Foetal Haemoglobin and Disease Severity in Nigerian Children with Sickle Cell Anaemia
Foetal Haemoglobin and Disease Severity in Nigerian Children with Sickle Cell Anaemia
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DOI：
10.4084/mjhid.2017.063
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发表时间：
2017-11-01
影响因子：
3.2
通讯作者：
Oseni, Saheed B. A.
中科院分区：
文献类型：
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作者：
Adeodu, Oluwagbemiga O.;Akinlosotu, Morenike A.;Oseni, Saheed B. A.
背景：胎儿血红蛋白（HbF）是影响镰状细胞病（SCD）严重程度的主要修饰因子。尽管如此，在尼日利亚并不常规进行HbF估计。HbF和SCD严重程度之间的关系，受影响的children也很差study.Methods：在这个描述性的横断面研究，我们确定了稳态HbF水平和疾病严重程度之间的关系，尼日利亚儿童1 - 15岁的纯合子SCD。对于每一个孩子，社会人口统计学特征和SCD的临床严重程度进行了确定。后者是根据过去12个月内显著疼痛发作、输血和住院的频率进行评估的; SCD相关并发症的终生累积发生率;脾脏和肝脏肿大的程度;目前的红细胞压积和白细胞计数。胎儿血红蛋白水平进行了定量与高效液相chromatography.Results：平均HbF水平的105名儿童与SCA为9.9 +/-6.0%。男性的平均HbF水平显著低于女性，8.0 +/- 5.6%对12.2 +/- 5.8%（p < 0.001）。没有一个孩子有严重的疾病。然而，32名中度疾病儿童的平均胎儿血红蛋白水平显著低于73名轻度疾病儿童（分别为7.7 +/- 5.6%和10.8 +/- 6.0%）。平均HbF水平也显着降低，在儿童谁有一个历史的急性胸部综合征和中风相比，没有这些并发症，p = 0.002和0.010.Conclusion：儿童SCA谁有中度疾病和那些有危及生命的并发症，如中风和急性胸部综合征的历史有显着低HbF水平。因此，建议提供早期定量胎儿血红蛋白和HbF诱导的设施，以降低这些儿童的发病率和死亡率。
Background: Foetal haemoglobin (HbF) is a major modifying factor influencing sickle cell disease (SCD) severity. Despite this, HbF estimation is not routinely done in Nigeria. The relationship between HbF and SCD severity among affected children is also poorly studied.Methods: In this descriptive cross-sectional study, we determined the relationship between steady state HbF levels and disease severity of Nigerian children aged 1 - 15 years with homozygous SCD. For each child, the socio-demographic characteristics and SCD clinical severity were determined. The latter was assessed based on the frequency of significant painful episodes, blood transfusion, and hospitalisation in the preceding 12 months; lifetime cumulative incidence of SCD-related complications; the degree of splenic and hepatic enlargement; current haematocrit and leucocyte count. Foetal haemoglobin levels were quantified with high-performance liquid chromatography.Results: The mean HbF level of the 105 children with SCA was 9.9 +/- 6.0%. Male had significantly lower mean HbF levels than females, 8.0 +/- 5.6% vs. 12.2 +/- 5.8% (p < 0.001). None of the children had severe disease. However, the 32 children with moderate disease had significantly lower mean foetal haemoglobin levels than the 73 with mild disease (7.7 +/- 5.6% vs 10.8 +/- 6.0% respectively). The mean HbF level was also significantly lower in children who had a history of acute chest syndrome and stroke compared to those without these complications, p = 0.002 and 0.010 respectively.Conclusion: Children with SCA who had a moderate disease and those with a history of life-threatening complications such as stroke and acute chest syndrome had significantly low HbF levels. Therefore, it is recommended that facilities for early quantification of foetal haemoglobin and HbF inducement were made available to reduce the morbidity and mortality among these children.