| 摘要: |
| 目的调查浙江血友病患儿的靶关节、靶关节相关因素、治疗现状和生活质量情况。方法对血友病诊疗中心登记的187例血友病患儿进行调查，总结血友病患儿的基本情况、靶关节状况及相关危险因素、治疗状况，采用CHO-KLAT评估患儿生活质量。结果共纳入187例患儿，靶关节发生率为44.4%（83/187），血友病A（HA）患儿存在靶关节66例（41.5%），血友病B（HB）患儿存在靶关节17例（60.7%）；年龄与靶关节呈正相关（r=0.313，P＜0.05）。35例（22.0%）HA患儿、4例（14.3%）HB患儿接受足量按需治疗。38例（23.9%）HA患儿接受预防治疗，5例接受足量预防治疗；5例（17.9%）HB患儿接受预防治疗。4岁及以上患儿及家长接受CHO-KLAT生活质量评估，儿童册得分为（58.17±10.33）分，家长册得分为（55.97±10.59）分，儿童得分高于家长（P＜0.05），且两者呈正相关（r=0.504，P＜0.05）。结论浙江省血友病儿童靶关节发生率高，其中HB比HA患儿更高，随年龄增大，靶关节发生率增高；大部分患儿出血后按需治疗不充分，在HB患儿中尤为突出，预防治疗比例不高，且预防治疗以小剂量预防为主。 |
| 关键词: 血友病 预防治疗 靶关节 CHO-KLAT |
| DOI：10.12056/j.issn.1006-2785.2017.39.9.2017-805 |
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| Status of target joint and quality of life in children with hemophilia |
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WANG Mengyuan, YAO Min, WAN Dandan, SHEN Juan, XU Weiqun
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the Children Hospital of Zhejiang University School of Medicine
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| Abstract: |
| Objective To analyze the status of target joint and quality of life in children with hemophilia. Methods A survey was conducted among 187 hemophilia children registered in Hemophilia Treatment Center, Children's Hospital affiliated to Zhejiang University School of Medicine between October 2013 and April 2016. The prevalence, treatment and associated risk factors of target joint were documented and the quality of life of hemophilia children was evaluated with CHO-KLAT2.0 questionnaire. Results The overall prevalence of target joint was 44.4% (83/187), and 41.5% (66/159) in hemophilia A (HA) patients and 60.7% (17/28) in hemophilia B (HB) patients, respectively. The age of patients was associated with the prevalence of
target joint (r=0.313, P＜0.05). For episodic treatment, only 22.0% (35/159) HA and 14.3% (4/28) HB patients received full dose.
23.9% (38/159) HA patients received prophylaxis, 5 of whom were on full dose and the rest on low dose; 17.9% (5/28) HB patients received prophylaxis and all on low dose. The CHO-KLAT scores reported from boys (58.17±10.33) and from parents (55.97± 10.59) were similar (r=0.504, P＜0.05). Conclusion Prevalence of target joint is high in hemophilia children and it is higher in HB than HA and increased with age. Most children did not receive full dose replacement therapy for episodic treatment, especially in HB, few children receive prophylaxis and most of them are on low dose. The quality of life of patients as assessed from children and their parents is less satisfactory. |
| Key words: Hemophilia Prophylaxis Target joint CHO-KLAT |
