Children and adolescents with haemophilia in Hong Kong: An epidemiological and clinical review

Abstract

A survey was performed in late-2003 by questionnaire to all paediatric and adolescent departments under the Hospital Authority of Hong Kong to study the epidemiology and clinical status of local haemophilia patients under 19 years of age. A total of 90 patients were recruited, corresponding to a local prevalence of 6.4 per 100,000. Among these 90 patients, 83.3% were haemophilia A and 16.7% were haemophilia B cases. Maternal carrier status was uncertain in 44.6% of patients and genotypic assessment was not performed in most of these cases. None had received primary prophylaxis although 42.9% of them were severe haemophiliacs and 46.4% had already suffered from chronic arthropathy. Inhibitors were detected only in 8.1% of patients. None was infected with human immunodeficiency virus but hepatitis C antibody was detected in 12 and hepatitis B surface antigen was positive in two patients. All but one of the infected patients were older than 13 years with only one patient suffering from chronic hepatitis. Twelve patients had history of intra-cranial haemorrhage. While infective complications were uncommon in our young haemophilia patients, improvement in carrier detection, prevention and treatment of haemophilic arthropathy should be considered in the future development of local haemophilia care.