Sinonasal

鼻窦
  • 文章类型: Journal Article
    背景:本研究旨在评估动态对比增强磁共振成像(DCE-MRI)和弥散加权成像(DWI)参数在区分鼻窦淋巴瘤和鼻窦癌方面的诊断效能。
    方法:42例经组织学证实的鼻腔鼻窦淋巴瘤和52例鼻腔鼻窦癌患者用3.0TMRI扫描仪进行成像。进行了DCE-MRI和DWI,和各种参数,包括时间-强度曲线(TIC)的类型,时间达到顶峰,峰值增强,峰值对比度增强,冲洗率,表观扩散系数(ADC),测量相对ADC。采用二元logistic回归和受试者工作特征(ROC)曲线分析来评估单独和组合指标对鼻窦淋巴瘤和鼻窦癌的诊断能力。
    结果:鼻窦淋巴瘤主要表现为II型TIC(n=20),而鼻腔鼻窦癌主要表现为III型TIC(n=23)。除冲洗比(p<0.05)外,所有参数均存在显着差异。ADC值成为单一参数中最可靠的诊断工具。与个别参数相比,DCE-MRI联合参数显示出更好的诊断效能。当组合DCE-MRI和DWI的所有参数时,效率最高(曲线下面积=0.945)。
    结论:涉及对比增强动态MRI和DWI的多参数评估在区分鼻窦淋巴瘤和鼻窦癌方面具有相当大的诊断价值。
    BACKGROUND: The study aimed to evaluate the diagnostic efficacy of dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) and diffusion-weighted imaging (DWI) parameters in distinguishing sinonasal lymphoma from sinonasal carcinoma.
    METHODS: Forty-two participants with histologically confirmed sinonasal lymphomas and fifty-two cases of sinonasal carcinoma underwent imaging with a 3.0T MRI scanner. DCE-MRI and DWI were conducted, and various parameters including type of time-intensity curve(TIC), time to peak, peak enhancement, peak contrast enhancement, washout rate, apparent diffusion coefficient (ADC), and relative ADC were measured. Binary logistic regression and receiver operating characteristic (ROC) curve analysis were employed to assess the diagnostic capability of individual and combined indices for differentiating nasal sinus lymphoma from nasal sinus carcinoma.
    RESULTS: Sinonasal lymphoma predominantly exhibited type II TIC(n = 20), whereas sinonasal carcinoma predominantly exhibited type III TIC(n = 23). Significant differences were observed in all parameters except washout ratio (p < 0.05), and ADC value emerged as the most reliable diagnostic tool in single parameter. Combined DCE-MRI parameters demonstrated superior diagnostic efficacy compared to individual parameters, with the highest efficiency (area under curve = 0.945) achieved when combining all parameters of DCE-MRI and DWI.
    CONCLUSIONS: Multiparametric evaluation involving contrast-enhanced dynamic MRI and DWI holds considerable diagnostic value in distinguishing sinonasal lymphoma from sinonasal carcinoma.
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  • 文章类型: Journal Article
    背景:鼻和鼻旁窦的疾病对患者的功能和结构方面有重大影响。它们影响所有年龄组和两性。由于它靠近重要结构,鼻子和鼻旁窦的疾病有时会导致非常严重的预后。
    方法:这是一项在印度中部最大的三级护理中心之一进行的回顾性研究。我们研究了227例非肿瘤性和肿瘤性两种类型的鼻窦肿块。在临床和影像学发现的帮助下分析肿块的临床病理特征,随后通过组织诊断证实。
    结果:就诊平均年龄为45.5岁,男女比例为1.25:1。我们的大多数患者来自中下层社会经济群体,其学历低于第10标准。鼻塞是最常见的症状。计算机断层扫描是首选的放射学检查。鼻窦未分化癌是最常见的恶性肿瘤,总共22人中有6人(27%)。
    结论:鼻窦肿块的评估应系统细致地进行。由于大多数鼻和鼻旁窦疾病的最初表现是相同的,一个适当的临床,放射学,应进行组织诊断,以免引起恶性病变。
    BACKGROUND: Diseases of the nose and paranasal sinuses have a significant impact on the patient\'s functional and structural aspects. They affect all age groups and both sexes. Due to its proximity to vital structures, diseases of the nose and paranasal sinuses sometimes lead to very grave prognoses.
