title: [An autopsy case of Marfan syndrome with bronchiectasis and multiple bullae].
abstract: We report an autopsy case (27-year-old male) with Marfan syndrome, who died of chronic respiratory failure due to bronchiectasis and multiple bullae in both lungs. He had suffered from expectoration of massive amounts of sputum since the age of 15 years. At this time, chest roentgenogram had revealed bronchiectatic changes in the bilateral lower lung fields. Seven years later at the age of 22 years, the formation multiple bullae in both lungs were added to the bronchiectatic changes on chest roentgenogram. Administration of erythromycin (400 mg/day) was started in February, 1987, and the massive sputum volume markedly decreased according to appearance of bullous formation. He was admitted to our department because of deterioration with chronic respiratory failure and right heart failure at 26 years in December, 1989. Although various therapy was performed, he died of chronic respiratory failure in February, 1990. Autopsy findings were as follows: (1) cyclindrical bronchiectatic changes in bilateral lower lobes and (2) extensive multiple bullae in the subpleural areas with bronchiectatic changes in the middle and bilateral lower lobes, with no bronchiectatic changes in the bilateral upper lobes. Several pulmonary disorders accompanying Marfan syndrome have been reported, especially in children. However, the present case demonstrated that fetal pulmonary involvement by Marfan syndrome may not present until adulthood, and affect both airways and lung parenchyma.
authors.affiliation: Department of Respiratory Medicine, Juntendo University, School of Medicine, Tokyo, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Saito
authors.forename: K
authors.initials: K
authors.lastname: Iijima
authors.forename: T
authors.initials: T
authors.lastname: Dambara
authors.forename: J
authors.initials: J
authors.lastname: Shiota
authors.forename: S
authors.initials: S
authors.lastname: Hirose
authors.forename: T
authors.initials: T
authors.lastname: Uekusa
authors.forename: S
authors.initials: S
authors.lastname: Saiki
authors.forename: S
authors.initials: S
authors.lastname: Kira
mesh_terms: D000328:Adult; D001987:Bronchiectasis / Q000150:complications* / Q000473:pathology; D003560:Cysts / Q000150:complications / Q000473:pathology; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000473:pathology; D011656:Pulmonary Emphysema / Q000150:complications* / Q000473:pathology; D012131:Respiratory Insufficiency / Q000209:etiology