Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of histiocytosis X (Langerhans type) associated with bullous pulmonary emphysema and acquired immune deficiency, regarding CD4 positive cells, is described. Previous history was remarkable for skin lesions which first appeared in 1981 and progressively worsened, diabetes insipidus diagnosed in 1986, and bullous pulmonary emphysema detected in 1988. Biopsy results of skin lesions were consistent with histiocytosis X. Thyroid gland involvement was found by means of cytological examination. The search for HIV infection (also performed by means of immunoblotting and PCR) was negative. To our knowledge the immunodeficiency detected in histiocytosis X affects the T suppressor lymphocyte subset, so we thought this peculiar case was worth describing.
 ...
PMID:A case of Langerhans histiocytosis with HIV-like immunodeficiency. 135 92

Between 1985 and 1989, 395 patients with primary lung cancer were treated at the Percy military teaching hospital, Paris. Among them were 16 patients (4%) aged between 29 and 40 years (mean: 34 years) and smokers (mean cumulative tobacco consumption 24 P.A.). Six of these (37%) had lung parenchyma dystrophy with bilateral apical bullae of emphysema, 1 had a clean cavity left by a previous lung abscess and 1 had microcytic fibrosis resulting from histiocytosis X of the lungs and bones. In all cases cancer had developed in contact with bullous lesions or sequelae. Cancer was discovered during radiological mass screening in 6 cases (37.5%), on the finding of systemic or thoracic symptoms in 5 cases and because of a distant metastasis in 5 other cases. Pathological examination revealed an adenocarcinoma in 8 out of 16 patients and only one small-cell carcinoma. Nearly two-thirds of the patients had reached an advanced stage: 3 were in stage III A, 1 in stage III B and 6 in stage IV. Eight patients underwent curative surgery (7 lobectomies, 1 pneumonectomy). Survival was known with precision in 14 patients: 8 died after a mean follow-up of 15 months (range: 3 and 31 months); 3 were alive with an active cancer and 4 are still alive in complete remission after curative surgery. Six published studies totalling 387 cases are concordant in demonstrating that primary lung cancer is severe in adults below 40 years of age and that surgery is useful in such cases, even with N2 lymph node involvement.
 ...
PMID:[Primary bronchial cancer in subjects aged 40 or younger]. 166 14

The authors retrospectively evaluated radiographs, computed tomographic (CT) scans, and results of pulmonary function tests (when available) for 17 patients with biopsy-proved pulmonary histiocytosis X. In 11 patients, high-resolution CT was used. In 12 patients, CT demonstrated cystic air spaces, usually less than 10 mm in diameter. In three of these 12, cysts were the only abnormality, but in six others, nodules (usually less than 5 mm in diameter) were also present. Two patients had only nodules and one, only emphysema. CT showed that many lesions that appeared reticular on plain radiographs were actually cysts. CT showed no central or peripheral concentration of lesions, but it did reveal that many small nodules were distributed in the centers of secondary lobules around small airways. CT findings correlated better with the diffusing capacity (rho = -0.71) than did the plain radiographic findings (rho = -0.57). Thus, CT was better than radiography at showing the morphology and distribution of lung abnormalities.
 ...
PMID:Pulmonary histiocytosis X: comparison of radiographic and CT findings. 278 35

A variety of diseases produces or mimics multiple, thin-walled, air-containing cavities or cysts in the lung. Although some causes of this pattern are common (bullous emphysema, multiple pneumatoceles), others are relatively rare (cystic bronchiectasis, histiocytosis X, tracheobronchial papillomatosis, and lucite ball plombage). To some extent, the radiographic features of these lesions allow their differentiation, but differential diagnosis can be difficult. This paper reviews their principal features.
 ...
PMID:Multiple, thin-walled cystic lesions of the lung. 677 84

