Two sisters with idiopathic pulmonary hemosiderosis

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage with unknown etiology. In the present report, the presentations of two sisters are described: one sister had IPH, eosinophilia and a high serum immunoglobulin E (IgE) level; and the other had IPH, pneumothorax, eosinophilia and a high serum IgE level. Both cases had quite unusual presentations. The first patient was 23 years of age, and had suffered from dry cough and progressive dyspnea for four years. Her hemoglobin level was 60 g/L, total serum IgE level was 900 U/mL and eosinophilia was 9%. Her chest radiography revealed diffuse infiltration. She died due to respiratory failure. The second patient was 18 years of age. She had also suffered from dry cough and gradually increasing dyspnea for two years. She had partial pneumothorax in the right lung and diffuse infiltration in other pulmonary fields on chest radiography. Her hemoglobin level was 99 g/L, total serum IgE level was 1200 U/mL and eosinophilia was 8%. IPH was diagnosed by open lung biopsy. All these findings suggested that familial or allergic factors, as well as immunological factors, might have contributed to the etiology of IPH.     

A 14-year-old with pulmonary hamartomatous lymphangiomyomatosis associated with bilateral pneumothoraces]

A 14-year-old girl was admitted because of cough, chest pain and hemosputum. Chest roentgenogram on admission showed a pneumothorax and a cavitary lesion with niveau formation in the right lung and cystic lesions in the bilateral lung fields. After bed rest and intravenous administration of antibiotics for two weeks, the right lung inflated well and the niveau formation disappeared, and the patient was discharged. One week later, she was readmitted with sudden-onset severe dyspnea, caused by bilateral pneumothoraces. Emergency tube thoracostomy and wedge resection of the bullous lesion was performed. Macroscopically, multiple small cystic changes were seen on the surface of the right lung. Histological examination revealed nodular proliferations of smooth muscle cells in the interstitium and vessel walls in the lung, which contained slit-like lymphatic channels. The diagnosis of pulmonary lymphangiomyomatosis was made. In this case, we could not measure receptors for estrogen and progesterone. Recently, hormonal therapy and oophorectomy have been reported as being useful. Tamoxifen (Norvadex) was therefore initiated, and the patient has remained well with slight dyspnea on exertion. There has been no recurrence of pneumothorax. Lymphangiomyomatosis is a rare disease of unknown etiology which occurs exclusively in women, mostly in those of reproductive age. We report a 14-year-old female patient with lymphangiomyomatosis associated with repeated pneumothorax, who had been under treatment for epilepsy. We believe this case to be of importance because of the long discussed relation between pulmonary lymphangiomyomatosis and tuberous sclerosis.     

Two cases of pneumothorax using the thoracic vent (TV) on an outpatient basis]

In case 1, a 73-year-old man developed dyspnea on exertion while travelling abroad. The symptom continued until the patient returned to Japan 5 days after onset and visited our clinic on the next day. SaO2 was 93% and chest radiography demonstrated pneumothorax with 75% collapse of the right lung. Insertion of a TV successfully prevented air leakage stopped within 2 days. The TV was removed after the lung collapse disappeared, as shown on the chest radiograph taken 4 days after its insertion. Case 2 occurred in a 62-year-old woman with underlying idiopathic interstitial pneumonitis, who had had dyspnea on exertion for approximately 5 years. The patient visited our clinic because the dyspnea on exertion became abruptly exacerbated. A TV was inserted after an SaO2 of 82% was measured and the chest radiograph revealed pneumothorax with a 50% collapse of the right lung. Air leakage stopped after 5 days, and the TV was removed on the 8th day after insertion. We concluded that TV is very useful for improving the QOL of patients and enables outpatient management of pneumothorax, resulting in a reduction in the cost of treatment.     

