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脂肪吸收正常的囊性纤维化患者呼吸预后改善

Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption.

作者信息

Gaskin K, Gurwitz D, Durie P, Corey M, Levison H, Forstner G

出版信息

J Pediatr. 1982 Jun;100(6):857-62. doi: 10.1016/s0022-3476(82)80501-5.

DOI:10.1016/s0022-3476(82)80501-5
PMID:7086584
Abstract

The clinical presentation, courses, and sweat chloride values of 72 CF patients with normal fat absorption are described. In general, these patients had milder clinical symptoms and a lower mean sweat chloride value than their counterparts with steatorrhoea. Pulmonary function tests, including FEV1, FVC, FEF25%-75%, PaO2, and RV/TLC%, were significantly better in patients with normal fat absorption compared with both male and female patients who had steatorrhoea. The maintenance of better pulmonary function, coupled with the low mortality, suggests that patients without steatorrhoea have a better prognosis. This difference remains unexplained, but may be contributed to by nutritional, genetic, or pancreatic factors.

摘要

本文描述了72例脂肪吸收正常的囊性纤维化(CF)患者的临床表现、病程及汗液氯化物值。总体而言,这些患者的临床症状较轻,平均汗液氯化物值低于脂肪泻患者。与有脂肪泻的男性和女性患者相比,脂肪吸收正常患者的肺功能测试,包括第一秒用力呼气容积(FEV1)、用力肺活量(FVC)、25%-75%用力呼气流量(FEF25%-75%)、动脉血氧分压(PaO2)和残气量/肺总量百分比(RV/TLC%)均显著更好。较好的肺功能维持以及低死亡率表明,无脂肪泻的患者预后更好。这种差异尚无法解释,但可能与营养、遗传或胰腺因素有关。

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Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption.脂肪吸收正常的囊性纤维化患者呼吸预后改善
J Pediatr. 1982 Jun;100(6):857-62. doi: 10.1016/s0022-3476(82)80501-5.
2
Improved prognosis in CF patients with normal fat absorption.脂肪吸收正常的囊性纤维化患者预后改善。
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Prognostic value of sweat-chloride levels in cystic fibrosis: a negative report.囊性纤维化中汗液氯化物水平的预后价值:一项否定性报告。
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Screening for cystic fibrosis with chloride electrode. An investigation of sweat chloride with chloride electrode orion 417 in normal persons and in patients with cystic fibrosis.
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Higher bioelectric potentials due to decreased chloride absorption in the sweat glands of patients with cystic fibrosis.囊性纤维化患者汗腺中氯化物吸收减少导致生物电位升高。
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