    METHODS: This was a retrospective study done in one of central India\'s largest tertiary care centers. We studied 227 cases of sinonasal masses in both the non-neoplastic and neoplastic categories. Clinicopathological characteristics of masses were analyzed with the help of clinical and imaging findings and subsequently confirmed by tissue diagnosis.
    RESULTS: The mean age of presentation was 45.5 years, with a male-to-female ratio of 1.25:1. Most of our patients were from lower-middle socioeconomic groups with educational qualifications below the 10th standard. Nasal obstruction was the most common symptom. A computed tomography scan was the preferred radiological investigation. Sinonasal undifferentiated carcinoma was the most common variant of malignancy, with a total number of six out of 22 (27%).
    CONCLUSIONS: The evaluation of sinonasal masses should be carried out systematically and meticulously. Since the initial presentation of most of the diseases of the nose and paranasal sinuses is the same, a proper clinical, radiological, and tissue diagnosis should be carried out to avoid causing malignant lesions.
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  • 文章类型: Journal Article
    目的:横纹肌肉瘤(RMS)是一种罕见的肿瘤,具有独特的形态学类型和挑战性的诊断。本研究旨在探讨临床病理特征,生存结果,影响成年鼻窦RMS患者预后的因素,解决知识上的关键差距。
    方法:这项回顾性队列研究采用各种统计分析来调查RMS患者。描述性统计总结了人口统计学和临床特征,而使用Kaplan-Meier方法和Cox比例风险模型的生存分析探讨了协变量与生存结局之间的关系。
    结果:我们分析了13例(7例男性,6名女性)鼻窦RMS。平均发病年龄为42.5岁(标准差18.9)。在多个位置观察到肿瘤,主要在上颌窦(n=7),其次是筛窦(n=5),和蝶窦(n=1)。研究显示存活率低,有12名患者死于这种疾病,只有一名患者幸存。随着时间的推移,生存概率从92.31%(0.5个月时)下降至7.69%(45个月时).分析表明,诊断年龄在40岁以下与生存率之间具有统计学上的正相关(p=0.05)。发现性别与生存率显着相关(p=0.03),男性患者的生存率更高(风险比=0.08,95CI=0.01-0.81)。
    结论:本研究强调了成人鼻窦RMS的复杂性。低生存率和不同的肿瘤位置强调需要进一步研究以改善诊断和治疗结果。
    OBJECTIVE: Rhabdomyosarcoma (RMS) is a rare tumor with distinct morphological types and challenging diagnosis. This study aimed to investigate clinicopathological characteristics, survival outcomes, and factors influencing prognosis in adult patients with sinonasal RMS, addressing a critical gap in knowledge.
    METHODS: This retrospective cohort study employed various statistical analyses to investigate patients with RMS. Descriptive statistics summarized demographic and clinical characteristics, while survival analysis using the Kaplan-Meier method and Cox proportional hazards model explored the relationship between covariates and survival outcomes.
    RESULTS: We analyzed 13 cases (7 males, 6 females) of sinonasal RMS. The average age at onset was 42.5 years (standard deviation 18.9). Tumors were observed in multiple locations, predominantly in the maxillary sinus (n=7), followed by the ethmoid sinus (n=5), and the sphenoid sinus (n=1). The study revealed a low survival rate, with 12 patients succumbing to the disease and only one patient surviving. Over time, survival probabilities declined from 92.31% (at 0.5 months) to 7.69% (at 45 months). The analysis indicated a borderline statistically significant positive association between age at diagnosis below 40 years and survival (p=0.05). Sex was found to be significantly associated with survival (p=0.03), with male patients exhibiting a higher survival rate (hazard ratio=0.08, 95%CI=0.01-0.81).
