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Increased mortality in patients with porphyria cutanea tarda—A nationwide cohort study

Journal of the American Academy of Dermatology Feb 11, 2020

Christiansen AL, et al. - In this nationwide cohort study, researchers contrasted all-cause and cause-specific mortality between a nationwide cohort of patients with porphyria cutanea tarda (PCT), a rare hepatocutaneous disease, and a matched population sample. All Danish patients who received a diagnosis of PCT from 1989 through 2012 were involved. They matched each patient by age and sex to 10 random population control people. Data reported that the 20-year survival was 42.9% for patients with PCT vs 60.5% for matched control people. It was noted that the hazard ratio of all-cause mortality was 1.80 before adjustment and 1.22 after adjustment. Findings suggested that PCT patients have increased mortality, primarily due to increased mortality from gastrointestinal diseases and gut, liver/gallbladder, and lung cancers.
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