从耳鼻喉科专科的角度探讨睫状固定综合征。

A M García de Hombre, C Garciía de Llanos, J Sánchez López
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引用次数: 0

摘要

原发性纤毛运动障碍是一种以反复出现的上、下呼吸道感染为特征的疾病。它是由一种隐性常染色体遗传疾病引起的。它的诊断往往很晚,因为其他的诊断可能性被首先考虑。在医疗记录中发现许多以前访问过耳鼻喉科专家是很常见的。最后的诊断是通过观察纤毛缺陷的电子显微镜给出的,这通常是由于手臂和负责运动的微管对结构中缺乏或异常。所研究的黏液通常来自下鼻甲。在其他情况下,只有在需要进行纤维支气管镜检查以评估支气管损伤或鼻腔样本不足时,才研究气管支气管装置的粘液。耳鼻喉科专家必须保持警惕,以便能够想到这个实体。遗传建议是最好的预防方法。
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[Revision of the immobile ciliary syndrome on the ENT speciality's point of view].

Primary ciliary dyskinesia is a disorder characterized by the presence of recurrent infections of the superior and inferior respiratory tract. It is caused by a recessive autosomic genetic disorder. Its diagnosis is often given late, because other diagnostic possibilities are thought of first. It is common to find many previous visits to the ENT specialist in a medical record. The final diagnosis is given by electronic microscopy observing the ciliary defect, which is frequently due to the absence or anomalies of dineine in the arms and in the structures of the microtubule pairs responsible of movement. The mucus to study is usually obtained from the inferior nasal concha. In the rest of cases the mucus of the tracheobronchial apparatus is only studied if a fibrobronchoscopy is necesary in order to evaluate bronchial compromise or when the nasal sample is not sufficient. The ENT specialist must be alert in order to be able to think of this entity. The genetic advice is the best way to prevent it.

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