    CONCLUSIONS: This study highlights the complex nature of sinonasal RMS in adults. The low survival rate and distinct tumor locations emphasize the need for further research to improve diagnosis and treatment outcomes.
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  • 文章类型: Journal Article
    目的:鼻腔鼻窦粘膜黑色素瘤(SNMM)常发生局部衰竭和远处转移。化疗的反应率低,很少检测到靶向突变。然而,越来越多的数据表明免疫检查点抑制(ICI)的有效性.这项回顾性单中心研究的目的是评估突变景观,并在现实世界中评估SNMM中手术治疗和ICI的结果。
    方法:回顾性分析了1999年至2020年间在我们机构接受治疗的38例SNMM患者。根据Kaplan-Meier生成存活曲线并通过对数秩检验进行比较。
    结果:60%的患者在治愈性治疗中出现局部失败。总的来说,所有患者中有24%患有区域性转移,66%患有远处转移。下一代测序揭示了BRAF的突变,NRAS和KRAS。用原发性ICI治疗的三名患者中有一名表现出完全反应(CR),两名表现出进行性疾病。11例患者接受ICI作为姑息治疗。在三名患者中可以观察到CR,在一名患者中可以观察到病情稳定。在整个研究人群中,5年总生存率(OS)为26%.在病程中接受ICI的患者OS较好。
    结论:在SNMM中复发和远处转移是常见的。在原发性和姑息性ICI之后可以观察到持久的CR。因此,ICI在姑息治疗中,佐剂甚至新佐剂设置可能在SNMM治疗中发挥有希望的作用,而很少检测到可靶向突变.
    OBJECTIVE: Local failure and distant metastases occur frequently in sinonasal mucosal melanoma (SNMM). Response rates to chemotherapy are low and targetable mutations are rarely detected. However, there is increasing data indicating efficacy of immune checkpoint inhibition (ICI). The aim of this retrospective monocenter study was to assess the mutational landscape and to evaluate the outcome of surgical treatment and ICI in SNMM in a real-world setting.
    METHODS: Thirty-eight SNMM patients being treated between 1999 and 2020 at our institution were retrospectively reviewed. Survival curves were generated according to Kaplan-Meier and compared by the log-rank test.
    RESULTS: Local failure was seen in 60% of patients treated in a curative intent. Overall, 24% of all patients suffered from regional and 66% from distant metastases. Next generation sequencing revealed mutations of BRAF, NRAS and KRAS. One out of three patients treated with a primary ICI showed a complete response (CR) and two showed progressive disease. Eleven patients received ICI as a palliative treatment. CR could be observed in three patients and stable disease in one patient. In the whole study population, the 5-year overall survival rate (OS) was 26%. OS was better for patients who received ICI during the course of disease.
    CONCLUSIONS: Recurrences and distant metastases are frequent in SNMM. Durable CR could be observed after primary and palliative ICI. Therefore, ICI in a palliative, adjuvant or even neoadjuvant setting might play a promising role in SNMM therapy while targetable mutations are rarely detected.
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  • 文章类型: Journal Article
    未经批准:在中国,关于鼻腔和鼻旁窦腺癌流行病学的报道有限。
    UNASSIGNED:本研究旨在描述中国单一机构治疗这些恶性肿瘤的经验。
    UNASSIGNED:我们对2019年1月至2021年12月收治的鼻腔和鼻旁窦腺癌患者进行了回顾性图表回顾。肿瘤分期是基于美国癌症联合委员会,第8版,鼻窦恶性肿瘤.
    未经评估:该系列包括10名男性和4名女性(平均年龄,54.5[范围,14-80]年)。鼻出血和鼻塞是10例(71.4%)患者最常见的临床表现。10人(71.4%)在诊断时患有T4期疾病,但无淋巴结或远处转移。后上隔(100.0%)和中鼻甲(92.8%)是最容易受到肿瘤侵袭的两个部位。所有患者均接受内镜切除术作为初始治疗;1例患者死亡。
    未经批准:在中国,这些恶性肿瘤与接触某些物质有关;然而,诊断可能会延迟。内窥镜切除术是鼻腔和鼻旁窦腺癌的合适治疗选择。
    UNASSIGNED: In China, reports on the epidemiology of adenocarcinomas of the nasal cavity and paranasal sinuses are limited.
