Mucoviscidosis of the lung

Summary A case of bronchiectasis due to mucoviscidosis without any clinical evidence of involvement of the pancreas is present. The importance of bearing this condition in mind in cases of recurrent chest infections in children in the absence of other manifestations of the disease is stressed. From the departments of Pediatrics and Pediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh.     

Mucoviscidosis and allergic bronchopulmonary aspergillosis]

Allergic bronchopulmonary aspergillosis (ABPA) is a severe disease inducing bronchopulmonary anatomic lesions which complicate those already present in patients with cystic fibrosis. The frequency of this association is estimated between 0.6 and 10% according to American studies. Diagnosis of ABPA is difficult, as findings such as sibilant rales, pulmonary infiltrates, bronchiectasies, anti-aspergillus precipitins may be present as single features in patients with cystic fibrosis. Thus it is important to ascertain the diagnosis as oral corticosteroid treatment is the only one able to prevent evolution towards bronchiectasies and pulmonary fibrosis.     

Survivin与肺动脉高压

肺动脉高压是慢性、严重的致命性疾病,可继发于心、肺、全身性疾患或原因不明,其特点是肺动脉压力和肺血管阻力增高,表现为进行性呼吸困难和运动受限,最终因右心室功能衰竭而死亡^[1]。对肺动脉高压的研究已有100多年的历史,但其发病机制至今尚未完全明了,现有的治疗手段有限,疗效也不确切。近年来,随着细胞生物学和分子生物学的飞速发展,对肺动脉高压发病机制有了深入的了解,     

Secondary pulmonary hypertension

Recent research has greatly improved our understanding of the pathophysiology of pulmonary hypertension. There is increasing recognition that pulmonary hypertension is an important complication of many childhood respiratory diseases including cystic fibrosis, interstitial lung diseases, upper airways obstruction and disorders of the respiratory muscles and chest wall. Chronic hypoxaemia and, in some cases, destruction of the vascular bed are the key factors. The clinical features of pulmonary hypertension are often overshadowed by those of the primary respiratory disease but newer imaging techniques allow earlier detection of this complication. This may be important in the future if new specific therapies for primary pulmonary hypertension are shown to be beneficial in secondary pulmonary hypertension. With some conditions, such as airways obstruction due to adenotonsillar hypertrophy, treating the underlying cause leads to rapid resolution of the hypertension. However, with most disorders, such as cystic fibrosis, management is focused on treating the lung disease intensively and correcting the chronic hypoxaemia with oxygen therapy, sometimes augmented by nasal mask ventilation. However, although several new selective therapies are effective in idiopathic pulmonary arterial hypertension, their role in secondary pulmonary hypertension remains unclear.