Pneumomediastinum, subcutaneous emphysema, and pulmonary fibrosis in a patient with idiopathic pneumonia syndrome after bone marrow transplantation

An adolescent female underwent bone marrow transplantation for relapsed leukemia and developed acute and chronic graft-versus-host disease and idiopathic pneumonia syndrome. Her lung disease responded to large doses of methylprednisolone but evolved to pulmonary fibrosis and pneumomediastinum and subcutaneous emphysema in the convalescent period. Pulmonary function tests revealed a restrictive pattern. Pneumomediastinum and subcutaneous emphysema are complications not only of obstructive but also of restrictive lung disease and vary with respect to time of onset.     

Familiärer mangel und α 1

Zusammenfassung Es wird über zwei Kinder aus zwei Familien mit Mangel an ₁ berichtet. Homozygote Defektträger wiesen etwa 15–25%, heterozygote 50–75% des mittleren normalen Serumspiegels auf. Unter 100 Blutspendern fanden sich 5 mit erniedrigten ₁ wie bei heterozygoten Defektträgern.Klinische Auswirkungen des Gendefektes waren im Kindesalter nicht zu erkennen, insbesondere keine Enthemmung der Fibrinolyse.

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₁ deficiency in two families

Two children from two different families with ₁ deficiency are described. Deficient subjects had 15–25% of normal serum ₁ levels in case of homozygosity and 50–75% in case of heterozygosity. Among one hundred healthy blood donors five had serum ₁ levels below 150 mg% corresponding to the levels found in heterozygous deficiency carriers.In the two children described the gene defect had no clinical consequence; fibrinolysis, in particular was not increased.

     

慢性阻塞性肺气肿上呼吸道病变的CT观察

目的　分析慢性阻塞性肺气肿上呼吸道的变化。方法　对 4 0例确诊为慢性阻塞性肺气肿患者和同期 39例正常对照者行肺部螺旋CT扫描。测量以下指标并进行统计学分析 :(1)气管横截面积 (Ta) ;(2 )气管指数 (Ti) ,即气管的最大横径 (Tc)和最大矢径 (Ts)的比率 ;(3)左、右主支气管直径 (分别记为Bl和Br) ;(4 )左、右主支气管的分叉角度 (Ba) ;(5 )胸廓指数 (Thi) ;(6 )气体潴留指数 (Ati)。结果　除Ba外 ,气肿组与对照组上述指标的均值差异都有显著性意义 ,气肿组的Ti与Ati呈明显的负相关 (r =- 0 5 93,P <0 0 0 1) ,Ti与Thi呈明显的正相关 (r =0 5 37,P <0 0 1)。Ta与Ati(r =0 10 5 ,P >0 0 5 )和Ta与Ti(r =- 0 12 5 ,P >0 0 5 )均无明显的相关关系。结论　Ti减小是慢性阻塞性肺气肿气管的主要变化 ,而且这种改变与肺气体潴留和胸廓改变相关     

阻塞性肺气肿模型的制作

目的在2周内对国际标准实验动物Beagle犬进行肺气肿模型的制作，建立较为标准的肺气肿模型，从而为治疗慢性阻塞性肺疾病（COPD）的方法提供良好的动物模型。方法Beagle犬8只，雌雄各半，2岁，体重13～18kg，雾化吸人木瓜蛋白酶12000U/kg，1次，周，共2周。实验前后测量试验动物肺功能，做胸部CT及病理学比较。结果实验前后动物肺功能，CT及病理学检查均显示较大的差异。8例实验动物的功能残气量（FRC）、肺总量（TLC）、功能残气量比肺总量（FRC/TLC％）、潮气量（VT）和呼吸频率（BR），在肺气肿模型制作前后做两两比较，结果显示均有统计学差异P〈0.05，血氧饱和度（SpO2）0.05两者间无统计学差异。CT显示肺体积增大，局部肺野透亮度增加有肺大泡形成。试验后病理学检查显示肺泡壁破坏，肺泡间质断裂，肺泡融合，形成典型的全小叶型肺气肿改变。实验后动物出现肺气肿征表现，如气喘，咳嗽及上呼吸道分泌物增等。结论在标准实验动物身上可以较为准确、快速地复制出近似于人类阻塞性肺气肿病理改变的动物模型。     

Risk factors for death while awaiting lung transplantation in Israeli patients: 1997–2006

