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)

Needle biopsy was performed in 100 children with diffuse interstitial pneumopathy. Severe histological lesions were noted in the biopsy sections in 4 out 5 cases. Those most frequently observed (57 cases) were non-specific lesions of diffuse interstitial pneumopathy (DIP) or idiopathic fibrosis, the course of which was followed, in some cases, by repeating the biopsy. Specific lesions, especially idiopathic pulmonary hemosiderosis (8 cases), were noted in 22 children, either associated with interstitial fibrosis or apparently better tolerated. The other 16 cases included the lipoidoses (Niemann-Pick, histiocytosis X), alveolar microlithiasis, and some specific inflammatory diseases (tuberculosis, syphilis) or those related to inhalation of a foreign body.
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PMID:[Results of lung biopsy in interstitial pneumopathies in children. A report on 100 cases (author's transl)]. 54 6

The accumulated experience and the literature data allow for the separation of a group of granulomatous pulmonary diseases among pulmonary diseases of different etiology. Etiologically heterogeneous granulomatous diseases are united by the general signs: granuloma development, immunologic disturbances mainly within the cellular system and mediators as well as systemic vascular affection in the form of vasculitis. In our opinion, granulomatous diseases include disseminated tuberculosis, sarcoidosis of respiratory organs, exogenic and idiopathic fibrous alveolitis, Wegener's granulomatosis, histiocytosis X, primary hemosiderosis, Goodpasture's syndrome and some other rare diseases. Granulomatous diseases are diagnosed on the basis of the ++clinico-roentgenologic findings with an obligatory cytological and histological study of the bioptic specimen; immunologic diseases are diagnosed proceeding from the study of the immunologic status and detection of specific antibodies; of great significance for the diagnosis of pulmonary granuloma caused by infectious pathogens are microbiologic studies which provide for the detection of microorganisms and fungi. Study of the clinicoroentgenologic and laboratory data made it possible to distinguish a number of features typical for each disease and to unite them into diagnostic symptom complexes. Despite the different course of granulomatous pulmonary diseases they may end in recovery and granuloma resolution, development of lung fibrosis in a chronic course and in certain diseases in lung tissue destruction with cavity formation (tuberculosis, Wegener's granulomatosis). A fatal outcome may also ensue due to an acute or chronic course of the diseases.
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PMID:[Diagnosis of granulomatous lung diseases]. 187 Oct 95

Fifty eight patients with disseminated lung lesions were investigated, including 19 with histiocytosis X, 5 with alveolar microlithiasis, 8 with pulmonary phospholipoproteinosis, 16 with idiopathic hemosiderosis, 1 with primary pulmonary amyloidosis and 9 with primary diffusive tumors of the lungs. In all the above cases, a complex bronchological examination was undertaken with the use of different biopsy transbronchial procedures as well as an open biopsy of a lung. Diagnostic value and efficiency of bronchobiopsies were considered. Recommendations concerning the use of different instrumental techniques were formulated.
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PMID:[Possibilities of instrumental biopsy methods in the diagnosis of rarely occurring disseminated processes of the lungs]. 233 37

A study is presented of 52 patients with disseminated pulmonary lesions. Among them: histiocytosis X--17, alveolar microlithiasis--5, pulmonary phospholipoproteinosis--7, idiopathic hemosiderosis--14, primary pulmonary amyloidosis--1, primary diffuse lung tumours--8. All patients were subjected to complex bronchological examination including use of different biopsy transbronchial methods as well as open biopsy of the lung. The diagnostic value of the employed bronchial biopsies is discussed. A diagnostic algorithm of rarely met pulmonary disseminations has been developed. Recommendations on the use of different instrumental methods are formulated.
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PMID:[The instrumental diagnosis of disseminated lesions of the lungs]. 260 83

Worldwide almost no epidemiologic data are available on the prevalence or incidence of interstitial lung diseases (ILD) in the general population. Therefore, a registration programme of ILD-prevalence was organised by the VRGT (Vereniging voor Respiratoire Gezondheidszorg en Tuberculosebestrijding), among about 100 Flemish pneumologists since 1990. Most categories of the classification by Crystal et al. (1) were included and the diagnostic criteria (histology, laboratory tests, clinic, radiology) were registered. The present paper presents the results of 1992-1994: twenty pneumologists had forwarded the summary files of 237 patients to the central office in 1992 (n = 68), 1993 (n = 90) and 1994 (n = 79). The diagnoses that were most frequently made were: sarcoidosis in 27%, idiopathic pulmonary fibrosis in 20%, hypersensitivity pneumonitis in 14% (of which 68% by birds) and collagen-vascular disease in 10% (of which 54% in rheumatoid arthritis). Less frequent causes were eosinophilic pneumonia (4%), inhalation of inorganic material (4%, anthracosilicosis being excluded), histiocytosis X (3%), drugs (3%), angiitis and granulomatosis (2%), pulmonary hemosiderosis (1%), lymphocytic infiltrative lung disease (1%) and lymphangioleiomyomatosis (1%). The order of relative frequencies of the different categories of diseases was the same in the 3 registration years. In 9% of the patients the diagnosis was confined to "undefined fibrosis". The diagnosis was confirmed by histology in 63% of the cases. The overall male-female ratio was nearly one, with, however, a male preponderance in hypersensitivity pneumonitis (22/12), UIP(8/3) and "undefined fibrosis" (14/7).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Epidemiology of interstitial lung disease (ILD) in flanders: registration by pneumologists in 1992-1994. Working group on ILD, VRGT. Vereniging voor Respiratoire Gezondheidszorg en Tuberculosebestrijding. 853 25