Cystic fibrosis:: an inherited susceptibility to bacterial respiratory infections

被引：91

作者：

Tümmler, B

Kiewitz, C

机构：

[1] Med Hsch Hannover, Klin Forschergrp, Zentrum Biochem, D-30623 Hannover, Germany

[2] Med Hsch Hannover, Zentrum Kinderheilkunde, D-30623 Hannover, Germany

来源：

MOLECULAR MEDICINE TODAY | 1999年 / 5卷 / 08期

关键词：

D O I：

10.1016/S1357-4310(99)01506-3

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Cystic fibrosis is a severe monogenic disorder of ion transport in exocrine glands. The basic defect predisposes to chronic bacterial airway infections with Staphylococcus aureus, Haemophilus influenzae, Pseudomonas aeruginosa and Burkholderia cepacia, The Pseudomonas infections in cystic fibrosis are a paradigm of how versatile environmental bacteria can conquer, adapt and persist in an atypical habitat and successfully evade defence mechanisms and chemotherapy in a susceptible host. Regular chemotherapy with aerosol and systemic antipseudomonal drugs has improved the course and prognosis of the disease, and research for effective vaccines is on the way.

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页码：351 / 358

页数：8

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