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比较 A 型和 B 型血友病患者的出血频率和因子浓缩物使用情况。

Comparing bleed frequency and factor concentrate use between haemophilia A and B patients.

机构信息

Hamilton Health Sciences, McMaster University, Hamilton, ON L8N3Z5, Canada.

出版信息

Haemophilia. 2011 Nov;17(6):872-4. doi: 10.1111/j.1365-2516.2011.02506.x. Epub 2011 Feb 22.

Abstract

Haemorrhagic manifestations in patients with haemophilia A and B are considered quite similar for comparable level of factor deficiency. We investigated the bleeding frequency and factor usage between HA and HB patients with comparable disease severities. We collected data on frequency of bleeds and factor concentrate utilization over 3 years, from January 2001 to December 2003. Information was gathered from home infusion logs recorded by patients or their parents, and treatment records from the Hemophilia Clinic or the Hospital Emergency Department. Data were available on 58 patients with severe HA (FVIII < 0.01 U mL(-1)), 10 with moderate HA (FVIII < 0.05 U mL(-1)), 15 with severe HB, and five with moderate HB who required treatment for episodic bleeds, postoperative haemostasis and for primary or secondary prophylaxis. The HA patients bled more frequently than HB patients (14.4 vs. 8.63 bleeds/patient/year), but used similar amounts of concentrate per year. HA patients underwent surgical procedures 3.2 times more frequently than HB patients to correct musculoskeletal complications. A total of 21,363,409 IU of recombinant FVIII was used by patients with HA (104,722 IU/patient/year) and 6,430, 960 IU of recombinant factor IX, by patients with HB (107,182 IU/patient/year). The difference in factor concentrate usage is not statistically significant (P > 0.05). The decrease in bleed frequency in haemophilia B indicates that the conclusions from randomized trials of prophylaxis in HA may not be accurately applied to HB.

摘要

甲型和乙型血友病患者的出血表现被认为在相当程度的因子缺乏时非常相似。我们研究了具有可比疾病严重程度的 HA 和 HB 患者的出血频率和因子使用情况。我们收集了 2001 年 1 月至 2003 年 12 月 3 年期间的出血频率和因子浓缩物使用数据。信息来自患者或其父母记录的家庭输注日志,以及血友病诊所或医院急诊部的治疗记录。有 58 名严重甲型血友病(FVIII < 0.01 U mL(-1))、10 名中度甲型血友病(FVIII < 0.05 U mL(-1))、15 名严重乙型血友病和 5 名需要治疗间歇性出血、术后止血以及原发性或继发性预防的中度乙型血友病患者的数据。HA 患者的出血频率高于 HB 患者(14.4 比 8.63 次/患者/年),但每年使用的浓缩物量相似。HA 患者因纠正肌肉骨骼并发症而接受手术的次数是 HB 患者的 3.2 倍。HA 患者共使用了 21363409IU 重组 FVIII(104722IU/患者/年),HB 患者使用了 6430960IU 重组因子 IX(107182IU/患者/年)。因子浓缩物使用量的差异无统计学意义(P > 0.05)。乙型血友病出血频率的降低表明,在 HA 中预防随机试验的结论可能不能准确应用于 HB。

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