[Lethal pulmonary involvement in neurofibromatosis].
A unusual case of a 61-year-old man who presented dyspnea and cough associated to skin and subcutaneous lesions typical of neurofibromatosis type 1 is reported. Chest roentgenograms and CT-scan showed emphysematous bullae. The patient underwent drainage of the greatest bulla, but death eventually ensured as a consequence of upper digestive bleeding. It can be expected that with the longer survival of neurofibromatosis patients with respiratory troubles, that are generally considered unusual will be recognized more frequently.
Faintuch
J J
JJ
Departamento de Clínica Médica, Faculdade de Medicina, Universidade de São Paulo.
Saito
R
R
Warth
M do P
Mdo P
Rocha
A dos S
Ados S
D006471:Gastrointestinal Hemorrhage / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis; D011656:Pulmonary Emphysema / Q000150:complications* / Q000175:diagnosis; D011658:Pulmonary Fibrosis / Q000150:complications* / Q000175:diagnosis; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed
Comprometimento pulmonar letal na neurofibromatose.