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首页> 外文期刊>Pediatric blood & cancer >Children with HbSβ 0 0 thalassemia have higher hemoglobin levels and lower incidence rate of acute chest syndrome compared to children with HbSS
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Children with HbSβ 0 0 thalassemia have higher hemoglobin levels and lower incidence rate of acute chest syndrome compared to children with HbSS

机译：与HBSS的儿童相比，HBSβ00 0月份的儿童血红蛋白血症具有更高的血红蛋白水平和急性胸综合征的发病率降低

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Abstract Background Based on the presumed clinical similarity between the two most severe sickle cell disease (SCD) genotypes, hemoglobin (Hb) Sβ 0 thalassemia and HbSS, randomized controlled trials (RCTs) have included both genotypes. Our group has demonstrated that healthcare providers inadequately distinguish the two diagnoses through clinical and laboratory parameters. Procedure In a retrospective cohort study utilizing data from the Silent Cerebral Infarct Transfusion trial (NCT00072761), we tested the hypothesis that children with genotypic diagnoses of HbSβ 0 thalassemia, when compared to HbSS, have significantly different rates of SCD comorbidities. Exclusion criteria included those with previous overt stroke or treatment with hydroxyurea or regular blood transfusion therapy. Results Among children with HbSβ 0 thalassemia (n?=?22) and HbSS (n?=?786), the mean hemoglobin was higher in children with HbSβ 0 thalassemia (9.2?g/dl) compared to HbSS (8.1?g/dl, P ??0.001). In children with HbSβ 0 thalassemia, when compared to HbSS, the incidence rate of acute chest syndrome (ACS) was 3.0 and 14.4 events per 100 patient‐years ( P ?=?0.028), and mean transcranial Doppler (TCD) velocities were 112.6 and 135.6?cm/sec, respectively ( P ?=?0.026). The number of children with HbSβ 0 thalassemia and HbSS with conditional TCD velocities were zero (0%) and 26 (4.9%), respectively ( P ?=?1.00), and the number with silent cerebral infarcts were five (27.8%) and 209 (30.8%), respectively ( P ?=?0.78). Conclusions We have provided preliminary evidence that clinically relevant differences occur in ACS rates and TCD velocities between children with HbSβ 0 thalassemia and HbSS. Future SCD RCTs should consider balanced allocation of these SCD genotypes, particularly when ACS and abnormal TCD velocities are primary outcome measures.
机译：摘要背景基于预测的临床相似性两种最严重的镰状细胞疾病（SCD）基因型，血红蛋白（HB）Sβ0的血红蛋白和HBSS，随机对照试验（RCT）包括两个基因型。我们的小组已经证明，医疗保健提供者通过临床和实验室参数来区分两种诊断。在叙述队列中的过程中利用来自无声脑梗死输血试验（NCT00072761）的数据，我们测试了与HBSS相比，HBSβ0的基因型诊断儿童的假设具有显着不同的SCD肝脏率。排除标准包括先前公开中风或用羟基脲或常规输血治疗的治疗方法。结果HBSβ0的儿童（N？= 22）和HBSS（N？=β786），与HBSS相比，HBSβ0的儿童（9.2μlΩ血管血症儿童（9.2μlΩ·g / dl），平均血红蛋白dl，p？0.001）。在HBSβ0的儿童中，与HBSS相比，急性胸部综合征（ACS）的发生率为3.0和14.4每100例患者年龄（P？= 0.028），平均经颅多普勒（TCD）速度为112.6分别为135.6？cm / sec（p？= 0.026）。 HBSβ0的儿童数量和有条件TCD速度的HBSβ0和HBSS分别为零（0％）和26（4.9％）（P？=？1.00），静音脑梗死的数量为五（27.8％）和209（30.8％）分别（p？= 0.78）。结论我们提供了初步证据，即临床相关差异在ACS率和HBSβ0的儿童与HBSS之间的TCD速度发生。未来的SCD RCT应该考虑平衡分配这些SCD基因型，特别是当ACS和异常的TCD速度是主要结果措施时。

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