A case of pulmonary sarcoidosis with pneumothorax and pleural effusion after improvement of pulmonary impairment]

A 23-year-old man was admitted to our hospital for a complete medical evaluation of abnormal pulmonary shadows found on a chest radiograph during his annual check-up. Chest radiography and chest CT showed a diffuse spread of micronodules in both lung fields and mediastinal lymphadenopathy. A transbronchial lung biopsy demonstrated evidence of noncaseating epithelioid cell granuloma with multinucleated giant cells, and a diagnosis of sarcoidosis was made. The pulmonary shadows improved without therapy. Twenty months later, the patient was readmitted to the hospital because of chest pain and dyspnea. Pneumothorax appeared on the right in a chest radiograph, but subsided after drainage therapy, and two weeks later, a right side pleural effusion was seen. We determined that the pleura was involved in the sarcoidosis, and the patient was treated with oral prednisolone 20 mg daily. The pleural effusion gradually subsided. This is the first reported case in Japan of pulmonary sarcoidosis with pneumothorax and pleural effusion after improvement of pulmonary impairment.     

A case of primary pulmonary proteinosis with bilateral pneumothorax]

A 40-year-old man was admitted to our hospital because of chronic cough and mild exertional dyspnea. Radiographs of the chest showed diffuse infiltrative shadows in both lung fields. A diagnosis of pulmonary alveolar proteinosis (PAP) was made by examination with a flexible bronchoscope and bronchoalveolar lavage, with transbronchial biopsy. After diagnosis, the patient underwent whole-lung lavage with temporary improvement. Due to the recurrence of his illness, he needed a total of four whole-lung lavages over the course of his illness. However, the exertional dyspnea became progressively worse. Bilateral pneumothorax developed suddenly and led to his death. This case indicates the possibility that deterioration of PAP despite whole-lung lavage may sometimes be followed by pneumothorax.     

Spontaneous pneumothorax and active pulmonary tuberculosis

We report three HIV-negative patients with spontaneous pneumothorax as clinical manifestation of active tuberculosis acute chest pain and dyspnea was the cause of admission. Chest roentgen grams showed lung collapse and parenchymal cavitation in two of therm and hydropneumothorax in the other. Outcome was favorable with antituberculous drug therapy and placement of chest tube. Although spontaneous pneumothorax is frequent in fibrosis pulmonary tuberculosis, it seldom complicated active tuberculosis in spite of increase of its incidence.     

Dyspnea secondary to pulmonary hematopoiesis as presenting symptom of myelofibrosis with myeloid metaplasia

We report a case of a patient with myelofibrosis with myeloid metaplasia (MMM) who presented with progressive dyspnea of unexplained origin. Splenomegaly, blood smear, and bone marrow findings allowed diagnosis of MMM. High-resolution CT chest scan revealed diffuse septal thickening, while echocardiography and electrocardiogram showed no indirect evidence of pulmonary hypertension. Finally, lung biopsy revealed irregularly distributed interstitial fibrosis with islands of erythroblasts, immature granulocytic elements, and dysplastic megakaryocytes, allowing diagnosis of pulmonary extramedullary hematopoiesis (EMH). The patient received hydroxyurea as cytoreductive agent, obtaining a good hematologic response and an improvement of dyspnea. Note that, in this patient, dyspnea was the first clinical symptom of MMM; the dyspnea was not associated with pulmonary hypertension and improved following cytoreductive treatment. This case points to the importance of suspecting pulmonary EMH when unexplained progressive dyspnea occurs in a patient with MMM. Early recognition of pulmonary EMH may prevent PH and favor a better response to therapy.     