We describe 12 patients with simultaneous bilateral spontaneous pneumothorax (SBSP). They represent 4 percent of patients with spontaneous pneumothorax seen at our hospital from 1971 to 1990. Five of the 12 had no underlying lung disease. In the seven remaining patients, SBSP was secondary to histiocytosis X, lymphangioleiomyomatosis, osteogenic sarcoma with pleural and pulmonary metastases, Hodgkin's disease, mesothelioma, cystic fibrosis, or miliary tuberculosis. Nineteen of the 56 patients with SBSP (34 percent) described in the literature (this series included) had pulmonary disease related to disorders of cells of mesenchymal origin. Emphysema and bullous lung disease were not associated with SBSP. Long-term prognosis was a function of pulmonary status. Four of the patients described herein died during the period reviewed. All suffered from severe underlying disease. In no case was SBSP the main cause of death. With timely treatment, the short-term prognosis is benign even for patients with underlying lung disease. Surgical pleurectomy should be attempted early, especially in SBSP secondary to underlying lung disease.
 ...
PMID:Simultaneous bilateral spontaneous pneumothorax. 816 40

The authors reviewed the radiographic and computed tomographic (CT) appearances of abnormal air-filled spaces in the lung that develop in response to lung diseases. The major types of these lung diseases include infection, vessel-related or vascular-embolic disorders, bronchiectasis, emphysema, pulmonary fibrosis, adult respiratory distress syndrome and air-block diseases, and unusual disorders of the lung (such as Langerhans cell histiocytosis, Klippel-Trenaunay syndrome, and tracheolaryngeal papillomatosis). After studying the CT scans, conventional radiographs, and medical records of 150 patients with various abnormal air-filled spaces in their lungs and 300 lung specimens and the corresponding high-resolution CT scans, the authors concluded that mechanisms of air-space formation fall into five basic categories: (a) vascular occlusion or ischemic necrosis, (b) dilatation of the bronchi, (c) disruption of the elastic fiber network of the lung, (d) remodeling of the lung architecture and retractile fibrosis, and (e) multifactorial or unknown mechanisms.
 ...
PMID:Abnormal air-filled spaces in the lung. 839 71

The health risks associated with cigarette smoking are well known. Cigarette smoking is the most important causative factor in the development of bronchogenic carcinoma. Pulmonary diseases such as chronic bronchitis, centrilobular and panacinar emphysema, respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), and pulmonary Langerhans cell histiocytosis are also related to cigarette smoking. In adenocarcinoma and squamous cell carcinoma, the most common manifestation at computed tomography (CT) is a solitary pulmonary nodule; in small cell carcinoma, hilar and mediastinal adenopathy secondary to metastases; and in large cell carcinoma, a mass with central necrosis or cavitation in the lung periphery. For chronic bronchitis, the most common CT finding is bronchial wall thickening, but this finding is nonspecific. Emphysema, both centrilobular and panacinar associated with alpha-1-antitrypsin deficiency, usually manifests as areas of decreased attenuation and may involve bullous changes. However, centrilobular emphysema more commonly involves the upper lungs, whereas panacinar emphysema more commonly involves the lower lungs. Most patients with RB-ILD have normal high-resolution CT scans; however, abnormalities may be present, the most common of which are areas of ground-glass attenuation. CT appearance of pulmonary Langerhans cell histiocytosis varies depending on the disease stage: In the early stage, typical CT findings include multiple nodules, usually accompanied by cystic spaces with thin, well-defined walls. As the disease progresses, the cystic spaces become more numerous and the number of nodules decreases.
 ...
PMID:Cigarette smoking: CT and pathologic findings of associated pulmonary diseases. 908 79

