Journal of the Korean Pediatric Society 1984;27(9):883-895.
Published online September 30, 1984.
Clinical Studies on 100 Cases of Hemophilia.
Mi Ja Shin, Kwang Wook Ko
Department of Pediatrics College of Medicine, Seoul National University
혈우병환아 100례의 임상적 연구
신미자, 고광욱
서울대학교 의과대학 소아과학교실
Abstract
A clincal studies were performed on 100 cases of hemophilia who had been visited to O.P.D. of pediatric department and emergency-room of Seoul Red Cross Hospital during 10 years period between July, 1973 and June, 1983. The results were obtained as follows: 1)Nin ty two cases were confirmed as hemophilia A and 8 cases were hemophilia B and. none of hemophilia C was detected. 2)All of the 100 cases were male. None of the cases was identified female. 3)There were positive family history in 42 cases out of 100 cases yielding 42% in positiverate, showing as brothers, maternal uncles, maternal cousins and maternal grandfather in decreasing frequency. 4) The age distribution at the first visit to the clinic due to bleeding episodes were in 65.2% before the age of 2 years, in 87% at the age of 5 years in hemophilia A and in 87.5% before the age of 3 years in hemophilia B. 5)The common complaints on first visit to the clinic were hemorrhagic symptoms such as prolonged bleeding following minor trauma, bruises, hematoma, gingival bleedings in 87% and hemarthrosis in 13%. 6)The major bleeding episodes in 100 cases were hemarthrosis and prolonged posttraumatic bleeding which were followed by hematoma, gingival bleeding and epistaxis in orders. 7)One hundred hemophiliacs had 1,667 visits due to the various bleeding episodes during the ten years. Almost the half of the bleeding episodes was caused by emarthrosis, showing 48.53% in frequency. 8)Partial thromboplastin time(PTT) was checked in 39 cases out of 100 cases. All of the 39 cases were prolonged over 50 seconds and 71.7% of 39 cases showed the severe prolongation over 90 seconds. 9)The specific assay of factor "Vffi and K was performed in total 62 cases (57 cases of hemophilia A and 5 cases of hemophilia B). The severe cases (less than 2% of normal value) were showed in 59.67% and moderate cases were in 25.8%. Most of the cases involved in this study group (85.48%) showed moderate to severe disease in severity. 10)Forty-four cases were tested for HBs-Ag and anti-HBs. Thirty one out of 44 cases already had anti-HBs (70.45%) and 6 cases carried HBs-Ag as healthy carriers (13.6%). Two of the 6 cases with antigenemia had the past history of transfusion.
Key Words: Hemophilia, Hemorrhagic symptoms, Hemarthrosis




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