Rogers D W, Clarke J M, Cupidore L, Ramlal A M, Sparke B R, Serjeant G R
Br Med J. 1978 Jun 10;1(6126):1515-6. doi: 10.1136/bmj.1.6126.1515.
In Jamaican children with homozygous sickle cell (SS) disease diagnosed at birth two-year survival was 87%, compared with 95% in children with sickle cell-haemoglobin C (CS) disease, and 99% in normal controls. Death among those with SS disease occurred most often between the ages of 6 and 12 months. Principal causes were acute splenic sequestration and pneumococcal infection. Neonatal diagnosis of haemoglobinopathies must be followed by close observation if mortality is to be reduced by early diagnosis and treatment of these complications.
在牙买加出生时被诊断为纯合子镰状细胞(SS)病的儿童中,两年生存率为87%,而镰状细胞-血红蛋白C(CS)病儿童的两年生存率为95%,正常对照组为99%。SS病患儿的死亡最常发生在6至12个月龄之间。主要原因是急性脾滞留和肺炎球菌感染。如果要通过对这些并发症的早期诊断和治疗来降低死亡率,那么对血红蛋白病进行新生儿诊断后必须进行密切观察。