idiopathic

特发性
  • 文章类型: Case Reports
    嗜酸性粒细胞增多综合征(HES)是一种以嗜酸性粒细胞水平升高为特征的疾病,根据严重程度可能与多器官受累有关。特发性HES的最新诊断标准要求嗜酸性粒细胞绝对计数(AEC)高于1500个细胞/mcL,并有组织损伤的证据。我们介绍了一例37岁的男性消防员,据称有嗜酸性粒细胞性支气管炎病史,他因晕厥发作和持续的生产性咳嗽而被转诊到医院。患者在入院时显示与高炎症标志物相关的4500个细胞/mcL的AEC。心脏成像显示急性心肌炎伴心力衰竭和射血分数降低。胸部成像最初提示社区获得性肺炎。检查为恶性病因阴性;排除了同样的感染原因。经过多学科评估,诊断为特发性HES,并开始使用类固醇,症状迅速缓解.我们的案例说明了在其他健康的成年人中,将嗜酸性粒细胞增多症视为急性心力衰竭的诱发因素的重要性。快速诊断可以导致类固醇的早期启动,以避免向多器官衰竭发展。
    Hypereosinophilic syndrome (HES) is a disorder characterized by elevated levels of eosinophils, which may be associated with multi-organ involvement depending on severity. The recent diagnostic criteria for idiopathic HES require an elevated absolute eosinophil count (AEC) above 1500 cells/mcL with evidence of tissue damage. We present a case of a 37-year-old male firefighter with a purported history of eosinophilic bronchitis who was referred to the hospital with syncopal episodes and a persistent productive cough. The patient showed an AEC of 4500 cells/mcL on admission associated with high inflammatory markers. Cardiac imaging demonstrated acute myocarditis with heart failure and a reduced ejection fraction. Chest imaging was initially suggestive of community-acquired pneumonia. Workup was negative for a malignant etiology; infectious causes similarly were excluded. After a multidisciplinary evaluation, a diagnosis of idiopathic HES was made and steroids were instituted with rapid resolution of symptoms. Our case illustrates the importance of considering hypereosinophilia as a precipitating factor for acute heart failure in an otherwise healthy adult. An expeditious diagnosis can lead to early initiation of steroids to avoid progression toward multi-organ failure.
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
    背景:特发性胸膜实质纤维弹性增生症(IPPFE)是一种非常罕见且缓慢明显进展的慢性肺部疾病。通常累及肺的上叶。这种不寻常的疾病,2013年首次被认为是罕见的特发性间质性肺炎,其特征是内脏胸膜和下肺实质的致密纤维化,并伴有主要在胸膜下肺泡壁的弹性变性。为了提高我们对这种罕见疾病的认识,我们报告一例由病理结果确定的IPPFE。病例报告:一名73岁的男性患者,吸烟者,有慢性阻塞性肺疾病病史,自2022年1月以来,劳累时呼吸困难逐渐恶化,并伴有体重减轻。胸部X线检查发现胸部扩张。胸部高分辨率计算机断层扫描显示双尖胸膜下实质凝结,右上叶有牵张性支气管扩张和胸膜回缩以及弥漫性双侧中央小叶气肿。扫描引导的跨顶叶肺活检显示肺实质纹身有炭疽病沉积,大部分被纤维组织重塑,在偏振光中与许多波浪状和屈光性畸形结构混合在一起。orcein染色证实了这些病变中存在过量的弹性纤维。所有病因调查均为阴性。他的肺功能研究显示可逆性阻塞性通气障碍。经过多学科的讨论,IPPFE的诊断是根据胸部计算机断层扫描在上肺的分布并结合病理类型来确定的。结论:该病例强调了IPPFE的非典型误导性放射学表现以及病理结果在建立诊断中的关键作用。因此,需要进一步的研究来提高我们对这种罕见疾病的认识,并建立明确的IPPFE诊断和治疗指南.
