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- 저자 : 권차희 (검색결과 3 건)
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권차희,조성윤,맹세현,정유진,진동규 대한소아내분비학회 2012 Annals of Pediatirc Endocrinology & Metabolism Vol.17 No.3
Purpose: Differences in phenotypes between the two most common subtypes of Prader-Willi syndrome (PWS) indicate that a distinct response to growth hormone (GH)treatment may exist. To test this hypothesis, we compared the results of GH treatment in individuals with PWS due to uniparental disomy (UPD) to those of individuals with deletions. Methods: Sixty-five children with PWS who had been treated with GH for more than two years were included in this study. Twenty-one individuals were confirmed as having UPD and 44 individuals had a deletion. Height, body weight, body mass index (BMI), and insulin like growth factor-1 (IGF-I) measurements were recorded before GH treatment and at intervals of 12 months thereafter. Results: After two years of GH therapy, no significant differences were noted for yearly improvements in height standard deviation scores (SDS) between the groups (second year SDS, 0.93 ± 0.94; deletion, 0.84 ± 1.31; UPD, P = 0.717). Body weight SDS, BMI SDS,and IGF-I SDS also showed no differences between the two groups. Conclusion: Our study showed no significant differences in yearly improvements in height SDS between the deletion and UPD groups, at least for the first two years.
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권차희,이호석,유정석,김지현,손영익,안강모 대한 소아알레르기 호흡기학회 2012 Allergy Asthma & Respiratory Disease Vol.22 No.3
Congenital short trachea is a rare congenital anomaly in which the trachea is composed of reduced number of cartilage rings, which result in an abnormally high position of the carina and an abnormal course of the main bronchi. Hazards of congenital short trachea in infants and children include inadvertent bronchial intubation, because it causes bronchiostenosis, pulmonary interstitial emphysema, pneumomediastinum, pneumothorax, and ipsilateral atelectasis. Laryngeal cleft is a rare condition, as well. Symptoms range from mild stridor to massive aspiration and respiratory distress, depending on the severity of the cleft. Until now, a case with combination of these two rare congenital defects has not been reported. Herein, we report a 13 month-old boy who has congenital short trachea with laryngeal cleft. 선천성 단축기관증은 드문 선천성 기형 질환으로 기관연골 개수가 정상 소아에서 17개인데 비해 15개 미만으로 감소한 것이 특징이다. 이러한 환아에서 기도삽관 시 기관지 삽관이 될 가능성이 높고 그 합병증으로 기관지 협착, 폐 간질 기종(pulmonary interstitial emphysema), 기종격동(pneumomediastinum), 폐 기종(pneumothorax), 반대쪽 무기폐 등이 발생할 수 있어 위험하다. 후두열 또한 드문 선천성 기형 질환으로 개열의 위치 및 정도에 따라 경한 천명에서 심각한 흡인 및 호흡 부전까지 다양한 스펙트럼의 임상 양상을 보인다. 현재까지 국내 및 국외에서 두 질환이 동시에 진단된 경우는 보고된 바가 없다. 이에 저자들은 선천성 후두열이 있어 교정 수술을 시행한 뒤 호흡 곤란 증상이 지속적으로 발생하여 시행한 검사상 선천성 단축기관증이 진단되었기에 이를 보고하는 바이다.
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권차희 ( Cha Hee Kwun ),이호석 ( Ho Seok Lee ),유정석 ( Jung Seok Yoo ),김지현 ( Ji Hyun Kim ),손영익 ( Young Ik Son ),안강모 ( Kang Mo Ahn ) 대한소아알레르기호흡기학회(구 대한소아알레르기 및 호흡기학회) 2012 소아알레르기 및 호흡기학회지 Vol.22 No.3
Congenital short trachea is a rare congenital anomaly in which the trachea is composed of reduced number of cartilage rings, which result in an abnormally high position of the carina and an abnormal course of the main bronchi. Hazards of congenital short trachea in infants and children include inadvertent bronchial intubation, because it causes bronchiostenosis, pulmonary interstitial emphysema, pneumomediastinum, pneumothorax, and ipsilateral atelectasis. Laryngeal cleft is a rare condition, as well. Symptoms range from mild stridor to massive aspiration and respiratory distress, depending on the severity of the cleft. Until now, a case with combination of these two rare congenital defects has not been reported. Herein, we report a 13 month-old boy who has congenital short trachea with laryngeal cleft.
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