[Familial alveolar proteinosis].
Pulmonary alveolar proteinosis is a progressive disease recognized 35 years ago. Many etiologies have been put forward, but the pathogenesis remains obscure. Familial alveolar proteinosis is a rare condition, probably transmitted by recessive autosomal inheritance, with deficiency in surfactant apoprotein SPB or defective expression of the common granulocyte colony stimulating factor (GM-CSF), IL3 and IL5 receptor. Gene therapy could be a future option for treatment in patients with specific gene defects.
Ayadi
H
H
Service de Pneumologie, CHU Hédi-Chaker, Sfax, Tunisie. Hajer@medscape.com
Ayoub
A K
AK
D000328:Adult; D018893:Bronchoalveolar Lavage; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis* / Q000235:genetics; D012075:Remission, Spontaneous
D007074:Immunoglobulin G