    Background: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a very rare and a slowly conspicuous progressing chronic lung disease, which usually involves the upper lobes of the lung. This unusual disease, first recognized as a rare idiopathic interstitial pneumonia in 2013, is characterized by dense fibrosis of the visceral pleura and the subjacent lung parenchyma accompanied by elastosis predominating in the subpleural alveolar walls. In the interest of improving our understanding of this uncommon disease, we report a case of IPPFE established by pathology results. Case report: A 73-year-old male patient, smoker, with a medical history of chronic obstructive pulmonary disease, presented since January 2022 with a gradual worsening of dyspnea on exertion and productive cough with weight loss. The chest X-ray detected a thoracic distention. The chest high resolution computed tomography revealed biapical subpleural parenchymatous condensations with tractive bronchiectasis and pleural retraction in the right upper lobe and diffuse bilateral cento-lobular emphysema. A scan-guided trans-parietal lung biopsy showed lung parenchyma tattooed with anthracosic deposits, largely remodeled by fibrous tissue, intermingled with numerous wavy and refractive dyselastotic structures in polarized light. The orcein staining confirmed the presence of excess elastosic fibers within these lesions. All etiological investigations were negative. His lung function studies revealed a reversible obstructive ventilatory disorder. Following a multidisciplinary discussion, the diagnosis of IPPFE was confirmed on the basis of the distribution in the upper lungs on chest computed tomography combined with pathology pattern. Conclusions: This case emphasizes the atypical misleading radiological presentation of IPPFE and the key role of pathological results in establishing the diagnosis. Hence, further studies are needed to improve our understanding of this uncommon disease and to establish clear-cut guidelines for IPPFE diagnosis and management.
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  • 文章类型: Case Reports
    巨大的舌头,一种罕见的解剖学异常,可以表现为先天性或获得性。舌头的大小随着年龄而变化,在8年达到峰值,在18年达到完全成熟。先天性巨舌症源于多种疾病,比如肌肉肥大,血管瘤,淋巴管瘤,唐氏综合症,和其他人。获得性巨舌症可由恶性肿瘤引起,内分泌和代谢紊乱,慢性传染病,头颈部感染,在其他因素中。此外,容易扩展的手术可以导致其发展。巨舌症的发病率可能被低估。这种情况很少见,文献中只有6例报告病例。
    Macroglossia, an uncommon anatomical anomaly, can manifest as either congenital or acquired. The size of the tongue undergoes variations with age, peaking at 8 years and reaching full maturity at 18 years. Congenital macroglossia stems from diverse conditions, such as muscular hypertrophy, hemangioma, lymphangioma, Down syndrome, and others. Acquired macroglossia can result from malignancies, endocrine and metabolic disorders, chronic infectious diseases, and head and neck infections, among other factors. Additionally, extended-prone surgery can lead to its development. The incidence of macroglossia is likely underreported. This presentation is rare with only six reported cases in the literature.
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  • 文章类型: Case Reports
    鼻中隔脓肿是一种罕见的耳鼻喉疾病,文献报道很少。最常继发于血肿,特发性脓肿形成罕见。一名先前健康的13岁男性,之前没有鼻腔血肿或外伤,表现为鼻漏,鼻痛,和阻塞。最初的切口和引流无法治愈;患者因新发头痛而返回护理。神经影像学检查显示脓肿复发以及全鼻窦炎和硬膜外脓胸。患者被转移到三级护理中心进行耳鼻喉和神经外科联合护理。患者的术后过程并不复杂。鼻中隔脓肿变得越来越少见,但它们仍然存在。及时治疗是必要的,以减轻进一步颅内扩散和面部畸形的潜在风险,正如在这个案例中看到的。
    Nasal septal abscess is an uncommon otolaryngologic condition with little reported in the literature. Most commonly arising secondary to hematoma, idiopathic abscess formation is rare. A previously healthy 13-year-old male with no preceding nasal hematoma or trauma presented with rhinorrhea, nasal pain, and obstruction. Initial incision and drainage were not curative; the patient returned to care with complaints of new-onset headache. Neuroimaging revealed abscess recurrence alongside pansinusitis and epidural empyema. The patient was transferred to a tertiary care center for joint otolaryngologic and neurosurgical care. The patient\'s postoperative course was uncomplicated. Nasal septal abscesses have become increasingly uncommon, but they still occur. Prompt treatment is warranted to mitigate the potential risk of further intracranial spread and facial deformity, as seen in this case.
