Babinski Dariusz, Trawinska-Bartnicka Maria
Department of Otolaryngology, Medical University of Gdansk, Debinki Street 7, 80-211 Gdansk, Poland.
Int J Pediatr Otorhinolaryngol. 2008 May;72(5):619-24. doi: 10.1016/j.ijporl.2008.01.010. Epub 2008 Feb 21.
Chronic inflammation of sinuses and nasal mucosa is found in 74-100% patients suffering from cystic fibrosis, whereas nasal polyps in 6-44% patients. The aim of this paper is to assess rhinosinusitis types taking into account the forms of cystic fibrosis and the kind of CFTR gene mutation.
The author presents material of 126 cystic fibrosis patients, 90 with typical clinical features and 36 with atypical phenotype. Genetic tests were carried out to determine the genotype of CFTR gene. The sample was divided into four groups according to the genotype effect on the chloride canal function. Cytological examination of nasal mucosa was carried out in all the patients.
In 71.5% of patients with cystic fibrosis, infectious chronic non-specific rhinosinusitis was found. Other types of rhinosinusitis--acute infectious, chronic allergic and non-allergic with eosinophilia were found in 21.4% of patients, whereas in 7.1% of patients no clinical symptoms of rhinosinusitis were found. Nasal polyps were found in 23 (18.3%) patients with cystic fibrosis: in 21 patients with a typical form and in 2 patients with an atypical form. Nasal polyps were more frequent in groups with the genotype consisting of both "strong" mutations than in the group with unknown or "mild" mutations.
Rhinosinusitis in cystic fibrosis patients is not homogenous pathology. Infectious chronic non-specific rhinosinusitis is found the most frequently, but other forms of rhinosinusitis appear quite often and they require proper treatment.
在74%-100%的囊性纤维化患者中发现鼻窦和鼻黏膜的慢性炎症,而6%-44%的患者存在鼻息肉。本文旨在根据囊性纤维化的形式和CFTR基因突变的类型评估鼻窦炎的类型。
作者展示了126例囊性纤维化患者的资料,其中90例具有典型临床特征,36例具有非典型表型。进行基因检测以确定CFTR基因的基因型。根据基因型对氯通道功能的影响将样本分为四组。对所有患者进行鼻黏膜细胞学检查。
在71.5%的囊性纤维化患者中发现感染性慢性非特异性鼻窦炎。其他类型的鼻窦炎——急性感染性、慢性过敏性和嗜酸性粒细胞增多性非过敏性鼻窦炎在21.4%的患者中被发现,而7.1%的患者未发现鼻窦炎的临床症状。23例(18.3%)囊性纤维化患者发现鼻息肉:21例为典型形式,2例为非典型形式。由两个“强”突变组成基因型的组中鼻息肉比基因型未知或为“轻度”突变的组更常见。
囊性纤维化患者的鼻窦炎并非同质病理。感染性慢性非特异性鼻窦炎最常见,但其他形式的鼻窦炎也很常见,需要适当治疗。