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Table 3 Incidence of long-term complications during observation period

From: Clinical features of acute attacks, chronic symptoms, and long-term complications among patients with acute hepatic porphyria in Japan: a real-world claims database study

 

Overall

AHP Population

AHP Population

Age ≥ 55 Years

Matched Population

Overall

Type

Overall

Type

AIP

HCP

VP

AIP

HCP

VP

Patients, n

391

120

46

11

149

34

15

8

3910

Complications for observation period (ICD-10), n (%)

         

Hepatitis, cirrhosis of liver, liver cell carcinoma

         

Hepatic failure, not elsewhere classified (K72)

26

(6.6)

7

(5.8)

2

(4.3)

1

(9.1)

15

(10.1)

2

(5.9)

1

(6.7)

1

(12.5)

31

(0.8)

Chronic hepatitis, not elsewhere classified (K73)

28

(7.2)

3

(2.5)

3

(6.5)

2

(18.2)

11

(7.4)

0

(0.0)

1

(6.7)

2

(25.0)

51

(1.3)

Fibrosis and cirrhosis of liver (K74)

62

(15.9)

13

(10.8)

6

(13.0)

1

(9.1)

31

(20.8)

5

(14.7)

3

(20.0)

1

(12.5)

118

(3.0)

Malignant neoplasm of liver and intrahepatic bile ducts, liver cell carcinoma (C22.0)

66

(16.9)

9

(7.5)

12

(26.1)

2

(18.2)

43

(28.9)

5

(14.7)

8

(53.3)

2

(25.0)

183

(4.7)

Kidney disease

         

Kidney failure (N17-N19)

64

(16.4)

19

(15.8)

4

(8.7)

0

(0.0)

47

(31.5)

11

(32.4)

4

(26.7)

0

(0.0)

167

(4.3)

Acute kidney failure (N17)

10

(2.6)

4

(3.3)

2

(4.3)

0

(0.0)

7

(4.7)

1

(2.9)

2

(13.3)

0

(0.0)

40

(1.0)

Chronic kidney disease (N18)

33

(8.4)

6

(5.0)

2

(4.3)

0

(0.0)

28

(18.8)

5

(14.7)

2

(13.3)

0

(0.0)

100

(2.6)

Unspecified kidney failure (N19)

31

(7.9)

13

(10.8)

1

(2.2)

0

(0.0)

20

(13.4)

7

(20.6)

1

(6.7)

0

(0.0)

79

(2.0)

Nerve injury

         

Polyneuropathies and other disorders of peripheral nervous system (G60-G64)

80

(20.5)

33

(27.5)

7

(15.2)

2

(18.2)

38

(25.5)

15

(44.1)

5

(33.3)

1

(12.5)

308

(7.9)

Hereditary and idiopathic neuropathy (G60)

0

(0.0)

0

(0.0)

0

(0.0)

0

(0.0)

0

(0.0)

0

(0.0)

0

(0.0)

0

(0.0)

0

(0.0)

Inflammatory polyneuropathy (G61)

25

(6.4)

11

(9.2)

2

(4.3)

1

(9.1)

7

(4.7)

3

(8.8)

1

(6.7)

0

(0.0)

3

(0.1)

Other polyneuropathies (G62)

63

(16.1)

26

(21.7)

6

(13.0)

2

(18.2)

28

(18.8)

11

(32.4)

4

(26.7)

1

(12.5)

238

(6.1)

Other disorders of peripheral nervous system (G64)

12

(3.1)

1

(0.8)

3

(6.5)

0

(0.0)

6

(4.0)

1

(2.9)

2

(13.3)

0

(0.0)

98

(2.5)

Hypertension

         

Essential (primary) hypertension (I10)

102

(26.1)

27

(22.5)

9

(19.6)

4

(36.4)

77

(51.7)

18

(52.9)

8

(53.3)

4

(50.0)

736

(18.8)

  1. AHP, acute hepatic porphyria; AIP, acute intermittent porphyria; AP, acute porphyria; HCP, hereditary coproporphyria; HP, hepatic porphyria; ICD-10, International Classification of Diseases, Tenth Revision; MDV, Medical Data Vision; VP, variegate porphyria