    UNASSIGNED: This study aimed to describe the experience of a single institution in China in treating these malignant tumours.
    UNASSIGNED: We conducted a retrospective chart review of patients with adenocarcinoma of the nasal cavity and paranasal sinuses admitted between January 2019 and December 2021. Tumour staging was based on the American Joint Committee on Cancer, 8th edition, for sinonasal malignancies.
    UNASSIGNED: The series included 10 men and 4 women (mean age, 54.5 [range, 14-80] years). Epistaxis and nasal obstruction were the most common clinical manifestations in 10 (71.4%) patients. Ten (71.4%) had stage T4 disease at diagnosis, but no patient had lymph node or distant metastasis. The posterosuperior septum (100.0%) and middle turbinate (92.8%) were the two sites most vulnerable to tumour invasion. All patients underwent endoscopic resection as the initial treatment; one patient died.
    UNASSIGNED: In China, these malignancies are related to exposure to certain substances; however, diagnosis can be delayed. Endoscopic resection is a suitable treatment option for adenocarcinomas of the nasal cavity and paranasal sinuses.
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  • 文章类型: Journal Article
    在过去的20年里,经鼻内镜手术(TES)已成为治疗鼻窦恶性肿瘤最常用的手术技术。鼻窦肿瘤的稀有性和异质性阻碍了大规模的非人群分析。
    纳入了1995年至2021年在5家转诊医院接受TES治疗的所有患者。进行了预后研究,将多变量模型转化为列线图。培训和验证集基于3个欧洲中心和2个非欧洲中心的结果,分别。
    训练和验证集包括940名和420名患者,分别。手术的平均年龄,原发性与复发性表现,组织学分布,手术类型,T类别和辅助治疗的类型在训练和验证集中分布不同。在训练集中,5年总生存率和无复发生存率,置信区间为95%,分别为72.7%(69.5-76.0%)和66.4%(63.1-69.8%),分别,随组织学显著变化。在多变量分析中,年龄,性别,以前的治疗,颅骨切除的程度,外侧和后外侧轴,等级/子类型,T类,节点状态,切缘状态和辅助治疗均与不同的预后结局相关,根据组织学显示异质意义和效应大小。除粘膜黑素瘤外,所有组织学的列线图的内部和外部验证均令人满意(乐观校正的C指数>0.7,曲线下累积面积>0.7)。
    基于TES的鼻窦癌治疗的结果随组织学而显著不同。这么大,非基于人群的研究提供了被认为适合包括TES在内的治疗的鼻窦癌预后的基准数据.
    Over the last 2 decades, transnasal endoscopic surgery (TES) has become the most frequently employed surgical technique to treat sinonasal malignancies. The rarity and heterogeneity of sinonasal cancers have hampered large non-population-based analyses.
    All patients receiving TES-including treatment between 1995 and 2021 in 5 referral hospitals were included. A prognostic study was performed, and multivariable models were transformed into nomograms. Training and validation sets were based on results from 3 European and 2 non-European centres, respectively.
    The training and validation set included 940 and 420 patients, respectively. The mean age at surgery, primary-versus-recurrent presentation, histology distribution, type of surgery, T category and type of adjuvant treatment were differently distributed in the training and validation set. In the training set, 5-year overall survival and recurrence-free survival with a 95%-confidence interval were 72.7% (69.5-76.0%) and 66.4% (63.1-69.8%), respectively, significantly varying with histology. At multivariable analyses, age, gender, previous treatment, the extent of resection on the cranial, lateral and posterolateral axes, grade/subtype, T category, nodal status, margin status and adjuvant treatment were all associated with different prognostic outcomes, displaying a heterogeneous significance and effect size according to histology. The internal and external validation of nomograms was satisfactory (optimism-corrected C-index >0.7 and cumulative area under curve >0.7) for all histologies but mucosal melanoma.
