Table 3

Comparison between different studied groups as regards clinical and laboratory data

G1 (35)

G2 (6)

G3 (9)

G4 (27)

Total (77)

P value


Male/Female

20/15

4/2

7/2

12/15

43/34

0.32


Median age of onset (yrs) (Range)

10 (6-15)

12 (10-14)

11 (9-18)

8 (1-17)

10 (1-18)

0.017 *


Duration between onset and diagnosis (yrs)

0.83 ± 3

4.75 ± 0.9

1.9 ± 0.6

-

1.03 ± 0.2

0.000*


Duration of follow up (ms) Mean (Range)

60.27 (0.25-156)

32.5 (12-48)

75.86 (12-156)

89.55 (5-156)

58.9 (0.25-156)


S. Ceruloplasmin

<7

25 (71.4)

6 (100)

7 (77.8)

19 (70.4)

57 (74)

(mg%)

7-20

6 (17.1)

0 (0)

2 (22.2)

7 (25.9)

15 (19.5)

No. (%)

>20

4 (11.4)

0 (0)

0 (0)

1 (3.7)

5 (6.5)


Hepatomegaly

22 (62.9)

2 (33.3)

5 (55.6)

22 (81.5)

51 (66.2)

0.134

Hepatic

Splenomegaly

22 (62.9)

1 (16.6)

6 (66.6)

9 (33.3)

38 (49.4)

0.006

signs

Jaundice

24 (68.6)

0 (0)

2 (22.2)

0 (0)

26 (33.8)

0.000*

No. (%)

Ascites

17 (48.6)

0 (0)

4 (44.4)

0 (0)

21 (27.3)

0.000*

LL Oedema

15(42.9)

0 (0)

5(55.6)

0 (0)

20 (26)

0.000*


Encephalopathy

2 (5.7)

0 (0)

0 (0)

0 (0)

2 (2.6)

ALT (x ULN)

2.8 ± 0.4

1.7 ± 0.8

1.7 ± 0.3

3.2 ± 0.5

2.7 ± 0.3

0.056

Liver

AST (x ULN)

3.9 ± 0.5

1.0

3.9 ± 0.2

2.4 ± 0.3

3.2 ± 0.3

0.001*

function

Bilirubin (mg%)

8.0 ± 1.7

2.5 ± 1.6

1.8 ± 0.5

0.8 ± 0.1

4.2 ± 0.8

0.000*

tests Mean ± SD

INR

3.0 ± 0.4

1.3 ± 0.2

3.8 ± 0.2

1.1 ± 0.04

2.3 ± 3.4

0.000*

Albumin (gm%)

2.6 ± 0.1

4.3 ± 0.1

3.0 ± 0.3

3.9 ± 0.2

3.2 ± 1.2

0.000*


Chronic hepatitis

4/11 (36.4)

1/4 (25)

0/4 (0)

7/20 (35)

12/39 (30.8)

Liver

Cirrhosis

7/11 (63.6)

1/4 (25)

4/4 (100)

9/20 (45)

21/39 (53.8)

biopsy

Steatosis

3/11 (27.3)

1/4 (25)

2/4 (50)

2/20 (10)

8/39 (20.5)

No. (%)

Glycogenated nuclei

2/11 (18.2)

1/4 (25)

0/4 (0)

7 (35)

10/39 (25.6)

Cu associated protein

4/11 (36.4)

0/4 (0)

1/4 (25)

5 (25)

10/39 (25.6)


Pigment

1/11 (9)

1/4 (25)

0/4 (0)

2 (10)

4/39 (10.3)


Positive KF rings No. (%)

27/28 (96.4)

6/6 (100)

8/8 (100)

4/23 (17.4)

45/65 (69.2)

0.000


Abdel Ghaffar et al. BMC Pediatrics 2011 11:56   doi:10.1186/1471-2431-11-56

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