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Current Practices for Severe Alpha-1 Antitrypsin Deficiency Associated COPD and Emphysema 治疗与严重 Alpha-1 抗胰蛋白酶缺乏症相关的慢性阻塞性肺病和肺气肿的现行方法
Pub Date : 2024-07-26 DOI: 10.29328/journal.jprr.1001058
Nicholson Mj, Seigo M
Alpha-1 antitrypsin deficiency is a genetic disorder that can lead to chronic obstructive pulmonary disease and emphysema. Although it is the most well-studied genetic risk factor for emphysema, data is still scarce. Traditionally, medical therapy is similar to standard chronic obstructive pulmonary disease patients. Over the past several decades, enzyme augmentation therapy has emerged as a highly utilized alpha-1 antitrypsin-specific therapy. It has become the standard of care for severe alpha-1 antitrypsin deficiency despite unclear effects on a multitude of clinical outcomes. Significant data supports interventional therapies, including lung volume reduction surgery and bronchoscopic lung volume reduction, for chronic obstructive pulmonary disease patients without alpha-1 antitrypsin deficiency. These interventions have less robust data in the treatment of alpha-1 antitrypsin-induced chronic obstructive pulmonary disease. This review will explore the data regarding various treatment options for severe alpha-1 antitrypsin deficiency associated with chronic obstructive pulmonary disease and emphysema.
α-1抗胰蛋白酶缺乏症是一种可导致慢性阻塞性肺病和肺气肿的遗传性疾病。虽然它是研究最充分的肺气肿遗传风险因素,但相关数据仍然很少。传统的药物治疗与标准的慢性阻塞性肺病患者类似。在过去的几十年中,酶增强疗法已成为使用率极高的α-1 抗胰蛋白酶特异性疗法。尽管对多种临床结果的影响尚不明确,但它已成为治疗严重α-1 抗胰蛋白酶缺乏症的标准疗法。有大量数据支持对无α-1 抗胰蛋白酶缺乏症的慢性阻塞性肺病患者采取介入疗法,包括肺容积缩小手术和支气管镜肺容积缩小术。在治疗α-1 抗胰蛋白酶引起的慢性阻塞性肺病方面,这些干预措施的数据不太可靠。本综述将探讨与慢性阻塞性肺病和肺气肿相关的严重α-1抗胰蛋白酶缺乏症的各种治疗方案的相关数据。
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引用次数: 0
Effect of Pulmonary Rehabilitation on Non-obstructive Disease Congenital Kyphoscoliosis Patient in Kuwait 肺康复对科威特非阻塞性疾病先天性脊柱侧凸患者的影响
Pub Date : 2024-07-18 DOI: 10.29328/journal.jprr.1001057
Almutairi Noura Mohammed
A case study of a non-COPD (Chronic Obstructive Pulmonary Disease) patient with congenital kyphoscoliosis to see the effect of pulmonary rehabilitation after 36 sessions given for chest disease in Kuwait with signs and symptoms, physical capacity, functional level, and Quality of life.
