Polycentric analysis of current situation of diagnosis of haemophilia in Chinese children
10.3760/cma.j.issn.2095-428X.2017.05.011
- VernacularTitle:中国儿童血友病诊断现状多中心研究
- Author:
Min ZHOU
;
Ling TANG
;
Runhui WU
;
Weiqun XU
;
Guoping HAO
;
Yuan LU
;
Huan WANG
;
Wanli LI
;
Ying WANG
;
Yongchun SU
;
Shuhong WANG
;
Qian FANG
;
Jianping LIU
;
Xiaoqin FENG
;
Xiaojing LI
- Keywords:
Haemophilia;
Child;
Diagnosis
- From:
Chinese Journal of Applied Clinical Pediatrics
2017;32(5):361-364
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the diagnostic status of haemophilia in Chinese children in recent years,and to provide information for increasing the life quality of children with haemophilia in China.Methods The pediatric haemophilia cases registration data were collected and analyzed by using questionnaire,from January 1,2008 to March 30,2014 in 13 haemophilia treatment centers of haemophilia treatment center collaboration network of China pediatric group.These centers were from 12 provinces / municipalities.Results A total of 554 cases were collected;median age was 7.0 years old(0.1 to 17.9 years old);among them,481 cases(86.8%) were hemophilia A,and 73 cases were hemophilia B (13.2%);55 mild cases (9.9%),290 moderate cases (52.4%),and 209 severe cases (37.7%);162 cases(29.2%) had family history,the other 392 cases(70.8%) had no family history.The diagnosis was made at a median age of 12.0 month-old(0 to 180.0 months);the diagnosis time was 0.5 months(0 to 144.0 months) after the first bleeding;diagnosis timing with short interval was in 356 cases(64.3%),long interval was in 198 cases(35.7%).The diagnostic timing was not correlated with disease severity (P =0.812) or the family history (P =0.243);but correlated with the severity of first bleeding(P =0.027) and per capita gross domestic product (P < 0.01) in patients' residence.From 1996 to 2013,the annual number of newly diagnosed cases was increasing year by year,with a higher proportion of short interval of diagnose timing.Conclusions With development of hemophilia work in China,the number of diagnosis of haemophilia children is increasing year by year.Moderate and severe hemorrhage are both taken seriously and diagnosed timely.But the diagnosis is delayed in some children.Chinese haemophilia work still need to be strengthen and the propaganda and diagnostic technology are to be popularized.