• 文章类型: Case Reports
    伪痛风的特征是焦磷酸钙二水合物晶体(CPPD)的沉积,主要影响大型关节。关节外表现,特别是在头部和颈部,非常罕见。我们报告了一例独特的中耳双侧孤立性假性发声,表现为进行性传导性听力损失,这是假性发声的第一个也是唯一的症状。耳镜和CT扫描通常会产生鉴别诊断,包括肿瘤或胆脂瘤。需要进行组织病理学检查的手术。在鉴定二水合焦磷酸钙晶体后确认了明确的诊断。在大多数情况下,晶体的去除导致传导性听力损失的解决。喉镜,2024.
    Pseudogout is characterized by the deposition of calcium pyrophosphate dihydrate crystals (CPPD), primarily affecting large joints. Extra-articular manifestations, particularly in the head and neck region, are exceedingly rare. We report a unique case of bilateral isolated pseudogout of the middle ear manifesting with progressive conductive hearing loss as the first and only symptom of pseudogout. Otoscopy and CT scan often yield a differential diagnosis that includes tumors or cholesteatoma, necessitating surgery with histopathological examination. The definitive diagnosis is confirmed upon identification of calcium pyrophosphate dihydrate crystals. In most cases, removal of the crystals results in resolution of conductive hearing loss. Laryngoscope, 2024.
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  • 文章类型: Journal Article
    该病例报告描述了一名34岁女性急性淋巴细胞白血病和移植物抗宿主病(GVHD)骨髓移植后罕见的并发过敏性真菌性鼻鼻窦炎(AFRS)和慢性肉芽肿性侵袭性真菌性鼻窦炎(CGIFS)。最初表现为鼻漏和鼻塞,患者被诊断为右上颌窦AFRS,随后在左鼻腔进行了CGIFS的术后疗程,展示独特的事件。她在诊断期间没有免疫受损。CGIFS可能是由于手术而发生的;然而,伏立康唑导致显著改善。该病例突出了慢性鼻窦炎和GVHD病史患者的非侵袭性和侵袭性真菌感染,并强调了诊断和治疗此类病例的复杂性。
    This case report describes rare concomitant allergic fungal rhinosinusitis (AFRS) and chronic granulomatous invasive fungal sinusitis (CGIFS) in a 34-year-old woman with acute lymphoblastic leukemia and graft-versus-host disease (GVHD) post bone marrow transplantation. Initially presenting with rhinorrhea and nasal obstruction, the patient was diagnosed with AFRS in the right maxillary sinus, followed by a postoperative course of CGIFS in the left nasal cavity, showcasing the unique occurrence. She was not immunocompromised during diagnosis. CGIFS may have occurred because of surgery; however, voriconazole led to significant improvement. This case highlights noninvasive and invasive fungal infections in patients with chronic rhinosinusitis and a history of GVHD and underscores the complexity of diagnosing and managing such cases.
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  • 文章类型: Case Reports
    背景:噬血细胞性淋巴组织细胞增生症(HLH)是一种可能危及生命的综合征,早期识别和治疗对于改善预后至关重要。HLH的特点是不受控制的免疫激活导致发烧,血细胞减少,肝脾肿大,凝血异常,和升高的典型标记。这种情况可以是遗传的或继发性的,后者通常由感染引发。这里,我们介绍了急性中耳炎(AOM)继发HLH的独特病例,常见的耳部感染.
    方法:我们描述了一个4岁男孩,他最初表现为高烧和耳痛,后来诊断为双侧AOM。尽管有抗生素治疗,他的病情恶化。
    方法:患者符合HLH诊断标准。
    方法:使用免疫球蛋白联合治疗的积极治疗,静脉注射类固醇(地塞米松),环孢菌素,并进行依托泊苷。
    结果:治疗1个月后,观察到耳科症状的改善,血液学检查结果逐渐好转并恢复正常。
    结论:AOM和HLH之间的联系可能与炎症反应和免疫机制有关,强调在严重感染病例中考虑HLH的重要性。这种情况强调需要及时诊断和管理,尤其是在继发性HLH情况下,改善患者预后。必须意识到这两个条件之间的潜在相关性,医疗保健专业人员应该考虑HLH的可能性。
    BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome for which early recognition and treatment are essential for improving outcomes. HLH is characterized by uncontrolled immune activation leading to fever, cytopenias, hepatosplenomegaly, coagulation abnormalities, and elevated typical markers. This condition can be genetic or secondary, with the latter often triggered by infections. Here, we present a unique case of HLH secondary to acute otitis media (AOM), a common ear infection.