通过对一名患有先天性脊柱侧弯症的非慢性阻塞性肺病(COPD)患者的病例研究,了解在对科威特的胸部疾病患者进行 36 次肺康复治疗后,在体征和症状、体能、功能水平和生活质量方面的效果。
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引用次数: 0
An Interesting Case of COPD Exacerbation Presenting with Mixed Features of Intracranial Hypertension and Hypercapnic Encephalopathy 一例有趣的慢性阻塞性肺疾病加重并伴有颅内高压和高碳酸血症脑病混合症状的病例
Pub Date : 2024-07-02 DOI: 10.29328/journal.jprr.1001056
Chaoneng Wu, Mendez Gustavo, Gandhi Aaron, Kambhatla Sujata, Siddiqui Furqan, Pasha Amin, Madhavan Ramesh
Background: Idiopathic intracranial hypertension (IIH or pseudotumor cerebri) has two major morbidities: papilledema with visual loss and disabling headache. Intracranial Venous Hypertension (IVH) is a fundamental mechanism of IIH. Although traditionally considered limiting to the central nervous system, evidence suggests IIH as a systemic disease associated with cardiorespiratory disorders, which has been far less comprehended. Case Report: A 60-year-old female with Chronic Obstructive Pulmonary Disease (COPD) was admitted for dyspnea and developed a coma with a pH of 7.01 and pCO2 of 158 mmHg. She was intubated and had persistent nuchal rigidity, a brief myoclonus episode with a negative electroencephalogram, and negative CT head studies. A Lumbar Puncture (LP) revealed elevated opening pressure (35 cmH2O) with normal Cerebral Spinal Fluid (CSF) studies. Her nuchal rigidity improved after the removal of 40 mL CSF. The ophthalmology examination the next day after her the large volume LP didn’t show visual loss or papilledema. The patient improved clinically and was extubated two days later. Her echocardiogram showed a dilated right ventricle with pulmonary hypertension. The patient was discharged home. Discussion: IIH is different from hypercapnic encephalopathy and characterized by increased intracranial pressure with papilledema, vision loss, and debilitating headache. Hypercapnia-induced increased intracranial venous flow and pulmonary hypertension-caused elevated central venous pressure with consequent outflow resistance lead to IVH. In hypercapnic encephalopathy, the presentation is mostly cognitive changes. In this case, nuchal rigidity with a negative CT head scan triggered the investigation of IIH. Conclusion: A deep understanding of the relationship between COPD and IIH is vital. There is insufficient evidence to recommend routine eye examinations in COPD patients for papilledema and to conduct a pulmonary function test for a newly diagnosed IIH patient. However, we highly suggest a timely ophthalmology exam prior to performing an LP in COPD patients with suspecting IIH to avoid unnecessary procedures and meanwhile improve clinical outcomes.
背景:特发性颅内高压(IIH 或假性脑瘤)有两大主要病症:视力丧失的乳头水肿和致残性头痛。颅内静脉高压(IVH)是 IIH 的基本机制。虽然传统上认为 IIH 仅局限于中枢神经系统,但有证据表明 IIH 是一种与心肺功能紊乱相关的全身性疾病,而人们对这种疾病的认识还远远不够。病例报告:一名患有慢性阻塞性肺病(COPD)的 60 岁女性因呼吸困难入院,随后出现昏迷,pH 值为 7.01,pCO2 为 158 mmHg。患者插管后出现持续性颈项强直、短暂的肌阵挛发作,脑电图呈阴性,头部 CT 检查呈阴性。腰椎穿刺(LP)显示开口压力升高(35 cmH2O),脑脊液(CSF)检查正常。取出 40 毫升 CSF 后,她的颈部僵硬症状有所改善。大容量腹腔穿刺术后第二天的眼科检查未发现视力下降或乳头水肿。患者的临床症状有所改善,两天后拔管。她的超声心动图显示右心室扩张并伴有肺动脉高压。患者出院回家。讨论IIH 不同于高碳酸血症脑病,其特点是颅内压增高并伴有乳头水肿、视力减退和头痛。高碳酸血症引起的颅内静脉流量增加和肺动脉高压引起的中心静脉压升高以及随之而来的流出阻力会导致 IVH。高碳酸血症脑病主要表现为认知改变。在本病例中,头部 CT 扫描阴性的颈部僵硬引发了对 IIH 的检查。结论深入了解慢性阻塞性肺病与 IIH 之间的关系至关重要。目前还没有足够的证据建议对慢性阻塞性肺病患者进行常规眼部检查,以确定是否存在乳头水肿,也没有足够的证据建议对新诊断的 IIH 患者进行肺功能检查。但是,我们强烈建议对怀疑患有 IIH 的 COPD 患者在进行 LP 检查之前及时进行眼科检查,以避免不必要的手术,同时改善临床预后。
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引用次数: 0
Stable COPD Picture through Exhaled Breath Condensate, Questionnaires and Tests. A Proof of Concept Study 通过呼出的冷凝液、问卷和测试获得稳定的慢性阻塞性肺病图像。概念验证研究
Pub Date : 2023-12-05 DOI: 10.29328/journal.jprr.1001049
Patsiris Stephanos, Afthinos Andreas, Augouste Ligeri, Valsami Katerina, Dimitras Antonis, Exarchos Themistoklis, Vlamos Panagiotis
Background: Changes in lung structures persist in stable Chronic Obstructive Pulmonary Disease (COPD), but their correlation with the clinical picture remains unclear. The purpose of this study was to assess the stable COPD picture via the relationship between exhaled breath condensate (EBC) particle concentration and the Saint George Respiratory Questionnaire (SGRQ), COPD Assessment Test (CAT), and six-minute walking test (6 MWT). Methods: 12 stable COPD and 12 healthy subjects participated in the study. The EBC was collected with Rtube and analyzed using the Accusizer FxNano. Particle concentration was measured and correlated with the findings of the tools used to assess the health status and functional profile of COPD. The results’ analysis was performed with the Spearman’s test and the Mann-Whitney U - test. Results: The COPD group presented a worse picture of health status and functional profile compared to the healthy group. Correlations were observed between components of the SGRQ and CAT. The two groups presented similar levels of EBC particle concentrations, but the number of small particles was higher in COPD subjects. A correlation of the EBC particle concentration with the activity and total score of the SGRQ was only observed in the healthy group. Conclusion: The total particle number was similar in the COPD and healthy groups. A few correlations between the EBC particles and tools used were also observed. The use of EBC particle concentration to monitor COPD status cannot be claimed with confidence because of the small sample size. Further research is necessary, particularly in large-scale groups.