A case of pulmonary tuberculosis associated with severe respiratory failure, DIC and intractable bilateral pneumothoraces]

We had a sixty-five year old male patient who suddenly complained of dyspnea and fever with pulmonary tuberculosis, severe respiratory failure, disseminated intravascular coagulation (DIC) and intractable bilateral pneumothoraces. From the first hospital day severe hypoxemia which did not respond to conventional oxygen therapy developed with a diffuse ill-defined reticulo-nodular shadow in the plain chest x-ray film. On the 2nd hospital day mechanical ventilation with 2cmH2O PEEP was introduced. Antituberculous agents as well as corticosteroids were started suspecting acute interstitial pneumonia with pulmonary tuberculosis and adult respiratory distress syndrome (ARDS). Medication was followed by the treatment of Gabexate mesilate and heparin against DIC on laboratory data. Though clinical findings and pulmonary infiltrate on chest x-ray film transiently improved, right pneumothorax occurred suddenly on the 6th day followed with left pneumothorax on the 36th day. Tube drainage of both pleural spaces and repeated instillation of thrombin-rich oxycel cotton via bronchofiberscope failed to stop air leakage. He ultimately expired on 49th hospital day. At postmortem lung had multiple bilateral bulla several of which ruptured to the pleural site and caseating necrotic area containing bacilli positively stained with Ziehl-Nielsen stain in the bilateral upper lobe. No typical caseating necrotic lesion, however, was found in the other lung tissue. Therefore, it seemed to show a chronic phase of diffuse alveolar damage (DAD).     

Simultaneous bilateral pneumothorax complicating Pneumocystis carinii pneumonia in a homeless man with AIDS]

Although pneumothorax is a well-known complication of AIDS related Pneumocystis carinii pneumonia, simultaneous bilateral pneumothorax has not been reported in Japan. A 54-year-old homeless man was admitted with emaciation and dyspnea. Chest X-ray showed diffuse ground glass opacity. Computed tomography of the chest demonstrated ground glass opacity and cyst-like lesions in both upper lobes of the lung. The patient was HIV positive. Grocott's stain of a bronchial lavage specimen demonstrated Pneumocystis carinii microorganisms. Intravenous trimethoprim-sulfamethoxazol and steroid administration was started. Seven days later, the patient developed tension pneumothorax of the right lung. Immediately after the thoracostomy, contralateral pneumothorax developed. Another chest tube was inserted. Subsequently, the patient died from bilateral pneumothorax. The endemicity of HIV infections among the homeless population is already a significant problem in Western countries. We should be aware that HIV infection may become a problem among the Japanese homeless as well.     

肺郎格罕细胞组织细胞增多症7例临床分析

目的探讨肺郎格罕(Langerhans)细胞组织细胞增多症的临床表现,以提高对本病的认识。方法回顾性分析1997—2006年北京协和医院确诊的7例肺郎格罕细胞组织细胞增多症的临床资料。结果7例患者均为男性,平均年龄26.7岁,其中4例吸烟,主要症状为咳嗽、活动后气短,5例在疾病过程中发生气胸。肺功能示阻塞性通气功能障碍3例、限制性通气功能障碍2例、混合性通气功能障碍2例,4例有不同程度的弥散功能障碍。胸部高分辨CT(HRCT)示6例表现为双上中肺野网格状改变及囊性变,仅有1例可见小结节影。外科肺活检标本病理学检查结果示7例光镜下均可见病理性郎格罕细胞浸润,6例可见囊样及气腔样结构。免疫组化阳性检出情况为7例S-100均为阳性;5例行CD1a检查者中4例阳性;6例行CD68检查者中5例阳性。结论肺郎格罕细胞组织细胞增多症常见于年轻吸烟男性,主要临床表现为咳嗽、活动后气短、反复气胸,胸部HTCT表现为双上中肺野为主的网结节或囊性变,肺功能无特异性改变,可伴有弥散功能异常,病理学检查可见病理性郎格罕细胞或免疫组化CD1a、S-100阳性可以明确诊断。     

Diffuse cystic disease of the lung associated with simultaneous bilateral spontaneous pneumothorax: an unknown component of Caroli disease?

