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Table 4 Annual incidence/mortality rates and subdistribution hazard ratios for malignancies and causes of death in persons with porphyria cutanea tarda (2000–2016, 18 years or older)

From: Porphyria cutanea tarda increases risk of hepatocellular carcinoma and premature death: a nationwide cohort study

  Incidence cases/no. at risk Person-years; annual mortality rate per 100 (95% CI) Subdistribution hazard ratios (95% confidence intervals)
Crude SHR (95% CI) Adjusted SHR (95% CI)
All sites – first primary C (C00–96, D45–47)
 Reference 3549/30468 393,347; 0.902 (0.873–0.932) 1.0 1.0
 PCT 78/572 8132; 0.959 (0.768–1.198) 0.7 (0.5–0.8) 0.7 (0.6–0.9)
HCC (155.0; C22.0)
 Reference 80/30,468 402,342; 0.020; 0.020 (0.16–0.025) 1.0 1.0
 PCT 5/572 8330; 0.060 (0.025–0.144) 2.2 (0.9–5.5) 3.1 (1.2–7.7)
Gallbladder & biliary tract (156; C23–24)
 Reference 19/30,468 402,403; 0.003 (0.003–0.007) 1.0 1.0
 PCT 3/572 8339; 0.036 (0.012–0.112) 4.4 (1.3–15.0) 4.0 (1.1–14.4)
All-cause mortality
 Reference 11,756/30468 402,757; 2.919 (2.867–2.972) 1.0 1.0
 PCT 150/595 8684; 1.728 (1.472–2.027) 0.4 (0.3–0.4) 0.4 (0.4–0.5)
Diseases of the liver (K70–77, B15–19, E83.1)
 Reference 735/30468 402,746; 0.182 (0.170–0.196) 1.0 1.0
 PCT 4/595 8683; 0.046 (0.017–0.123) 0.2 (0.1–0.6) 0.2 (0.1–0.6)
Malignant neoplasms (C00–96)
 Reference 2323/30,468 402,746; 0.577 (0.554–0.601) 1.0 1.0
 PCT 45/595 8683; 0.518 (0.387–0.694) 0.5 (0.4–0.7) 0.6 (0.4–0.8)
Drug and alcohol overdose (X40–49)
 Reference 484/30,468 402,746; 0.134 (0.134–0.157) 1.0 1.0
 PCT 5/597 8.683; 0.058 (0.024–0.138) 0.5 (0.2–1.1) 0.5 (0.2–1.2)
  1. Disease codes defined using the International Classification of Primary Care – 2nd Edition (ICPC-2) and the International Statistical Classification of Diseases 7th (ICD-7) and 10th (ICD-10) revisions. Reference group = Chronic alcohol abuse/dependence (ICD-10 = F10, ICD-9 = 303, 305.0, ICPC2 = P15). 17 persons with both PCT and a diagnosis of chronic alcohol abuse or dependence, including one person with HCC, were excluded from the analyses. 23 persons with a cancer diagnosis prior to their PCT diagnosis were excluded from any analysis in which cancer was the outcome
  2. Mean age at death for persons with chronic alcohol abuse = 63.6 (SD: 11.7) vs. PCT = 72.5 (SD = 11.7), proportion of males = 78% vs. PCT = 50%