Exertional dyspnea as initial manifestation of Takayasu's arteritis – A case report and literature review

Background  

Takayasu's arteritis is a chronic systemic inflammatory disease that usually affects the aorta, its primary branches and occasionally the pulmonary and coronary arteries. Female gender in reproductive age and Asian origin are known factors associated with higher disease prevalence. The clinical manifestations vary considerably and are typically caused by limb or organ ischemia illness and fever. The estimated incidence rate in the western world is 2.6 cases per million persons per year. Occasionally, exertional dyspnea can be the sole primary clinical manifestation of Takayasu's arteritis.     

Clinical characteristics and epidemiology of pulmonary pseudallescheriasis

BackgroundSome members of the Pseudallescheria (anamorph Scedosporium) have emerged as an important cause of life-threatening infections in humans. These fungi may reach the lungs and bronchial tree causing a wide range of manifestations, from colonization of airways to deep pulmonary infections. Frequently, they may also disseminate to other organs, with a predilection for the brain. In otherwise healthy patients, the infection is characterized by non-invasive type involvement, while invasive and/or disseminated infections were mostly seen in immunocompromised patients.AimsWe reviewed all the available reports on Pseudallescheria/Scedosporium pulmonary infections, focusing on the geographical distribution, immune status of infected individuals, type of infections, clinical manifestations, treatment and outcome.Results and conclusionsThe main clinical manifestations of the 189 cases of pulmonary pseudallescheriasis reviewed were pneumonia (89), followed by fungus ball (26), and chest abscess (18). Some patients had more than one type of invasive pulmonary manifestations. Among patients with pneumonia, several cases of pneumonia associated with near-drowning (10/89, 11.2%) have also been reported in immunocompetent hosts. Major underlying conditions for non-invasive pulmonary infection were preexisting lung cavities and medical immunosuppression for invasive pulmonary infection. Saprobic airway colonization was mostly seen in patients with mucosal dysfunction, i.e. patients with cystic fibrosis. The mortality rate was closely related to the infection type, being 26.8% in non-invasive type (fungus balls) and 57.2% in invasive type.     

31例非获得性免疫缺陷综合征肺隐球菌病临床分析

目的通过研究非获得性免疫缺陷综合征肺隐球菌病的临床特点,提高医务人员对肺隐球菌病的认识,做到早期诊断和治疗。方法回顾性分析2001年1月至2011年12月在南京医科大学附属杭州市第一人民医院确诊的31例非获得性免疫缺陷综合征肺隐球菌病患者的性别、年龄分布、职业情况、伴随的基础疾病、临床症状、实验室检查、影像学特征、治疗方案及预后等各方面特点。结果肺隐球菌病发病年龄有年轻化趋势,临床特点及影像学表现缺乏特异性,极易误诊及漏诊。治疗方法及药物少,病程长。结论肺隐球菌病虽然临床表现缺乏特异性,但从病例分析结果来看诊治该病仍有一定规律可循,亟需提高对本病的认识,遇到疑似病例应行血乳胶凝集试验及肺活检或手术病理予以明确,药物治疗需要足够疗程。     

