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구교연,이준석,이순민,남궁란,박국인,이철,윤춘식,정우희,최홍식,박민수 대한소아청소년과학회 2010 Clinical and Experimental Pediatrics (CEP) Vol.53 No.2
Lymphangioma is a rare benign congenital tumor of the lymphatic system, which is commonly diagnosed before 2 years of age. In the natronal report, cystic lymphangioma was usually reported as a huge translucent mass located in the head and neck area. It's occurrence in retropharyngeal space with respiratory obstruction and swallowing difficulty in neonate is extremely rare and postoperative nasopharyngeal reflux has rarely been reported. Complete resection is the standard therapy. However, involvement of the upper airway may be determining prognosis in the extensive lymphangiomas because of the difficulty of complete excision. We present a case of cystic lymphangioma in neonate which was initially asymptomatic but gradually progressed to cause respiratory obstruction due to enlargement. After resection, nasopharyngeal reflux developed with dysfunction of the soft palate and gradually improved with conservative care over 5 months.
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건설기업 실행예산 편성업무의 애로요인 분석에 관한 연구
구교연,김한수,Koo, Kyo Youn,Kim, Han Soo 한국건설관리학회 2016 한국건설관리학회 논문집 Vol.17 No.3
국내 건설기업들의 수익성 악화로 인한 내실 경영이 중요한 화두가 되고 있는 현 시점에서 본 연구는 실행예산 편성 업무에서 나타나는 애로요인을 도출하고 이들 애로요인에서 나타나는 주요 특징과 시사점을 분석하기 위한 목적으로 수행되었다. 종합건설사의 견적과 공무의 경험이 있는 전문가들을 대상으로 설문조사와 인터뷰조사를 통하여 실행예산 편성업무의 6개 애로요인군(群)과 18개 애로요인에 대하여 분석을 실시하였다. 그 결과는 실행 편성 업무에서 다양한 애로요인이 존재하지만 각 애로요인에는 경중이 있으며 우선적으로 주목하고 대비할 애로요인이 존재하는 것으로 나타났다. 또한 실행 예산 편성업무에서 나타나는 어려움을 최소화하기 위해 해결해야 할 가장 중요한 현안은 협력업체의 견적능력 역량 강화와 견적전문가 양성 및 안정적인 운영인 것으로 나타났다. Under the circumstances where the profitability has become a more critical issue for general contractors, the objective of the study is to identify and discuss obstacles and their key characteristics in the construction budget estimating. Through the questionnaire survey and interviews with experienced estimators of general contractors, the study identified 18 obstacles by 6 groups. It discussed that although various obstacles exist, there are key obstacles to which general contractors should pay attention for the realistic construction budget estimating. In particular, improving subcontractors' estimating competency and incubating estimating experts are identified as the key issues to overcome obstacles in the construction budget estimating.
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Duarte Variant/Classical Galactosemia (D/G) Heterozygote으로 진단된 일란성 쌍둥이 1례
구교연,이철호,양정윤,이진성,Koo, Kyo Yeon,Lee, Chul Ho,Yang, Jeong Yoon,Lee, Jin-Sung 대한유전성대사질환학회 2012 대한유전성대사질환학회지 Vol.12 No.1
고전적 갈락토오스혈증(classical galactosemia; OMIM #230400)은 상염색체 열성 유전의 갈락토오스 대사장애로, 9번 염색체에 위치하는 GALT 유전자(OMIM *606999)로부터 전사되는 galactose-1-phosphate uridylyltransferase (GALT; E.C.2.7.7.12)의 심각한 결손으로 인해 유발되는 질환이다. GALT의 결함은 galactose-1-phosphate의 체내 축적을 일으켜, 신생아 시기부터 구토, 수유 곤란, 황달, 복수, 경련 발작, 기면 상태 등의 증상을 유발하고, 장기적으로는 백내장, 성장지연, 지능저하를 초래한다. 반면 Duarte형 갈락토오스혈증은 적혈구에서의 GALT 효소 활성도가 감소되어 있어 혈중 galactose와 galactose-1-phosphate의 농도가 증가하지만 임상적으로는 거의 증상을 보이지 않는 아형이다. 최근 신생아 선별검사의 발달과 함께, 무증상의 양성 판정 환아들이 늘고 있으며, 이들 환아들이 Duarte형 갈락토오스혈증일 가능성이 제기되고 있다. 이에 저자들은 국내에서 그 동안 드물게 보고되었던 N314D와 -119_-116delGT CA/E363K이형접합체의 Duarte형 갈락토오스혈증 일란성 쌍둥이 1례를 보고하고자 한다. Classical galactosemia (OMIM# 230400) is an autosomal recessive disorder of carbohydrate metabolism, due to a complete loss in galactose-1-phosphate uridyltransferase (GALT; E.C.2.7.7.12) enzyme activity. It caused by mutations in the GALT gene (OMIM$^*$ 606999) that is located at chromosome 9p13. The GALT enzyme deficiency results in a build-up of galactose and galactose-1-phosphate, causing life threatening complications such as feeding problems, failure to thrive, hepatocellular damage, bleeding and sepsis. However, Duarte galactosemia, a variant form of GALT deficiency, has residual GALT enzyme activities in erythrocytes and do not have manifest the symptoms of classical galactosemia. Since the advent of newborn screening (NBS) for galactosemia, we rarely encounter such overwhelmingly ill newborns. The positive NBS with no symptoms indicates the possibility of Duarte galactosemia besides a simple false positive and it has to be differentiated from classical galactosemia which is a medical emergency. In Korea, detection rate of Duarte galactosemia is very low and its genetic information is restrictive, too. We report a case of monozygotic twins with D/G galactosemia compound heterozygote in proven by the mutational analysis of GALT gene, which revealed N314D polymorphism and -119 to -116 delGTCA.
