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Table 3 Annual mortality rates and hazard ratios for 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

Sample Mortality cases/no. at risk Person-years; annual mortality rate per 100 (95% CI) Hazard ratios (95% confidence intervals) E-value
Crude Adjusted
Premature death
 Reference 748,900/5451,951 71,559,947; 1.047 (1.044–1.049) 1.0 1.0 1.0
 PCT (total) 158/612 8921; 1.771 (1.515–2.070) 1.5 (1.3–1.8) 1.5 (1.2–1.7) 2.4
  Familial PCT 27/255 3933; 0.686 (0.471–1.001) 0.7 (0.5–1.1) 0.8 (0.1–1.1) 1.0
  Sporadic PCT 64/255 3828; 1.672 (1.309–2.136) 1.5 (1.2–1.9) 1.4 (1.1–1.8) 2.2
  Unclassified PCT 67/102 1160; 5.776 (4.546–7.339) 2.6 (2.1–3-4) 2.5 (1.9–3.1) 4.4
Malignant neoplasms (C00–96)
 Reference 181,205/5,451,951 71,559,947; 0.254 (0.253–0.255) 1.0 1.0 1.0
 PCT (total) 48/612 8921; 0.538 (0.405–0.714) 1.4 (1.1–1.9) 1.4 (1.0–1.9) 2.2
  Sporadic PCT 23/255 3828; 0.601 (0.399–0.904) 1.6 (1.0–2.4) 1.5 (1.0–2.2) 2.4
  Familial PCT 11/255 3933; 0.280 (0.155–0.505) 0.9 (0.5–1.7) 0.9 (0.5–1.6) 1.0
  Unclassified PCT 14/102 1160; 1.207 (0.715–2.038) 2.0 (1.2–3.4) 1.8 (1.1–3.1) 3.3
Diabetes mellitus (E10–14)
 Reference 11,826/5452,010 71,559,947; 0.016 (0.016–0.017) 1.0 1.0 1.0
 PCT 3/612 8921; 0.034 (0.011–0.104) 2.0 (0.7–6.3) 1.7 (0.5–5.3) 2.8
Ischemic heart disease (I20–25)
 Reference 5,451,951 71,559,947; 0.135 (0.134–0.136) 1.0 1.0 1.0
 PCT 7/612 8921; 0.079 (0.037–0.165) 0.6 (0.3–1.2) 0.6 (0.3–1.2) 1.0
Cerebrovascular diseases (I60-I69)
 Reference 57,639/5,451,951 71,559,947; 0.081 (0.080–0.081) 1.0 1.0 1.0
 PCT 8/612 8915; 0.090 (0.045–0.179) 1.2 (0.6–2.4) 1.1 (0.6–2.3) 1.4
Chronic obstructive pulmonary disease (J43–44)
 Reference 31,172/5,451,951 71,559,947; 0.043 (0.043–0.044) 1.0 1.0 1.0
 PCT 7/612 8921; 0.078 (0.037–0.165) 1.4 (0.6–2.9) 1.4 (0.6–2.8) 2.2
Diseases of the liver (K70–77, B15–19, E83.1) a
 Reference 4661/5,452,010 71,559,947; 0.007 (0.00–0.007) 1.0 1.0 1.0
 PCT 6/612 8921; 0.067 (0.030–0.150) 5.9 (2.7–13.2) 5.5 (2.5–12.2) 10.5
Renal failure (N17–19)
 Reference 6686/5,452,010 71,559,947; 0.009 (0.009–0.010) 1.0 1.0 1.0
 PCT 2/612 8921; 0.022 (0.006–0.090) 2.9 (0.7–11.8) 2.9 (0.7–11.7) 5.3
Drug and alcohol overdose (X40–49) b
 Reference 4961/5452,010 71,559,947; 0.007 (0.007–0.007) 1.0 1.0 1.0
 PCT 7/612 8921; 0.078 (0.037–0.165) 12.1 (5.8–25.5) 9.9 (4.7–20.8) 19.3
  1. Disease codes defined using the International Statistical Classification of Diseases 10th (ICD-10) revision
  2. aSpecific diagnoses of the 6 PCT patients with liver disease were: alcoholic cirrhosis of liver (K70.3), alcohol hepatic failure (K70.4) primary biliary cirrhosis (K74.3), hepatic failure (K72.9), 2 x chronic viral hepatitis C (B18.2). Familial PCT, n=0, sporadic PCT, n=2, Not tested, n=3. None of the six persons with liver disease were diagnosed with HCC or gallbladder and biliary tract cancer.
  3. bSpecific diagnosis of the 7 persons with PCT and drug and alcohol overdose included: 4 x accidental poisoning by and exposure to narcotics and psychodysleptics [hallucinogens], not elsewhere classified (ICD-10: X42); 1 x accidental poisoning by and exposure to non-opioid analgesics, antipyretics and antirheumatics (ICD-10: X40); 1 x accidental poisoning by and exposure to antiepileptic, sedative-hypnotic, antiparkinsonism and psychotropic drugs, not elsewhere classified (ICD-10: X41); 1 x accidental poisoning by and exposure to alcohol (X45). Familial PCT, n=1, sporadic PCT, n=3, Not tested, n=3