Hulbert Monica L, Panepinto Julie A, Scott J Paul, Liem Robert I, Cook Lawrence J, Simmons Timothy, Brousseau David C
Department of Pediatrics, Washington University School of Medicine, St Louis, Missouri.
Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin.
Transfusion. 2017 Aug;57(8):1891-1897. doi: 10.1111/trf.14155. Epub 2017 May 12.
Little is known about red blood cell (RBC) transfusion practices for children hospitalized for a sickle cell vaso-occlusive pain crisis (VOC). We hypothesized that transfusion would be associated with the development of acute chest syndrome (ACS), lower hemoglobin (Hb) concentration, and lack of hydroxyurea therapy.
This is a secondary analysis of all children admitted for a sickle cell pain crisis enrolled in the Magnesium in Crisis (MAGiC) randomized trial; all had HbSS or S-β thalassemia. ACS development and transfusion administration were prospectively collected during the parent trial. All Hb values during the hospitalization were recorded, as was parent report of child receiving hydroxyurea. Relative risks (RRs) of transfusion were compared between groups.
Of 204 enrolled children, 40 (19.6%) received a transfusion. Of the 30 children who developed ACS, 22 (73.3%) received transfusions compared to 18 of 174 (10.3%) without ACS: the RR of transfusion in children with ACS was 7.1 (95% confidence interval [CI], 4.4-11.5). Among those without ACS, the lowest Hb was most strongly associated with transfusions: RR was 3.1 (95% CI 2.0 - 4.7) for each 1 g/dL decrease in lowest Hb. In a binary recursive partitioning model for those without ACS, a lowest recorded Hb level of less than 6.3 g/dL was significantly associated with transfusion during admission (p < 0.01). Hydroxyurea use was not associated with transfusions in any analysis.
ACS increased the RR of transfusion in children hospitalized for VOC sevenfold. In children without ACS, transfusion was associated with lowest Hb concentration, particularly Hb concentration of less than 6.3 g/dL.
对于因镰状细胞血管阻塞性疼痛危象（VOC）住院的儿童，关于其红细胞（RBC）输血治疗的情况知之甚少。我们推测输血与急性胸综合征（ACS）的发生、较低的血红蛋白（Hb）浓度以及未接受羟基脲治疗有关。
这是对参与“危机中的镁”（MAGiC）随机试验的所有因镰状细胞疼痛危象入院儿童的二次分析；所有患儿均为HbSS或S-β地中海贫血。在原试验期间前瞻性收集ACS的发生情况和输血情况。记录住院期间的所有Hb值，以及患儿接受羟基脲治疗的家长报告。比较各组输血的相对风险（RRs）。
在204名登记患儿中，40名（19.6%）接受了输血。在发生ACS的30名患儿中，22名（73.3%）接受了输血，而在未发生ACS的174名患儿中有18名（10.3%）接受了输血：发生ACS患儿输血的RR为7.1（95%置信区间[CI]，4.4 - 11.5）。在未发生ACS的患儿中，最低Hb与输血的相关性最强：最低Hb每降低1 g/dL，RR为3.1（95% CI 2.0 - 4.7）。在针对未发生ACS患儿的二元递归划分模型中，入院期间记录的最低Hb水平低于6.3 g/dL与输血显著相关（p < 0.01）。在任何分析中，羟基脲的使用与输血均无关联。
ACS使因VOC住院儿童的输血RR增加了7倍。在未发生ACS的儿童中，输血与最低Hb浓度有关，尤其是Hb浓度低于6.3 g/dL。