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  • 文章类型: Case Reports
    鼻中隔脓肿(NSA)被认为是鼻急症。幸运的是,由于引入了抗生素和易于获得医疗护理,NSA的发病率显着降低。NSA通常是由鼻中隔和上覆的粘液膜和/或粘液膜骨膜之间的空间感染引起的。通常继发于鼻中隔血肿,但也可能是特发性的.及时的诊断和干预对于避免进一步的并发症至关重要。本文报道了一名46岁的男性,没有已知的NSA危险因素。他接受了广谱抗生素治疗,手术治疗包括切开引流,并在术后第5天在术中放置Penrose引流管和硅橡胶片。患者出院,无鼻中隔穿孔、鞍状鼻畸形等并发症。
    Nasal septal abscess (NSA) is considered a rhinologic emergency. Fortunately, the incidence of NSA has markedly reduced due to the introduction of antibiotics and easy access to medical care. NSA commonly results from infection in the space between the nasal septum and the overlying mucoperichondrium and/or mucoperiosteum, typically secondary to nasal septal hematoma, but it can also be idiopathic. Prompt diagnosis and intervention are critical to avoid further complications. This paper reports the case of a 46-year-old man with no known risk factors for NSA. He was treated with broad-spectrum antibiotics, and the surgical treatment involved incision and drainage with the intraoperative placement of a Penrose drain and a silastic sheet on postoperative day five. The patient was discharged without complications such as septal perforation or saddle nose deformity.
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  • 文章类型: Case Reports
    急性特发性颈椎后凸畸形(AICK)是一种罕见的疾病,它的管理仍然存在争议。术前手术计划和个人决策似乎是必要的。迄今为止,缺乏足够的证据和明确的指导方针。
    方法:一名21岁男性在3个月前发现进行性宫颈畸形。该患者患有严重的进行性脊髓病,既没有颈部创伤,也没有类似预期疾病的家族史。他的宫颈成像显示宫颈后凸畸形95度。经过3次单独的360度固定手术,颈椎后凸缩小了90度。没有观察到小平面错位,椎板切除术是不必要的.术后神经系统检查发现明显改善。6个月和2年的随访是有利的。就作者所知,该病例是文献中报道的最广泛程度的颈椎后凸畸形。
    AICK的多阶段校正将导致有利的结果并降低并发症的风险。在骨膜下夹层中,应特别注意高度后凸畸形的棘突间间隙,以防止医源性脊髓损伤。
    结论:目前的工作可能提供了关于宫颈姿势习惯在阿片类药物滥用障碍患者中的作用的第一份报告,这可能引发了这位特殊患者的颈椎后凸畸形。AICK的多阶段校正将导致有利的结果并降低并发症的风险。
    UNASSIGNED: Acute idiopathic cervical kyphosis (AICK) represents a rare entity, and its management remains controversial. Preoperative surgical planning and individual decision-making seem necessary. To date, there is a lack of sufficient evidence and clear guidelines.
    METHODS: A 21-year-old male was referred with a progressive cervical deformity detected 3 months earlier. The patient suffered from severe progressive myelopathy and represented neither neck trauma nor a familial history of similar expected conditions. His cervical imaging revealed 95 degrees of cervical kyphosis. After 3 separate surgical sessions for 360-degree fixation, the cervical kyphosis was reduced by 90 degrees. No facet dislocation was observed, and laminectomy was unnecessary. Post-operative neurological examination detected significant improvement. Six months and 2-year follow-ups were favorable. To the authors\' knowledge, the current case had the most extensive degree of cervical kyphosis reported in the literature.
    UNASSIGNED: Multistage correction of AICK would result in a favorable outcome and reduce the risk of complications. Particular attention should be paid to the wide inter-spinous spaces in high grades of kyphosis during sub-periosteal dissection to prevent iatrogenic spinal cord injuries.
    CONCLUSIONS: The present work may provide the first report on the role of cervical postural habits in patients with opiate substance abuse disorder, which could have triggered cervical kyphosis in this particular patient. Multistage correction of AICK would result in a favorable outcome and reduce the risk of complications.
