All issues > Volume 35(11); 1992

Original Article

J Korean Pediatr Soc. 1992;35(11):1501-1508. Published online November 15, 1992.

Clinical Characteristics in Hemophilia Patients

Kun Soo KS Lee¹, Eun Sook ES Kim¹, Jang Soo JS Seo²

¹Department of Pediatrics, School of Medicine, Kyungpook National University, Taegu, Korea
²Department of Clinical Pathology, School of Medicine, Kyungpook National University, Taegu, Korea

Abstract

The clinical characteristics and laboratory data in the twenty six patients of hemophilia diagnosed in the Department of Pediatrics, Kyungpook National University Hospital for 8 years from 1983 were evaluated. The following results were obtained: Twenty one cases (80.7%) were diagnosed as hemophilia A and five cases (19.3%) as hemophilia B. Eleven cases (42.3%) were diagnosed under one year of age and the mean age was 8.2 years. All patients were male. Eight cases (50%) had a family history including brother (5 cases), maternal cousin (4 cases), maternal uncle (3 cases). There was no correlation between the family history and the severity of disease. Most common initial symptom was easy bruising (69.2%), then bleeding after trauma were in 3 cases (11.5%), hemarthrosis in 2 cases (7.6%), intracranial hemorrhage in 1 case (3.8%), and nasal bleeding in 1 case (3.8%). According to the level of factor VIII or IX, mild hemophilia were in 15 cases (57.7%), moderate in 6 cases (23.1%) and severe in 5 cases (19.2%). In severe and moderate caeses, all patients had been diagnosed under one year of age with a mean of 5.6 months in severe and 6.5 months in moderate. But 46.7% of mild cases were diagnosed after one year with a mean of 4.3 years. This is statistically significant with p value below 0.005. Activated partial thromboplastin time (aPTT, normal range of 20 to 35 seconnds) was prolonged in 24 cases (92.4%), but in two cases (7.6%) aPTT was normal. The level of factor VIII in these two were 28% and 29%. There was no correlation between the level of factor VIII or IX and aPTT. The complications were intracranial hemorrhage in 3 cases and limping gate from repeated hemoarthroses in 2 cases. Serologic tests revealed positive HBs antigen in 1 case, positive HBs antibody in 10 cases, positive HCV antibody in 8 cases and positive HIV antibody in 3 cases.

Keywords :Hemophilia A and B, aPTT, Factor assay