We aim to compare clinical severity of atopic and non-atopic eczema in children and examine the relationship between total-IgE, eosinophil counts, Eosinophil, Eosinophil cationic protein (ECP) and clinical severity of atopic dermatitis (AD). Methods: A total of 271 children diagnosed with AD at the Pediatric Allergy Respiratory Center in Soonchunhyang University Hospital from October 2005 to March 2008 were enrolled for this study and divided into 2 groups: atopic and non-atopic eczema. Serum concentrations of total- and specific-IgE, eosinophil counts and ECP were measured. Allergy skin tests were also performed and the SCORAD index was used to evaluate clinical severity. Comparisons the SCORAD index and serum total-IgE, eosinophil count and ECP between the 2 groups were made. Results: Of the 271 patients, 162 (59.8%) were included in the atopic eczema group, while 109 (40.2%) were included in the non-atopic group according to the laboratory results. Serum total-IgE, eosinophil counts, ECP, the SCORAD index and the frequency of a family history of eczema were relatively higher in the atopic group. In the atopic group, serum total-IgE, eosinophil counts and ECP each had a statistically significant correlation with the SCORAD index with eosinophil counts showing the highest correlation. However, only eosinophil counts had a statistically significant correlation with the SCORAD index in the non-atopic group. Conclusion: Serum total-IgE, eosinophil counts, and ECP can be used as markers for clinical severity in patients with atopic eczema, while eosinophil counts be used as marker for clinical severity in those with non-atopic eczema. [Pediatr Allergy Respir Dis(Korea) 2008;18:219-227]

소아 천식 환자에서 게임을 통한 천식 교육법의 유용성

홍예슬 ( Ye Seul Hong ),전유훈 ( You Hoon Jeon ),양현종 ( Hyeon Jong Yang ),편복양 ( Bok Yang Pyun ) 대한천식알레르기학회 2009 천식 및 알레르기 Vol.29 No.4

Background: Education for understanding the disease itself is important in the management of asthma. Objective: The purpose of this study is to evaluate the effect of the repeated education for the asthmatic children through various games. Method: A 15 school-aged children with mild-to-moderate asthma who participated in the Soonchunhyang asthma camp were enrolled. We compared each score of the test which consisted of 4 components of asthma (triggers, preventions, treatments and courses). The first test was performed without any education. Thereafter, the second, third and fourth tests were conducted repeatedly with various games. Result: The average score of the first test was 11.93±5.20. Meanwhile, the scores of the repeated tests showed gradual improvement; the scores of the second, third and fourth tests were 15.64±4.77, 18.06±2.96 and 18.6±2.47. The score of the second test showed improvement as compared to that of the first test (P<0.01). The score of the third test compared also showed improvement as compared to the second test (P=0.03). Conclusion: The interesting games as a tool for the education of asthma could be helpful in not only understanding the disease itself but also improving the compliance of patients. (Korean J Asthma Allergy Clin Immunol 2009;29:262-268)

기관지확장제 반응 검사에서 속효성 기관지확장제 투여 용량의 차이가 소기도 반응도에 영향을 줄까?

백지현 ( Ji Hyeon Baek ),장호민 ( Homin Jang ),전유훈 ( You Hoon Jeon ),서보선 ( Bo Seon Seo ),이승진 ( Seung Jin Lee ),지혜미 ( Hye Mi Jee ),이경석 ( Kyung Suk Lee ),정영호 ( Young-ho Jung ),신윤호 ( Youn Ho Sheen ),한만용 ( Man 대한천식알레르기학회(구 대한알레르기학회) 2016 Allergy Asthma & Respiratory Disease Vol.4 No.4

Purpose: It is recommended to use 200 (2 puffs) or 400 (4 puffs) μg of salbutamol in the bronchodilator response (BDR) test. We aimed to compare the difference between these 2 doses with regard to small airway dysfunction.Methods: One hundred sixteen subjects who visited the hospital for diagnosis or follow-up of asthma were consecutively enrolled between June 1 and November 31, 2013. The subjects were randomly assigned to the BDR test at the 2 doses (200 or 400 μg of salbutamol), with physicians blinded to the group each subject was assigned to and undertook the BDR test using the spirometry and impulse oscillometry system (IOS).Results: A total of 116 subjects participated in this study; the mean age was 7.8±3.6 years. The number of participants who were assigned to 2 and 4 puffs groups was 59 and 57, respectively. The mean age was older in the 4 puffs group than in the 2 puffs group (P=0.008). There were no significant difference in spirometric and oscillometric parameters between the 2 and 4 puffs groups. However, in subgroup analysis of asthmatic patients on maintenance therapy (n=21), there was a significant difference in relative changes in Rrs5 between the 2 and 4 puffs groups (16.4%±9.6% vs. 28.7%±8.8%, P=0.035). The forced expiratory volume of 1 second showed a significant correlation with resistance in the 2 puffs group and with reactance in the 4 puffs group.Conclusion: There was a significant relationship between the amounts of bronchodilators administered and the small airway dysfunction in children with asthma on maintenance therapy. Further research is warranted to delineate changes in spirometric and IOS measures in accordance with the different amounts of bronchodilators administered. (Allergy Asthma Respir Dis 2016:4:284-289)

한국 소아 알레르기비염 환자의 삶의 질에 대한 설문 개발 및 유용성 평가를 위한 연구

진장용 ( Jang Yong Jin ),양현종 ( Hyeon Jong Yang ),전유훈 ( You Hoon Jeon ),김경원 ( Kyung Won Kim ),김우경 ( Woo Kyung Kim ),박용민 ( Yong Mean Park ),윤혜선 ( Hae Sun Yoon ),염혜영 ( Hye Yung Yum ),편복양 ( Bok Yang Pyun ) 대한천식알레르기학회 2009 천식 및 알레르기 Vol.29 No.4

Background: A questionnaire for assessing the quality of life (QOL) of children with allergic rhinitis has not been developed in Korea. Objective: Our study was designed to develop and validate a Questionnaire for QOL Specific to Allergic Rhinitis in Korean Children (QQOL-ARK). Method: The QQOL-ARK consisted of 4 domains including 18 items with a 5-point scale: symptoms (3 items), physical factors (8 items), psychosocial factors (6 items) and prognosis (1 item). A survey with the QQOL-ARK was performed on 168 children with allergic rhinitis twice, 4 weeks apart. We analyzed their responsiveness, reproducibility and validity. Result: The mean score of QOL according to the severity showed a statistically significant difference between the first and second visits (P<0.01). The scores of QOL were decreased in the improved group (P<0.01) and increased in the worsen group (P<0.01). The differences in scores between the first and second visits showed a statistically significant difference in intergroup analysis (P<0.01). The QQOL-ARK also showed a good reproducibility (R=0.97). There was no strong correlation between QOL and clinical severity. Conclusion: The QQOL-ARK showed a good reproducibility and validity. However, the assessment of reliability on 18 items of the questionnaire could not be performed. Although the items of this questionnaire are limited, they could provide the basis for the development of the QOL questionnaire for Korean children with allergic rhinitis in conjunction with clinical evaluation. (Korean J Asthma Allergy Clin Immunol 2009;29:242-248)

반복적인 영아 천명에서 지속성 천명으로의 진행에 관여하는 위험 인자

양현종 ( Hyeon Jong Yang ),홍용희 ( Yong Hee Hong ),전유훈 ( You Hoon Jeon ),편복양 ( Bok Yang Pyun ) 대한소아알레르기 및 호흡기학회 2007 소아알레르기 및 호흡기학회지 Vol.17 No.1