Stanislao Faraone

Increased Endothelin-Like Immunoreactive Material on Bronchoalveolar Lavage Fluid from Patients with Bronchial Asthma and Patients with Interstitial Lung Disease

Endothelin (ET) is an endothelial regulatory peptide present also in pulmonary tissue where it exerts several biological actions both on bronchial and vascular smooth muscle cells. It has been shown to increase in bronchoalveolar lavage fluid (BALF) from asthmatic patients; but changes in other chronic respiratory disease have not been well studied. We measured by a radioimmunoassay (RIA) ET-immunoreactive (IR) levels on BALF (BALF-ETIR, pg/ml) from 5 normal subjects (NS), 5 patients with chronic extrapulmonary disease (ED) without signs of lung involvement, 5 patients with allergic bronchial asthma (BA), 10 patients with idiopathic lung fibrosis (ILF) and 9 patients with miscellaneous interstitial lung disease (MILD). In 5/5 NS and 4/5 ED BALF-ETIR was lower than sensibility of RIA test used (0.8 pg/ml). BALF-ETIR was dosable in all patients with bronchopulmonary disease; means were 2.45 pg/ml in BA, 12.37 pg/ml in ILF, 2.90 pg/ml in MILD--Wilcoxons rank test (two tailed) versus NS, p < 0.05. There was an inverse correlation between BALF-ETIR values and the degree of ventilatory impairment (forced vital capacity % of predicted value, r = -0.61 p < 0.01; forced expiratory volume in 1 s % of predicted value, r = -0.71 p < 0.01) and the level of arterial pressure of O2 (PaO2; r = -0.75 p < 0.01); a positive correlation was found with number of neutrophils/ml of BALF (r = 0.52 p < 0.01)--Spearmans rank correlation. Though rarely detected on BALF from normal lungs, ET increases on BALF in patients with bronchopulmonary disease, raising the question of its involvement in pathogenic mechanisms or evolution of bronchial asthma and interstitial lung disease.

Exhaled nitric oxide in severe obesity: Effect of weight loss

Exhaled nitric oxide (NO) is a recognized biomarker in the lower respiratory tract. The effect of large variation in body mass on exhaled NO in the same individuals is not well known. The aim of the study was to evaluate both the effect of severe obesity and the influence of weight reduction on exhaled NO. A consecutive series of 24 uncomplicated obese patients (OB), who had laparoscopic adjustable gastric banding (LAGB) and 15 healthy controls (HC) were studied. Body mass index (BMI), exhaled NO and respiratory function tests were assessed. Exhaled NO was lower in obese in comparison to HC (12.0+/-3.6ppb versus 15.8+/-4.0ppb, p=0.0035). A significant positive correlation was found between exhaled NO and BMI in HC, which was not evident in OB. Among the respiratory indexes, functional residual capacity was significantly associated to exhaled NO. After 1 year, 12 obese patients undergone to LAGB were re-evaluated. Mean BMI (kg/m(2)) decreased from 44.8 before surgery to 32.3 post-operatively. The exhaled NO increased from 11.8+/-3.2ppb before surgery to 14.9+/-3.1ppb 1 year post-operatively (p=0.0023, n=12). In conclusion exhaled NO is consistently reduced in severe obesity and it is restored after weight reduction. The relationship between exhaled NO, large body mass excess and decrease of resting lung volume in severe obesity deserves further studies.

Association of Adams–Oliver syndrome with pulmonary arterio-venous malformation in the same family: A further support to the vascular hypothesis

Adams–Oliver syndrome (AOS) is a rare disease characterized by congenital scalp defects, terminal transverse limb defects and cutis marmorata telangiectatica. A significant incidence of cardiac and vascular malformations has been reported, leading to the hypothesis of a vascular defect early involved in the pathogenesis. We report two members of the same family with previously diagnosed AOS based on clinical phenotype and later recognized to have pulmonary arterio‐venous malformation (PAVM). None of the subjects fulfilled current diagnostic criteria of hereditary hemorrhagic telangiectasia, which is the most common cause of PAVM. The occurrence of PAVM in AOS lends support to the hypothesis that endothelial specific abnormalities could be a patho‐physiological mechanism in its development. Therefore, the role of screening for PAVM in clinical management of subjects with AOS should deserve further studies.