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[囊性纤维化:在专业中心的长期治疗及其预后价值(作者译)]

[Cystic fibrosis: long-term treatment at a specialized centre, and its prognostic value (author's transl)].

作者信息

Tümmler B, von der Hardt H, Müller W, Rieger C

出版信息

Monatsschr Kinderheilkd. 1982 Mar;130(3):157-64.

PMID:6806608
Abstract

Data are presented on morbidity, mortality, and age at diagnosis of 90 patients with cystic fibrosis who were seen at the Medical School of Hannover from 1975 to 1981. The status of patients was characterized by the clinical score and the score of chest roentgenogram. Course and prognosis in groups of patients defined by the items "false diagnosis", "false negative sweat test", "meconium ileus", "late onset of clinical symptoms after age 5", and "comprehensive long-term treatment at a centre for cystic fibrosis" were compared. In patients with intestinal or pulmonary symptoms a delay in diagnosis for patients, who died, died in early childhood. However, for those patients who receive proper medical care at a specialized CF-centre a clinical course can be achieved which may resemble the course of disease in patients with late onset of symptoms.

摘要

本文呈现了1975年至1981年间在汉诺威医学院就诊的90例囊性纤维化患者的发病率、死亡率及诊断时的年龄数据。患者的状况通过临床评分和胸部X光片评分来表征。对按“误诊”、“假阴性汗液试验”、“胎粪性肠梗阻”、“5岁后临床症状出现较晚”以及“在囊性纤维化中心接受全面长期治疗”等项目定义的患者组的病程和预后进行了比较。对于有肠道或肺部症状的患者,死亡患者的诊断存在延迟,且多在幼儿期死亡。然而,对于那些在专门的囊性纤维化中心接受适当医疗护理的患者,可以实现一种临床病程,其可能类似于症状出现较晚的患者的疾病病程。

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