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To investigate the incidence, risk factors, clinical manifestations and prognosis of intracranial hemorrhage (ICH) in children with hemophilia A in a center of China, we conducted a retrospective analysis of 126 children with hemophilia A at our hospital in recent 4 years. Thirty-six children with hemophilia A (including 19 severe cases, and 17 moderate cases complicated with joint diseases) received low dose factor VIII (FVIII) prophylaxis, and none of them had ICH. However, 13 cases of hemophilia A not given prophylaxis were complicated with ICH (12 severe cases, and 1 moderate case) and demonstrated an incidence of 10.3% (13/126) in all patients, and 28.6% (12/42) in severe cases. Of the 13 cases, 9 severe ICH cases had a definite history of head injury, accounting for 69.2%. Headache was common in children >3 years, but somnolence, irritability, gaze or convulsions in children <3 years. The most common findings of cranial CT scan included intracranial hematoma (9/13), and less commonly observed were subependymal hemorrhage and intraventricular hemorrhage. After administration of FVIII, all patients survived. Hematoma of 6 cases was observed during CT reexamination after 1-3 months. During the follow-up period, only one case had slight activity limitation on one side of the limb, but steadily recovered. Besides the decreased concentration of FVIII, trauma is the most common risk factor of ICH in children with hemophilia A. The active treatment can improve the prognosis of ICH in children with hemophilia A.
This article was published in the following journal.
Name: Current medical science
Intracranial hemorrhage (ICH) is a life-threatening condition in children. Inherited bleeding disorders (IBD) have high risk of ICH.
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The classic hemophilia resulting from a deficiency of factor VIII. It is an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage.
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A deficiency of blood coagulation factor IX inherited as an X-linked disorder. (Also known as Christmas Disease, after the first patient studied in detail, not the holy day.) Historical and clinical features resemble those in classic hemophilia (HEMOPHILIA A), but patients present with fewer symptoms. Severity of bleeding is usually similar in members of a single family. Many patients are asymptomatic until the hemostatic system is stressed by surgery or trauma. Treatment is similar to that for hemophilia A. (From Cecil Textbook of Medicine, 19th ed, p1008)
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