肺结核合并下呼吸道感染患者CT影像特点及IL-23R水平表达的临床意义

摘要 目的：探讨肺结核合并下呼吸道感染患者CT影像特点及IL-23R水平表达的临床意义。方法：选取2015年1月-2018年8月于我院就诊的123例肺结核患者,将所选取的患者按是否合并下呼吸道感染分为单纯肺结核组73例和肺结核合并下呼吸道感染组50例。分析两组组患者临床表现、CT影像学图片、CT影像学表现、血清IL-23R的表达水平的差异。结果：单纯肺结核组患者出现14例低热、11例胸闷、17例消瘦、14例气短、18例痰中带血、14例颈部淋巴结肿大、23例咳嗽、21例胸痛。肺结核合并下呼吸道感染组患者出现16例低热、13例胸闷、21例消瘦、19例气短、20例痰中带血、15例颈部淋巴结肿大、26例咳嗽、23例胸痛,两组患者临床表现差异均无统计学意义（P＞0.05）。单纯肺结核组CT影像学图片显示可见结节影或包块影,呈明显分叶征、短毛刺征、空泡征并发肺不张、棘状突起,边缘较清晰,增强扫描后明显强化。肺结核合并下呼吸道感染组CT影像学图片显示有分叶、毛刺,同时肺内肿物周边存在晕征、血管聚集征、卫星灶征、胸膜凹陷等表现,此外伴纵膈及肺门淋巴结明显肿大,胸壁侵犯。肺结核合并下呼吸道感染组CT影像中毛刺状结节、胸膜凹陷、空洞、分叶征、肿块、条索影比例均高于单纯肺结核组,差异有统计学意义（P＜0.05）。肺结核合并下呼吸道感染组患者IL-23R水平明显高于单纯肺结核组,差异有统计学意义（P＜0.05）。结论：与单纯肺结核患者相比较,肺结核合并下呼吸道感染患者临床表现更为严重,CT影像学特点更为明显,患者血清中IL-23R表达水平明显升高,临床可通过CT影像学结合血清IL-23R检测,以提高肺结核合并下呼吸道感染患者的临床诊断。     

43例术后疑似急性肺栓塞分析

目的:探讨影响术后急性肺栓塞(Acute Pulmonary Embolism,APE)的发生和预后的相关因素,以提高对术后肺栓塞的认识和诊疗水平。方法:收集2009.01-2014.12期间南方医院术后疑似急性肺栓塞患者的临床资料,总结其临床特征,分析其诱发因素、临床表现、治疗和预后,探讨其发病的高危因素。结果:共收集术后疑似肺栓塞43例,平均年龄56.09±14.08岁(17~80岁),明确诊断为肺栓塞15例(34.9%),共死亡20例(死亡率46.5%)。其临床表现和体征均具有非特异性,呼吸困难、心悸和晕厥是主要的临床表现。不仅可以发生于下肢、胸腹部(包括妇产科)、颅内等大手术后,也可能发生在介入栓塞术后。相关危险因素很多,包括性别、年龄、恶性肿瘤、全身麻醉、手术时间长等。具有高危因素的患者并具有可疑肺栓塞的临床表现时,结合D-二聚体、动脉血气分析、心电图、胸部X线、超声心动图、下肢彩超可检查协助APE的诊断,而胸部增强CT作为检查手段有利于明确诊断。结论:肺栓塞是手术后致命的并发症之一,早期诊断、早期治疗,能降低术后肺栓塞患者的死亡率。     

7例不典型肺真菌病的临床分析

目的 肺部真菌感染往往症状不典型,肺部影像学表现多样,缺乏特异性,为早期诊断和治疗带来困难。尤其足近年来无免疫缺陷人群中也出现肺部真菌感染．使临床判断更加复杂。方法 对我院于2006年上半年收治的7例无免疫缺陷的肺部真菌感染病例,从发病特点、临床表现、影像学、病原学、诊断依据以及治疗策略等方面对其进行了详细分析。结果 7例肺真菌病患者均无明显免疫缺陷,肺部影像学表现多样。通过血清学、肺活检、痰培养等方法确诊,对治疗反应均较好。结论 肺真菌病的临床表现趋向复杂多样,临床医生应予以高度重视。     

Multiples défauts segmentaires et sous-segmentaires de perfusion discordants avec une angiographie pulmonaire normale et une forte suspicion de maladie veino-occlusive pulmonaire

We report the case of a 55-year-old woman presenting stage-3-4 dyspnea over the last 5 years with no other clinical symptoms. Pulmonary arterial hypertension (PAH) was evidenced by echocardiography. However, there was no argument for chronic thromboembolic disease during pulmonary angiography. The pulmonary scintigraphy showed homogeneous ventilation on both lung fields, with multiple bilateral segmental and subsegmental perfusion defects, however. These perfusion abnormalities with a normal pulmonary angiography led to the diagnosis of pulmonary veno-occlusive disease (PVOD) taking into account the clinical context, imaging findings, and laboratory and functional assessments. PVOD is a clinicopathological entity characterized by the occlusion or narrowing of the pulmonary veins and venules by fibrous tissue, leading to clinical manifestations that are, in many ways similar to PAH. PVOD remains poorly understood and it is difficult to diagnose and treat. However, PVOD needs to be known by clinicians and considered in the differential diagnosis of PAH.     

