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Table 4 Age at NTBC-start and sequelae: odds ratio of patients diagnosed and treated beyond the perinatal period compared to those treated below the age of 1 month (OR = Odds ratio; 95% LCI = lower limit of 95% confidence interval; UCI = upper limit of 95% confidence interval)

From: Cross-sectional study of 168 patients with hepatorenal tyrosinaemia and implications for clinical practice

Complications

1-6 mn=45 patients

7-12 mn= 20 patients

>12 mn= 46 patients

 

OR

LCI 95%

UCI 95%

OR

LCI 95%

UCI 95%

OR

LCI 95%

UCI 95%

LTx

2.5

0.2

25.8

4

0.3

47.1

12.7

1.5

103

acute liver failure

2.5

0.1

63.9

15

0.7

306.4

2.4

0.09

62.4

Carcinoma

2.5

0.2

25.8

6.3

0.6

65.6

12.7

1.5

103

Cirrhosis

8.1

0.4

156.1

41.6

2.2

779.9

40.5

2.3

704.1

hepatomegaly

3.3

0.9

11.3

4.4

1.1

17.7

3.9

1.1

13.3

Rickets

4.3

0.2

92.6

10.1

0.5

222.1

19

1.1

338.3

renal dysfunction

1.7

0.2

9.8

5.8

1.0

33.4

5.5

1.1

26.6

renal tubular dysfunction

1.2

0.1

7.9

1.9

0.2

14.9

4.3

0.8

21.6

nephromegaly

0.8

0.1

6

3.0

0.4

20.2

2.6

0.4

13.8

nephrocalcinosis

4.3

0.2

92.6

5.7

0.2

148.3

2.5

0.09

62.5

impaired growth

2,5

0.1

63.9

1.8

0.03

95.6

2.5

0.09

62.5

Adipositas

2.5

0.1

63.9

1.8

0.03

95.6

2.5

0.09

62.5

neurological concomitant disease

1.4

0.3

6.3

0.5

0.05

6.1

0.2

0.02

2.5

neurological crises

0.2

0.02

2.5

0.5

0.05

6.1

0.2

0.02

2.5

Epilepsy/convulsion

0.8

0.1

3.4

0.9

0.1

5.5

0.1

0.02

1.7

ADS, behavioural disorders

1.4

0.3

6.3

1.2

0.1

8.2

0.5

0.08

3.2

learning/language difficulties

0.8

0.05

13.5

0.5

0.02

15.2

0.2

0.01

6.6

Impaired psychomotor development

0.7

0.2

2.3

1.5

0.4

5.34

0.7

0.2

2.27

Death

0.8

0.01

42.5

15

0.7

306.4

2.5

0.09

62.5