Pulmonary manifestations of POEMS syndrome: case report and literature review.

Phrenic nerve paresis is an unusual complication of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein spike and skin changes) syndrome. In this report, we describe a case of POEMS syndrome in which a 56-year-old woman presented with dyspnea and ventilatory failure due to bilateral phrenic nerve paralysis. To our knowledge, only one other case of phrenic neuropathy in POEMS syndrome has been reported.     

Pulmonary fibrosis associated with tocainide: report of a case with literature review

A report is presented of an 85-yr-old woman who developed severe pulmonary fibrosis while receiving tocainide for treatment of ventricular arrhythmias. Severe bilateral interstitial fibrosis was documented in this patient by open lung biopsy and at autopsy. Review of the literature revealed other cases with an association between the use of tocainide and the development of pulmonary complications such as pulmonary fibrosis and interstitial pneumonitis, and suggested that clinical improvement occurs when the correct diagnosis is made early and tocainide therapy is stopped immediately. Because tocainide is currently being widely used for the treatment of ventricular arrhythmias, the pulmonary status of patients receiving tocainide should be monitored closely. Hopefully, awareness of this potential complication of tocainide therapy will minimize the number of cases of serious pulmonary toxicity.     

Cystocerebral syndrome: a case report and literature review.

Delirium is a frequent cause of hospitalization in the elderly patient, and can be sustained by several factors, which are not always evident. In 1990 Blackburn and Dunn described a clinical picture characterized by the presence of acute urinary retention presenting as delirium, and named it "cystocerebral syndrome". In 1991 Liem and Carter advanced a possible pathophysiological explanation for this phenomenon, suggesting that adrenergic tension might increase in the central nervous system when micturition cannot occur at the usual threshold. The consequent increase in catecholamines level might produce delirium. We report the case of a very old subject with delirium and acute urinary retention, suggestive of the "cystocerebral syndrome", in order to call the attention of geriatricians to acute urinary retention as a possible precipitating factor of delirium.     

肺母细胞瘤1例并文献复习

目的提高对肺母细胞瘤的认识。方法结合1例肺母细胞瘤患者的临床资料和文献复习,详细分析该病的组织起源、临床病理特点、分类、诊断、鉴别诊断、治疗及预后等。结果该病临床症状少且轻微,影像学提示“良性肿瘤”;病理学检查主要特征是镜下瘤组织由胎儿型腺管样的上皮成分和原始的肉瘤样间质成分混杂存在。免疫组化示瘤组织由上皮和间叶两种成分构成。目前尚无有效治疗措施,预后差。结论肺母细胞瘤罕见,极易误诊,应予重视。     

Pulmonary toxocariasis: a case report and literature review

Toxocariasis is a parasitic disease caused by Toxocara canis or T. cati. We report a patient with toxocariasis who presented with dyspnea, high-grade eosinophilia, and bilateral pulmonary nodules. To further characterize the pulmonary manifestations of toxocariasis, we have reviewed 11 previously published pulmonary toxocariasis cases. The most common pulmonary symptoms in our review were cough and dyspnea, and the most common finding on chest imaging was bilateral pulmonary nodules. Risk factors for Toxocara infection primarily included exposure to dogs. Most patients received albendazole and responded well. A high index of suspicion is needed to diagnose this otherwise preventable parasitic disease.     

原发性抗磷脂抗体综合征合并肺血栓栓塞症1例并文献复习

目的：了解原发性抗磷脂抗体综合征合并肺血栓栓塞症的临床特征。方法对近期北京医院收治的1例原发性抗磷脂抗体综合征合并肺血栓栓塞症患者进行分析,并复习32例国内外文献发表的原发性抗磷脂抗体综合征合并肺血栓栓塞症病例。结果33例患者中,男18例,女15例,主要症状包括呼吸困难、胸痛、咯血和下肢疼痛等,其中合并呼吸困难、胸痛、咯血三联征的比例为42.4%,双下肢静脉血栓占38.7%,双侧肺动脉栓塞占87.1%,右房和/或右室增大者占56.3%,合并肺动脉高压者占75.0%。部分患者合并血小板减少,活化部分凝血活酶时间明显延长。及时诊断和治疗能取得良好的疗效。结论原发性抗磷脂抗体综合征合并肺血栓栓塞症患者合并三联征(胸痛、咯血、呼吸困难)的比例高,血栓范围广泛,常合并右房和/或右室扩大、血小板和凝血机制异常,及时诊断和治疗可获得良好的疗效。     

Rapunzel syndrome: a case report and review of literature.

