Hill I D, MacDonald W B, Bowie M D, Ireland J D
Department of Paediatrics and Child Health, University of Cape Town.
S Afr Med J. 1988 Feb 6;73(3):147-9.
A retrospective study of all patients with cystic fibrosis (CF) seen at the Red Cross War Memorial Children's Hospital was undertaken. A total of 106 patients were identified and of these 64 still attend the CF clinic regularly. By 1 year of age 95% had symptoms but only 63% had been correctly diagnosed. In 27% the diagnosis was only made after 5 years of age. Chronic lung disease, failure to thrive, and diarrhoea or steatorrhoea were the most common presenting symptoms. Clinical scores of those still attending the clinic show that 79% are in the mild or better categories. The overall mortality rate was 21% and death was due to severe chest disease in the vast majority of cases. On the basis of the number of new patients with CF born during a 4-year period and the total number of live births recorded in this time, the calculated incidence of CF in Cape Town was 1/2,000 for whites and 1/12,000 for coloureds. Calculated prevalence rates for both whites and coloureds were much higher in urban Cape Town than in the rural areas of the western Cape. This suggests that many cases remain undiagnosed in the rural areas.
对在红十字战争纪念儿童医院就诊的所有囊性纤维化（CF）患者进行了一项回顾性研究。共确定了106例患者，其中64例仍定期前往CF门诊。到1岁时，95%的患者出现了症状，但只有63%得到了正确诊断。27%的患者直到5岁以后才被确诊。慢性肺病、发育不良以及腹泻或脂肪泻是最常见的首发症状。仍在门诊就诊的患者的临床评分显示，79%属于轻度或更好的类别。总死亡率为21%，绝大多数病例的死亡原因是严重的胸部疾病。根据4年期间出生的CF新患者数量以及同期记录的活产总数，计算出开普敦白人CF的发病率为1/2000，有色人种为1/12000。开普敦市区白人和有色人种的计算患病率均远高于西开普农村地区。这表明农村地区有许多病例仍未被诊断出来。