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  • 文章类型: Case Reports
    大十二指肠是一种罕见的临床综合征,其特征是十二指肠扩张。伸长率,和肥大。鉴于其罕见和非特异性临床表现,十二指肠可能会被误诊,导致手术治疗的延误和发病率的增加。我们描述了一名年轻的白人女性的特发性巨十二指肠病例,有五年口臭史的人,反复打气,腹胀,恶心和呕吐,和餐后上腹部腹痛。通过造影造影检查发现,她被诊断为巨大十二指肠。她出现了十二指肠扭转,需要紧急剖腹探查术,在此期间进行了十二指肠折叠术和十二指肠空肠侧侧吻合术。剖腹探查术和组织病理学分析没有发现任何明确的异常来解释她的巨十二指肠。术后,她出现了两个早期小肠梗阻,两者都来自随后的粘连,需要重复剖腹手术和粘连溶解。她随后康复,没有发生任何事故。巨大十二指肠的诊断和准确分类需要手术探查,并进行全层活检和随后的组织病理学分析,以排除十二指肠的阻塞性或功能性疾病。巨大十二指肠的治疗取决于十二指肠扩张的根本原因和程度。临床医生了解各种手术选择是至关重要的,他们的适应症,以及可能出现的术后并发症。
    Megaduodenum is a rare clinical syndrome characterized by significant duodenal dilation, elongation, and hypertrophy. Given its rarity and nonspecific clinical manifestations, megaduodenum may be misdiagnosed, leading to delays in surgical care and increased morbidity. We describe a case of idiopathic megaduodenum in a teenage Caucasian female, who presented with a five-year history of halitosis, recurrent belching, bloating, nausea and vomiting, and postprandial epigastric abdominal pain. She was diagnosed with megaduodenum by dramatic findings on contrast radiography. She developed a duodenal volvulus necessitating emergency exploratory laparotomy, during which a duodenal plication and a side-to-side duodenojejunostomy were performed. Exploratory laparotomy and histopathological analysis were unrevealing of any definitive abnormalities to explain her megaduodenum. Postoperatively, she developed two early small bowel obstructions, both from subsequent adhesions requiring repeat laparotomy with adhesiolysis. She has subsequently recovered without incident. Diagnosis and accurate classification of megaduodenum requires surgical exploration with a full-thickness biopsy and subsequent histopathologic analysis to rule out obstructive or functional disorders of the duodenum. Treatment of megaduodenum depends on the underlying cause and degree of duodenal distention. It is crucial that clinicians are knowledgeable of the various surgical options, their indications, and the potential postoperative complications that may arise.
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  • 文章类型: Case Reports
    腹茧综合征(ACS),作为机械性肠梗阻的罕见原因,可分为原发性/特发性vs.次要类型。原发性ACS通常无症状,仅在剖腹探查术中诊断。手术的主要治疗方法可能具有挑战性。由于肠壁和腹膜紧密粘附,肠穿孔可能发生在粘连松解术期间。因此,有一个有经验的外科医生来进行手术是很重要的。
    作者介绍了一个50岁男性的主要ACS病例。患者入院时表现出难以忍受的上腹痛。计算机断层扫描(CT)扫描显示严重的肠梗阻。需要剖腹探查术,导致ACS的诊断,排除次要因素后被认为是特发性的。成功地进行了粘连分解。注意,在手术期间测量的整个肠仅为2.1m。无术后并发症。患者顺利康复。
    原发性ACS的病因未知。发病率相对较低,男女之间被认为是平等的。作为肠梗阻的罕见原因,应加强对诊断的怀疑。包括粘连松解术和肠切除术的手术仍然是主要的治疗方法。如果粘连溶解失败,肠切除将是不可避免的。将测试外科医生的知识和经验。
    应进一步探讨原发性ACS的病因。肠梗阻的鉴别诊断应涵盖ACS,以便外科医生在手术前做好准备。
    UNASSIGNED: Abdominal cocoon syndrome (ACS), as a rare cause of mechanical intestinal obstruction, can be divided into primary/idiopathic vs. secondary type. The primary ACS is often asymptomatic and only diagnosed in exploratory laparotomy. The major treatment of surgery can be challenging. Since the gut wall and peritoneum are densely adhered, gut perforation might occur during adhesiolysis. Thus, it is important to have an experienced surgeon to perform the surgery.
