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1.
Cystic fibrosis (CF) is one of the most common inherited life-shortening diseases with an incidence of 1:2.500–3500 and a carrier frequency of 4–5% [1]. It is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, which encodes a protein expressed in the apical membrane of exocrine epithelial cells, resulting in changes to the fluid and electrolytes on cell surface. More than 1500 mutations have been described so far in the CFTR gene, grouped in 5 classes on the basis of the impact on protein synthesis or activity [2].In the last years a mounting number of reports described patients that did not meet all diagnostic criteria for CF [4]; in particular, atypical or nonclassic CF is characterized by normal or borderline sweat test, pancreatic sufficiency and a monosymptomatic phenotype; the most studied forms are: congenital bilateral absence of vasa deferentes, acute or chronic recurrent pancreatitis, idiopathic bronciectasis [5]. These patients typically bear a severe and a mild (classes 4–5) CFTR mutation, the latter being dominant [6].The involvement of upper airways is observed in up to 100% of classic CF patients, including recurrent sinusitis and rhinitis. Nasal polyps are present in 6–48% of overall CF patients and in 5–15.2% of less than 10 years old CF patients.Surgery is required for syntomatic polyps. It is the second most common class of operations performed on CF patients. In spite of the best of operations CF patients will have recurrence, because of systemic disease.We describe a patient diagnosed as “atypical CF” on the basis of persisting chronic rhinosinusitis, that showed a very rare CFTR genotype.  相似文献   

2.
目的:探讨慢性鼻-鼻窦炎(CRS)鼻内镜术后迁延不愈者鼻腔分泌物的细菌学特征。方法:选取单纯鼻中隔偏曲患者20例(对照组),取其中鼻道分泌物;选取首次接受鼻内镜手术的CRS患者30例(手术组),术中取中鼻道分泌物;取FESS术后随访3个月以上未达治愈标准的患者20例(迁延组),在鼻内镜下取中鼻道分泌物,分别做细菌培养和药物敏感试验,3组结果进行比较分析。结果:对照组检出细菌13株,手术组检出15株,迁延组检出15株,其检出阳性率差异无统计学意义;迁延组G-菌检出率明显高于对照组和手术组;对常用抗生素耐药的菌株比例迁延组显著高于手术组。结论:需氧菌可存在于正常鼻腔内;CRS术后迁延不愈者,G-杆菌的感染和细菌的耐药性明显增加。因此,强调要合理应用抗生素,对术后迁延性鼻窦炎的抗生素治疗,应建立在细菌培养和药敏试验的基础上。  相似文献   

3.
Rozsasi A  Keck T 《Laryngo- rhino- otologie》2003,82(10):715-25; quiz 726-30
Patients with cystic fibrosis (CF) show a high morbidity with otorhinolaryngological disorders, especially of the nasal airways and the paranasal sinuses. Because of the underlying basic genetic defect of the respiratory mucosa in CF many patients suffer from chronic rhinosinusitis, partially combined with the development of nasal polyps. Chronic rhinosinusitis in CF is different from chronic sinusitis in Non-CF-patients. In CF mainly neutrophils are found in the nasal epithelium, whereas in Non-CF-patients eosinophils predominate. In CF-sinuses a characteristic mucosal thickening and increase of high viscous mucus is seen which is not typically found in Non-CF-patients with chronic sinusitis. The respiratory mucosa in CF is mostly colonized with Staphylococcus und Pseudomonas. These bacteria participate in the damage of the respiratory epithelia and infection of the paranasal sinuses. Repeated administration of antibiotics frequently fails to reduce subjective symptoms of the patients or polyp formations, and sinus surgery is often necessary. New therapeutic approaches, such as postoperative inhalation with Dornase-alpha, are only empiric and not frequently recommended yet. Besides the strict indication for sinus surgery, which should only be performed on an inpatient basis, the hygienic handling with CF-patients needs special attention, particularly in medical centers with many patients. An effective genetic therapy for CF is currently not available.  相似文献   

