Nasal polyposis from cystic fibrosis in children - the experience of a single center.

IF 1.2 4区 医学 Q4 DEVELOPMENTAL BIOLOGY Romanian Journal of Morphology and Embryology Pub Date : 2023-04-01 DOI:10.47162/RJME.64.2.14
Gheorghe Iovănescu, Liviu Laurenţiu Pop, Daniela Roxana Vintilă, Karina Cristina Marin, Carmen Aurelia Mogoantă, Dorela Codruţa Lăzureanu, Veronica Mădălina Borugă, Ioana Mihaela Ciucă
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Abstract

Background: Cystic fibrosis (CF) is the most common monogenic disease, characterized by clinically notable polymorphism. Respiratory disease is the main factor that influences the disease outcome and prognosis of the patient with CF, bacterial infections being responsible for severe exacerbations and rhinosinusitis a difficult complication, besides lung disease.

Aim: The aim of the paper was to present a case series of CF-associated nasal polyposis and our management experience, providing new data for nasal and sinus complications.

Patients, materials and methods: Patients attending the National Cystic Fibrosis Center, Timişoara, Romania, were evaluated for nasal polyposis. Besides clinical examination, endoscopy, and computed tomography (CT) was performed for comprehensive evaluation. Patients with persistent symptoms or with complicated sinusitis underwent surgical approach.

Results: Fourteen (18.18%) children were diagnosed with nasal polyposis and had surgery, with positive outcomes. One patient received Omalizumab for an associated, uncontrolled asthma with a subsequent substantial effect with the significant polyp reduction and lack of recurrence.

Conclusions: Even if extremely difficult to manage, complicated nasal polyposis CF related might have an improved outcome and better life quality.

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儿童囊性纤维化引起的鼻息肉病——单一中心的经验。
背景:囊性纤维化(CF)是最常见的单基因疾病,具有临床显著的多态性。呼吸系统疾病是影响CF患者疾病结果和预后的主要因素,细菌感染导致严重恶化,鼻窦炎是除肺部疾病外的一种困难并发症。目的:本文旨在介绍一系列CF相关鼻息肉病的病例和我们的治疗经验,为鼻腔和鼻窦并发症提供新的数据。患者、材料和方法:对罗马尼亚Timişoara国家囊性纤维化中心的患者进行鼻息肉病评估。除了临床检查外,还进行了内窥镜检查和计算机断层扫描(CT)以进行综合评估。有持续症状或有复杂鼻窦炎的患者接受了手术治疗。结果:14名(18.18%)儿童被诊断为鼻息肉病并接受了手术,结果呈阳性。一名患者接受了奥马珠单抗治疗相关的、未控制的哮喘,随后取得了显著的效果,息肉明显减少,没有复发。结论:即使极难处理,与CF相关的复杂鼻息肉病可能会改善预后和提高生活质量。
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来源期刊
CiteScore
1.70
自引率
20.00%
发文量
221
审稿时长
3-8 weeks
期刊介绍: Romanian Journal of Morphology and Embryology (Rom J Morphol Embryol) publishes studies on all aspects of normal morphology and human comparative and experimental pathology. The Journal accepts only researches that utilize modern investigation methods (studies of anatomy, pathology, cytopathology, immunohistochemistry, histochemistry, immunology, morphometry, molecular and cellular biology, electronic microscopy, etc.).
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