    METHODS: We describe a 4-year-old boy who initially presented with a high fever and otalgia, later diagnosed with bilateral AOM. Despite antibiotic treatment, his condition deteriorated.
    METHODS: The patient fulfilled diagnostic criteria for HLH.
    METHODS: Aggressive treatment by using combination therapy with immunoglobulins, intravenous steroids (dexamethasone), cyclosporine, and etoposide was performed.
    RESULTS: After 1 month of treatment, improvement in the otologic symptoms was observed, and hematological findings gradually improved and normalized.
    CONCLUSIONS: The link between AOM and HLH may be associated with inflammatory responses and immunological mechanisms, highlighting the importance of considering HLH in severe infection cases. This case emphasizes the need for prompt diagnosis and management, especially in secondary HLH scenarios, to improve patient outcomes. It is imperative to be aware of the potential correlation between these 2 conditions, and healthcare professionals should consider the likelihood of HLH.
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  • 文章类型: Case Reports
    急性化脓性中耳炎偶尔会导致面瘫,这需要及时诊断和治疗。面瘫,贝尔麻痹的同义词,是导致面部肌肉一侧快速减弱的一种情况,导致那一边的脸下垂。决定病情进程的主要因素是通过物理治疗进行康复。这里,我们介绍了一名26岁女性的病例,她在2024年2月21日左耳感到不适,但当时选择忽略.她观察到嘴里突然出现偏差,并参观了一家乡村医院,她被录取的地方.在检查过程中观察到面部不对称,她被发现在House-Brackmann量表上有V级.通过哑剧治疗开始了接近正常的肌肉动作,通过面部本体感觉神经肌肉促进和电刺激给予本体感觉刺激。所有这些方法都以显著的方式使患者受益。
    Acute suppurative otitis media can occasionally result in facial paralysis, which calls for prompt diagnosis and treatment. Facial paralysis, a synonym for Bell\'s palsy, is a condition that causes rapid weakening of one side of the face muscles, leading to drooping of the face on that side. A major factor in determining the course of the condition is rehabilitation through physiotherapy. Here, we present a case of a 26-year-old female who felt discomfort in her left ear on February 21, 2024, but chose to ignore it then. She observed an abrupt deviation in her mouth and visited a rural hospital, where she was admitted. Facial asymmetry was observed during the examination, and she was found to have a grade V on the House-Brackmann scale. A near-normal muscle action was initiated by mime therapy, and proprioceptive stimulation was given by facial proprioceptive neuromuscular facilitation along with electrical stimulation. All these approaches benefited the patient in a significant manner.
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  • 文章类型: Journal Article
    听力障碍是人类最普遍的感觉疾病,可以对发育产生巨大影响,和保存,我们的认知能力和社交互动。目前,世界上20%的人口患有某种形式的听力障碍;预计到2050年,这一比例将上升到25%。尽管有如此惊人的疾病负担,以及它对社会造成的巨大破坏,人类的医疗和经济结构,我们的预测能力,或防止,听力损失确实非常差。我们在这里提出了我们研究耳聋方法的范式转变。通过在形态不同的模型中更有力地利用人类听觉和听觉之间的分子遗传保守性,比如果蝇果蝇,我们相信,可以对听力和耳聋有更深入的了解。一种超越“耳聋基因”表面的理解,以探测听力的潜在基石,它在整个类群中共享,并部分跨模式共享。当谈到了解人类感官功能的运作(和故障)时,简单的果蝇可以提供很多东西,而苍蝇眼有时可能是人耳的强大模型。特别是使用飞行化身,其中人类(例如特定患者)的特定分子(遗传或蛋白质组)状态通过实验复制,为了在受控而自然的环境中研究相应的分子机制(例如特定疾病),是一种承诺多种前所未有的见解的工具。使用苍蝇-和飞行化身-将使人类受益,并有助于增强其他科学模型的功能,比如老鼠。
    Hearing impairment is the most prevalent sensory disease in humans and can have dramatic effects on the development, and preservation, of our cognitive abilities and social interactions. Currently 20 % of the world\'s population suffer from a form of hearing impairment; this is predicted to rise to 25 % by 2050. Despite this staggering disease load, and the vast damage it inflicts on the social, medical and economic fabric of humankind, our ability to predict, or prevent, the loss of hearing is very poor indeed. We here make the case for a paradigm shift in our approach to studying deafness. By exploiting more forcefully the molecular-genetic conservation between human hearing and hearing in morphologically distinct models, such as the fruit fly Drosophila melanogaster, we believe, a deeper understanding of hearing and deafness can be achieved. An understanding that moves beyond the surface of the \'deafness genes\' to probe the underlying bedrock of hearing, which is shared across taxa, and partly shared across modalities. When it comes to understanding the workings (and failings) of human sensory function, a simple fruit fly has a lot to offer and a fly eye might sometimes be a powerful model for a human ear. Particularly the use of fly avatars, in which specific molecular (genetic or proteomic) states of humans (e.g. specific patients) are experimentally reproduced, in order to study the corresponding molecular mechanisms (e.g. specific diseases) in a controlled yet naturalistic environment, is a tool that promises multiple unprecedented insights. The use of the fly - and fly avatars - would benefit humans and will help enhance the power of other scientific models, such as the mouse.
