Pulmonary changes in patients with type i neurofibromatosis and tuberous sclerosis
Ljubić, S.; Peros-Golubicić, T.
Lijecnicki Vjesnik 115(3-4): 95-98
1993
ISSN/ISBN: 0024-3477 PMID: 8231624 Document Number: 420820
Two patients with pulmonary affections in the course of neurofibromatosis and tuberous sclerosis are presented. We wanted to draw attention to the possibility of occurrence of diffuse interstitial pulmonary changes during congenital neurocutaneous syndromes, of which no data are reported in our literature. The affection of the lungs in 10-20% of the patients with neurofibromatosis and 1% with tuberous sclerosis is estimated, commonly in women. The most frequent changes in neurofibromatosis are fibrosing alveolitis and interstitial fibrosis, and in tuberous sclerosis cystic lung changes and lymphangiomyomatosis, what agrees with our findings. It is important to recognize such patients in order not to perform unnecessary diagnostic procedures, and not to draw wrong conclusions, thus being able to avoid an unnecessary therapy, ineffective in such cases. The patients should be followed up, and in the case of complaints symptomatic therapy has to be administered.