Why do some adults with PiMZ alpha1-antitrypin develop bronchiectasis?
N Aggarwal, B Martinez Delgado Delgado, S Salipalli, N Matamala, J Rademacher, N Schwerk, T Welte, S Janciauskiene, FC Ringshausen · Pneumologie · 2016 · Heft 10
Bibliografische Angaben
Zusammenfassung
Several reports indicate an association among inherited alpha-1-antitrypsin deficiency (A1ATD), pulmonary infections and bronchiectasis. Most reported A1ATD cases with bronchiectasis are elderly homozygous PiZZ (Glu342Lys) smokers with emphysema. Whether there is an increased risk of pulmonary diseases, including bronchiectasis, in heterozygous PiMZ A1ATD carriers is debated. We describe an 18-year old, non-smoking male who had neonatal hyperbilirubinemia and suffered from productive cough, chronic rhinosinusitis and recurrent otitis since early childhood. A high-resolution CT scan of the chest revealed bronchie…