Simultaneous bilateral spontaneous pneumothorax is an uncommon and serious medical problem frequently related to an underlying lung disease such as parenchymal lung disease, inflammatory events or neoplasms. Recurrent pneumothorax associated with persistent air leakage may pose a therapeutic dilemma in patients with underlying lung diseases. Caroli disease (CD) is a very rare congenital disorder characterised by intrahepatic biliary cystic dilatations and frequently associated with polycystic kidney and hepatic fibrosis. To date, no relation between CD and the bilateral diffuse cystic structure of pulmonary parenchyma has been described. In this paper we present a patient with the diagnosis of CD in whom simultaneous bilateral spontaneous pneumothorax was the initial finding due to severe underlying pulmonary disease. Our patient's demographic and clinical characteristics, laboratory findings and course made us exclude the other aetiologies leading to such diffuse multi-bullous pulmonary involvement. The coincidence of recurrent pneumothorax and severe pulmonary disease has led to a big therapeutic dilemma. Open or minimally invasive surgery could be morbid or even mortal because of the nature of the parenchyma and the operative risk due to CD. Therefore, we suggest a unique conservative management including tube thoracostomy by locating the correct air spaces with the aid of high-resolution computed tomography followed by talc pleurodesis in patients with severe multi-bullous lung disease associated with potential risks due to co-morbidities.     

Erdheim-Chester disease presenting with pneumothorax

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis most commonly characterized by symmetrical skeletal involvement and may present with pulmonary involvement leading to chronically progressive pulmonary symptoms. Characteristics on chest radiography include non-specific findings of diffuse interstitial and pleural thickening, micronodules, ground-glass opacities and parenchymal condensation as a result of infiltration by lipid-laden histiocytes. We present the case of a 50-year-old man with ECD presenting with acute pulmonary symptoms due to rupture of a large cystic lesion with resultant pneumothorax. He was brought by ambulance to our hospital, complaining of acute anterior chest pain and severe dyspnea. Chest radiography showed right-sided pneumothorax with a collapsed lung, a large, left-sided cystic lesion in the upper lung field and accentuated interstitial markings. Bullectomy and surgical biopsy were performed, demonstrating histologically histiocytic infiltrates that were strongly positive for CD68, but negative for S-100 protein and CD1a. Subsequent systemic examinations indicated widespread symmetrical skeletal involvement, leading to a definitive diagnosis of ECD.     

Persistent spontaneous pneumothorax in an adolescent with Marfan's syndrome and pulmonary bullous dysplasia.

A 16-year-old boy with Marfan's syndrome was admitted with progressive dyspnea due to a large spontaneous pneumothorax. Bullous pulmonary dysplasia was confirmed and pleural tube drainage did not affect the air leak. Complete recovery required surgical resection of the bulla responsible for the ongoing air leak. This case report highlights the issue of management for severe spontaneous pneumothorax in general, showing that the choice of treatment should not depend on the presence of pulmonary bullous dysplasia but on the clinical evaluation of the individual patient.     

COPD并自发性气胸行肺大疱切除加单侧肺减容术临床分析

目的报道急诊或亚急诊肺大疱切除加单侧肺减容术治疗COPD并自发性气胸的临床结果。方法对32例COPD并自发性气胸患者实施肺大疱切除加单侧肺减容术,术后随访24个月,分别于术后第6、12、24个月测定动脉血气分析值。结果术后第6、12、24个月与术前相应数值比较,均有明显好转（P〈0.05）。全组无手术死亡,术后并发症发生率在35%左右。结论肺大疱切除加单侧肺减容术可以改善COPD并自发性气胸患者的临床症状,且手术死亡率低,尽量切除肺大疱及病变侧过度气肿的肺组织和防止残肺漏气为手术关键,做好围手术期的处理,能明显减少术后并发症。     

肺减容术37例临床分析

目的对临床肺减容术效果进行探究。方法针对在我院肺减容术37例患者的预后研究分析。结果本组37例患者均治愈出院,无一例死亡。术后所有患者呼吸困难的症状得到明显改善,FEV1和动脉血氧饱和度均有所增加,高碳酸血症也有明显的改善。术后15例患者出现肺漏气,占40.5%;3例发生对侧气胸,占8%,经胸腔闭式引流后痊愈;7例肺部感染,占18.9%,经大量抗生素治疗后痊愈。结论 LRVS有良好的近期疗效,能明显改善患者的呼吸功能和生活质量,为广大重症COPD带来了福音。     

