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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