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Sickle Cell-Related Disease: A Case Study

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Madsen et al. developed this classification to asthmatic, cardiac, lymphatic, and sickle cell-related disease states(10, 13, 14). Madsen et al.(10, 13) defined this classification depending on the analysis of prior case studies. Cardiac patients mentioned by them consist of eight Fontan patients, one Glenn shunt patient and three Blalock–Taussig shunt patients. The casts can be immediately expectorated and sometimes patients will cough up large amount from their tracheobronchial tree(14). If the patients do not expectorate, it may delay the diagnosis(14). Patients often need bronchoscopy for the expulsion of cast(14). It is a rare condition that is mentioned in asthma and sickle cell disease patients and has higher mortality in patients with congenital heart disease(10, 13) and death is not unusual due to the occlusion of airway from a cast(14). The pathophysiology and management of plastic bronchitis is not clear due to insufficient clinical data. Over the years, plastic bronchitis has been labeled by various names as Hoffman's bronchitis, cast bronchitis, fibrinous …show more content…

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Brogan et al. used acetylcysteine, urokinase, tPA and DNase to incubate a cast from a Fontan patient in vitro(13). It indicated that acetylcysteine had the huge impact to soften the casts. The practice of inhaled DNAse has produced the favorable outcome(11, 18). Use of corticosteroid was also favorable to treat an 8-year-old patient who had Fontan circulation with casts and mild eosinophilic infiltration(46). Based on limited data, it seems that the use of anti-inflammatory therapy may primarily benefit eosinophilic-type

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