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KMID : 0366219880230010255
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Korean Journal of Hematology
1988 Volume.23 No. 1 p.255 ~ p.265
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Statistical Studies on Hemophilia in Korea
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김길영/Kir Young Kim
양창현/조명준/이문호(이상집필대표)/강성귀 그외 30명/Chang Hyun Yang/M J Cho/M H Lee/S K Kang 그외 30명
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Abstract
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498 cases of hemophilia which were reported by sixteen medical centers in Korea
were reviewed and analyzed.
The results are summarized as follows;
1) 425 cases (85.3%) were diagnosed as hemophilia A and the remainder 73 cases (14.
7%) as hemophilia B.
2) One case was female and the other cases were males.
3) Family history was positive in 43.0% and the involved members were brothers,
maternal cousins, maternal uncles and maternal grandfathers in descending order of
frequency.
4) The major symptoms of the patients were hemorrhagic symptoms such as easy
bruising and hemarthrosis followed by prolonged bleeding after trauma and soft tissue
hematoma. The incidence of hemarthrosis increased significantly with age.
5) The pediatric age group below the age of 15 year was 67.1%.
6) According to the age at the time of diagnosis, the half (54.2%) of the severe cases
was diagnosed before the age of 1 year.
7) PTT was prolonged over 40 sec in all cases and 261 cases showed severe
prolongation over 80 second.
8) 273 cases (54.8%) belonged to severe form (factor Ⅷ less than 1%), whereas 182
cases (36.5%) and 43 cases (8.7%) belonged to moderate form (factor 2∼5%) and mild
form (factor 6∼25%), respectively.
9) Chronic arthropathy was present in 236 cases (49.6%) and the incidence increased
significantly with age. The management of chronic arthropathy most commonly
employed was rehabilitation in 25.4%, but in 50.8% no management was given at all.
10) The involved joints of patients with chronic arthropathy were knee, elbow and
ankle joint in descending order of frequency
11) The complications were intracranial hemorrhage in 48 cases, GⅠ bleeding in 24
cases and nerve palsy in 13 cases.
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