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)

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.
 ...
PMID:A case of multiorgan Langerhans' cell histiocytosis presented with pneumothorax. 1558 54

We report a case of sudden death due to bilateral pneumothorax in a previously healthy 16-year-old adolescent white girl. She presented with sudden onset of shortness of breath followed by loss of consciousness. Postmortem chest radiograph showed bilateral pneumothoraces. Autopsy confirmed the bilateral pneumothorax and additionally showed emphysematous changes and bullae throughout the lung tissue. Microscopic sections of the lungs showed Langerhans cell histiocytosis. To the best of our knowledge, this is the first reported case of fatal presentation of pulmonary Langerhans cell histiocytosis.
 ...
PMID:Sudden death of a patient with pulmonary Langerhans cell histiocytosis. 1591 33