T-cell acute lymphoblastic lymphoma presenting as chylothorax in a child

摘要

The median survival of Cystic Fibrosis patients has increased remarkably in the last decade, due to precocious and aggressive respiratory and nutritional intervention, and the multidisciplinary management of the disease based on systematized protocols. The knowledge of the basic defect of the disease, the cuantitative or functional fault of the CFTR protein, on which the hidroelectrolytic balance of the epitelial airways depends, has allowed the development of specific therapies targeted to its correction, in advanced stages of investigation. The hope has been opened to have therapies directed to the cause of the Cystic Fibrosis and not only to alleviate its consequences.