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  • 文章类型: Journal Article
    目的:探讨外科,Osia2植入物的听力学和患者报告结果。方法:对2022年4月至2023年11月期间接受植入的14名连续受试者的数据进行了回顾。十名受试者有传导性听力损失,3人患有混合性听力损失,1人患有单侧耳聋(SSD)。高音阈值,在安静和噪音中的纯音平均(PTA4)和言语歧视得分(SDS)在没有帮助的情况下进行了测定。从两个标准化问卷中确定主观结果:(1)国际助听器结果清单(IOI-HA)和(2)言语,听力量表12的空间和质量(SSQ12b)。结果:4例患者术后出现意外疼痛。颤音阈值在所有频率上都表现出一致的降低,有助于辅助PTA4的平均降低27dB。SDS表现出显著的改进,在50dB时增加57.3%,在65dB时增加55.6%。在噪音中,SDS表现出43.9%的改善。平均IOI-HA评分为3.8,SSQ12b的平均总分为6.6,各亚组的结果一致。结论:对于传导性或混合性听力损失的个体,Osia装置是一个有希望的推荐,也可能是那些有SSD的人。其安全性和有效性与更广泛的有源经皮设备类别一致,与经皮替代方法相比,伤口感染的风险降低。听力学评估和主观评估均显示出积极的结果。
    Objective: To examine the surgical, audiological and patient-reported outcomes of the Osia 2 implant. Methods: Data from 14 consecutive subjects undergoing implantation between April 2022 and November 2023 were reviewed. Ten subjects had conductive hearing loss, three had mixed hearing loss and one had single-sided deafness (SSD). Warble tone thresholds, Pure Tone Average (PTA4) and Speech Discrimination Score (SDS) in quiet and in noise were determined unaided and aided. The subjective outcome was determined from two standardized questionnaires: (1) International Outcome Inventory for Hearing Aids (IOI-HA) and (2) Speech, Spatial and Qualities of Hearing Scale 12 (SSQ12b). Results: Unexpected postoperative pain was found in four cases. The warble tone thresholds exhibited a consistent reduction across all frequencies, contributing to a mean decrease of 27 dB in the aided PTA4. SDS demonstrated notable improvements, with a 57.3% increase at 50 dB and a 55.6% increase at 65 dB. In noise, SDS exhibited a 43.9% improvement. The mean IOI-HA Score was 3.8, and the mean overall score for SSQ12b was 6.6, with consistent findings across the subgroups. Conclusions: The Osia device emerges as a promising recommendation for individuals with conductive or mixed hearing loss, possibly also for those with SSD. Its safety and efficacy profile aligns with the broader category of active transcutaneous devices, demonstrating a reduced risk of wound infection compared to percutaneous alternatives. Both audiological assessments and subjective evaluations revealed positive outcomes.
