Senent Sánchez C J, Piris Pinilla M A, González Gutiérrez M L, Panadero Sevilla P, Menéndez Lozano A
Sección de Alergología, Hospital Virgen del Valle, Toledo.
Rev Clin Esp. 1990 Oct;187(6):263-8.
Eight patients are studied who are suspected to have primary ciliary dyskinesia (PCD). All cases presented from the first year of live repetitive respiratory infections, chronic cough, mucopurulent rhinorrhea, radiologic signs of sinusitis and one patient also presented situs inversus. Bronchiectasis were found in four cases, they were discarded in two cases, and in two other cases they could not be found nor discarded. The definite diagnosis was achieved by the study of the ultrastructure of the cilia by nasal biopsy. In three cases, nasal biopsy discarded the diagnosis of PCD and confirmed such diagnosis in other three cases. One case of PCD was diagnosed by a bronchial biopsy after two unsuccessful attempts to obtain a nasal sample containing ciliary epithelium. One case remains undiagnosed since after a nonvalid biopsy, we did not consider necessary to obtain another one given that the patient was asymptomatic during the last three years.
对8名疑似患有原发性纤毛运动障碍(PCD)的患者进行了研究。所有病例均在出生后第一年出现反复呼吸道感染、慢性咳嗽、黏液脓性鼻漏,有鼻窦炎的影像学表现,1例患者还出现了内脏反位。4例发现有支气管扩张,2例被排除,另外2例既未发现也无法排除支气管扩张。通过鼻活检对纤毛超微结构进行研究得出明确诊断。3例鼻活检排除了PCD诊断,另外3例则确诊为此病。在两次尝试获取含纤毛上皮的鼻样本均未成功后,通过支气管活检确诊了1例PCD。1例患者仍未确诊,因为在一次无效活检后,鉴于该患者在过去三年中无症状,我们认为没有必要再进行一次活检。