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早期诊断和治疗对囊性纤维化的影响:一项对16对同胞兄弟姐妹的七年研究。

The effect of early diagnosis and treatment in cystic fibrosis: a seven-year study of 16 sibling pairs.

作者信息

Orenstein D M, Boat T F, Stern R C, Tucker A S, Charnock E L, Matthews L W, Doershuk C F

出版信息

Am J Dis Child. 1977 Sep;131(9):973-5. doi: 10.1001/archpedi.1977.02120220039005.

Abstract

Data on 16 sibling pairs with cystic fibrosis were analyzed to test the hypothesis that early treatment of this condition improves prognosis. Younger siblings' conditions were diagnosed before 1 year of age, usually before the onset of pulmonary disease. Older siblings' conditions were diagnosed after 1 year of age and after the onset of pulmonary disease. Although the sibling pairs received similar treatment, comparison at 7 years of age showed that the younger siblings had significantly better chest roentgenogram scores, total clinical scores, residual lung volumes, and ratios of residual volume to total lung volume. Younger siblings also required fewer hospital admissions to control their lung disease. The results suggest that, in general, early initiation of therapy is beneficial for patients with cystic fibrosis.

摘要

对16对患有囊性纤维化的同胞进行了数据分析,以检验该病早期治疗可改善预后这一假设。年幼同胞的病情在1岁之前被诊断出来,通常是在肺部疾病发作之前。年长同胞的病情在1岁之后且在肺部疾病发作之后被诊断出来。尽管这些同胞对接受了相似的治疗,但7岁时的比较显示,年幼同胞在胸部X光片评分、总临床评分、残气量以及残气量与肺总量的比值方面明显更好。年幼同胞控制肺部疾病所需的住院次数也更少。结果表明,总体而言,早期开始治疗对囊性纤维化患者有益。

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