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  • 文章类型: Journal Article
    背景:无创通气(NIV)是高碳酸血症慢性呼吸衰竭(CRF)的标准护理。阻塞性睡眠呼吸暂停综合征(OSA)通常会导致CRF患者的通气不足。CPAP可以改善某些COPD和肥胖患者的高碳酸血症,比如NIV.我们旨在描述长期通气患者从NIV转换到CPAP的情况,并确定与成功转换相关的因素。
    方法:在本病例对照研究中,在2015年至2020年期间在第戎大学医院接受治疗的394名通气患者中,有88名连续接受NIV-CPAP转换的患者与266名对照进行了比较。他们遵循了标准化的协议,包括在NIV戒断三晚后记录的poly(somno)图。如果证实重度OSA,则进行CPAP试验。在CPAP下1和23[14-46]个晚上后检查患者的反复通气不足。
    结果:患者为53%的男性,中位年龄65[56-74]岁,和中位数BMI34[25-38.5]kg/m2。64%的患者安全切换并保持长期CPAP。在多变量分析中,NIV-CPAP转换的概率与年龄相关(OR:1.3[1.01-1.06]),BMI(OR:1.7[1.03-1.12]),CRF病因(或COPD:20.37[4.2-98,72],或肥胖:7.31[1.58-33.74]),NIV开始的情况(急性加重OR:11.64[2.03-66.62]),较低的压力支持(或:0.90[0.73-0.92]),较低的基线PaCO2(OR:0.85[0.80-0.91])和较低的依从性(OR:0.76[0.64-0.90])。在72名接受CPAP治疗回家的患者中,压力支持水平是与NIV-CPAP转换结果相关的唯一因素,即使在调整BMI和年龄(p=0.01)后,也存在非线性相关性。慢性呼吸衰竭的病因,年龄,BMI,基线PaCO2,NIV启动情况,家庭NIV时间或NIV依从性不能预测NIV-CPAP转换的结果.
    结论:NIV-CPAP转换在稳定性肥胖和COPD合并OSA患者的现实生活中是可能的。
    BACKGROUND: Non-invasive ventilation (NIV) is a standard of care for hypercapnic chronic respiratory failure (CRF). Obstructive sleep apnea syndrome (OSA) frequently contributes to hypoventilation in CRF patients. CPAP improves hypercapnia in selected COPD and obese patients, like NIV. We aimed to describe the profile of patients switching from NIV to CPAP in a cohort of patients on long-term ventilation and to identify the factors associated with a successful switch.
    METHODS: In this case-control study, 88 consecutive patients who were candidates for a NIV-CPAP switch were compared with 266 controls among 394 ventilated patients treated at the Dijon University Hospital between 2015 and 2020. They followed a standardized protocol including a poly(somno)graphy recorded after NIV withdrawal for three nights. CPAP trial was performed if severe OSA was confirmed. Patients were checked for recurrent hypoventilation after 1 and 23[14-46] nights under CPAP.
    RESULTS: Patients were 53% males, median age 65 [56-74] years, and median BMI 34 [25-38.5] kg/m2. Sixty four percent of patients were safely switched and remained on long-term CPAP. In multivariate analysis, the probability of a NIV-CPAP switch was correlated to older age (OR: 1.3 [1.01-1.06]), BMI (OR: 1.7 [1.03-1.12]), CRF etiology (OR for COPD: 20.37 [4.2-98,72], OR for obesity: 7.31 [1.58-33.74]), circumstances of NIV initiation (OR for acute exacerbation: 11.64 [2.03-66.62]), lower pressure support (OR: 0.90 [0.73-0.92]), lower baseline PaCO2 (OR: 0.85 [0.80-0.91]) and lower compliance (OR: 0.76 [0.64-0.90]). Among 72 patients who went home under CPAP, pressure support level was the only factor associated with the outcome of the NIV-CPAP switch, even after adjustment for BMI and age (p=0.01) with a non-linear correlation. Etiology of chronic respiratory failure, age, BMI, baseline PaCO2, circumstances of NIV initiation, time under home NIV or NIV compliance were not predictive of the outcome of the NIV-CPAP switch.
    CONCLUSIONS: A NIV-CPAP switch is possible in real life conditions in stable obese and COPD patients with underlying OSA.
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  • 文章类型: Case Reports
    腮腺原发性恶性黑色素瘤是一种罕见且复杂的临床实体,由于肿瘤中不存在黑色素及其与其他低分化病变的组织学相似性,因此提出了许多诊断挑战。准确的诊断通常需要先进的成像技术和免疫组织化学程序来识别特定的黑色素瘤标志物。治疗学上,经常推荐同时进行宫颈解剖的全腮腺切除术,虽然结合手术的方法,化疗,放射治疗,甚至免疫疗法也在探索中。尽管取得了这些进步,恶性腮腺黑色素瘤仍然与严峻的预后有关,强调正在进行的研究的重要性,以改善治疗方案和了解这种罕见的病理。
    The primary malignant melanoma of the parotid is a rare and complex clinical entity, posing numerous diagnostic challenges due to the absence of melanin in the tumor and its histological similarities with other poorly differentiated lesions. Accurate diagnosis often requires advanced imaging techniques and immunohistochemical procedures to identify specific melanoma markers. Therapeutically, total parotidectomy with simultaneous cervical dissection is frequently recommended, although approaches combining surgery, chemotherapy, radiotherapy, or even immunotherapy are also being explored. Despite these advancements, malignant parotid melanoma continues to be associated with a grim prognosis, emphasizing the importance of ongoing research to improve therapeutic options and understanding of this rare pathology.