Bezoars consist of ingested foreign material or organic matter which forms a mass in the gastrointestinal tract, usually in stomach. Trichobezoars formed by swallowed hair present with malnutrition, weight loss, abdominal pain with signs of gastrointestinal obstruction. Gastric trichobezoar with a tail reaching the small intestine is called Rapunzel syndrome. Although endoscopic methods are available to remove bezoars, majority of trichobezoars with Rapunzel syndrome undergo surgical removal.     

Pulmonary hyalinizing granuloma with ureteric fibrosis: A case report and review of relevant literature

A 52-year-old, asymptomatic patient presented with bilateral lung nodules on chest radiograph. She was diagnosed to have "pulmonary hyalinizing granuloma" on an open lung biopsy. We review the clinical features of this rare disease.     

Pulmonary veno-occlusive disease: a case report and a review of therapeutic possibilities.

We describe the observation of a 12 yr old girl who died of pulmonary veno-occlusive disease (PVOD). Diagnosis was based on histological examination of an open lung biopsy. The differential diagnosis, pathogenesis and possible therapies are discussed. Although medical therapy can sometimes give some temporary relief, lung transplantation might offer these patients a better chance of survival and a better quality of life.     

Pulmonary amyloidosis in Sjogren's syndrome: A case report and systematic review of the literature

Amyloidosis is a heterogeneous group of disorders characterized by misfolding of extracellular proteins. The two most common forms are light‐chain (AL) and reactive (AA) amyloidosis. Pulmonary amyloidosis secondary to Sjogren's syndrome (SS) is rare, and there is a paucity of data on its clinical and radiological features. We describe the case of a 41‐year‐old woman with diffuse septal pulmonary AL (λ) amyloidosis related to SS, in the absence of systemic amyloidosis. A systematic search for pulmonary amyloidosis in SS yielded 37 cases. Amyloidosis occured almost exclusively in women (96.5%). Cough and dyspnoea (56%) were the most common symptoms. The diagnosis of pulmonary amyloidosis was made subsequent to that of SS, with a median delay of 7 years. Radiologically, diffuse nodules with (45.5%) or without (33.3%) multiple cysts were the most common pattern. Most cases were related to nodular AL amyloidosis (both κ and λ). Diffuse septal AA amyloidosis has been reported in SS. The index patient is the first documented case of diffuse septal pulmonary AL amyloidosis in SS, without systemic amyloidosis. Surgical lung biopsy (78%) is usually required to establish the diagnosis and rule out lymphoma. There is no data to support any definitive therapeutic intervention for SS‐related pulmonary amyloidosis. Observation is sufficient for nodular amyloidosis. Further studies are necessary to assess the efficacy of current therapies in diffuse septal amyloidosis secondary to SS.     

Sezary综合征1例并文献复习

目的:通过报告1例sezary综合征以及文献复习提高对此病的认识。方法:报告1例sezary综合征,并对国内文献进行总结分析。结果:共26例sezary综合征患者,男性23例占88.5%,中位年龄59.5岁,主要表现为全身红皮病,伴有瘙痒,可出现淋巴结肿大、肝脾肿大。实验室检查发现白细胞升高,外周血中可找到sez-ary细胞。皮肤或淋巴结活检可发现sezary细胞。部分患者经以激素为主的治疗后获得缓解。结论:sezary综合征是一罕见的淋巴瘤,对此病的认识还需提高,国内尚需加强对细胞免疫分型和分子生物学的检测。     

Pulmonary capillary hemangiomatosis: report of a case and review of the literature.

We describe a case of pulmonary capillary hemangiomatosis in a 60-year-old woman with a 1-year history of progressive exertional dyspnea. Four years before admission a diagnosis of breast cancer was made, and she underwent mastectomy plus radiation therapy and treatment with oral antiestrogens. The chest X-ray showed bilateral interstitial infiltrates. Pulmonary function studies revealed a severe restrictive pattern. Abundant red blood cells were found in the bronchoalveolar lavage fluid. On the basis of open lung biopsy, interstitial fibrosis was diagnosed. Cardiac catheterization revealed pulmonary hypertension. Steroids were prescribed, but the patient's condition continued to deteriorate and she died approximately 3 years after presentation. The identification of proliferating and invasive capillaries, which are unique to pulmonary capillary hemangiomatosis, led to the correct diagnosis at autopsy.     