4.
OBJECTIVES/HYPOTHESIS: The objective was to clinically characterize and determine disease severity parameters for chronic recurrent rhinosinusitis (CRRS). STUDY DESIGN: Prospective. METHODS: A consecutive series of adult patients undergoing evaluation for CRRS was prospectively evaluated. Patients with four or more acute rhinosinusitis episodes in the previous calendar year with an absence of symptoms between episodes were considered as manifesting CRRS. Symptom severity and disease data from the Rhinosinusitis Symptom Inventory was obtained, as well as Lund staging information from the paranasal sinus CT scan. The Lund staging scores for patients with CRRS were compared with a control group of patients without CRRS. Symptom domain scores and disease severity parameters were compared between the CRRS group and a third group of patients with chronic persistent rhinosinusitis. RESULTS: In all, 30 patients met inclusion criteria for the diagnosis of CRRS. Mean age was 40.9 years with a 3:1 female preponderance. The mean Lund score for patients with CRRS was 3.79. Patients with CRRS failed to demonstrate a statistically different Lund score from control patients (mean Lund score, 4.26 [P = .538]). Symptom severity scores according to Rhinosinusitis Symptom Inventory domains were largely similar for the nasal, facial, and total symptom domains between patients with CRRS versus chronic persistent rhinosinusitis. However, patients with CRRS demonstrated statistically significant increases in oropharyngeal and systemic symptom domain scores. Patients with CRRS also had significant increases in number of antibiotic courses (4.8 vs. 2.9 [P < .001]) and number of missed workdays (8.8 vs. 4.6 d [P = .046]) attributable to rhinosinusitis. CONCLUSION: Chronic recurrent rhinosinusitis is a distinct form of chronic rhinosinusitis differing somewhat from chronic persistent rhinosinusitis. However, patients with CRRS still experience significant symptoms associated with this diagnosis, which results in significant medication usage and workplace impact.  相似文献   

5.
Chronic rhinosinusitis is a symptomatic inflammation of the mucosa of the nose and paranasal sinuses lasting for at least 12 weeks. Atypical bacteria Chlamydophila pneumoniae and Mycoplasma pneumoniae are important causes of human respiratory tract infection. Also, they were identified in bronchial respiratory epithelium of patients with chronic obstructive pulmonary disease or asthma. Having in mind the unified airway concept, it is also possible that these bacteria can cause persistent infection of sinus mucosa in patients with chronic rhinosinusitis. Sixty consecutive patients with chronic rhinosinusitis who underwent the functional endoscopic sinus surgery due to medical therapy failure were included in the study. During the operation, sinuses were irrigated with sterile 0.9 % NaCl solution and this lavage was immediately aspirated. Aspirates were used for the detection of C. pneumoniae and M. pneumoniae DNA using real-time PCR. C. pneumoniae and M. pneumoniae DNA were not detected in samples analysed. Atypical bacteria C. pneumoniae and M. pneumoniae did not cause persistent infection of sinus mucosa in patients with chronic rhinosinusitis.  相似文献   

6.
PURPOSE OF REVIEW: The current recommendation for the choice of empiric antibiotic therapy of chronic rhinosinusitis is based on the bacteriology of acute rhinosinusitis. Staphylococcus aureus has been found consistently in cultures from patients with chronic rhinosinusitis. New studies have examined the role of Staphylococcus aureus in the pathogenesis and clinical course of chronic rhinosinusitis, both before and after surgical therapy. The consequence of antibiotic resistance, on the course of chronic rhinosinusitis and its treatment, has also been examined. RECENT FINDINGS: Several recent studies have confirmed past literature demonstrating a high incidence of sinus cultures positive for Staphylococcus aureus growth in patients with chronic rhinosinusitis. Moreover, one study reported that Staphylococcus aureus is common in patients with persistent chronic rhinosinusitis after endoscopic sinus surgery. These studies, however, have not shown that this bacterium is a significant factor in the development of chronic rhinosinusitis or a predictor of the severity of symptoms. Other studies did not support the notion of increased antibiotic resistance in chronic rhinosinusitis, although there is some evidence that antibiotic resistance may lead to refractory cases of the disease. The standard treatment, of multiple courses of empiric antibiotics followed by surgery, was not shown to increase the prevalence of methicillin-resistant Staphylococcus aureus. SUMMARY: A review of recent literature shows a high incidence of positive cultures for Staphylococcus aureus from the sinuses of patients with chronic rhinosinusitis, both before and after surgery, but has not produced convincing evidence that Staphylococcus aureus has a significant role in the pathogenesis or clinical course of the disease.  相似文献   

