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囊性纤维化患者的颞骨气化:与基因型有关吗?

Temporal bone pneumatization in cystic fibrosis: a correlation with genotype?

作者信息

Berkhout Maaike C, van Rooden Cornelis J, Aalbers Ralph C, el Bouazzaoui Lahssan H, Fokkens Wytske J, Rijntjes Evert, Heijerman Harry G M

机构信息

Departments of Pulmonology, Haga Teaching Hospital, the Hague, the Netherlands.

出版信息

Laryngoscope. 2014 Jul;124(7):1682-6. doi: 10.1002/lary.24575. Epub 2014 Feb 6.

DOI:10.1002/lary.24575
PMID:24374715
Abstract

OBJECTIVES/HYPOTHESIS: Paranasal sinus pneumatization in patients with cystic fibrosis (CF) is less extensive compared to the general population and seems to be correlated to CF genotype. Interestingly, in CF patients temporal bone pneumatization (TBP) is more extensive compared to the general population, and middle ear pathology is generally uncommon in CF. It is debated whether TBP is influenced environmentally or genetically. The aim of the present study was to investigate pneumatization of the temporal bone in patients with CF and to correlate this with genotype and paranasal sinus volume.

STUDY DESIGN

Prospective collection of data.

METHODS

In 104 adult CF patients, computed tomography of the temporal bone and the paranasal sinuses was performed. TBP was graded using a validated scoring system. Patients were divided into two groups, mild and severe CF, based on their mutations in the CF transmembrane conductance regulator gene.

RESULTS

Of the 31 patients with mild CF, 71% had extensive TBP, and of the 73 patients with severe CF, 82% had extensive pneumatization of the temporal bone. TBP did not differ significantly for CF genotype, and TBP was not correlated to paranasal sinus volume.

CONCLUSIONS

Whereas paranasal sinus pneumatization in CF patients seems to be related to CF genotype among other influencing factors, this study showed no correlation between TBP and CF genotype. TBP was not correlated to paranasal sinus volume. Hypothetically, in CF, pneumatization of the temporal bone is under a different influence than paranasal sinus pneumatization.

LEVEL OF EVIDENCE

摘要

目的/假设:与普通人群相比,囊性纤维化(CF)患者的鼻窦气化程度较低,且似乎与CF基因型相关。有趣的是,与普通人群相比,CF患者的颞骨气化(TBP)程度更高,且CF患者中耳病变通常并不常见。目前尚不清楚TBP是受环境因素还是遗传因素的影响。本研究的目的是调查CF患者的颞骨气化情况,并将其与基因型和鼻窦容积相关联。

研究设计

前瞻性数据收集。

方法

对104例成年CF患者进行颞骨和鼻窦的计算机断层扫描。使用经过验证的评分系统对TBP进行分级。根据CF跨膜传导调节基因的突变情况,将患者分为轻度和重度CF两组。

结果

在31例轻度CF患者中,71%有广泛的TBP;在73例重度CF患者中,82%有广泛的颞骨气化。TBP在CF基因型之间无显著差异,且TBP与鼻窦容积无关。

结论

虽然CF患者的鼻窦气化似乎在其他影响因素中与CF基因型有关,但本研究表明TBP与CF基因型之间无相关性。TBP与鼻窦容积无关。假设在CF中,颞骨气化与鼻窦气化受到的影响不同。

证据级别

4级。

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