Labbé A, Déchelotte P, Creveaux I, Poitrineau P, Gaulme J
Service de Pédiatrie A, Hôtel-Dieu, Clermont-Ferrand.
Rev Mal Respir. 1992;9(3):324-6.
A case of bronchiolitis of insidious evolution appearing in an unweened infant aged six months is reported. Initially an acute episode of bronchial obstruction was followed by respiratory failure with failure to thrive. The total inefficacy of conventional treatment (corticosteroids, nebulised and oral bronchodilators) led to assisted ventilation for three weeks, four months after the onset of symptoms. All investigations aimed at achieving a diagnosis were negative and this led to an open lung biopsy. This showed characteristic lesions of bronchiolitis and follicular bronchitis without other parenchymatous disease. With continuous antibiotics and physiotherapy the respiratory status improved, both clinically and radiologically. Amongst the explanations of the pathophysiology of follicular bronchitis they also discussed the existence of heterozygous delta F 508 in their observation to explain the chronicity of the problems. They stress the need to look for a mutation of delta F 508 in infants who present with unexplained obstructive bronchial pathology.
报告了一例隐匿性演变的细支气管炎病例，该病例出现在一名6个月大未断奶的婴儿身上。最初是急性支气管阻塞发作，随后出现呼吸衰竭和发育不良。常规治疗（皮质类固醇、雾化和口服支气管扩张剂）完全无效，症状出现4个月后导致辅助通气3周。所有旨在确诊的检查均为阴性，这导致进行了开放性肺活检。活检显示为细支气管炎和滤泡性支气管炎的特征性病变，无其他实质疾病。通过持续使用抗生素和物理治疗，呼吸状况在临床和放射学上均有所改善。在对滤泡性支气管炎病理生理学的解释中，他们还讨论了在其观察中存在杂合性δF 508以解释问题的慢性化。他们强调，对于出现不明原因阻塞性支气管病变的婴儿，有必要寻找δF 508突变。
