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囊性纤维化患儿的鼻息肉

Paediatric nasal polyps in cystic fibrosis.

作者信息

Mohd Slim Mohd Afiq, Dick David, Trimble Keith, McKee Gary

机构信息

Department of Ear, Nose and Throat, Royal Victoria Hospital, Belfast, UK.

出版信息

BMJ Case Rep. 2016 Jun 21;2016:bcr2016214467. doi: 10.1136/bcr-2016-214467.

DOI:10.1136/bcr-2016-214467
PMID:27329094
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4932358/
Abstract

Patients with cystic fibrosis (CF) are at increased risk of nasal polyps. We present the case of a 17-month-old Caucasian patient with CF who presented with hypertelorism causing cycloplegic astigmatism, right-sided mucoid discharge, snoring and noisy breathing. Imaging suggested bilateral mucoceles in the ethmoid sinuses. Intraoperatively, bilateral soft tissue masses were noted, and both posterior choanae were patent. Polypectomy and bilateral mega-antrostomies were performed. Histological examination revealed inflammatory nasal polyposis typical of CF. The role of early functional endoscopic sinus surgery (FESS) in children with CF nasal polyposis remains questionable as the recurrence rate is higher, and no improvement in pulmonary function has been shown. Our case, however, clearly demonstrates the beneficial upper airway symptom relief and normalisation of facial appearance following FESS in a child with this condition.

摘要

囊性纤维化(CF)患者患鼻息肉的风险增加。我们报告一例17个月大的患有CF的白种人患者,该患者表现为眼距过宽导致睫状肌麻痹性散光、右侧黏液性分泌物、打鼾和呼吸嘈杂。影像学检查提示筛窦双侧黏液囊肿。术中发现双侧软组织肿块,双侧后鼻孔通畅。进行了息肉切除术和双侧上颌窦扩大开窗术。组织学检查显示为CF典型的炎性鼻息肉病。早期功能性内镜鼻窦手术(FESS)在CF鼻息肉病儿童中的作用仍存在疑问,因为复发率较高,且未显示出肺功能改善。然而,我们的病例清楚地表明,FESS可使患有这种疾病的儿童上呼吸道症状得到缓解,面部外观恢复正常。

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Paediatric nasal polyps in cystic fibrosis.囊性纤维化患儿的鼻息肉
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Chronic Rhinosinusitis in Children: Pathophysiology, Evaluation, and Medical Management.儿童慢性鼻-鼻窦炎:病理生理学、评估和医学管理。
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本文引用的文献

1
Cystic fibrosis genetics: from molecular understanding to clinical application.囊性纤维化遗传学:从分子理解到临床应用
Nat Rev Genet. 2015 Jan;16(1):45-56. doi: 10.1038/nrg3849. Epub 2014 Nov 18.
2
Paranasal sinus pathogens in children with cystic fibrosis: do they relate to lower respiratory tract pathogens and is eradication successful?囊性纤维化患儿的鼻窦病原体:它们与下呼吸道病原体有关吗?根除是否成功?
J Cyst Fibros. 2014 Jul;13(4):449-54. doi: 10.1016/j.jcf.2014.03.003. Epub 2014 Apr 6.
3
Sinonasal inhalation of tobramycin vibrating aerosol in cystic fibrosis patients with upper airway Pseudomonas aeruginosa colonization: results of a randomized, double-blind, placebo-controlled pilot study.对患有上呼吸道铜绿假单胞菌定植的囊性纤维化患者经鼻吸入妥布霉素振动气雾剂:一项随机、双盲、安慰剂对照试验研究的结果
Drug Des Devel Ther. 2014 Feb 10;8:209-17. doi: 10.2147/DDDT.S54064. eCollection 2014.
4
Medical management of chronic rhinosinusitis in cystic fibrosis: a systematic review.囊性纤维化慢性鼻-鼻窦炎的医学管理:系统评价。
Laryngoscope. 2014 Jun;124(6):1308-13. doi: 10.1002/lary.24503. Epub 2013 Dec 11.
5
Epidemiology and differential diagnosis of nasal polyps.鼻息肉的流行病学与鉴别诊断
Am J Rhinol Allergy. 2013 Nov-Dec;27(6):473-8. doi: 10.2500/ajra.2013.27.3981.
6
Cystic fibrosis chronic rhinosinusitis: a comprehensive review.囊性纤维化慢性鼻-鼻窦炎:全面综述。
Am J Rhinol Allergy. 2013 Sep-Oct;27(5):387-95. doi: 10.2500/ajra.2013.27.3919.
7
Bacterial sinusitis can be a focus for initial lung colonisation and chronic lung infection in patients with cystic fibrosis.细菌性鼻窦炎可成为囊性纤维化患者肺部初始定植和慢性肺部感染的病灶。
J Cyst Fibros. 2013 Sep;12 Suppl 2:S1-20. doi: 10.1016/S1569-1993(13)00150-1.
8
Topical drug delivery in chronic rhinosinusitis patients before and after sinus surgery using pulsating aerosols.经鼻窦手术前后慢性鼻-鼻窦炎患者的经皮药物递送:脉冲气溶胶法。
PLoS One. 2013 Sep 11;8(9):e74991. doi: 10.1371/journal.pone.0074991. eCollection 2013.
9
Nasal endoscopic and CT scan alterations of the paranasal sinuses as predictors of severity in patients with cystic fibrosis.鼻内镜和鼻窦 CT 扫描改变对囊性纤维化患者严重程度的预测作用。
Braz J Otorhinolaryngol. 2013 Aug;79(4):480-6. doi: 10.5935/1808-8694.20130086.
10
Surgical management of chronic rhinosinusitis in cystic fibrosis: a systematic review.囊性纤维化患者慢性鼻-鼻窦炎的外科治疗:系统评价。
Int Forum Allergy Rhinol. 2013 Oct;3(10):814-22. doi: 10.1002/alr.21190. Epub 2013 Jul 9.