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2000-2008년 수도권 지역의 가와사끼병의 역학적 연구
박형준,김영대,김우경,송영환,김동욱,박용원 인제대학교 2009 仁濟醫學 Vol.30 No.-
Objectives : The aim of this study was to investigate the clinical features and epidemiologic pattern of Kawasaki disease(KD) in Seoul metropolitan area for the 9-year-period. Methods : An epidemiologic survey on KD was retrospectively performed. The questionnaire was sent to 3 hospitals including Seoul Paik hospital, Sanggye Paik hospital and Ilsan Paik hospital, and obtained data were analyzed. Results : Data from a total of 1,297 cases were collected. The male-to-female ratio was 1.33:1(741:556), and the mean age of onset was 32.2 months. The monthly number of patients was slightly higher in May, June and December. There was no sibling case and rate of recurrence cases was 2.31%(30/1,297), and the proportion of patients with KD among total hospitalized pediatric patients was 2.05%. Coronary arterialu..A) abnormalities were detected at follow-up by echocardiogram in 17.2%(204/1,185) of cases including 0.76% of aneurysms. Conclusion : Comparing with the data of the KD patients in Korea(1.48%), our data of 1,297 KD patients area showed higher proportion of patients with KD among total hospitalized pediatric patient(2.05%).
박형준;김영대;김우경;박용원 인제대학교 2011 仁濟醫學 Vol.32 No.-
Congenital factor VII deficiency is a rare bleeding disorder that has an estimated prevalence of 1/300,000 to 1/500,000. Transmission is autosomal recessive. Because factor VII is important in initiation the coagulation cascade, a factor VII deficiency can result in significant bleeding with pr oiongation of the prothrombin time. In severely affected patients, bleeding starts early in life and encompasses life-threatening hemorrhages such as Intracranial and gastrointestinal hemorrhages. These are the most frequent bleeding events in the first 6 months of life. Definitive diagnosis tests on a specific assay for factor VII clotting activity. Human factor VII from plasma is used to treat factor VII deficiency. Either fresh frozen plasma or factor VII concentrate can be used. We experienced a case of intraventricular hemorrhage in a newborn infant who had been diagnosed as congenital factor VII deficiency. So, we report the case with a review of literatures.