In order to determine the presence and distribution of Haemophilus influenzae in lung tissue sections, we obtained lung explants from 49 lung transplant recipients with cystic fibrosis (CF) (n = 16), chronic obstructive pulmonary disease (COPD) including emphysema (n = 16), bronchiectasis (n = 5), pulmonary hypertension (n = 9), Langerhans cell histiocytosis (n = 1), and idiopathic pulmonary fibrosis (n = 2). Analysis was done by selective culturing, immunoperoxidase (IP) staining, and by polymerase chain reaction (PCR). H. influenzae was cultured from specimens of the lung explants from one CF and one COPD patient. IP staining of tissue sections was positive in 24 patients (10 CF patients, eight COPD patients, two bronchiectasis patients, and four patients with noninfectious pulmonary diseases). IP-positive tissue sections were PCR-positive, and IP-negative sections were PCR-negative. H. influenzae was more frequently detected in tissue sections of lung explants from CF and COPD patients than from patients with bronchiectasis or noninfectious pulmonary diseases. H. influenzae was diffusely present in the epithelium, the submucosa of the bronchi, the bronchioles, the interstitium, and the alveolar epithelium. H. influenzae was localized extracellularly alone and in bacterial clusters, and was also associated with macrophages in CF patients. The results of this study demonstrate that H. influenzae is often present in the lungs of patients with end-stage pulmonary disease, especially CF and COPD patients. H. influenzae is diffusely present in the respiratory epithelium and subepithelial layers of the lungs of these patients.
 ...
PMID:Haemophilus influenzae in lung explants of patients with end-stage pulmonary disease. 951 16

To estimate the number of patients with intractable respiratory diseases, we conducted a two-stage nationwide epidemiological survey in 1997. The first survey was performed at randomly sampled hospitals to identify the number of patients treated. The second survey sought detailed clinico-epidemiological data on the patients reported in the first survey. The response rates were 54% for the first survey and 62% for the second. Based on the survey findings, we derived the following nationwide estimates: 450 patients (95% confidence interval: 360-530) with chronic thromboembolic pulmonary hypertension; 230 (200-260) with primary pulmonary hypertension; 180 (150-210) with obesity-associated hypoventilation syndrome; 40 (30-50) with primary alveolar hypoventilation syndrome; 160 (140-180) with histiocytosis X; and 190 (150-230) with juvenile pulmonary emphysema.
 ...
PMID:[Estimated numbers of patients with intractable respiratory diseases]. 1006 52

Histiocytosis X (HX), also referred as Langerhans cell granulomatosis is a disorder characterized by the presence of destructive granulomas containing Langerhans cells, lymphocytes, eosinophils and fibroblastes in the involved organs. Three presentations are commonly observed: 1) nonproductive cough or effort dyspnea, 2) spontaneous pneumothorax 3) incidental pulmonary infiltrates on chest X-ray in asymptomatic patients. HRCT may be helpful in the initial diagnosis of pulmonary HX. HRCT scans show nodules, cysts and estimate the extent of disease. But the final diagnosis of histiocytosis X requires the histologic demonstration of specific histiocytosis X cell in biopsy specimens of the lung. The aim of this study was to define the importance of the detection of Langerhans cells in bronchoalveolar lavage fluid (BALF) for the diagnosis of HX. The searched cells express a specific CD1 antigen, recognized by the monoclonal antibody OKT-6. In our study the demonstration of more than 5% of CD1 positive cells was defined to confirm HX. We have studied the BALF in 21 patients with suspected histiocytosis X. In BALF of 4 patients more than 5% of CD1 positive cells were found. In 1 of them HX was confirmed with open lung biopsy. Two patients displayed 5% of CD1 positive cells. The final diagnosis of the first patient was hypersensitivity pneumonitis and of the second one was bronchitis chronica. In 5 patients out of 15 patients in whom less than 5% of CD1 positive cells were found histiocytosis X was histologically proven. In other 10 patients the following disorders were histologically recognised: pulmonary emphysema 3 cases, pneumoconiosis-3, LMA-BOOP-1, sarcoidosis-1 and pleuritis eosinophilica-1. The estimation of Langerhans cells in BALF can be a useful method among the diagnostic procedures for histiocytosis X. It is necessary to remember that demonstration of less than 5% of CD1 positive cells do not exclude histiocytosis X.
 ...
PMID:[Usefulness of CD1 expression on surfaces of cells in bronchoalveolar fluid for diagnosis of histiocytosis X--our experience]. 1064 82


1 2 3 4 5 Next >>