7.
The paper presents recommendations of the current European and Russian documents concerning pathogenesis of polypous rhinosinusitis (International Consensus Conference on Nasal Polyposis (2006), European documents EAACI - EP3OS (2007), and Summit of the Russian Society of Rhinologists "Nasal polyposis and inflammation" (2009)). The bilateral polypous process in the nasal cavity is considered to be a "special form of rhinosinusitis" in which bacterial superantigens or fungal infection induce chronic eosinophilic inflammation. Researchers of the ENT Department, Rostov State Medical University, undertook analysis of the results of long-term comprehensive examination of patients with polypous rhinosinusitis that included clinical, bacteriological, histomorphological, and allergological studies as well as evaluation of local and systemic immunity. The data obtained allowed to describe one of the forms of polypous rhinosinusitis as chronic infection-dependent allergic rhinosinusitis with the manifestation of all four types of allergic reactions, formation of the autoimmune component, and development of persistent immune inflammation leading to remodeling of endonasal mucosa. In all these cases, the process progressed parallel to the development of combined secondary immune deficiency (SID). A hypothetical scheme of pathogenesis of chronic polypous allergic rhinosinusitis is proposed.  相似文献   

8.
Conclusions: Chronic rhinosinusitis diagnosed according to the European Position Paper on Rhinosinusitis and Nasal Polyps 2012, not by computed tomography alone, is one of the prognostic factors affecting long-term survival in patients with lung transplantation. Endoscopic sinus surgery might play a beneficial role in the management of lung transplantation recipients with chronic rhinosinusitis.

Objective: To show the effect of paranasal sinus infection on post-lung transplantation survival.

Method: Lung transplantation recipients were included in this study. Computed tomography was performed before and after lung transplantation. The severity of chronic rhinosinusitis was evaluated by Lund–Mackay scoring system. The survival rate was calculated by the Kaplan–Meier method.

Results: One hundred and forty-eight patients received lung transplantation for various indications. Chronic rhinosinusitis was found in 18.9% (28/148) of the lung transplantation recipients. Of 28 patients with chronic rhinosinusitis, seven patients underwent endoscopic sinus surgery due to persistent post-nasal drip. The recipients with chronic rhinosinusitis who did not receive endoscopic sinus surgery (n?=?21) showed a significantly lower survival rate as compared to the patients without chronic rhinosinusitis. There was no statistically significant difference in the survival rate between the recipients with (n?=?50) and without (n?=?98) paranasal sinus abnormality on computed tomography.  相似文献   

9.
OBJECTIVE: To explore whether there is an increased prevalence of chronic rhinosinusitis (CRS) in known cystic fibrosis (CF) carriers. Self-reported CRS affects 13% to 14% of the US population and clusters in families, which suggests that genetic factors may play an etiologic role. Cystic fibrosis is an inherited recessive disorder that invariably affects the sinuses. The frequency of CF mutations has been reported to be higher in patients with CRS than in unaffected controls. PATIENTS: Obligate CF carriers (parents of patients with CF) were recruited from the Johns Hopkins CF clinic. The presence of signs and symptoms of CRS was assessed by a sinus disease questionnaire. A subgroup of participants was evaluated by a physician experienced in the diagnosis of CRS. RESULTS: Fifty-three (36%) of 147 obligate CF carriers who returned a completed questionnaire had self-reported CRS. Twenty-three CF carriers (14 with and 9 without CRS based on self-reporting in the questionnaire) were clinically evaluated. Seven were diagnosed as having CRS (all 7 with self-reported CRS), while another 6 had allergic rhinitis or recurrent acute rhinosinusitis (all 6 with self-reported CRS), and 10 had no evidence of active sinus disease (1 with self-reported CRS). The sensitivity (100%) and specificity (56%) of the questionnaire for physician-diagnosed CRS was similar to that of other survey instruments used to estimate the prevalence of self-reported CRS in the general population. CONCLUSION: Carriers of a single CF mutation have a higher prevalence of CRS than the general population.  相似文献   

