Berchel C, Diara J P, Loret H, Foucan L, Le Turdu C, Samuel Y
Service de pédiatrie, centre hospitalier régional et universitaire, Pointe-à-Pitre, Guadeloupe.
Rev Prat. 1992 Oct 1;42(15):1885-91.
Sickle-cell anaemia is characterized by three categories of clinical signs: anaemia, vaso-occlusive phenomena and infective complications, which are described here according to age. The natural history of the disease can be divided into four periods: the neonatal period which is asymptomatic but important to organize an effective protection; the first 5 years of life are characterized by a high risk of mortality, a high level of morbidity due to severe infections, episodes of acute anaemia and painful crises typical of that age-group; the life of older children and adolescents is dotted with painful crises; it is in this period that degenerative tissue pathology begins; in adulthood, the acute episodes are less frequent, but multiple complications develop. Some of them (cerebral vascular accidents or lung diseases) may be fatal, while others are the source of chronic and disabling lesions, notably ocular, orthopaedic and renal lesions, which affect the functional prognosis. Pregnancy remains a high risk. There is, therefore, a striking contrast between the basic physiopathological mechanism (polymerization of haemoglobin S) and the various clinical manifestations which depend on the type of haemoglobin, on the social and sanitary conditions in each country and on other reasons which remain to be elucidated.
贫血、血管阻塞现象和感染性并发症，本文将根据年龄对其进行描述。该疾病的自然史可分为四个阶段：新生儿期无症状，但对于组织有效的保护措施很重要；生命的前5年死亡率高，因严重感染、急性贫血发作和该年龄组典型的疼痛危象而发病率高；大龄儿童和青少年时期常有疼痛危象；正是在这个时期开始出现退行性组织病变；在成年期，急性发作较少，但会出现多种并发症。其中一些（脑血管意外或肺部疾病）可能致命，而另一些则是慢性致残性病变的根源，尤其是眼部、骨科和肾脏病变，这些会影响功能预后。妊娠仍然风险很高。因此，基本的生理病理机制（血红蛋白S的聚合）与取决于血红蛋白类型、每个国家的社会和卫生条件以及其他有待阐明的原因的各种临床表现之间存在显著差异。