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The First Case of Familial Mediterranean Fever Associated with Renal Amyloidosis in Korea
구교연,이진성,박세진,왕지영,신재일,정현주,임범진 연세대학교의과대학 2012 Yonsei medical journal Vol.53 No.2
Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterized by periodic episodes of fever and recurrent polyserositis. It is caused by a dysfunction of pyrin (or marenostrin) as a result of a mutation within the MEFV gene. It occurs mostly in individuals of Mediterranean origin; however, it has also been reported in non-Mediterranean populations. In this report, we describe the first case of FMF in a Korean child. As eight-year-old boy presented recurrent febrile attacks from an unknown cause, an acute scrotum and renal amyloidosis. He also showed splenomegaly, lymphadenopathy, pleural effusion, ascites and elevated acute phase reactants. After MEFV gene analysis, he was diagnosed as FMF combined with amyloidosis.
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The First Case of Familial Mediterranean Fever Associated with Renal Amyloidosis in Korea
구교연,박세진,왕지영,신재일,정현주,임범진,이진성 연세대학교의과대학 2012 Yonsei medical journal Vol.53 No.3
Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterized by periodic episodes of fever and recurrent polyserositis. It is caused by a dysfunction of pyrin (or marenostrin) as a result of a mutation within the MEFV gene. It occurs mostly in individuals of Mediterranean origin; however, it has also been reported in non-Mediterranean populations. In this report, we describe the first case of FMF in a Korean child. As eight-year-old boy presented recurrent febrile attacks from an unknown cause, an acute scrotum and renal amyloidosis. He also showed splenomegaly, lymphadenopathy, pleural effusion, ascites and elevated acute phase reactants. After MEFV gene analysis, he was diagnosed as FMF combined with amyloidosis.
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Effect of severe neonatal morbidities on long term outcome in extremely low birthweight infants
구교연,김정은,이순민,박민수,박국인,이철,남궁란 대한소아청소년과학회 2010 Clinical and Experimental Pediatrics (CEP) Vol.53 No.6
Purpose: To assess the validity of individual and combined prognostic effects of severe bronchopulmonary dysplasia (BPD), brain injury,retinopathy of prematurity (ROP), and parenteral nutrition associated cholestasis (PNAC). Methods: We retrospectively analyzed the medical records of 80extremely low birthweight (ELBW) infants admitted to the neonatal intensive care unit (NICU) of the Severance Children’s Hospital, and who survived to a postmenstrual age of 36 weeks. We analyzed the relationship between 4 neonatal morbidities (severe BPD, severe brain injury, severe ROP, and severe PNAC) and poor outcome. Poor outcome indicated death after a postmenstrual age of 36 weeks or survival with neurosensory impairment (cerebral palsy, delayed development, hearing loss, or blindness) between 18 and 24 months of corrected age. Results: Each neonatal morbidity correlated with poor outcome on univariate analysis. Multiple logistic regression analysis revealed that the odds ratios (OR) were 4.9 (95% confidence interval [CI], 1.0-22.6; P=0.044) for severe BPD, 13.2 (3.0-57.3; P<.001) for severe brain injury, 5.3 (1.6-18.1; P=0.007) for severe ROP, and 3.4 (0.5-22.7; P=0.215) for severe PNAC. Severe BPD, brain injury, and ROP were significantly correlated with poor outcome, but not severe PNAC. By increasing the morbidity count, the rate of poor outcome was significantly increased (OR 5.2; 95% CI, 2.2-11.9; P<.001). In infants free of the above-mentioned morbidities, the rate of poor outcome was 9%, while the corresponding rates in infants with 1, 2,and more than 3 neonatal morbidities were 46%, 69%, and 100%,respectively. Conclusion: In ELBW infants 3 common neonatal mornidifies,severe BPD, brain injury and ROP, strongly predicts the risk of poor outcome.
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The First Case of Familial Mediterranean Fever Associated with Renal Amyloidosis in Korea
구교연,박세진,왕지영,신재일,정현주,임범진,이진성 연세대학교의과대학 2012 Yonsei medical journal Vol.53 No.4
Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterized by periodic episodes of fever and recurrent polyserositis. It is caused by a dysfunction of pyrin (or marenostrin) as a result of a mutation within the MEFV gene. It occurs mostly in individuals of Mediterranean origin; however, it has also been reported in non-Mediterranean populations. In this report, we describe the first case of FMF in a Korean child. As eight-year-old boy presented recurrent febrile attacks from an unknown cause, an acute scrotum and renal amyloidosis. He also showed splenomegaly, lymphadenopathy, pleural effusion, ascites and elevated acute phase reactants. After MEFV gene analysis, he was diagnosed as FMF combined with amyloidosis.
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