Cela Elena, Vélez Ana G, Aguado Alejandra, Medín Gabriela, Bellón José M, Beléndez Cristina
Hematología Pediátrica, Hospital General Universitario Gregorio Marañón, Facultad de Medicina, Universidad Complutense de Madrid, Madrid, España.
Estudiante de pregrado de Medicina, Hospital General Universitario Gregorio Marañón, Facultad de Medicina, Universidad Complutense de Madrid, Madrid, España.
Med Clin (Barc). 2016 Dec 16;147(12):531-536. doi: 10.1016/j.medcli.2016.07.028. Epub 2016 Sep 29.
Sickle cell anaemia causes progressive organ damage. The objective is to describe school performance of patients with sickle cell anaemia and their clinical parameters and quality of life that may have an influence. The hypothesis is that if school alterations occur without other objective data, additional factors must be present besides the disease itself.
Transversal study performed in November 2015 considering analytical variables, complications and neuroradiological images of children with sickle cell anaemia, and family survey on school performance and quality of life.
Median age was 6.8 years and 78% were diagnosed at birth. Sixty patients were included. School performance was altered in 51% of cases and was related to nocturnal hypoxemia. Acute stroke incidence was 6.7%. Transcranial ultrasound was abnormal in 4% of cases and magnetic resonance imaging in 16% of cases. Quality of life showed pathological findings in all areas and the low values increased proportionally in older ages. The stroke affected the physical and social sphere, and lung disease affected the physical and emotional spheres.
Poor school performance affects half of the patients and it is related to nocturnal hypoxemia, although other socio-cultural factors may have an influence. Quality of life is affected in most of these cases independently of academic results. The absence of alterations in neuroimaging or the apparent lack of severe clinical parameters do not mean that quality of life and schooling are normal.
镰状细胞贫血会导致进行性器官损伤。目的是描述镰状细胞贫血患者的学业表现以及可能产生影响的临床参数和生活质量。假设是，如果在没有其他客观数据的情况下出现学业改变，那么除了疾病本身之外，必然还存在其他因素。
2015年11月进行横断面研究，分析镰状细胞贫血患儿的变量、并发症和神经放射影像，并对其学业表现和生活质量进行家庭调查。
中位年龄为6.8岁，78%在出生时被诊断出患病。纳入60例患者。51%的病例存在学业表现改变，且与夜间低氧血症有关。急性卒中发生率为6.7%。4%的病例经颅超声异常，16%的病例磁共振成像异常。生活质量在所有领域均显示出病理结果，且低值在年龄较大时成比例增加。卒中影响身体和社会领域，肺部疾病影响身体和情感领域。
学业表现不佳影响一半的患者，且与夜间低氧血症有关，尽管其他社会文化因素可能也有影响。在大多数此类病例中，生活质量受到影响，与学业成绩无关。神经影像学未显示改变或明显缺乏严重临床参数并不意味着生活质量和学业正常。