    Outcomes of TES-based treatment of sinonasal cancers vary substantially with histology. This large, non-population-based study provides benchmark data on the prognosis of sinonasal cancers that are deemed suitable for treatment including TES.
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  • 文章类型: Journal Article
    鼻窦癌(SNCs)的分类是一个难题。因此,预后和对非手术治疗反应的预测通常是不可靠的。预测和预测措施的可用性是一个未满足的需求,要研究的第一个逻辑信息来源是该疾病的临床病理特征。该研究的假设是,可以利用SNC的临床病理信息来更好地预测预后和放化疗敏感性。
    所有接受治愈性治疗的SNC患者,包括手术,研究纳入了1998年10月至2019年2月在布雷西亚大学耳鼻咽喉头颈外科的研究.审查了机构系列并进行了生存分析。采用机器学习和多变量统计方法来开发,分析,并测试3种实验分类(分类#1,基于细胞形态学,组织形态学,和分化信息;分类#2,基于分化信息;和分类#3,基于SNC的局部区域扩展),基于固有的临床病理信息。测试了实验分类与预后和放化疗敏感性的关联。
    该研究包括145名患者。从预后的角度来看,机器学习生成的SNC分类比目前的世界卫生组织分类提供了更好的预测。然而,SNC的化学放射敏感性的预测无法实现.
    重组临床病理信息,特别提到与肿瘤分化有关的那些,可以提高SNC预后的可靠性。预测化学放射敏感性仍然是一个未满足的需求,需要进一步的研究。
    UNASSIGNED: The classification of sinonasal carcinomas (SNCs) is a conundrum. Consequently, prognosis and prediction of response to non-surgical treatment are often unreliable. The availability of prognostic and predictive measures is an unmet need, and the first logical source of information to be investigated is represented by the clinicopathological features of the disease. The hypothesis of the study was that clinicopathological information on SNC could be exploited to better predict prognosis and chemoradiosensitivity.
    UNASSIGNED: All patients affected by SNC who received curative treatment, including surgery, at the Unit of Otorhinolaryngology-Head and Neck Surgery of the University of Brescia between October 1998 and February 2019 were included in the analysis. The institutional series was reviewed and a survival analysis was performed. Machine learning and multivariable statistical methods were employed to develop, analyze, and test 3 experimental classifications (classification #1, based on cytomorphological, histomorphological, and differentiation information; classification #2, based on differentiation information; and classification #3, based on locoregional extension) of SNC, based on the inherent clinicopathological information. The association of experimental classifications with prognosis and chemoradiosensitivity was tested.
    UNASSIGNED: The study included 145 patients. From a prognostic standpoint, the machine learning-generated classification of SNC provided better prediction than the current World Health Organization classification. However, the prediction of the chemoradiosensitivity of SNC was not achievable.
    UNASSIGNED: Reorganization of clinicopathological information, with special reference to those related to tumor differentiation, can improve the reliability of prognosis of SNC. Prediction of chemoradiosensitivity remains an unmet need and further research is required.
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  • 文章类型: Journal Article
    目的:评估基于多参数MRI的影像组学特征(RS)对术前评估鼻腔鼻窦恶性肿瘤Ki-67增殖状态的预测能力。
    方法:回顾性分析了在两个医疗中心接受多参数MRI检查的共128例鼻窦恶性肿瘤患者。来自一个医疗中心(n=77)的数据用于开发预测模型,来自其他医疗中心(n=51)的数据构成测试数据集。回顾了临床数据和常规MRI检查结果,以确定重要的预测因素。使用最大相关性最小冗余和最小绝对收缩和选择算子算法来确定影像组学特征。随后,使用逻辑回归(LR)算法建立RS。使用校准评估RS的预测性能,决策曲线分析(DCA),准确度,AUC。
    结果:根据临床数据和常规MRI结果,未观察到高Ki-67增殖的独立预测因子。基于单参数MRI建立RS-T1,RS-T2和RS-T1c(对比增强T1WI)。RS组合(组合T1WI,FS-T2WI,和T1c特征)是基于多参数MRI开发的,并实现了0.852(0.733-0.971)和86.3%的AUC和准确性,分别,在测试数据集上。校准曲线和DCA在临床实践中显示出改善的适应性和益处。
    结论:基于MRI的多参数RS可用作非侵入性,可靠,和术前评估Ki-67增殖状态的准确工具,以克服鼻窦恶性肿瘤的采样偏倚。
    结论:•基于MRI的多参数影像组学特征(RS)用于术前评估Ki-67在鼻窦恶性肿瘤中的增殖状态。•使用最大相关性最小冗余(mRMR)和最小绝对收缩和选择算子(LASSO)算法来确定影像组学特征。•使用逻辑回归(LR)算法建立RS。
    OBJECTIVE: To assess the predictive ability of a multi-parametric MRI-based radiomics signature (RS) for the preoperative evaluation of Ki-67 proliferation status in sinonasal malignancies.