OBJECTIVE: Patients with end-stage lung disease very frequently die while awaiting lung transplantation. The aim of this study was to identify factors associated with mortality in patients referred for lung transplant assessment. METHODS: The files of all consecutive patients listed for lung transplantation in Israel between 1997 and 2006 were reviewed and the data were compared statistically between those who survived to transplantation. RESULTS: A total of 229 patients were listed for lung transplantation, of whom 42 (18.3%) died while awaiting transplantation. Comparison of the patients who survived to transplantation with those who did not using univariate analysis revealed that the died-waiting group was significantly older, used steroids to a greater extent, had more IPF patients and less emphysematous, and lower mean oxygen saturation at rest (p=0.005). There were no between-group differences in comorbid diseases or pulmonary function measurements. The 6 min walk distance was strongly and inversely correlated with risk of death before transplantation (p=0.005). On multivariate analysis, only oxygen saturation at rest was a significant independent risk factor for death while awaiting transplantation (OR 0.886; C.I. 0.805-0.974). CONCLUSIONS: There are several risk factors for death in the Israeli population listed for LTX, including age, steroid use, emphysematous patients and lower saturation at rest.     

Secondary spontaneous pneumothorax associated with emphysema and ruptured bullae at the azygoesophageal recess

Purpose  With secondary spontaneous pneumothorax (SSP) associated with emphysema, lesions responsible for pneumothorax can be located anywhere along the lung surface. Among such lesions, ruptured bullae at the azygoesophageal recess (AER) have received little attention thus far. Methods  We conducted a retrospective study of 38 right SSP patients with emphysema who underwent surgery. Among them, we reviewed the clinical characteristics and technical problems of patients with surgically proven ruptured bullae at the AER. Results  Ruptured bullae at the AER were found in 10 of 38 patients. They accounted for 26.3% of all 38 patients and for 66.7% of 15 patients whose bullae at the AER were identified by preoperative computed tomography (CT). On CT, all the bullae were relatively large and oriented in a predominantly vertical axis. At surgery, they were confirmed as white, thin-walled structures originating from the mediastinal part of the apical segment of the right lower lobe. Surgery typically consisted of stapling bullectomy with video-assisted thoracic surgery. Technical problems in surgical treatment included poor mobilization of the base of the bulla and a restricted working space. Conclusion  Bullae at the AER are common and possibly lead to rupture. The presence of a bulla at the AER seen by CT can be predictive of rupture. Although the AER is a unique location, video-assisted bullectomy is the method of choice for treating these lesions.     

Differential Diagnosis of Chronic Airflow Obstruction

Chronic obstructive pulmonary disease is a syndrome including illnesses such as asthma, chronic bronchitis, and emphysema. Although these diseases share a common obstructive component, their optimal treatment and prognosis differ. This article examines the salient features of the history, physical exam, pulmonary function tests, and radiological evaluation which may allow the clinician to differentiate the various diseases that make up COPD; thus allowing the clinician to better target the multiple therapeutic modalities available.     

The nature and causes of chronic obstructive pulmonary disease: A historical perspective: The Christie Lecture 2007, Chicago, USA

Chronic obstructive pulmonary disease (COPD) is the currently favoured name for the diseases formerly known as emphysema and bronchitis. COPD has been recognized for more than 200 years. Its cardinal symptoms are cough, phlegm and dyspnea, and its pathology is characterized by enlarged airspaces and obstructed airways. In the 19th century, the diagnosis of COPD depended on its symptoms and signs of a hyperinflated chest, and reduced expiratory breath sounds. The airflow obstruction evident on spirometry was identified in that century, but did not enter into clinical practice. Bronchitis, and the mechanical forces required to overcome its obstruction, was believed to be responsible for emphysema, although the inflammation present was recognized. The causes of bronchitis, and hence emphysema, included atmospheric and domestic air pollution, as well as dusty occupations. Cigarette smoking only became recognized as the dominant cause in the 20th century. The lessons learned of the risks for COPD in 19th-century Britain are very pertinent to the world today.     

Pulmonary barotrauma including orbital emphysema following inhalation of toxic gas

Severe pulmonary barotrauma occurred following smoke and toxic gas inhalation in a 20-year-old male. He developed pneumothorax, pneumomediastinum, and extensive facial subcutaneous emphysema which intensified during treatment with positive pressure ventilation. Following the appearance of diplopia and exotropia, orbital emphysema was demonstrated radiologically. The diplopia and exotropia were manifestations of mechanical interference in extra-ocular muscle function by the intra-orbital air, an unusual expression of pulmonary barotrauma.