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用氨苯砜治疗以呼吸道受累为主的复发性多软骨炎:肺功能测试的价值(作者译)

[Relapsing polychondritis with predominant respiratory involvement treated with Dapsone :value of pulmonary function tests (author's transl)].

作者信息

Liot F, Lemaigre D, Dessanges J F, Leroy M

出版信息

Ann Med Interne (Paris). 1980;131(8):519-24.

PMID:7224463
Abstract

A 31-year-old patient had developed lesions in the nasal cartilage during infancy, followed by laryngotracheal lesions when 9 years old. The latter lesions recurred when he was 21, followed several years later by an affection of the main left bronchus and the diagnosis of relapsing polychondritis. Initial respiratory function tests showed mainly a reduction in maximum inspiratory flow, due to sequelae in the upper respiratory tract. Repeated tests were able to demonstrate lesions in the main left bronchus, determining severe expiratory slowing. No significant improvement occurred after one year of corticoïd therapy. Treatment with sulfones (Disulone) produced clinical improvement (reappearance of a vesicular murmur in the left lung) and a twofold increase in FEV1. Functional improvement was even better demonstrated by successive flow-volume curves.

摘要

一名31岁患者在婴儿期鼻软骨出现病变,9岁时出现喉气管病变。21岁时后者病变复发,几年后左主支气管受累,诊断为复发性多软骨炎。最初的呼吸功能测试主要显示最大吸气流量降低,这是由于上呼吸道后遗症所致。重复测试发现左主支气管有病变,导致严重的呼气减慢。皮质类固醇治疗一年后无明显改善。使用砜类药物(二磺法宗)治疗后临床症状改善(左肺出现肺泡呼吸音),第一秒用力呼气容积(FEV1)增加了两倍。连续的流量-容积曲线更清楚地显示了功能改善。

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