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)

Interstitial lung disease encompasses a large number of clinical disorders that affect the epithelium, the endothelium or both cell surfaces of alveolar wall and satellite structures including terminal and respiratory bronchioles. Causative factors are over 200 from bacteria, fungi, viruses, protozoans, to collagen disease, hypersensitivity and inorganic pneumoconiosis. Clinical and histological findings of open lung and transbronchial biopsies from 50 patients are reported, 18 patients were affected by diffuse alveolar damage (DAD), 15 patients by usual interstitial pneumonia (UIP), 8 patients by non specific interstitial pneumonia-fibrosis (NSIP-F), 9 patients by bronchiolitis obliterans organizing pneumonia (BOOP) correlated with conventional chest radiography in 30 patients and with HRCT in 31 patients. Interstitial lung disease other than histiocytosis X share anatomoradiologic features indicative for activity, chronic progression, chronic quiescence, chronic advanced and irreversible disease. In general, the histologic features correlate with radiographic patterns and even if radiologic findings do not always supply definitive diagnosis, some HRCT patterns are highly suggestive and usually classified into 4 categories: normal; with ground glass attenuation; linear, nodular or reticulo-nodular; honeycombing, suggestive for end-stage fibrosis. Correlation of HRCT with histologic findings in 31 patients with idiopathic interstitial fibrosis (IIF) allowed assessment of disease activity, follow-up and therapeutic result. HRCT definitely better than conventional radiology detects the presence, type and extent of parenchymal alterations, differentiating potential reversible lesions (inflammatory) from potentially irreversible (fibrotic) lesions. In IIF, for diagnostic accuracy the specimen of open lung (the gold standard), transbronchial or video-thoracoscopic biopsy must be preoperative, HRCT-assisted and centered on ground glass opacties (or nodules in suspected histiocytosis X) since a diagnostically reliable biopsy correlates with HRCT morphology of histologic specimen. Interstitial lung disease includes benign as well as malignant forms, thus only a multidisciplinary approach can prevent long term hazardous effects as severe cor pulmonale or a fatal outcome. The histologic HRCT-assisted assessment of "active" lesions is crucial for correct careful treatment of these patients.
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PMID:Idiopathic interstitial lung disease: anatomoradiologic pathogenesis. 914 19

Langerhans cell histiocytosis (LCH) is an uncommon disorder affecting primarily young adult smokers. It is characterized by abnormal proliferation of Langerhans cells, specialized monocyte-macrophage lineage antigen-presenting cells. LCH can affect the lungs in isolation or as part of a systemic disease. Most commonly, the disease presents in the third or fourth decade without gender predominance. Symptoms typically include dyspnea and cough. Commonly, physical examination is unremarkable but cor pulmonale may be observed in advanced disease. The chest radiograph is typically abnormal with nodular or interstitial infiltrates and cystic changes. High-resolution computed tomography of the chest with these findings in the middle and upper lobes of an adult smoker is virtually diagnostic of LCH. Pulmonary function assessment is variable. Asthma has rarely been reported in association with this disorder. There are only three reported cases of the diagnosis of concomitant asthma which have been made in association with the diagnosis of LCH. We present a case in which our patient presented with signs and symptoms of asthma to include confirmatory findings of airway hyperresponsiveness. The diagnosis of LCH was established after the patient failed to respond to conventional treatment for asthma, and further evaluation was completed.
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PMID:Langerhans cell histiocytosis presenting as uncontrolled asthma. 2406 95