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体重和体脂肪质量指数是预测成人甲型血友病患者体内因子 VIII 恢复的强预测因子。

Body weight and fat mass index as strong predictors of factor VIII in vivo recovery in adults with hemophilia A.

机构信息

Institute of Health and Society, Université catholique de Louvain, Brussels, Belgium.

出版信息

J Thromb Haemost. 2011 Sep;9(9):1784-90. doi: 10.1111/j.1538-7836.2011.04431.x.

Abstract

BACKGROUND

The treatment of hemophilia A requires infusions of factor VIII (FVIII) concentrates. The number of units to be given in order to obtain the target level is calculated using the formula: [body weight (BW) × desired FVIII increase]/2, which assumes that each unit infused per kg of BW increases the FVIII level by 2%.

OBJECTIVES

The present observational study evaluated the dependence of FVIII recovery on different morphometrical variables: BW, fat mass index (FMI), body mass index, and the difference between actual and ideal BW.

PATIENTS AND METHODS

FVIII recovery was measured in 46 non-actively bleeding hemophilia A patients, being treated with a recombinant FVIII concentrate. Regression trees were used to identify morphometrical predictors of recovery.

RESULTS

The median recovery was 2.08 for all patients, 2.63 for those with a BW ≥ 81.0 kg and 1.87 for others (P < 0.001). The recovery was significantly higher when FMI was ≥ 20% compared with FMI < 15% (median recovery: 2.35 vs. 1.74; P = 0.007). Using regression trees, three groups were created: BW < 80.5 kg and FMI < 22.3%, BW < 80.5 kg and FMI ≥ 22.3% and BW ≥ 80.5 kg. Median recovery in these groups was 1.80, 2.16 and 2.63, respectively (P < 0.001).

CONCLUSIONS

The dose calculation of FVIII should take into account both BW and FMI, and be adapted to underweight or overweight patients. Comparison of the average recovery after different FVIII concentrates should keep in mind morphometrical patient characteristics.

摘要

背景

治疗甲型血友病需要输注凝血因子 VIII(FVIII)浓缩物。为了达到目标水平,所需给予的单位数是通过公式计算的:[体重(BW)×所需 FVIII 增加量]/2,该公式假设每公斤 BW 输注的单位数可使 FVIII 水平增加 2%。

目的

本观察性研究评估了 FVIII 恢复对不同形态变量的依赖性:BW、脂肪质量指数(FMI)、体重指数以及实际 BW 与理想 BW 之间的差异。

患者和方法

对 46 名非活动性出血甲型血友病 A 患者进行了 FVIII 恢复的测量,他们正在接受重组 FVIII 浓缩物的治疗。回归树用于确定恢复的形态学预测因子。

结果

所有患者的中位数恢复为 2.08,BW≥81.0kg 的患者为 2.63,其他患者为 1.87(P<0.001)。与 FMI<15%相比,FMI≥20%时恢复明显更高(中位数恢复:2.35 vs. 1.74;P=0.007)。使用回归树,创建了三组:BW<80.5kg 且 FMI<22.3%,BW<80.5kg 且 FMI≥22.3%和 BW≥80.5kg。这些组的中位数恢复分别为 1.80、2.16 和 2.63(P<0.001)。

结论

FVIII 的剂量计算应同时考虑 BW 和 FMI,并适应于体重不足或超重的患者。比较不同 FVIII 浓缩物后的平均恢复应考虑患者的形态学特征。

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