    METHODS: A total of 128 patients with sinonasal malignancies that underwent multi-parametric MRIs at two medical centres were retrospectively analysed. Data from one medical centre (n = 77) were used to develop the predictive models and data from the other medical centre (n = 51) constitute the test dataset. Clinical data and conventional MRI findings were reviewed to identify significant predictors. Radiomics features were determined using maximum relevance minimum redundancy and least absolute shrinkage and selection operator algorithms. Subsequently, RSs were established using a logistic regression (LR) algorithm. The predictive performance of RSs was assessed using calibration, decision curve analysis (DCA), accuracy, and AUC.
    RESULTS: No independent predictors of high Ki-67 proliferation were observed based on clinical data and conventional MRI findings. RS-T1, RS-T2, and RS-T1c (contrast enhancement T1WI) were established based on a single-parametric MRI. RS-Combined (combining T1WI, FS-T2WI, and T1c features) was developed based on multi-parametric MRI and achieved an AUC and accuracy of 0.852 (0.733-0.971) and 86.3%, respectively, on the test dataset. The calibration curve and DCA demonstrated an improved fitness and benefits in clinical practice.
    CONCLUSIONS: A multi-parametric MRI-based RS may be used as a non-invasive, dependable, and accurate tool for preoperative evaluation of the Ki-67 proliferation status to overcome the sampling bias in sinonasal malignancies.
    CONCLUSIONS: • Multi-parametric MRI-based radiomics signatures (RSs) are used to preoperatively evaluate the proliferation status of Ki-67 in sinonasal malignancies. • Radiomics features are determined using maximum relevance minimum redundancy (mRMR) and least absolute shrinkage and selection operator (LASSO) algorithms. • RSs are established using a logistic regression (LR) algorithm.
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  • 文章类型: Journal Article
    粘液纤维肉瘤是一种恶性多形性成纤维细胞肉瘤,具有不同的粘液样间质,并以独特的曲线血管模式为特征。在头部和颈部,粘液纤维肉瘤极为罕见,迄今为止,文献中只有少数病例报告。我们报告了两个机构的第一例原发性鼻窦黏液纤维肉瘤。在这5例病例中(2名女性,3男,52-82岁),四个来自上颌窦,一个来自蝶窦。4例患者接受了手术切除,3例接受了辅助放疗。肿瘤的最大尺寸为2.9至5.6cm。所有肿瘤均表现出广泛的粘液样间质(>50%粘液样成分),具有特征性曲线,细长的,肿瘤细胞血管周围凝结的薄壁脉管系统。除一个外,所有其他均被归类为中高级粘液纤维肉瘤。在有随访信息的四名患者中,3例报告无局部复发或远端转移,其中一人局部复发。粘液纤维肉瘤应包括在具有多形性梭形细胞形态和“无效”免疫表型的鼻窦肿瘤的鉴别诊断中。
    Myxofibrosarcoma is a malignant pleomorphic fibroblastic sarcoma with variably myxoid stroma, and is characterised by a distinctive curvilinear vascular pattern. In the head and neck area, myxofibrosarcoma is extremely rare, with only a handful of case reports in the literature to date. We report the first case series of primary sinonasal myxofibrosarcoma across two institutions. Among the five cases (2 female, 3 males, aged 52-82 years old), four arose from the maxillary sinus and one from the sphenoid sinus. Four patients received surgical resection and three with adjuvant radiotherapy. The tumours ranged from 2.9 to 5.6 cm in greatest dimensions. All tumours demonstrated extensive myxoid stroma (>50% myxoid component) with a characteristic curvilinear, elongated, thin-walled vasculature with perivascular condensation of tumour cells. All but one were classified as intermediate to high grade myxofibrosarcoma. Among the four patients with follow-up information, three reported no local recurrence or distal metastasis, and one had local recurrence. Myxofibrosarcoma should be included in the differential diagnosis of sinonasal tumours with a pleomorphic spindle cell morphology and a \'null\' immunophenotype.
