Combined Pulmonary Fibrosis and Emphysema

The combination of pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, in which an upper lobe emphysema and lower lobe fibrosis coexist in a single patient. These patients have a characteristic lung function profile, with apparently normal or minimally altered dynamic and static lung volumes, contrasting with a significant reduction of carbon monoxide transfer (DLco), and hypoxemia, which worsens with exercise. Pulmonary hypertension is highly prevalent and is the principal negative prognostic factor for this condition. High resolution computed axial tomography (HRCT) is the main tool to confirm the diagnosis. Cigarette smoking has been proposed as the main factor in its etiology; however, neither pathogenic mechanisms nor the sequence of events involved in this syndrome has been clarified yet. Experimental studies in animal models are providing information on the involvement of some inflammatory mediators in the pathogenesis. There is currently no consensus on the therapeutic approach to be followed in these patients, since the studies published to date on this subject are limited to wellcharacterized series of cases. Therefore, it is a pathology with many unknowns yet to be resolved and highly likely to be underdiagnosed, unless its functional clinical characteristics are taken into account.     

Gastroesophageal Reflux and Idiopathic Pulmonary Fibrosis

Background

Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease of unknown origin that affects about 40,000 new patients every year in the USA. Albeit the disease is labelled as idiopathic, it is thought that pathologic reflux, often silent, plays a role in its pathogenesis through a process of microaspiration of gastric contents.

Aims

The aim of this study was to review the available evidence linking reflux to IPF, and to study the effect of medical and surgical therapy on the natural history of this disease.

Results

Medical therapy with acid-reducing medications controls the production of acid and has some benefit. However, reflux and aspiraion of weakly acidic or alkaline gastric contents can still occur. Better results have been reported after laparoscopic anti-reflux surgery, as this form of therapy re-establishes the competence of the lower esophageal sphincter, therefore stopping any type of reflux.

Conclusions

A phase II NIH study in currently in progress in the USA to determine the role of antireflux surgery in patients with GERD and IPF. The hope is that this simple operations might alter the natural history of IPF, avoiding progression and the need for lung transplantation.

     

特发性肺间质纤维化患者的护理

对18例特发性肺间质纤维化患者采用糖皮质激素、免疫抑制剂及对症支持治疗,在此基础上给予心理护理、呼吸功能训练、氧疗、药物治疗等措施.结果 18例患者住院期间临床症状明显缓解,无护理并发症发生.     

牛黄解毒片乙醇浸泡液外敷治疗小儿输液外渗

对18例特发性肺间质纤维化患者采用糖皮质激素、免疫抑制剂及对症支持治疗，在此基础上给予心理护理、呼吸功能训练、氧疗、药物治疗等措施。结果18例患者住院期间临床症状明显缓解，无护理并发症发生。     

Guidelines for the Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis is defined as a chronic fibrosing interstitial pneumonia limited to the lung, of unknown cause, with poor prognosis and few treatment options. In recent years there has been an increase in their prevalence, probably due to the optimization of diagnostic methods and increased life expectancy. The ATS/ERS Consensus (2000) established the diagnostic criteria and recommendations for the assessment of the disease course and treatment. Later studies have helped to redefine diagnostic criteria and treatment options. In 2011, an international consensus was published, establishing diagnostic criteria and new treatment strategies. These guidelines have been updated with the newest aspects of diagnosis and treatment of idiopathic pulmonary fibrosis. A level of evidence has been identified for the most relevant questions, particularly with regard to treatment options.     

Familial Pulmonary Fibrosis in 2 Mexican Sisters With Hermansky-Pudlak Syndrome

Hermansky-Pudlak syndrome is an autosomal recessive disorder commonly found in individuals of Puerto Rican ancestry. We present 2 cases of familial pulmonary fibrosis in 2 Mexican sisters with Hermansky-Pudlak syndrome. Pulmonary fibrosis was biopsy-proven in 1 of the patients. This report shows that Hermansky-Pudlak syndrome may occur in individuals of Mexican ancestry.