10.
慢性鼻-鼻窦炎(CRS)是多因素作用下鼻及鼻窦黏膜的慢性持续性炎症,具有高度异质性,病理机制非常复杂,临床表现型相同,其内在型和预后可能不同。CRS内在型是对不同个体CRS发病内在本质的研究,是从细胞和分子生物学角度探索疾病的发病本质,随着CRS内在型研究的不断深入,期望能找到某些特定的分子标志物,以这些分子标志物为靶标实现个体化的精准治疗。  相似文献   

11.
The aim of the study was to define the characteristic changes revealed by computed tomography (CT) examination of the paranasal sinuses in patients with cystic fibrosis (CF). The group of 30 CF patients was evaluated in the outpatient clinic of the Department of Otorhinolaryngology, Medical University of Warsaw, from 1996 to 1998. The control group consisted of 30 patients with chronic rhinosinusitis (CRS). CT scans were obtained from both groups of patients, and findings revealed more advanced pathological changes in the CF than CRS group. The extensive inflammatory process observed in CT scans of CF patients resulted in the impairment of frontal and maxillary sinus development, destruction of bony structures, and medial projection of the lateral nasal wall.  相似文献   

12.
The authors report a prospective study in which the aim was to analyse the usefulness of different criteria in optimizing the diagnosis of allergic fungal rhinosinusitis. From 1995 to 1998, 165 patients were operated on for chronic rhinosinusitis. Investigations used in this study for the diagnosis of allergic Aspergillus rhinosinusitis consisted of an analysis of clinical, radiological, immuno-allergic criteria. Fourteen patients presented with allergic Aspergillus rhinosinusitis. One hundred and fifty-one patients did not present any of the necessary criteria for the diagnosis of allergic Aspergillus rhinosinusitis. The results show that the characteristic macroscopic appearance, the maxillary sinus localization, and the presence of positive specific IgE to Aspergillus fumigatus are arguments that reinforce the diagnostic certitude of allergic fungal sinusitis. No specific clinical or radiological criteria orients a diagnosis of chronic rhinosinusitis toward that of allergic fungal rhinosinusitis. The other immuno-allergic tests do not contribute to the diagnosis of allergic fungal rhinosinusitis. pathological, mycological, and  相似文献   

13.

Objective

Obstructive sleep apnea syndrome (OSAS) in cystic fibrosis (CF) patients may be associated with the presence of upper airway obstruction caused by chronic infection and nasal polyposis that may also contribute to OSAS severity. Our objective was to identify the profile of findings in CF and OSAS patients by performing upper airway examinations.

Methodology

Observational, cross-sectional study involving 63 children and adolescents between the ages of 2 and 14 with CF. All patients answered a questionnaire and underwent a standard otolaryngology examination, including an endoscopic nasal and nocturnal polysomnography. OSAS diagnosis was confirmed if the obstructive apnea index was ≥1.

Results

OSAS was identified in 35 (55.6%) patients. The upper airway findings were evaluated using multiple correspondence factorial analysis. The OSAS group presented with overjet >2 mm, enlarged pharyngeal pillars, palatine tonsils and pharyngeal tonsils hypertrophy, ogival hard palates and characteristics of chronic rhinosinusitis.

Conclusion

Bone and soft tissue structural alterations of the upper airway and chronic rhinosinusitis were associated with the OSAS group patients.  相似文献   

14.
OBJECTIVES: Evaluation was made of eosinophilic cationic protein (ECP) in nasal secretion for measuring the degree of nasal inflammation and monitoring response to therapy in cystic fibrosis (CF) patients with chronic rhinosinusitis. Symptoms and findings in regard to ECP levels before and after treatment were described. STUDY DESIGN: Study was prospective, with 21 CF patients aged 4 to 19 years; 20 healthy volunteers served as controls. Collection of nasal secretion by a sponge was performed, and blood samples were obtained for serum. Cystic fibrosis (CF) patients were classified according to nasal symptoms and findings. METHODS: ECP was measured by fluoroimmunoassay. Age, sex, nasal symptoms, and endoscopic and histological findings were obtained, and examinations were conducted before and after treatment; recurrences were recorded. RESULTS: In CF patients with chronic nasal inflammation, increased nasal levels of ECP were detected when compared with asymptomatic CF patients or healthy nonatopic subjects. ECP concentrations were strongly related to the extent of nasal disease; patients with nasal polyps had higher levels than those without. Checked at 1 and 4 months after treatment, ECP levels declined with regression of symptoms, and in patients with exacerbation of nasal disease, ECP levels rose. CONCLUSIONS: According to our study, there is a relationship between levels of ECP in nasal secretions and the degrees of nasal inflammation. In addition, the measurement of ECP could be useful in monitoring nasal disease in CF patients.  相似文献   

