Table 3 Comparison between different studied groups as regards clinical and laboratory data
From: Phenotypic and Genetic Characterization of a Cohort of Pediatric Wilson Disease Patients
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 | |