Characterization of pulmonary T cell response to helper-dependent adenoviral vectors following intranasal delivery

In spite of the extensive research in the field of gene therapy, host immune responses continue to be the major barrier in translating basic research to clinical practice. Helper-dependent adenoviral (HD-Ad) vectors show great potential for pulmonary gene therapy, but the knowledge of pulmonary immune responses toward these vectors is very limited. In this study, we show that HD-Ad vectors are potent stimulators of dendritic cell (DC) maturation, thus leading to stimulation of T cell proliferation with approximately 6% of naive CD4(+) T cells from pulmonary mediastinal lymph node responding to HD-Ad-treated DCs. In contrast to the belief that HD-Ad vectors are unable to prime adaptive immune response, we show for the first time, through in vivo pulmonary studies in mice, that HD-Ad vectors can prime CD4(+) and CD8(+) T cell responses in the lung at high and substantially low doses. This indicates cross-presentation of HD-Ad-derived epitopes by DCs to prime CD8(+) T cell responses. To assess the basis of pulmonary T cell response against HD-Ad vectors, we examined the response of conventional DCs (cDCs) and plasmacytoid DCs (pDCs) in the lung. In response to HD-Ad delivery, there is induction of maturation in both cDC and pDC subsets, but it is the cDCs, not pDCs, that migrate rapidly to draining lymph nodes within the first 2 days after vector delivery to prime adaptive immune response against these vectors. These findings have implications for development of strategies to prevent adaptive immune responses against gene therapy vectors.     

Occurrence of subcutaneous zygomycosis (Entomophthoromycosis Basidiobolae) caused by Basidiobolus haptosporus with pulmonary involvement

A case of a two-year-old boy with multiple subcutaneous lesions caused by Basidiobolus haptosporus is presented.The child had also a non-toxic familial goiter and clinical and radiological features of a pulmonary illness. The pulmonary manifestations only disappeared with the treatment with potassium iodide. The authors think that the pulmonary lesions must have arisen by direct spread of the fungus from the subcutaneous lesions of the chest.     

圣乔治教堂诺卡菌引起的肺部感染1例

诺卡菌肺部感染可引起肺诺卡菌病,因其无特异性的临床表现,容易误诊、漏诊。因此,临床实验室的培养鉴定能力非常重要,若不能及时诊治,则会导致病死率较高。诺卡菌病临床较为少见,为引起临床实验室对诺卡菌的鉴定和药敏试验的重视,本文报道了上海市嘉定区中心医院2019年2月收治的1例由圣乔治教堂诺卡菌(Nocardia cyriacigeorgica)感染引起的肺诺卡菌病病例,针对其临床特征、实验室检测及治疗等进行分析,期望对临床诊治诺卡菌病有所帮助。     

肺结核合并肺癌患者的临床分析

目的:探讨肺结核合并肺癌的临床特点,提高临床医师诊断肺结核合并肺癌的警惕性.方法:对2000年1月至2009年12月在我院诊断为肺结核、肺结核合并肺癌、肺癌患者各40例进行回顾性分析.结果:三组之间的临床表现与实验室检查进行相互比较,发现其临床表现并无统计学差异,影像学与支气管镜检查有明显统计学差异外,其他实验室检查无差异.结论:结核合并肺癌与肺结核、肺癌等临床表现、实验室检查近似,易造成漏诊、误诊,临床医师应提高警惕,及时行影像学及支气管镜等检查.     

Philopatric reproduction, a prime mover in the evolution of termite sociality?

