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KMID : 0387820020090020226
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Clinical Pediatric Hematology-Oncology
2002 Volume.9 No. 2 p.226 ~ p.233
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Intracranial Hemorrhage in Hemophilia Patients
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Lee Kun-Soo
Hwang Sung-Kyoo
Lee Sun-Min
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Abstract
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Purpose: Intracranial hemorrhage (ICH) in hemophilia patients is the most common cause of death in Korea. Early suspicion and prompt treatment of ICH is a very important for saving their life and minimizing neurologic sequelae. We investigate the prognosis of the hemophilia patients with ICH who registered in Daegu & Kyungpook area.
Methods:We evaluated the clinical courses, laboratory findings, brain image, effect of treatment and prognosis of ICH. Nine of 161 patients registered in our Department of Pediatrics suffered 16 episodes of ICH from Dec. 1996 to Mar. 2002.
Results:were male hemophilia A patients (severe; 5, moderate; 3 and mild; 1) with median age of 48 (17~312) months at diagnosis of ICH. Two patients who had inhibitor became negative and one patient who had found inhibitor on annual routine follow-up has had inhibitor until now. One had trauma history and seven had family history. The median time interval from first symptom to hospital visit was 7 hours (15 minutes~10 days). Chief complaints were vomiting in 6 patients, headache in 4, seizure in 3 and mental change in 1. All patients except one were initially given factor VIII concentrate, 50 units/kg and then continuous infusion, 2~3 units/kg/hour. One patient who had factor VIII inhibitor was given factor IX concentrates, 100 units/kg with activated prothrombin complex (FEIBA ), 75 units/kg at every 12 hours. All except one with hematoma in cerebellar vermis and the third ventricle are alive without any neurologic sequela.
Conclusion:ICH in hemophilia patients can be severe with no trauma history, or can be recurred several times with life-threatening event. So, it is desirable to have early treatment, prophylaxis, regular follow-up and patient education about abnormal symptoms to reduce the complications of them.
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KEYWORD
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Hemophilia A, Inhibitor, Intracranial hemorrhage, Prophylactic therapy
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