背景:在慢性阻塞性肺病(COPD)病情稳定期,肺部结构会持续发生变化,但这些变化与临床表现的相关性仍不清楚。本研究旨在通过呼出气体冷凝物(EBC)颗粒浓度与圣乔治呼吸问卷(SGRQ)、慢性阻塞性肺病评估测试(CAT)和六分钟步行测试(6 MWT)之间的关系来评估慢性阻塞性肺病的稳定期症状。方法:12 名病情稳定的慢性阻塞性肺病患者和 12 名健康受试者参加了研究。使用 Rtube 收集 EBC,并使用 Accusizer FxNano 进行分析。测量粒子浓度,并将其与用于评估慢性阻塞性肺病患者健康状况和功能状况的工具的结果相关联。结果分析采用斯皮尔曼检验和曼-惠特尼 U 检验。结果显示与健康组相比,慢性阻塞性肺病组的健康状况和功能状况更差。SGRQ和CAT之间存在相关性。两组的 EBC 颗粒浓度水平相似,但慢性阻塞性肺病受试者的小颗粒数量更多。仅在健康组中观察到 EBC 颗粒浓度与活动和 SGRQ 总分之间存在相关性。结论慢性阻塞性肺病组和健康组的粒子总数相似。还观察到 EBC 颗粒与所用工具之间存在一些相关性。由于样本量较小,因此不能肯定使用 EBC 颗粒浓度来监测慢性阻塞性肺病的状况。有必要开展进一步研究,尤其是在大规模群体中开展研究。
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引用次数: 0
Clinical Approach to Immunotherapy-induced Type 1 Diabetes Mellitus: A Case of Pembrolizumab Associated Insulin-dependent Diabetes in a Patient with NSCLC 免疫治疗诱导的1型糖尿病的临床方法:1例非小细胞肺癌患者的派姆单抗相关胰岛素依赖性糖尿病
Pub Date : 2023-09-25 DOI: 10.29328/journal.jprr.1001047
Ntertsos Nikolaos, Christantoniou George, Kyrka Krystallia, Pezirkianidou Persefoni, Bikos Vasileios, Konstantina Papadaki, Tsiouda Theodora
As the introduction of immune checkpoint inhibitors in the treatment of various cancers is now proven to be already acquired knowledge, so does a new challenge arise for clinicians; the understanding, diagnosis, and management of the rarest adverse effects of immunotherapy. We present a case of type-1 diabetes Mellitus (T1DM) in a patient with non-small cell lung carcinoma (NSCLC) treated with pembrolizumab. Following ten cycles of treatment, our patient was diagnosed with T1DM after being admitted for diabetic ketoacidosis and stayed hospitalized in the ICU. Later, they continued treatment with insulin, having shown disease response to pembrolizumab, and resumed immunotherapy while on insulin. Immunotherapy-induced T1DM can sometimes occur with PD1/PD-L1 blockage therapies. It has a rapid onset, is characterized by insulin deficiency due to the autoimmune destruction of beta-cells, and usually presents itself with diabetic ketoacidosis. Unlike most of the other adverse effects of immunotherapy, glucocorticoids don’t seem to be of therapeutic value, and insulin substitution is required. Regular glucose monitoring can be key to early diagnosis and prevention of hospitalization.