Summary: Philopatric reproduction commonly occurs even in the most basic isopteran lineages and is almost certainly a primitive potentiality of these insects. Several authors hypothesized that opportunities for philopatric reproduction may have been a prime mover for the three most remarkable events in the evolution of termite sociality: the origin of helping behaviour, the origin of soldiers, and the origin of a permanently apterous worker caste. A critical assessment of these hypotheses and a review of relevant evidence are presented here. I first discuss the hypothesis that termite helpers derive from individuals choosing to postpone dispersal because of nest inheritance prospects. Such individuals would have developed helping as a strategy yielding indirect reproductive benefits while they wait for reproductive opportunities. However, it appears that prospects of philopatric reproduction need not be invoked to explain the origin of helping behaviour, which can be justified more parsimoniously by a favourable intrinsic benefit/cost ratio. Second, the hypothesis that soldiers originated through selection for fighting abilities among neotenic reproductives is found to face important difficulties, the major one being how to explain the absence of fighting devices in almost all present-day neotenics. Finally, the hypothesized link between apterism and chances of philopatric reproduction, which might have favoured the onset of the worker caste, is poorly supported by empirical evidence. It appears thus that philopatric reproduction, notwithstanding its importance in the biology of many extant termite species, is unlikely to have been a prime mover in the evolution of helping and of altruistic castes in termites.     

探讨CT技术在肺结节疾病诊断中的应用

目的：探讨CT技术在肺结节疾病诊断中的应用。利用CT技术提供的影像学特征,及时准确的诊断肺结节疾病,从而更具体更全面的提高对该疾病的认识,减少误诊的发生。方法：回顾性分析我院2010年-2012年确诊的符合相应临床诊治标准的肺结节患者48例的临床资料,通过CT扫描分析其具体的特征表现。结果：在所选的48例中,纵隔淋巴结增大45例,双侧肺门淋巴结增大44例,胸部淋巴结增大患者48例。肺部病变者33例,其中单发结节患者2例,多发结节患者25例,支气管血管束增粗者14例,磨玻璃样影案例者9例,实变案例5例。胸膜病发患者13例。结论：我们通过分析发现,胸部有典型影像学表现形式的肺结节病例诊断较容易,无典型影像学表现的患者诊断较为困难。因此我们认为利用CT技术诊断肺结节疾病具有特异性意义,值得临床医生重视。     

肺结核合并肺癌患者的临床分析

目的：探讨肺结核合并肺癌的临床特点,提高临床医师诊断肺结核合并肺癌的警惕性。方法：对2000年1月至2009年12月在我院诊断为肺结核、肺结核合并肺癌、肺癌患者各40例进行回顾性分析。结果：三组之间的临床表现与实验室检查进行相互比较,发现其临床表现并无统计学差异,影像学与支气管镜检查有明显统计学差异外,其他实验室检查无差异。结论：结核合并肺癌与肺结核、肺癌等临床表现、实验室检查近似,易造成漏诊、误诊,临床医师应提高警惕,及时行影像学及支气管镜等检查。     

Clinical polymorphism in amenorrhea of chromosomal and nonchromosomal etiologies

In search of the basis of distinguishing amenorrhea, due to chromosomal mosaicism and other causes, 179 females affected by primary or secondary amenorrhea were examined, 83 of them being 45,X/46,XX mosaics. 119 traits characterizing the morphological status of the musculoskeletal and reproductive systems, as well as skin, hair and nails were scored. By means of statistical approaches, a group of 21 traits were specified, which makes it possible to diagnose the amenorrhea of chromosomal origin. Statistically significant association between the clinical manifestations and the rate of mosaicism was shown.     

Enigmas in sarcoidosis.

