1/7. Spiral computerized tomography and video thoracoscopy in catamenial pneumothorax.Catamenial pneumothorax is a rare disease that is usually diagnosed on clinical grounds. Delay in diagnosis can lead to considerable morbidity. We describe a case in which spiral CT scan and videothoracoscopy led to early objective diagnosis and management.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/7. pulmonary fibrosis with intractable pneumothorax: new pulmonary manifestation of relapsing polychondritis.Relapsing Polychondritis is a rare disease which causes the repetitive inflammation of cartilage and connective tissues. Although the large airway is sometimes involved and the stenosis of them often influences the prognosis of the patients, there have been few reports concerning the manifestation of the peripheral lung. A 60-year-old man with pulmonary fibrosis was admitted to a regional hospital due to sudden deafness, and then he suffered from relapsing polychondritis. During the steroid therapy, he also suffered from bilateral pneumothoraces. His computed tomogram revealed many bilateral bullae, emphysematous changes, and fibrotic changes in bilateral lungs. The mechanism of generating peripheral pulmonary manifestations is also discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/7. Familial clustering of dendriform pulmonary ossification.Dendriform pulmonary ossification is an extremely rare disease, which is usually found and analysed postmortem. Pathogenesis of pulmonary ossification is therefore still unknown. We describe two males in the same family (a 29 year-old patients and his 58 year-old father) with pulmonary ossification. The young male was symptomatic pneumothorax but his father had been asymptomatic since youth. Familial clustering of pulmonary ossifications strongly suggests a genetic predisposition for the pathogenesis. This is the first report of two cases of dendriform pulmonary ossification in the same family.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/7. Difficulties in the diagnosis of rare immunological diseases manifesting with cystic lung disease and spontaneous pneumothorax: case reports.Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of an unknown cause affecting women of reproductive age and characterised by smooth muscle proliferation along lung lymphatic channels. pneumothorax develops in up to 80% of patients with LAM and may be the presenting manifestation of the disease. pneumothorax also precedes or complicates the clinical course of 25% of patients with Langerhans'-cell histiocytosis (LCH, histiocytosis X) pathologically characterised by involved tissue infiltration with large numbers of unusual Langerhans' cells, often organised as granulomas. A 41-year-old female patient was treated twice by simple tube drainage due to left pneumothorax in 1996. She was then diagnosed with chronic obstructive lung disease demonstrating with dyspnea, cough and wheeze. Abnormalities found in the high-resolution computed tomography (HRCT) scanning were characteristic of LAM with thin-walled parenchymal cysts distributed homogeneously in both lungs and with thickening of interlobular septa. A 38-year-old man was hospitalised due to chronic lung failure in the course of LCH characterised by small, poorly limited nodular lesions and thin-walled cysts revealed in HRCT scans. For two years of observation, he has suffered five episodes of right pneumothorax. When diagnosing pneumothorax in the middle-age patients, the possible cause of the disease is LAM or LCH. The use of HRCT scanning may enable good determination of the nature and distribution of parenchymal abnormalities found in the diseases.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/7. Hyperimmunoglobulinemia E syndrome: pulmonary imaging considerations.The Hyper IgE syndrome is a rare disease consisting of recurrent sinusitis and pneumonia, pneumatoceles, chronic dermatitis, and elevated serum levels of IgE. The primary radiographic abnormalities are recurrent alveolar lung disease and pneumatoceles. pneumothorax may occasionally occur as in one of our cases. Other causes of pneumatoceles are usually easily excluded by the history and other clinical data. Pulmonary scintigraphy and computed tomography may add information valuable to the management of these patients.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/7. tuberous sclerosis and associated pleuropulmonary lesions.tuberous sclerosis (TS) is a rare disease of the nervous system, being characterized by seizures, mental retardation and adenoma sebaceum. Concomitant pleuropulmonary lesions and spontaneous pneumothorax are extremely rare during the evolution of this disease. To date, only 19 cases of TS and spontaneous pneumothorax have been described in the literature. Here we present a case of TS and associated pleuropulmonary lesions with spontaneous pneumothorax in a 29-year-old female patient. Clinical, roentgenographic and histological aspects of this disease are commented upon.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/7. Pulmonary lymphangiomyomatosis.Pulmonary lymphangiomyomatosis is a rare disease of smooth muscle proliferation in the walls of lymphatic vessels and in the interstitial areas of the lungs. It only affects women of reproductive age. The majority of patients die from respiratory failure within 10 years. A new observation of pulmonary lymphangiomyomatosis is reported in a woman of 36 years. The disease was discovered at an advanced stage of respiratory failure. Left-sided pleurectomy and treatment with medroxy-progesterone and tamoxifen did not improve the patient's condition.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |