Congenital lobar emphysema (CLE) is an uncommon cause of progressive respiratory distress that typically presents in the first few days of life. There has been a previous report of CLE in a mother and daughter. The authors describe 2 cases involving the right upper and middle lobes in a father and son secondary to relative deficiency of the bronchial cartilage. This provides additional evidence for inherited factors in the etiology of CLE.
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