Parviz Vabedi,
Volume 2, Issue 4 (2-1989)
Abstract
A 25 year old male and his 46 year old aunt presented with shortness of
breath and a dramatic response to steroids. The other two patients are sisters
with more advanced disease. One of these responded partially to steroids,
while the other died within 4 months of treatment. The genetic basis and
pathogenesis are discussed.