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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Spontaneous pneumothorax (SP) is commonly encountered by general surgeons and is usually caused by rupture of subpleural blebs. Although uncommon, SP may be due to primary pulmonary histiocytosis X, a disease characterized by eosinophilic and histiocytic infiltration of lung tissue. It primarily affects young smokers and usually presents with symptoms of cough, dyspnea, and chest pain. A young adult male with this disease presented with recurrent SP and was successfully treated with thoracoscopic stapling of bullae and pleural abrasion.
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PMID:Recurrent spontaneous pneumothorax in pulmonary histiocytosis X. 979 65

Pulmonary Langerhans' cell histiocytosis (LCH) represents an uncommon clinical disorder with unpredictable clinical presentation and outcome. Lung involvement may occur either in isolation or as part of a multiorgan disease. A 43-year-old woman was admitted to our hospital with acute left chest pain and shortness of breath. Spontaneous left pneumothorax was detected. All laboratory tests, including pulmonary function studies, were normal. Radiological findings posed high suspicion for LCH and lung biopsy confirmed this diagnosis. Further studies identified small cystic lesions in the scalp and liver. The diagnosis of multiple organ LCH involvement was made. Spontaneous pneumothorax might be the presenting clinical symptom of LCH. The present case emphasizes the capricious nature of LCH and the importance of an individualized therapeutic approach.
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PMID:A case of multiorgan Langerhans' cell histiocytosis presented with pneumothorax. 1558 54

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by infiltration of either single or multiple organs by a distinct cell type that is S-100 and CD1a positive and contains ultrastructural Birbeck granules on electron microscopy. Historically, LCH included four main clinical forms: Letter-Siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma (together grouped as histiocytosis) and Hashimoto-Pritzker disease. The writing group of the Histiocytotic Society in 1987 proposed the uniform term of 'Langerhans cell histiocytosis' to encompass all the aforementioned eponymous forms. Lung involvement occurs in up to half of all children with multisystem disease and usually parallels overall disease activity. Spontaneous pneumothorax (SP) occurs in approximately 10% of children with pulmonary disease and may be a fatal complication. Patients with pulmonary LCH are likely predisposed to the development of pneumothorax based on destructive changes in the lung parenchyma. Here, we report a case of multisystem LCH in which the patient presented at 2 months of age because of simultaneous bilateral pneumothorax.
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PMID:Langerhans cell histiocytosis manifesting as recurrent simultaneous bilateral spontaneous pneumothorax in early infancy. 1804 18

Pulmonary Langerhans cell histiocytosis is an interstitial lung disease that results from the accumulation of specific histiocytic cells in the lung. Spontaneous pneumothorax is a recognized feature of pulmonary Langerhans cell histiocytosis and results from destruction of lung parenchyma with associated cystic changes. We report on a 2-year-old boy with recurrent bilateral spontaneous pneumothorax; a computed tomography scan showed marked interstitial changes, fibrosis, cystic spaces and bilateral bullae. The diagnosis was confirmed by the histology and the immunohistochemistry examination of the pulmonary biopsy with CD1 and S100 positive antibodies. The child was treated with prednisone and etoposide, and had a good clinical response and favorable changes in the second thoracic CT scan.
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PMID:[Bilateral spontaneous pneumothorax as a setting of Langerhans cell histiocytosis]. 2486 20