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)

Over the past 19 years, 278 patients (238 men-40 women) have undergone 325 parietal pleurectomies: 311 transaxillary apical and 14 full pleurectomies. Mean age was 32 +/- 12 years. The overall male:female ratio was 6:1. Surgical treatment was performed for either recurrence of spontaneous pneumothorax or failure to respond adequately to management by tube drainage. Thirty-six patients underwent one stage bilateral apical pleurectomy. On the basis of our experience (idiopathic pneumothorax: 311; chronic obstructive lung disease: 12; histiocytosis X: 2), we advocate the use of transaxillary apical pleurectomy for the younger age group with recurrent idiopathic pneumothorax and a full pleurectomy in the older age group. Major complications occurred in 1.9% of cases (re-exploration for haemorrhage: 5 cases; for air leak: 1 case). The recurrence rate in this group of patients was 1% (3 in 278 patients: one of them has been reoperated).
 ...
PMID:[Spontaneous and recurrent pneumothorax. Surgical treatment. Apropos of 278 cases]. 160 40

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

Diminished exercise capacity in advanced pulmonary histiocytosis X does not appear to be related to ventilatory limitation but may be related to pulmonary vascular dysfunction. Pulmonary hemodynamics and respiratory function were studied in 21 consecutive patients with advanced pulmonary histiocytosis X, and compared with parameters of patients with other severe chronic lung diseases (29 patients with chronic obstructive pulmonary disease and 14 patients with idiopathic pulmonary fibrosis). All patients with pulmonary histiocytosis X displayed severe pulmonary hypertension: mean pulmonary arterial pressure, 59 +/- 4 mm Hg; cardiac index, 2.6 +/- 0.8 L/min/m(2); and total vascular pulmonary resistance, 25 +/- 3 IU/m(2) (p < 0.05, as compared with patients with other chronic lung diseases). Pa(O(2)) was similar in the three groups, whereas FEV(1) was lower in patients with other chronic lung diseases (p < 0.05). In contrast to other chronic lung diseases, the degree of pulmonary hypertension was not related to variables of pulmonary function in pulmonary histiocytosis X. Histopathology was available for 12 patients with pulmonary histiocytosis X and revealed proliferative vasculopathy involving muscular arteries and veins, with prominent venular involvement. Two consecutive lung samples (taken before and after the occurrence of pulmonary hypertension) were available for six patients with pulmonary histiocytosis X, and showed that pulmonary vasculopathy worsened, whereas parenchymal and bronchiolar lesions remained relatively unchanged. These results indicate that pulmonary hypertension in pulmonary histiocytosis X might be related to an intrinsic pulmonary vascular disease, in which the pulmonary circulation is involved independent of small airway and lung parenchyma injury.
 ...
PMID:Severe pulmonary hypertension in histiocytosis X. 1061 23

Bronchiolar abnormalities are relatively common and occur in a variety of clinical settings. Various histopathologic patterns of bronchiolar injury have been described and have led to confusing nomenclature with redundant and overlapping terms. Some histopathologic patterns of bronchiolar disease may be relatively unique to a specific clinical context but others are nonspecific with respect to either etiology or pathogenesis. Herein, we present a scheme separating (1) those disorders in which the bronchiolar disease is the predominant abnormality (primary bronchiolar disorders) from (2) parenchymal disorders with prominent bronchiolar involvement and (3) bronchiolar involvement in large airway diseases. Primary bronchiolar disorders include constrictive bronchiolitis (obliterative bronchiolitis, bronchiolitis obliterans), acute bronchiolitis, diffuse panbronchiolitis, respiratory bronchiolitis, mineral dust airway disease, follicular bronchiolitis, and a few other rare variants. Prominent bronchiolar involvement may be seen in several interstitial lung diseases, including hypersensitivity pneumonitis, respiratory bronchiolitis-associated interstitial lung disease, cryptogenic organizing pneumonia (idiopathic bronchiolitis obliterans organizing pneumonia), and pulmonary Langerhans' cell histiocytosis. Large airway diseases that commonly involve bronchioles include bronchiectasis, asthma, and chronic obstructive pulmonary disease. The clinical relevance of a bronchiolar lesion is best determined by identifying the underlying histopathologic pattern and assessing the correlative clinico-physiologic-radiologic context.
 ...
PMID:Bronchiolar disorders. 1464 23

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.
 ...
PMID:Difficulties in the diagnosis of rare immunological diseases manifesting with cystic lung disease and spontaneous pneumothorax: Case reports. 1498 35