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  • 文章类型: Journal Article
    目的:该项目旨在前瞻性和客观地评估原发性纤毛运动障碍(PCD)儿童的耳鼻喉表现和生活质量,并将这些发现与健康的儿科对照进行比较。
    方法:横断面。
    方法:两个高容量儿科PCD专业中心。
    方法:标准化临床评估;鼻窦结果测试22(SNOT-22);听力环境和反射生活质量(HEAR-QL);反流症状指数(RSI);鼻窦标准化体检,喉,和耳科系统;收集了包括纯音听力图(PTAs)和鼻窦培养在内的调查。
    结果:招募了47名PCD儿童和25名对照参与者。PCD患儿上呼吸道症状多于健康儿童。他们在SNOT-22和RSI中的得分明显更高,显示更严重的鼻窦和反流症状,与健康儿童相比,HEAR-QL指数的生活质量较差(P<0.05)。52%的PCD相关听力损失儿童没有意识到他们在听力学评估中存在的听力缺陷。只有23%的有通风管的儿童有慢性耳漏,其中大多数是很容易控制与耳滴剂。此外,尽管所有PCD患儿都有慢性鼻-鼻窦炎,其中只有36%的人使用局部鼻腔治疗。从中鼻道培养的最常见细菌是金黄色葡萄球菌,占47人中的11人(23%),其次是肺炎链球菌,占47人中的10人(21%)。
    结论:此多中心队列研究强调了耳鼻咽喉科参与治疗儿童PCD的重要性。更严格的耳鼻喉科管理可能会降低PCD儿童的总体发病率并改善其生活质量。
    OBJECTIVE: This project aims to prospectively and objectively assess otolaryngological manifestations and quality of life of children with primary ciliary dyskinesia (PCD) and compare these findings with healthy pediatric controls.
    METHODS: Cross-sectional.
    METHODS: Two high-volume pediatric PCD specialty centers.
    METHODS: Standardized clinical assessment; Sino-Nasal Outcome Test 22 (SNOT-22); Hearing Environment and Reflection Quality of Life (HEAR-QL); Reflux Symptom Index (RSI); standardized physical examination of the sinonasal, laryngeal, and otological systems; and investigations including pure-tone audiograms (PTAs) and sinonasal cultures were collected.
    RESULTS: Forty-seven children with PCD and 25 control participants were recruited. Children with PCD had more upper airway symptoms than healthy children. They had significantly higher scores in both SNOT-22 and RSI, indicating worse sinonasal and reflux symptoms, with worse quality of life on the HEAR-QL index compared to healthy children (P < .05). Fifty-two percent of children with PCD-related hearing loss were not aware of their hearing deficit that was present on audiological assessment, and only 23% of children who had ventilation tubes had chronic otorrhea, most of which was easily controlled with ototopic drops. Furthermore, although all children with PCD had chronic rhinosinusitis, only 36% of them were using topical nasal treatment. The most common bacteria cultured from the middle meatus were Staphylococcus aureus in 11 of 47 (23%), followed by Streptococcus pneumoniae in 10 of 47 (21%).
    CONCLUSIONS: This multisite cohort highlights the importance of otolaryngology involvement in the management of children with PCD. More rigorous otolaryngological management may lead to reductions in overall morbidity and improve quality of life for children with PCD.
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