Clinical correlates of steady-state oxyhaemoglobin desaturation in children who have sickle cell disease

Clinical correlates of steady-state oxyhaemoglobin desaturation in children who have sickle cell disease
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DOI:
10.1111/j.1365-2141.2005.05738.x
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发表时间:
2005-10-01
影响因子:
6.5
通讯作者:
Ahmad, N
Ahmad, N
中科院分区:
医学2区
文献类型:
--
作者:
Quinn, CT;Ahmad, N

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镰状细胞病(SCD)患者在稳定状态下可能会出现氧合血红蛋白去饱和,其原因尚不完全清楚。我们研究了一组585名患有镰状细胞贫血(SS)、镰状β(0)-地中海贫血(S β(0))、镰状血红蛋白C病(SC)或镰状β(+)-地中海贫血(Sb+),以确定稳态氧合血红蛋白饱和度(SpO(2))与SCD基因型、年龄、性别、稳态血红蛋白(Hb)和网织红细胞计数之间的关系,急性胸部综合征(ACS)的发生率。SS/S β(0)组(n 390)的平均SpO(2)低于SC/S β(+)组(n 195)(96.3% vs. 98.7%,P < 0.001)。在SS/Sb-0受试者中,稳态SpO(2)降低与Hb降低、网织红细胞增加、年龄较大和男性相关。在SC/Sb+组中未发现这些相关性。既往ACS与稳态SpO无关(2)。多变量模型解释了SpO(2)中45%的变异性,但Hb仅解释了SpO(2)中5%的变异性。我们的结论是稳态去饱和在SCD患者中很常见,但它似乎与之前的ACS发作无关,并且很大程度上无法用慢性贫血解释。
Individuals with sickle cell disease (SCD) may have oxyhaemoglobin desaturation during the steady-state, the causes of which are incompletely known. We studied a cohort of 585 children who have sickle cell anaemia (SS), sickle beta(0)-thalassaemia (S beta(0)), sickle-haemoglobin C disease (SC), or sickle beta(+)-thalassaemia (Sb+) to determine the relationships between steady-state oxyhaemoglobin saturation (SpO(2)) and SCD genotype, age, gender, steady-state haemoglobin (Hb) and reticulocyte count, and rate of acute chest syndrome (ACS). The SS/S beta(0) group (n 390) had lower mean SpO(2) than the SC/S beta(+) group (n 195) (96.3% vs. 98.7%, P < 0.001). Among SS/Sb-0 subjects, a decrease in steady-state SpO(2) correlated with a decrease in Hb, an increase in reticulocytes, older age and male gender. These correlations were not found in the SC/Sb+ group. Prior ACS did not correlate with steady-state SpO(2). A multivariate model explained 45% of the variability in SpO(2), but only 5% of the variation in SpO(2) was explained by Hb. We conclude that steady-state desaturation is common in individuals with SCD, but it appears to be unrelated to prior episodes of ACS and largely unexplained by chronic anaemia.