Analysis of hemophilia case information of Shandong province / 中华医学遗传学杂志

ABSTRACT

<p><b>OBJECTIVE</b>To summarize the general condition, regional distribution, prevalence and clinical characteristics of Shandong province based on hemophilia case registry information.</p><p><b>METHODS</b>A retrospective study was carried out on 1979 hemophilia patients registered at Shandong Hemophilia Registration Center.</p><p><b>RESULTS</b>The 1979 cases have included 1704 hemophilia A and 275 hemophilia B patients. Hemophilia A was characterized as severe in 1021 patients (59.9%), moderate in 483 patients (28.4%), and mild in 200 patients (11.7%); while hemophilia B was characterized as severe in 125 patients (45.4%), moderate in 116 patients (42.2%), and mild in 34 patients (12.4%). The median age was 23 years (ranging from 1 month to 81 years), and most were young patients. Joint deformity occurred in 963 patients, and 948 patients had a family history of hemophilia. All counties of Shandong province had patients except for Changdao county and Fushan district of Yantai city. The prevalence of Heze city and Dongying city (3.39/100 000 and 3.05/100 000, respectively) were relatively higher.</p><p><b>CONCLUSION</b>The above data revealed epidemiological and clinical characteristics of Shandong Province. Patient-centered registry system allowed a more detailed and accurate patient information, and promoted the comprehensive care of hemophilia, which also suggested the necessity for the establishment and improvement of the National Hemophilia Registry System.</p>