Risk for incident comorbidities, nonhepatic cancer and mortality in acute hepatic porphyria: A matched cohort study in 1244 individuals.
Mattias LissingDaphne VassiliouYlva FloderusPauline HarperJacinth YanHannes HagströmEliane SardhStaffan WahlinPublished in: Journal of inherited metabolic disease (2022)
The acute hepatic porphyrias (AHP) are associated with long-term complications such as primary liver cancer, hypertension, and chronic kidney disease. Data on other related comorbidities are scarce. In this register-based, matched cohort study, we assessed the risks of nonhepatic cancers, cardiovascular diseases, renal diseases, psychiatric disorders, and mortality in relation to porphyria type, sex, and biochemical disease activity. All patients in the Swedish porphyria register with a verified AHP diagnosis during 1987-2015 were included. The biochemical activity of acute intermittent porphyria was assessed using recorded maximal urinary porphobilinogen (U-PBG). Data on incident comorbidities and mortality were collected from national health registries. Cumulative incidences, rates, and hazards were compared to reference individuals from the general population, matched 1:10 by age, sex, and county. We identified 1244 patients with AHP with a median follow-up of 19 years. Health registries identified 149 AHP-subjects (12.0%) with nonhepatic cancer, similar to 1601 (13.0%) in the matched reference population (n = 12 362). Patients with AHP had a higher risk of kidney cancer (0.8% vs. 0.2%, p < 0.001), hypertension, and chronic kidney disease but no increase in risk for cardiovascular disease, except for cerebrovascular disease in patients with elevated U-PBG, (aHR = 1.40 [95% CI:1.06-1.85]). Mortality risk during follow-up was higher among patients with AHP (21% vs. 18%, p = 0.001), and associated with primary liver cancer, female sex, and biochemical activity. In conclusion, AHP is associated with an increased risk of kidney cancer, hypertension, chronic kidney disease, and mortality but not with cardiovascular disease or other nonhepatic cancers.
Keyphrases
- cardiovascular disease
- chronic kidney disease
- end stage renal disease
- papillary thyroid
- cardiovascular events
- blood pressure
- squamous cell
- liver failure
- disease activity
- peritoneal dialysis
- risk factors
- rheumatoid arthritis
- childhood cancer
- type diabetes
- systemic lupus erythematosus
- drug induced
- respiratory failure
- coronary artery disease
- public health
- cardiovascular risk factors
- metabolic syndrome
- squamous cell carcinoma
- heart rate
- aortic dissection
- newly diagnosed
- acute respiratory distress syndrome
- ejection fraction
- high intensity
- body composition
- climate change
- big data
- human health
- intensive care unit
- data analysis
- extracorporeal membrane oxygenation