Pulmonary benign metastasizing leiomyoma: a case report and literature review

Benign metastasizing leiomyoma (BML) is a rare condition that occurs in all age groups and that is particularly prevalent among women of late childbearing age. All patients have a history of uterine leiomyoma and/or myomectomy, often associated with distant metastases from the uterus, which commonly occurs in the lung. We report the case of a 32-year-old young woman suffering from chest stuffiness, labored respiration and weakness after a myomectomy performed one month earlier. The chest CT showed a diffuse miliary shadow in both sides of her lungs, but serum tumor markers such as CA125, CA199, carcinoembryonic antigen (CEA), neuron specific enolase (NSE), and CYFRA21-1 were normal. The patient underwent a lung biopsy by thoracoscopic surgery after four weeks of anti-TB treatment; there were no significant changes in the chest CT. H&E staining showed that the tumor cells had characteristics of smooth muscle cell differentiation. Immunohistochemical staining showed a low tumor cell proliferation index, which indicated that the likelihood of a malignancy was not high. There was no expression of CD10, indicating a diagnosis of pulmonary benign metastasizing leiomyoma (PBML). Smooth muscle actin (SMA) and desmin as specific markers of smooth muscle and the estrogen receptor (ER) and progesterone receptor (PR) were all strongly positive, which is characteristic of PBML. The patient was given the anti-estrogen tamoxifen for 3 months. With no radiological evidence of disease development and further distant metastasis, the patient will continue to be followed.     

Pulmonary nodules in early fat embolism syndrome: a case report

The radiologic abnormalities in a patient with mild clinical manifestations of fat embolism are reported. The findings consisted of small nodular opacities, which were shown on computed tomography (CT) scans to be located predominantly in the centrilobular and subpleural regions. The nodules presumably represented alveolar edema or hemorrhage secondary to the fat embolism syndrome.     

肺球孢子菌感染一例并文献复习

目的 提高对肺球孢子菌病的认识.方法 结合1例肺球孢子菌病患者的临床资料及诊治经过并复习相关文献,对球孢子菌病的临床表现、病理结果及治疗进行分析.结果 患者男,74岁,因"体检发现双肺占位性病变9个月,咳嗽2周"于2009年2月10日收入南京医科大学第一附属医院呼吸内科.患者9个月前体格检查发现双肺多发占位性病变,于2008年6月17日在外院行肺穿刺活检,病理结果提示隐球菌感染,给予氟康唑治疗6个月后,复查胸部CT病灶无明显改变.入院后13 d行胸腔镜下双肺楔形切除术,术后病理结果提示球孢子菌病、孢子菌瘤形成.术后先后给予伏立康唑和氟康唑治疗共6个月,复查胸部CT未见复发.结论 原发性肺球孢子菌病少见,临床表现不典型,其病理所见易与隐球菌混淆.随着免疫抑制剂的广泛使用,本病可能会逐渐增多,临床医生应提高对该病的认识.     

Capillary leakage syndrome: a case report and a review

Capillary leakage Syndrome (CLS) is a rare clinical syndrome, that was first described in 1960, characterized by acute episodes of generalized edema, hemoconcentration, hypoproteinemia and monoclonal gammopathy, in the vast majority of cases. We describe a 39-year-old man with anasarca, bilateral pleural and pericardial effusions, ascites and diffuse alveolo-intersticial edema. Clinical and laboratory findings were consistent with an acute episode of CLS. Treatment with prednisone, furosemide and aminophylline was started, which lead to a gradual improvement in 48 hours. Pathophysiologically there is an increase in capillary permeability with the extravasation of fluid and plasmatic proteins to the extravascular space that can lead to hypovolaemic shock. In the second phase there is a reentry of the fluid overload leading to pulmonary edema. The etiology of this hyperpermeability still remains unclear. The role of cytokines has become central in the comprehension of pathophysiology of CLS. Adhesion molecules are probably also involved in the genesis of capillary leakage. CLS treatment remains empirical. However, at present it seems that the association of steroids with furosemide, aminophylline and terbutaline are capable of controlling the clinical manifestation of the acute episodes in most cases. To our knowledge no prophylatic therapy has clearly proven its efficacy. There are only a few series analyzing the long-term evolution of patients with CLS. Further studies are necessary with the objective to collect enough patients with CLS to observe natural history of the disease and evaluate the efficacy of empiric treatments.     

Carotid stump syndrome. A case report and literature review.

Carotid stump syndrome occurs infrequently. Diagnosis and management requires flow studies with duplex ultrasound scan as well as anatomical delineation of the affected vessel by magnetic resonance angiography. Surgical exclusion of the internal carotid artery is safe and effective and is the gold standard of treatment with best medical therapy.