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  • 文章类型: Journal Article
    前庭神经鞘瘤是一种良性肿瘤;然而,神经鞘瘤本身和干预措施可导致感音神经性听力损失。大多数前庭神经鞘瘤是单侧肿瘤,仅在一侧影响听力。注意力集中在改善单侧听力损失患者的生活质量上,并强调了解决这一问题的治疗干预措施。在这里,我们遇到了一名患者,根据术前发现,他是听力保留手术的候选人,并且在手术后听力不可用,根据加德纳-罗伯逊分类。术后,患者的听力理解能力和定位声源的能力下降。他配备了双侧助听器,他定位声源的能力提高了。尽管患者患侧有术后不可用的听力,未患侧有与年龄相关的听力损失,双耳助听器对他的日常生活很有用。因此,患者能够保持双耳听觉效果,声源定位能力得到改善。该报告强调了前庭神经鞘瘤保留听力的重要性,作为术后并发症,对听力损失康复的需求会增加,即使听力损失是无用的。
    A vestibular schwannoma is a benign tumor; however, the schwannoma itself and interventions can cause sensorineural hearing loss. Most vestibular schwannomas are unilateral tumors that affect hearing only on one side. Attention has focused on improving the quality of life for patients with unilateral hearing loss and therapeutic interventions to address this issue have been emphasized. Herein, we encountered a patient who was a candidate for hearing preservation surgery based on preoperative findings and had nonserviceable hearing after the surgery, according to the Gardner-Robertson classification. Postoperatively, the patient had decreased listening comprehension and ability to localize sound sources. He was fitted with bilateral hearing aids, and his ability to localize sound sources improved. Although the patient had postoperative nonserviceable hearing on the affected side and age-related hearing loss on the unaffected side, hearing aids in both ears were useful for his daily life. Therefore, the patient was able to maintain a binaural hearing effect and the ability to localize the sound source improved. This report emphasizes the importance of hearing preservation with vestibular schwannomas, and the demand for hearing loss rehabilitation as a postoperative complication can increase, even if hearing loss is nonserviceable.
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  • 文章类型: Journal Article
    支持或反对使用干预措施的建议需要考虑理想和不良效果以及患者的价值观和偏好(V&P)。在决策背景下,患者V&P代表人们对决策结果的相对重要性。因此,干预的理想和不良效果之间的平衡不仅应取决于益处和危害之间的差异,还应取决于患者对它们的价值。因此,V&P是在建议分级制定的证据到决策框架中制定指南建议时要考虑的标准之一,评估,发展和评价(等级)工作组。患者V&P可以通过公用事业进行量化,可以使用直接方法(例如,标准赌博或时间权衡)或间接方法(使用经过验证的仪器来测量与健康相关的生活质量,如EQ-5D)。等级方法建议进行系统审查,以总结所有可用证据,并评估V&P的确定性程度。在这篇文章中,我们讨论了考虑患者V&P的重要性,并举例说明了2024年以人为中心的变应性鼻炎及其对哮喘(ARIA)指南的影响.
    Recommendations for or against the use of interventions need to consider both desirable and undesirable effects as well as patients\' values and preferences (V&P). In the decision-making context, patients\' V&P represent the relative importance people place on the outcomes resulting from a decision. Therefore, the balance between desirable and undesirable effects from an intervention should depend not only on the difference between benefits and harms but also on the value that patients place on them. V&P are therefore one of the criteria to be considered when formulating guideline recommendations in the Evidence-to-Decision framework developed by the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) Working Group. Patients\' V&P may be quantified through utilities, which can be elicited using direct methods (e.g., standard gamble or time trade-off) or indirect methods (using validated instruments to measure health-related quality of life, such as EQ-5D). The GRADE approach recommends conducting systematic reviews to summarise all the available evidence and assess the degree of certainty on V&P. In this article, we discuss the importance of considering patients\' V&P and provide examples of how they are considered in the 2024 person-centred Allergic Rhinitis and its Impact on Asthma (ARIA) guidelines.
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