肺结核并自发性气胸的临床特征及治疗

目的探讨肺结核合并自发性气胸的临床及治疗。方法对360例肺结核合并自发性气胸的临床表现和治疗方法进行回顾性分析。结果肺结核并气胸的临床特征是症状重、呼吸困难多见(26例,74.7%)、呼吸衰竭发生率高(217例,60.3%)、胸腔闭式引流率高(283例,78.6%)、多肋间插管率高(59例,16.4%),55例(15.3%)患者进行了纤维支气管镜介入治疗,共治愈356例,治愈率98.9%,肺复张时间平均6.5d。结论肺结核并自发性气胸病情危重者多,及时有效的排气减压预后良好,辅以纤支镜介入治疗可提高疗效。     

Case of pneumothorax associated with pulmonary Mycobacterium fortuitum infection

A 39-year-old man with dyspnea was revealed to have severe pneumothorax and received partial resection of the left upper lobe after unsuccessful drainage. Necrotizing epitheloid granuloma was found in the resected lung and Mycobacterium fortuitum was detected from the lesion. Chemotherapy with levofloxacin and clarithromycin was started one year after surgery because of the newly found nodular shadow near the lesion. The case experienced pyothorax due to pulmonary tuberculosis three years before and Mycobacterium avium pleuritis one year before this episode. Three-time mycobacterial pleural infection in three years seems to be uncommon. Furthermore this is the first report of pneumothorax associated with pulmonary Mycobacterium fortuitum infection.     

肺淋巴管平滑肌瘤病

目的　提高对罕见病肺淋巴管平滑肌瘤病 (PL AM)的认识。方法　对我院诊治的患者进行分析 ,并结合文献复习。结果　 PL AM是一种罕见的弥漫性肺部疾病 ,临床反复发作自发性气胸或 /和乳糜胸、活动后呼吸困难和痰血等。肺功能呈阻塞性或混合性通气功能障碍 ,低氧血症。胸部 X线表现为两肺弥漫分布网格状阴影 ,高分辨 CT(HRCT)示弥漫分布囊状改变。病理学检查示 :肺组织淋巴管增生和扩张 ,管外平滑肌明显增生。结论　育龄期妇女如反复发生气胸及出现原因不明的呼吸困难、咯血或乳糜胸 ,应疑诊 PL AM,及时行 HRCT及肺功能检查 ,必要时肺活检 ,以明确病理诊断。     

Pulmonary involvement in tuberous sclerosis

Tuberous sclerosis is a neurocutaneous syndrome involving many tissues. Pulmonary involvement is rare. Pulmonary cases are usually women of child-bearing age who present with spontaneous pneumothorax or progressive dyspnea. In childhood and, even more, in male patients, pulmonary involvement by tuberous sclerosis is exceptional. We report on a 4-year-old male patient diagnosed in the first months of life with tuberous sclerosis with predominantly respiratory symptoms (frequently with acute RDS criteria), in whom lung involvement was pathologically confirmed.     

A case of silicoproteinosis with pneumothorax]

A 46-year-old man was admitted because of an increasingly severe cough and dyspnea on exertion. For 13 years, he had inhaled sand containing 100% crystalline silica (SiO2). Chest radiographs revealed right pneumothorax and diffuse small nodular and ground-glass opacities in both lungs (especially in the upper lung fields). A chest CT scan disclosed several bullae in both upper lobes, and an open lung biopsy was performed along with resection of these bullae. Subsequently, silicotic nodules containing silica and PAS-positive materials were recognized in the alveolar spaces in the histological findings, and a diagnosis of silicoproteinosis was made. We have reported on this case of silicoproteinosis with pneumothorax which progressed for over one year and which showed unusual radiological findings dissimilar to those of primary pulmonary alveolar proteinosis.