Sarcoidosis is a multisystem granulomatous disorder of unknown cause that presents most frequently in young adults with bilateral hilar adenopathy, pulmonary infiltrates, and skin or eye lesions. The multisystem clinical manifestations of this disease are a diagnostic challenge to all physicians. Although the clinical and pathologic characteristics of sarcoidosis are well described, the decision to treat and the optimal therapy are less well defined. This review focuses on the natural history, clinical manifestations, controversies in therapy, including steroid-sparing agents, and current concepts of how the disease''s activity can be monitored.     

Clinical presentations of nosocomial aspergillosis

Aspergillus is a very ubiquitous fungal in our environment, including hospitals, being the second in frequency in colonization and infection, just after Candida spp. Aspergillus nosocomial infections have increased, because the number of immunocompromised patients has also increased. Nosocomial infections can be caused by different species of Aspergillus, being pulmonary manifestations the most frequent. Primary or secondary nonpulmonary infections can affect the brain, heart, kidney, eyes and other organs. The mortality due to invasive aspergillosis is very high, and a clinical-radiological suspicion and, specially the instauration of a rapid treatment with high doses of amphotericin B or its new formulations (associated with surgery in many times) may modify the mortality observed in this patients.     

A case of sparganosis mimicking a varicose vein

Recent advances in radiological and serological techniques have enabled easier preoperative diagnosis of sparganosis. However, due to scarcity of cases, sparganosis has been often regarded as a disease of other etiologic origin unless the parasite is confirmed in the lesion. We experienced a case of sparganosis mimicking a varicose vein in terms of clinical manifestations and radiological findings. Sparganosis should be included among the list of differential diagnosis with the varicose vein.     

Pulmonary vascular development goes awry in congenital lung abnormalities

Pulmonary vascular diseases of the newborn comprise a wide range of pathological conditions with developmental abnormalities in the pulmonary vasculature. Clinically, pulmonary arterial hypertension (PH) is characterized by persistent increased resistance of the vasculature and abnormal vascular response. The classification of PH is primarily based on clinical parameters instead of morphology and distinguishes five groups of PH. Congenital lung anomalies, such as alveolar capillary dysplasia (ACD) and PH associated with congenital diaphragmatic hernia (CDH), but also bronchopulmonary dysplasia (BPD), are classified in group three. Clearly, tight and correct regulation of pulmonary vascular development is crucial for normal lung development. Human and animal model systems have increased our knowledge and make it possible to identify and characterize affected pathways and study pivotal genes. Understanding of the normal development of the pulmonary vasculature will give new insights in the origin of the spectrum of rare diseases, such as CDH, ACD, and BPD, which render a significant clinical problem in neonatal intensive care units around the world. In this review, we describe normal pulmonary vascular development, and focus on four diseases of the newborn in which abnormal pulmonary vascular development play a critical role in morbidity and mortality. In the future perspective, we indicate the lines of research that seem to be very promising for elucidating the molecular pathways involved in the origin of congenital pulmonary vascular disease. Birth Defects Research (Part C) 102:343–358, 2014. © 2014 Wiley Periodicals, Inc.     

Cystic-fibrosis-like disease unrelated to the cystic fibrosis transmembrane conductance regulator

Cystic fibrosis (CF) is considered to be a monogenic disease caused by molecular lesions within the cystic fibrosis transmembrane conductance regulator (CFTR) gene and is diagnosed by elevated sweat electrolytes. We have investigated the clinical manifestations of cystic fibrosis, CFTR genetics and electrophysiology in a sibpair in which the brother is being treated as having CF, whereas his sister is asymptomatic. The diagnosis of CF in the index patient is based on highly elevated sweat electrolytes in the presence of CF-related pulmonary symptoms. The investigation of chloride conductance in respiratory and intestinal tissue by nasal potential difference and intestinal current measurements, respectively, provides no evidence for CFTR dysfunction in the siblings who share the same CFTR alleles. No molecular lesion has been identified in the CFTR gene of the brother. Findings in the investigated sibpair point to the existence of a CF-like disease with a positive sweat test without CFTR being affected. Other factors influencing sodium or chloride transport are likely to be the cause of the symptoms in the patient described. Received: 25 August 1997 / Accepted: 20 January 1998