15.
BACKGROUND: Chronic rhinosinusitis has several features of a prolonged bacterial infection including positive bacterial cultures and abnormal computed tomography findings such as mucosal thickening and hyperostotic bone. Recent studies have suggested that chronic rhinosinusitis may be treated successfully by outpatient parenteral antibiotic treatment (OPAT). In this setting, antibiotics are administered through a peripherally inserted central catheter (PICC). This study evaluates complications arising from OPAT for chronic rhinosinusitis. METHODS: A chart review was performed of 177 patients who underwent OPAT for chronic rhinosinusitis. RESULTS: PICC line-related infections (4/177, 2%)) included line thrombosis in three patients and septicemia in one patient. In the three patients with line thrombosis, the PICC lines were removed and replaced, allowing for completion of the antibiotic course. Antibiotic complications (29/177, 16%) included four patients with transient neutropenia and one patient with elevated liver function tests. Of the four patients with neutropenia, only one required a change in antibiotics. The patient with elevated liver function tests did not require a change in antibiotics. Minor complications from antimicrobial treatment such as rash, itchiness, flushing, and diarrhea were reported by 25 patients, 9 of these patients required a change in antibiotics. There were no permanent complications or deaths in this study. CONCLUSION: Intravenous antimicrobial treatment administered through a PICC line in an outpatient setting is well-tolerated for chronic rhinosinusitis. Although PICC line and antibiotic-related complications are relatively infrequent, the physician should be aware of these complications and consider them in selecting patients for OPAT.  相似文献   

16.
The objective of the present work was to assess the efficacy of the treatment of chronic rhinosinusitis (CR) in the patients treated with sinuforte using the contact microendoscpic technique. This randomized open clinical and instrumental study included 30 patients presenting with chronic rhinosinusitis. All of them were treated with sinuforte as monotherapy at a dose of 1.3 mg per procedure given once daily during 7 consecutive days. The efficacy of the treatment was evaluated by means of endoscopic and microendoscopic examination supplemented by functional tests on days 1, 2, 4, 6, and 8 after the beginning of therapy. In addition, a questionnaire study was carried out. It was demonstrated that the use of sinuforte for the treatment of the patients with chronic rhinjsinusitis is safe and efficacious as confirmed by the morphological picture revealed by contact microendoscopy.  相似文献   

17.
Aspirin intolerance in patients with chronic sinusitis is often a cause of early recurrence of symptoms after surgical treatment. This study assesses 84 patients who were tested for acetylsalicylic acid intolerance after presenting with symptoms like chronic rhinosinusitis, sometimes bronchial asthma, coexisting allergies or a history of aspirin sensitivity. Nasal polyposis was found in a majority of cases, often recurrent after previous surgery. The levels of eicosanoids such as peptido-leukotrienes and prostaglandin E2 were analyzed in isolated blood cells and compared with a healthy control group. Aspirin-intolerant patients showed elevated basal levels of peptido-leukotrienes and reduced basal levels of prostaglandin E2. Test results were graded in a system ranging from positive (68%), signifying aspirin intolerance, to borderline (18%) and negative results (14%). After screening patients with clinical findings indicating a possible aspirin intolerance, the results of this investigation reveal a strong correlation between the clinical symptomatology and the in vitro parameters of eicosanoid levels in isolated blood cells, indicating the need to induce aspirin tolerance to reduce the risk of recurrent rhinosinusitis.  相似文献   

