Scott-Jupp R, Lama M, Tanner M S
Department of Child Health, University of Leicester.
Arch Dis Child. 1991 Jun;66(6):698-701. doi: 10.1136/adc.66.6.698.
To assess the prevalence, demography, and clinical features of liver disease among patients with cystic fibrosis the case notes of 524 patients of all ages who were attending the cystic fibrosis clinic were studied. Computer databases were used to establish the condition of the liver in a further 576 such patients. The overall prevalence of overt liver disease indicated by the presence of an enlarged liver or spleen (or both) was 4.2%. The age related prevalence rose to a peak in adolescence, and then fell in patients over 20 years old. The implied increase in mortality among those with liver disease was not explained by deaths from liver disease, which were rare. Male patients were significantly more affected than female, the ratio being 3:1 among adolescents. Increasing prevalence of liver disease in patients with cystic fibrosis is, therefore, not just a result of longevity.
为评估囊性纤维化患者中肝脏疾病的患病率、人口统计学特征及临床特点,我们研究了在囊性纤维化门诊就诊的524例各年龄段患者的病历。我们还利用计算机数据库对另外576例此类患者的肝脏状况进行了评估。肝脏或脾脏肿大(或两者皆有)所提示的显性肝脏疾病的总体患病率为4.2%。与年龄相关的患病率在青春期达到峰值,然后在20岁以上的患者中下降。肝病患者中隐含的死亡率增加并非由肝病死亡导致,因为肝病死亡很罕见。男性患者受影响的程度明显高于女性,在青少年中这一比例为3:1。因此,囊性纤维化患者中肝病患病率的增加不仅仅是寿命延长的结果。