Eggert W, Bernardino L, de Cruz M, Eggert S, Schmidt G
Klinik und Poliklinik für Kinderheilkunde, des Bereichs Medizin (Charité), Humboldt-Universität zu Berlin.
Folia Haematol Int Mag Klin Morphol Blutforsch. 1987;114(6):857-65.
In a period of 6 years 819 children affected with drepanocytosis (S. E.) were treated at the children's clinic in Luanda. At the time of diagnosis the age of the 457 boys amounted to 3.4 +/- 2.7 years and that of girls to 4.3 +/- 3.4 years. The age distribution shows that 40.2% of the patients were in their first 2 years of life. Conditions of crisis which affected 70% of the diseased children are typical of this disease. The family anemnesis demonstrated that 13.6% of all brothers and sisters covered were HbSS positive. 34.1% of them died at an age of 3.5 +/- 3.5 years (boys) or 2.4 +/- 2.3 years (girls) respectively. The physical development measured in the body mass shows that it is significantly diminished in comparison with healthy children of the same age. The characteristic symptom of this disease, splenomegaly, existed in 27.8% of the children with a mean age of 2.8 +/- 2.3 years. In one age group of 4.4 +/- 2.6 years it could even be identified in 11.3% of the cases.
在6年的时间里，罗安达儿童诊所对819名镰状细胞贫血症（S.E.）患儿进行了治疗。确诊时，457名男孩的年龄为3.4±2.7岁，女孩的年龄为4.3±3.4岁。年龄分布显示，40.2%的患者年龄在2岁以下。影响70%患病儿童的危象情况是这种疾病的典型症状。家族病史显示，在所有接受调查的兄弟姐妹中，13.6%的人血红蛋白SS呈阳性。其中34.1%的人分别在3.5±3.5岁（男孩）或2.4±2.3岁（女孩）时死亡。通过体重测量的身体发育情况表明，与同龄健康儿童相比，患病儿童的身体发育明显减缓。这种疾病的典型症状脾肿大存在于27.8%的儿童中，这些儿童的平均年龄为2.8±2.3岁。在一个平均年龄为4.4±2.6岁的年龄组中，甚至有11.3%的病例出现脾肿大。
