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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