    UNASSIGNED: The authors present a primary ACS case of a 50-year-old man. The patient demonstrated an unbearable upper abdominal pain upon admission. A computed tomography (CT) scan showed a severe bowel obstruction. An exploratory laparotomy was indicated, leading to the diagnosis of ACS, which was considered idiopathic after ruling out secondary factors. An adhesiolysis was performed successfully. Note that the entire intestine measured was only 2.1 m during the surgery. There was no post-surgical complication. The patient was recovered uneventfully.
    UNASSIGNED: The aetiology of primary ACS is unknown. The incidence is comparatively low and considered equal between men and women. As a rare cause of gut obstruction, the suspicion of the diagnosis should be strengthened. Surgery including adhesiolysis and bowel resection remains the major treatment. If adhesiolysis fails, bowel resection will be inevitable. The knowledge and experience of surgeon will be tested.
    UNASSIGNED: The aetiology of primary ACS should be further explored. And the differential diagnosis of bowel obstruction should cover ACS in order for the surgeon to be prepared before surgery.
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  • 文章类型: Case Reports
    背景:肠膀胱囊肿性气肺病(PCI)是一种以小肠或大肠的粘膜下层和浆膜下存在气体填充的囊肿样结构为特征的疾病,在某些情况下还伴有气腹。PCI通常被认为是良性疾病,而不是在危及生命的情况下,例如肠系膜缺血。只有少数PCI病例被认为是原发性或特发性的,大多数是由各种潜在疾病引起的。PCI的症状是非特异性的或可能完全不存在。只要不怀疑潜在的危及生命的疾病,PCI可以非手术治疗。
    方法:我们介绍了一例71岁的肠管积气患者,腹腔内游离气体已知3年。由于自限性症状和缺乏威胁生命的潜在疾病的证据,到目前为止,尚未进行任何特定的治疗。没有发现潜在的疾病。由于反复发作的腹痛和新诊断的部分小肠梗阻伴有肠系膜扭转的影像学征象,成功切除了受影响的小肠.
    结论:非手术治疗PCI是可能的,前提是已经排除了危及生命的肺炎原因。肠梗阻是PCI的罕见并发症,需要手术治疗。
    结论:我们的病例报告表明,PCI的症状可能会随着时间的推移而恶化,并且可能发生需要手术干预的并发症。我们建议定期监测主要非手术治疗的患者。
    BACKGROUND: Pneumatosis cystoides intestinalis (PCI) is a condition characterized by the presence of gas-filled cyst-like structures in the submucosa and subserosa of the small or large intestine and in some cases accompanied by pneumoperitoneum. PCI is commonly considered a benign condition as opposed to pneumatosis intestinalis in life-threatening conditions such as mesenteric ischemia. Only a minority of cases of PCI are assumed to be primary or idiopathic with the majority being caused by a variety of underlying conditions. Symptoms of PCI are non-specific or may be absent altogether. Provided that there is no suspicion of an underlying life-threatening disease, PCI can be treated non-operatively.
    METHODS: We present the case of a 71-year-old patient with pneumatosis intestinalis with free intraperitoneal gas known for three years. Due to self-limiting symptoms and lack of evidence of a life-threatening underlying disease, no specific therapy had been carried out so far. No underlying diseases could be found. Because of recurrent worsening abdominal pain and newly diagnosed partial small bowel obstruction with radiological signs of mesenteric torsion, resection of the affected small bowel was successfully performed.
    CONCLUSIONS: Non-surgical management of PCI is possible provided that life-threatening causes of pneumatosis have been ruled out. Bowel obstruction is a rare complication of PCI which requires surgical treatment.
    CONCLUSIONS: Our case report illustrates that symptoms of PCI may worsen over time, and that complications requiring surgical intervention may occur. We recommend regular monitoring of patients who are primarily treated non-operatively.
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