title: [Hermansky-Pudlak syndrome in 2 brothers with lung fibrosis].
abstract: This is a report on two brothers with the Hermansky-Pudlak syndrome who developed pulmonary fibrosis. In the first, a gradual course lasting 13 years was observed, the patient finally died of long-standing colitis. His brother died of progressive respiratory failure 15 months after the subjective onset of the disease. Immunosuppressive therapy proved ineffective.
authors.affiliation: Deutsche Klinik für Diagnostik, Wiesbaden.
authors.forename: J
authors.initials: J
authors.lastname: Sültz
authors.forename: K
authors.initials: K
authors.lastname: Häussinger
authors.forename: W
authors.initials: W
authors.lastname: Wöckel
authors.forename: G
authors.initials: G
authors.lastname: Hübner
mesh_terms: D000328:Adult; D001706:Biopsy; D002869:Chromosome Aberrations / Q000235:genetics*; D025063:Chromosome Disorders; D005808:Genes, Recessive; D006801:Humans; D008168:Lung / Q000473:pathology; D008297:Male; D011658:Pulmonary Fibrosis / Q000235:genetics*; D013577:Syndrome
vernacular_title: Hermansky-Pudlak-Syndrom bei zwei Brüdern mit Lungenfibrose.