随着免疫检查点抑制剂在各种癌症治疗中的应用已经被证明是已经获得的知识,因此对临床医生提出了新的挑战;了解、诊断和处理免疫治疗最罕见的不良反应。我们报告一例1型糖尿病(T1DM)患者与非小细胞肺癌(NSCLC)用派姆单抗治疗。经过10个周期的治疗,患者因糖尿病酮症酸中毒入院后被诊断为T1DM,在ICU住院治疗。后来,他们继续用胰岛素治疗,对派姆单抗显示出疾病反应,并在胰岛素治疗期间恢复免疫治疗。免疫治疗诱导的T1DM有时会发生在PD1/PD-L1阻断治疗中。它起病迅速,特点是由于自身免疫破坏β细胞导致胰岛素缺乏,通常表现为糖尿病酮症酸中毒。与免疫治疗的其他副作用不同,糖皮质激素似乎没有治疗价值,需要胰岛素替代。定期血糖监测是早期诊断和预防住院的关键。
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引用次数: 0
COVID-19 detection and classification: key AI challenges and recommendations for the way forward COVID-19的检测和分类:人工智能的主要挑战和未来的建议
Pub Date : 2023-05-25 DOI: 10.29328/journal.jprr.1001044
Althinyan Albatoul, Mirza Abdulrahman, Aly Sherin, Nouh Thamer, Mahboub Bassam, Salameh Laila, Alkubeyyer Metab, AlSalamah Shada A
Coronavirus disease (COVID-19) is a viral pneumonia that is found in China and has spread globally. Early diagnosis is important for effective and timely treatment. Thus, many ongoing studies attempt to solve key COVID-19 problems such as workload classification, detection, and differentiation from other pneumonia and healthy lungs using different imaging modalities. Researchers have identified some limitations in the deployment of deep learning methods to detect COVID-19, but there are still unmet challenges to be addressed. The use of binary classifiers or building classifiers based on only a few classes is some of the limitations that most of the existing research on the COVID-19 classification problem suffers from. Additionally, most prior studies have focused on model or ensemble models that depend on a flat single-feature imaging modality without using any clinical information or benefiting from the hierarchical structure of pneumonia, which leads to clinical challenges, and evaluated their systems using a small public dataset. Additionally, reliance on diagnostic processes based on CT as the main imaging modality, ignoring chest X-rays. Radiologists, computer scientists, and physicians all need to come to an understanding of these interdisciplinary issues. This article first highlights the challenges of deep learning deployment for COVID-19 detection using a literature review and document analysis. Second, it provides six key recommendations that could assist future researchers in this field in improving the diagnostic process for COVID-19. However, there is a need for a collective effort from all of them to consider the provided recommendations to effectively solve these issues.
冠状病毒病(COVID-19)是一种在中国发现并在全球传播的病毒性肺炎。早期诊断对于有效和及时的治疗非常重要。因此,许多正在进行的研究试图通过不同的成像方式解决COVID-19的关键问题,如工作量分类、检测以及与其他肺炎和健康肺的区分。研究人员已经发现,在部署深度学习方法检测COVID-19方面存在一些局限性,但仍有未解决的挑战需要解决。使用二元分类器或仅基于少数类构建分类器是大多数现有研究COVID-19分类问题所面临的一些局限性。此外,大多数先前的研究都集中在模型或集成模型上,这些模型或集成模型依赖于扁平的单一特征成像模式,而不使用任何临床信息或受益于肺炎的分层结构,这导致了临床挑战,并使用小型公共数据集评估了他们的系统。此外,依赖以CT为主要成像方式的诊断过程,忽视了胸部x光片。放射科医生、计算机科学家和内科医生都需要了解这些跨学科的问题。本文首先通过文献综述和文档分析强调了在COVID-19检测中部署深度学习的挑战。其次,它提出了六项关键建议,可以帮助该领域的未来研究人员改进COVID-19的诊断过程。但是,需要所有这些国家共同努力,考虑提供的建议,以有效地解决这些问题。
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Journal of pulmonology and respiratory research
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