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

Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) designates interstitial lung changes in smokers, characterized histologically by bronchiolocentric accumulation of pigmented alveolar macrophages and fibrotic or cellular inflammatory changes of pulmonary interstitium. The definition is nearly identical to that of condensate pneumopathy, smoker's pneumopathy or smoker's lung, defined by accumulation of pigmented alveolar macrophages with bland alveoloseptal or peribronchial fibrosis and cellular inflammation of the bronchial wall. In addition to respiratory bronchiolitis, which is found in nearly all smokers, RB-ILD comprises a broad spectrum of varying degrees of the interstitial reaction to the exogenous injury of inhalation smoking with gradual transition to desquamative interstitial pneumonia (DIP). In most cases RB-ILD manifestations are subclinical and detected coincidentally. Radiographic features are reticulonodular and ground glass opacities of the lung. The high resolution computed tomography reveals centrilobular nodules, ground glass opacities, thickening of bronchial walls, and in some cases a reticular pattern. Mild emphysema is frequent. Lung function analysis reveals only minor restrictive or obstructive defects in most cases, often combined with hyperinflation. CO diffusing capacity is slightly to moderately impaired. Pronounced interstitial lung diseases with serious restrictive defects and arterial hypoxemia have been reported infrequently. In differential diagnosis smoking related interstitial lung diseases (DIP, Langerhans cell histiocytosis, idiopathic pulmonary fibrosis) and other interstitial lung diseases have to be excluded. In most cases diagnosis can be achieved by bronchoalveolar lavage and transbronchial lung biopsy. In cases of pronounced interstitial lung disease or assumption of an additional interstitial lung disease besides RB-ILD a thoracoscopic or open lung biopsy can be necessary. RB-ILD has a favourable prognosis. After smoking cessation lung changes are reversible. Corticosteroid therapy is not necessary. A fatal outcome of RB-ILD has not been reported. Follow-up examinations are advisable in order to preclude other interstitial lung diseases. RB-ILD seems to be more frequent than it is assumed at present. The clinical picture is masked in most cases by the concomitant smoking induced chronic bronchitis. Thus only pronounced cases with structural changes and resulting differential diagnostic problems are diagnosed.
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PMID:[Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)]. 1278 81

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.
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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.
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PMID:Simultaneous bilateral spontaneous pneumothorax report of 12 cases and review of the literature. 1557 Oct 26

A cystic lesion in the lung is defined as a well-demarcated epithel-lined cavity, that can be mostly filled with air, water, as well as solid material content. This definition includes a wide variety of diseases such as bronchogenic cyst, abscess formation, lymphangioleiomyomatosis, Langerhans cell histiocytosis, emphysema, bronchiectasis, and pneumatoceles. Despite the difficulties in differential diagnosis, there are some diagnostic criteria for CT-scanning helping the radiologist to differentiate between these cystic entities. Moreover, clinical informations are extremely important. The most important clinical parameters include age, sex, clinical history and symptoms. Thus, a better understanding of classic CT appearance of cystic lung disease will allow more definitive diagnosis and could, in some cases, avoid biopsy.
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PMID:[Computed tomography of cystic lung diseases]. 1558 57

Erdheim-Chester disease is a disseminated non-Langerhans' cell histiocytosis involving multiple organs with characteristic sclerotic musculoskeletal lesions. This is the report of the case of a 53-year-old woman with extensive and progressive pulmonary disease. Computed tomography scans revealed diffuse infiltrative lung disease. Thoracoscopic lung biopsy and a biopsy of the right femur lesion were performed. The histopathology revealed that she had non-Langerhans' cell histiocytosis; Erdheim-Chester disease. The characteristic lesions of Erdheim-Chester disease, including involvement of the orbit, pericardium, periaorta, and bone were detected. This helped to further confirm that the patient had Erdheim-Chester disease with associated pulmonary involvement. As Erdheim-Chester disease is a rare non-Langerhans' cell histiocytosis that may be misdiagnosed as interstitial lung disease or other pulmonary disorders, this diagnosis should be considered in the differential diagnosis of such lung lesions.
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PMID:Pulmonary involvement in Erdheim-Chester disease. 1595 55

