[Hermansky-Pudlak syndrome in 2 brothers with lung fibrosis].
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.
Sültz
J
J
Deutsche Klinik für Diagnostik, Wiesbaden.
Häussinger
K
K
Wöckel
W
W
Hübner
G
G
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
Hermansky-Pudlak-Syndrom bei zwei Brüdern mit Lungenfibrose.