ABSTRACT
Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of smooth muscle proliferation that generally leads to death, due to respiratory failure, within 10 yrs. The disease almost exclusively affects women of child-bearing age and rarely in postmenopausal years. High resolution computed tomographic (CT) patterns are characteristic and almost pathognomonic for LAM. Attempts to treat this disease with hormonal therapy have shown that a number of hormonal manipulations may not be helpful, but that surgical or radiant oophorectomy alone is the most effective treatment, even if the disease is so rare as to preclude randomized trials. Castration, as a means of early therapy, has been associated with a stable respiratory picture in our patients.
Subject(s)
Lymphangioleiomyomatosis/diagnosis , Adult , Female , Humans , Lymphangioleiomyomatosis/therapy , Middle AgedABSTRACT
In the present study the response of the pulmonary circulation to a vasodilator agent (nifedipine) and to low-flow oxygen (FiO2 35%) has been studied both at rest and during exercise (steady state, 25 W) in nine patients with severe scoliosis (spinal curve + 100 degrees, FVC 38%). All patients were dyspneic on exertion and had had at least one episode of right heart failure. The mean pulmonary artery pressure (Pap) ranged from 13 to 37 mmHg at rest and ranged from 30 to 75 mmHg during exercise. Standard exercise, performed 60 min after 20 mg sublingual nifedipine, was associated with a reduction in mean Pap (49 mmHg) as compared with exercise performed during the control period (58 mmHg, p less than 0.001). Pulmonary vascular resistance (PVR) on exercise decreased more on nifedipine (28.9%) than on oxygen (13.9%) (p less than 0.05). Our results suggest that nifedipine improves pulmonary haemodynamics in patients with severe scoliosis complicated by cor pulmonale.
Subject(s)
Nifedipine/pharmacology , Oxygen/pharmacology , Pulmonary Circulation/drug effects , Scoliosis/physiopathology , Adult , Aged , Female , Hemodynamics/drug effects , Humans , Male , Middle Aged , Physical Exertion/drug effects , Pulmonary Heart Disease/drug therapy , Pulmonary Heart Disease/etiology , Scoliosis/complicationsABSTRACT
A comparison between the radiographical findings and the haemodynamic data was made in 53 cases of chronic bronchial obstruction. The results were used in an assessment of the reliability of standard chest X-rays in the diagnosis of pulmonary hypertension. Reference is also made to quantitative indices. Several cases are presented by way of example.
Subject(s)
Bronchial Diseases/diagnostic imaging , Heart/physiopathology , Hypertension, Pulmonary/diagnostic imaging , Pulmonary Circulation , Adult , Aged , Airway Obstruction/complications , Airway Obstruction/diagnostic imaging , Airway Obstruction/physiopathology , Bronchial Diseases/complications , Bronchial Diseases/physiopathology , Female , Humans , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/physiopathology , Lung/diagnostic imaging , Male , Middle Aged , Pulmonary Emphysema/physiopathology , Pulmonary Heart Disease/physiopathology , RadiographyABSTRACT
This study evaluates the usefulness of chest x-ray in the diagnosis of pulmonary arterial hypertension. Twelve patients with severe interstitial fibrosis were studied by respiratory functional tests and right heart catheterization. A quantitative radiologic diagnosis of pulmonary arterial hypertension in pulmonary fibrosis results more difficult than in chronic obstructive airway disease, mitral stenosis or pulmonary thromboembolism. Nevertheless in case of severe interstitial fibrosis pulmonary hypertension was regularly present even if specific radiologic findings were not available. We have found some interesting correlations, i.e. a trend to a relationship between mean pulmonary pressure and right descending pulmonary artery diameter.
Subject(s)
Hypertension, Pulmonary/diagnostic imaging , Pulmonary Fibrosis/complications , Adult , Aged , Female , Hemodynamics , Humans , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/physiopathology , Male , Middle Aged , Pulmonary Circulation , Pulmonary Fibrosis/diagnosis , Pulmonary Fibrosis/diagnostic imaging , Radiography , Respiratory Function TestsABSTRACT
Lymphangioleiomyomatosis (LAM) is an uncommon disease affecting women of reproductive capacity. It is characterized by non-neoplastic proliferation of smooth muscle in the lungs and, occasionally, in lymph nodes and in the thoracic duct. The patients present with dyspnea, chylous pleural effusion, pnx, and hemoptysis. The authors evaluated 4 patients with biopsy-proven LAM, by means of CT. In all cases, CT revealed small well-defined cystic air spaces with smooth and thin walls, scattered in the lungs. CT was very useful because it was more sensitive and specific than conventional radiography. The CT appearance of LAM distinctly differed from that of other diseases than can produce cystic air spaces in the lungs, such as UIP, eosinophilic granuloma, centri-acinar emphysema, and bronchiectasis. Our experience confirms the clinical usefulness of CT in diagnosing LAM.
Subject(s)
Lung Neoplasms/diagnostic imaging , Lymphangiomyoma/diagnostic imaging , Tomography, X-Ray Computed , Adult , Biopsy , Diagnosis, Differential , Female , Humans , Lung Neoplasms/pathology , Lymphangiomyoma/pathology , Middle AgedABSTRACT
We report two cases of pulmonary involvement in ulcerative colitis. The first patient, a 37-year-old woman, had bilateral basal bronchiectasis full of mucopurulent secretion, with a marked improvement of pulmonary function and roentgenographic appearance after a conservative approach. The second patient had severe pulmonary fibrosis of autoimmune nature and died owing to a pulmonary infection. In the second patient, a sulfasalazine reaction as an etiologic factor was excluded, while in the first this possibility seemed unlikely. Therefore, we take these two cases as examples of the extraintestinal manifestations of ulcerative colitis.