18.
目的:旨在评价鼻窦球囊扩张术(sinus balloon catheter dilation,SBCD)对慢性鼻-鼻窦炎(CRS)患者的临床疗效。方法计算机检索Pubmed、EMBASE、MEDLINE、中国知网、万方数据库、中国生物医学文献数据库和维普数据库中关于CRS患者SBCD后疗效的自身前后对照试验,时限为从建库到2015年4月。对纳入研究的质量进行严格评价与提取资料,对符合标准的文献进行系统评价,统计学分析应用RevMan 5.0软件。结果共纳入8篇自身前后对照试验。结果显示CRS患者SBCD后3个月、6个月及1年的SNOT-20评分,术后3个月的Lund-Kennedy评分与VAS评分,以及术后1年的Lund-Mackay评分均低于术前,其差异均有统计学意义(P<0.05)。结论 CRS患者行SBCD后恢复快,并发症减少,SBCD在CRS的治疗中具有较好的应用前景。  相似文献   

19.
IntroductionChronic rhinosinusitis with nasal polyps is a heterogeneous disease and appropriate diagnostic algorithms in individual cases are necessary for effective medical treatment.ObjectiveThe purpose of this study was to clarify the relationship between the pendrin expression of nasal polyps and clinical and pathological characteristic features of eosinophilic chronic rhinosinusitis.MethodsA total of 68 patients were classified into eosinophilic chronic rhinosinusitis or non-eosinophilic chronic rhinosinusitis groups according to the degree of eosinophilic infiltration into the nasal polyps. Clinical, hematological, and immunohistochemical analyses were performed and statistically compared between both groups.ResultsThirty-eight were classified into eosinophilic chronic rhinosinusitis and 30 into non-eosinophilic chronic rhinosinusitis groups. There were no significant differences in age distribution, sex ratio, prevalence of asthma, or any other complications between the groups. The mean Lund–Mackay score and the number of serum eosinophils was significantly higher in the eosinophilic chronic rhinosinusitis than in the non-eosinophilic chronic rhinosinusitis groups. The pendrin expression was more frequently detected in the epithelial surface layer of nasal polyps in the eosinophilic chronic rhinosinusitis than in the non-eosinophilic chronic rhinosinusitis groups. In addition, mucin 5AC was more widely expressed in the eosinophilic chronic rhinosinusitis than in the non-eosinophilic chronic rhinosinusitis.ConclusionIncreased expression of pendrin and mucin 5AC in the nasal polyps would be associated with development of eosinophilic chronic rhinosinusitis. This finding could allow the development of a novel therapeutic agent targeted specifically to patients with eosinophilic chronic rhinosinusitis.  相似文献   

20.
PURPOSE OF REVIEW: Chronic rhinosinusitis represents a challenge with its poorly understood pathophysiology and limited treatment options. Potential roles of fungi and eosinophils in the etiology and pathophysiology of chronic rhinosinusitis are summarized. RECENT FINDINGS: Previously, the fungal role in chronic rhinosinusitis was limited to the rare subgroup, allergic fungal rhinosinusitis. Critical examination of earlier diagnostic criteria for allergic fungal rhinosinusitis reveals limitations. By using updated diagnostic standards and novel sensitive techniques to detect fungi, a higher number of patients can now be diagnosed with fungal rhinosinusitis. A novel non-IgE-mediated immunologic mechanism in chronic rhinosinusitis patients links the predominant eosinophilic inflammation to certain fungi. Overall, these new findings have implications for surgical and medical approaches, including anti-inflammatory and antifungal medications. SUMMARY: Several classification schemes and diagnostic criteria describe chronic rhinosinusitis and make comparisons difficult. Preselection of patient groups within the chronic rhinosinusitis population and the lack of sensitive diagnostic techniques have prevented a full understanding of the syndrome complex of chronic rhinosinusitis. New results suggest a broader role for fungi in the pathophysiology of chronic rhinosinusitis, linking the eosinophilic inflammation to the presence of certain molds in the nasal and paranasal cavities. Although fungi are commonly found in nearly everyone, only chronic rhinosinusitis patients respond to them with an eosinophilic inflammation. These findings support a shift in the etiologic understanding of chronic rhinosinusitis away from a bacteriologic infectious pathogenesis to a fungal-driven inflammatory pathophysiology. Herein, the authors review earlier studies and describe an updated view on an old paradigm.  相似文献   

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