Response by the lung to inhaled cigarette smoke varies widely between individuals and ranges from subclinical respiratory bronchiolitis to end-stage Langerhans' cell histiocytosis. The variety of interstitial lung diseases associated with cigarette smoking is wider than generally appreciated, and these often coexist. An understanding of the pathological basis of smoking-related lung disease helps to explain the sometimes complex patterns encountered on high-resolution computed tomography (HRCT). The recent refinement of the classification of the idiopathic interstitial pneumonias and the longitudinal study of cigarette smokers with HRCT has furthered understanding of what should be regarded as an overlapping group of entities. In this article the individual interstitial diseases ascribable to cigarette smoking are discussed in terms of their pathology and corresponding HRCT signs. Emphasis is placed on the frequent coexistence of various forms of smoking-related interstitial lung disease.
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PMID:Smoking-related diffuse parenchymal lung disease: HRCT-pathologic correlation. 1608 59

Pulmonary Langerhans' cell histiocytosis (PLCH) remains an important diagnostic consideration in the differential diagnosis of diffuse infiltrative lung disease, particular among smokers. This review highlights recent progress in our understanding of the etiology, clinical presentation, and diagnostic and therapeutic approaches to this unusual lung disorder. Evolving studies strongly link the basic pathogenesis of PLCH as an uncommon reaction to tobacco smoke. Recent progress in the clinical approach to these patients has emphasized important roles for high-resolution computed tomographic (CT) scanning and immune reactive tissue markers including cluster differentiation (CD) 1a antigen in the diagnosis of this disorder. A rationale diagnostic algorithm and current management strategies are summarized.
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PMID:Pulmonary Langerhans' cell histiocytosis. 1608 2

Adult pulmonary Langerhans cell histiocytosis (APLCH) is a rare lung disease. We report a case of APLCH and pregnancy with detailed lung function tests. There was no significant change in the lung function tests during pregnancy. It is apparent that pregnancy does not influence the evolution of pulmonary Langerhans cell histiocytosis.
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PMID:Effect of pregnancy on lung function in adult pulmonary Langerhans cell histiocytosis. 1649 95

Emphysema is defined as a condition of the lung characterized by abnormal, permanent enlargement of airspaces distal to the terminal bronchiole accompanied by destruction of the alveolar walls and without obvious fibrosis. It is a very common disease with high morbidity and mortality. Histopathologically, there are two types of emphysema: panlobular emphysema, typically occurring in alpha1-antitrypsin deficiency, and centrilobular emphysema, which is strongly associated with cigarette smoking. Computed tomography (CT) allows detection of emphysema with higher sensitivity than conventional chest radiography and pulmonary function tests. CT also allows quantification of emphysema and depicts associated changes and complications. The differential diagnosis of emphysema, which is characterized by the absence of clearly definable walls on CT, includes cystic lung disease, bullae, lung laceration, Langerhans cell histiocytosis, and lymphangioleiomyomatosis -which are all characterized by visible walls on CT.
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PMID:[Imaging of emphysema]. 1722 84

Pulmonary hypertension (PH) in patients with interstitial lung diseases (ILDs) is not well recognized and can occur in the absence of advanced pulmonary dysfunction or hypoxemia. To address this topic, we identified relevant studies in the English language by searching the MEDLINE database (1966 to November 2006) and by individually reviewing the references of identified articles. Connective tissue disease-related ILD, sarcoidosis, idiopathic pulmonary fibrosis, and pulmonary Langerhans cell histiocytosis are the ILDs most commonly associated with PH. Pulmonary hypertension is an underrecognized complication in patients with ILDs and can adversely affect symptoms, functional capacity, and survival. Pulmonary hypertension can arise in patients with ILDs through various mechanisms, Including pulmonary vasoconstriction and vascular remodeling, vascular destruction associated with progressive parenchymal fibrosis, vascular inflammation, perivascular fibrosis, and thrombotic angiopathy. Diagnosis of PH in these patients requires a high index of suspicion because the clinical presentation tends to be nonspecific, particularly in the presence of an underlying parenchymal lung disease. Doppler echocardiography is an essential tool in the evaluation of suspected PH and allows ready recognition of cardiac causes. Right heart catheterization is needed to confirm the presence of PH, assess its severity, and guide therapy. Management of PH in patients with ILDs is guided by identification of the underlying mechanism and the clinical context. An increasing number of available pharmacologic agents in the treatment of PH allow possible treatment of PH in some patients with ILDs. Whether specific treatment of PH in these patients favorably alters functional capacity or outcome needs to be determined.
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PMID:Pulmonary hypertension in patients with interstitial lung diseases. 1735 70


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