Cigarette smoking is a common cause of lung disease. It is clearly implicated in the development of chronic obstructive pulmonary disease and lung cancer. Importantly, cigarette smoking has also been implicated in the development of interstitial lung diseases such as respiratory bronchiolitis interstitial lung disease, desquamative interstitial pneumonia, pulmonary Langerhans' cell histiocytosis,and idiopathic pulmonary fibrosis. The exact role of cigarette smoking in the development of interstitial lung diseases is still being defined; the relatively low prevalence of interstitial lung disease makes epidemiologic studies difficult.
 ...
PMID:Cigarette smoking in interstitial lung disease: concepts for the internist. 1546 18

Simultaneous bilateral spontaneous pneumothorax (SBSP) is a very rare condition, mainly seen in patients with underlying lung disease. Up to now, there are 65 patients who have been published. Twelve consecutive patients who presented with SBSP as definitive diagnosis were recruited for this study. They represented 1 % of all patients with spontaneous pneumothorax. All patients had immediate bilateral chest tubes on admission. Five of the 12 patients (42%) had no underlying lung disease. In 7 patients, SBSP was secondary to pulmonary metastases, histiocytosis X, undefined interstitial pulmonary disease, tuberculosis, pneumonia and chronic obstructive pulmonary disease. None of the patients died during hospitalization. Eleven patients were treated with chemical pleurodesis, whereas thoracotomy and pleurectomy were necessary in 7 patients. Reexpansion of the lungs was achieved in all patients. Immediate bilateral chest tube insertion and pleurodesis are of major importance in the treatment of SBSP although a subset of patients needed surgical pleurectomy. Combination of these treatments provides successful and uneventful treatment of the disease.
 ...
PMID:Simultaneous bilateral spontaneous pneumothorax report of 12 cases and review of the literature. 1557 Oct 26

The tobacco smoke intake by the smoker or by the non-smoker is a toxic product and a risk factor for diseases as acne in teenagers, respiratory and non respiratory infection, or histiocytosis X. The four main diseases are: chronic obstructive pulmonary disease, which is more 80% related to tobacco; cancer (lung carcinoma, pharyngeal, laryngeal and oesophageal carcinoma, kidney, bladder carcinoma); cardiovascular diseases (myocardial infarction, cerebral stoke and sudden cardiac death); adverse consequences of smoking related to pregnancy.
 ...
PMID:[Tobacco-related diseases]. 1565 11

Pulmonary diseases associated with tobacco smoking are a complex group of disorders ranging from chronic obstructive pulmonary disease (COPD) to lung cancer. Interstitial lung diseases (ILDs) have only recently been linked to smoking. The ILDs related to smoking include respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and pulmonary Langerhans cell histiocytosis. The relationship of smoking with each of these entities has been largely established on the weight of epidemiologic evidence. Although they have been retained as distinct and separate conditions in various classifications of interstitial lung diseases, these 3 entities share a number of clinical, radiologic, and pathologic features suggesting that they represent a spectrum of patterns of interstitial lung disease occurring in predisposed individuals who smoke. Evaluation of histologic features, particularly in surgical lung biopsy samples, is important in making the distinction between these disorders. However, even after tissue biopsy, it may sometimes be difficult to clearly separate these entities. The importance of making the distinction between them lies in the different clinical management strategies used. Further experimental evidence, including genetic information, may be important in improving our understanding of these diseases.
 ...
PMID:Smoking-related interstitial lung disease. 1899 11

Early-onset chronic obstructive pulmonary disease (COPD) is designated as onset under age 50. We report a case of early-onset COPD with recurrent pneumothorax. A 29-year-old woman visited our hospital with productive cough and dyspnea on exertion. CT scan of the chest demonstrated severe panlobular emphysema. A pulmonary function test showed a reduction in FEV1.0 (41% of the predicted value). A diagnosis of severe COPD was made. Her symptoms and pulmonary function improved after the treatment of inhaled corticosteroid, long-acting beta2-agonist, and anti-cholinergic drugs. She had pneumothorax at least 8 times in the right lung. The level of alpha1-antitrypsin was normal. On the basis of the characteristics of the appearance of the chest X-ray and CT scan, the possibility of bronchiolitis obliterans, lymphoangioleiomyomatosis or Langerhans cell histiocytosis was thought to be low. We considered that several factors, such as high susceptibility, pulmonary infection during her childhood, bronchial asthma, malnutrition, smoking history from an early age, and long-term passive exposure to cigarette smoke may have contributed to the development of early-onset COPD in the present case.
 ...
PMID:[A case of early-onset COPD with recurrent pneumothorax]. 1926 May 33


1 2 3 Next >>