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Neurofibromatosis Type-1 Lung Disease Complicated by Pleuroparenchymal Fibroelastosis
Abhir Nainani1, Hugh Buzacott1, Nicole Goh1
1Department of Respiratory and Sleep Medicine Austin Health Melbourne Victoria Australia.
Abstract:
Neurofibromatosis type-1 is a rare autosomal dominant disease, due to the loss of the NF1 tumour suppressor gene. Here we present a case of a 28-year-old man with neurofibromatosis type-1 lung disease and pleuroparenchymal fibroelastosis leading to recurrent pneumothoraxes requiring intervention.
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