Heme oxygenase-1 deficiency presenting with interstitial lung disease and hemophagocytic flares

• Main Authors: Alice S. Chau (Author), Bonnie L. Cole (Author), Jason S. Debley (Author), Kabita Nanda (Author), Aaron B. I. Rosen (Author), Michael J. Bamshad (Author), Deborah A. Nickerson (Author), Troy R. Torgerson (Author), Eric J. Allenspach (Author)
• Format: Book
• Published: BMC, 2020-10-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Abstract Background Heme oxygenase-1 (HMOX1) catalyzes the metabolism of heme into carbon monoxide, ferrous iron, and biliverdin. Through biliverdin reductase, biliverdin becomes bilirubin. HMOX1-deficiency is a rare autosomal recessive disorder with hallmark features of direct antibody negative hem...