
2Department of Hepatobiliary Surgery, National Liver Institute, Menoufiya University, Egypt
1. Clearance of jaundice.
2. Proportions of native liver survival [5].
Long term (10 years or greater) survival have been reported in the range of 27%-53%. Although in another study, only about 15% had true long term survival without jaundice, normal liver biochemistry and no signs of liver disease or portal hypertension (PHT). However, even in this apparently "cured" group, liver histology still remains abnormal [6].
Severity of liver disease at time of last follow up was assessed by the Pediatric End-stage Liver Disease (PELD) score. It is calculated according to the following formula:
PELD Score = 0.480 x Loge (bilirubin mg/dL) + 1.857 x Loge (INR) - 0.687 x Loge (albumin g/dL) + 0.436 if the patient is less than 1 year old (scores for patients listed for liver transplantation before the patient’s first birthday continue to include the value assigned for age (< 1 Year) until the patient reached the age of 24 months) + 0.667 if the patient has growth failure (<-2 Standard deviation)
BA Patients | ||
N = 106 | ||
No. | % | |
Classification | ||
Post-KPE | 86 | (81.1%) |
Neglected BA | 20 | (18.9%) |
Outcome | ||
Living | 47 | (44.3%) |
Dead | 50 | (47.2%) |
Lost during follow up | 9 | (8.5%) |
Age Groups | Successful Outcome N = 15 |
Failed Outcome N = 45 |
P-value |
Mean ± SD | Mean ± SD | ||
Age at KPE (Days) | 12.02 ± 64.40 | 17.85 ± 70.98 | 0.226 |
Time LFTs | At Diagnosis N = 15 | Pre KPE N = 15 | One Month post KPE N = 15 | Six Months post KPE N = 15 | Last Lab N = 15 |
SD ± Mean | SD ± Mean | SD ± Mean | SD ± Mean | SD ± Mean | |
BILI.T (mg/dl) | 6.22 ± 11.99 | 4.71 ± 11.03 | 3.36 ± 5.53 | 0.45 ± 1.08 | 041 ± 0.85 |
BILI.D (mg/dl) | 9.52 ± 10.16 | 3.94 ± 8.12 | 2.49 ± 3.79 | 0.46 ± 0.51 | 0.28 ± 0.35 |
ALT (U/L) | 78.36 ± 118.97 | 63.49 ± 132.20 | 58.56 ± 85.60 | 79.85 ± 86.85 | 128.73 ± 99.85 |
AST (U/L) | 134.37 ± 194.82 | 90.53 ± 210.73 | 74.32 ± 134.33 | 48.98 ± 97.46 | 223.30 ± 136.69 |
GGT (U/L) | 682.78 ± 864.53 | 850.13 ± 1234.67 | 616.63 ± 882.57 | 244.12 ± 553.36 | 320.36 ± 538.50 |
ALP (U/L) | 176.69 ± 442.60 | 191.62 ± 450.00 | 302.05 ± 467.57 | 143.84 ± 319.78 | 165.82 ± 328.88 |
Albumin (g/dl) | 0.29 ± 3.37 | 0.36 ± 3.24 | 0.69 ± 3.30 | 0.46 ± 3.18 | 7.05 ± 3.68 |
PT (sec.) | 0.26 ± 12.60 | 0.44 ± 12.72 | 1.04 ± 12.78 | 0.33 ± 12.77 | 0.26 ± 12.73 |
INR | 0.75 ± 1.05 | 0.07 ± 1.05 | 0.07 ± 1.05 | 0.00 ± 1.10 | 0.09 ± 1.09 |
Time LFTs | At Diagnosis N = 45 |
Pre KPE N = 45 |
One Month post KPE N = 45 |
Six Months post KPE N = 45 |
At last follow up N = 45 |
SD ± Mean | SD ± Mean | SD ± Mean | SD ± Mean | SD ± Mean | |
BILI.T (mg/dl) | 2.85 ± 11.02 | 3.21 ± 12.00 | 3.69 ± 8.87 | 5.55 ± 11.37 | 6.09 ± 14.19 |
BILI.D (mg/dl) | 1.93 ± 7.48 | 2.51 ± 8.36 | 2.45 ± 6.18 | 4.09 ± 8.30 | 4.76 ± 10.78 |
ALT (U/L) | 113.63 ± 135.62 | 66.83 ± 121.02 | 103.64 ± 127.98 | 78.05 ± 81.49 | 57.13 ± 73.90 |
AST (U/L) | 126.39 ± 180.80 | 98.25 ± 193.76 | 82.05 ± 162.51 | 83.49 ± 120.91 | 86.04 ± 126.24 |
GGT (U/L) | 526.86 ± 785.67 | 646.84 ± 868.58 | 630.94 ± 1023.31 | 550.39 ± 771.97 | 437.84 ± 462.06 |
ALP (U/L) | 256.21 ± 458.24 | 303.18 ± 522.78 | 295.26 ± 480.91 | 387.52 ± 588.84 | 212.89 ± 453.24 |
Albumin (g/dl) | 0.48 ± 3.38 | 0.43 ± 3.29 | 1.49 ± 3.32 | 0.64 ± 2.88 | 0.69 ± 2.60 |
PT (sec.) | 1.19 ± 12.99 | 0.68 ± 12.86 | 1.77 ± 12.79 | 1.75 ± 14.07 | 7.51 ± 16.35 |
INR | 0.13 ± 1.07 | 0.11 ± 1.08 | 0.11 ± 1.06 | 0.23 ± 1.29 | 0.31 ± 1.31 |
Groups LFTs | Successful Outcome N = 15 | Failed Outcome N = 45 | P - Value |
SD ± Mean | SD ± Mean | ||
BILI.T (mg/dl) | 3.36 ± 5.53 | 3.69 ± 8.87 | 0.01 |
BILI.D (mg/dl) | 2.49 ± 3.79 | 2.45 ± 6.18 | 0.005 |
ALT (U/L) | 58.56 ± 85.60 | 103.64 ± 127.98 | 0.189 |
AST (U/L) | 74.32 ± 134.33 | 82.05 ± 162.51 | 0.213 |
GGT (U/L) | 616.63 ± 882.57 | 630.94 ± 1023.31 | 0.318 |
ALP (U/L) | 302.05 ± 467.57 | 295.26 ± 480.91 | 0.887 |
Albumin (g/dl) | 0.69 ± 3.30 | 1.49 ± 3.32 | 0.423 |
PT (sec.) | 1.04 ± 12.78 | 1.77 ± 12.79 | 0.480 |
INR | 0.07 ± 1.05 | 0.11 ± 1.06 | 0.854 |
Groups LFTs | Successful Outcome N = 15 | Failed Outcome N = 45 | P - Value |
SD ± Mean | SD ± Mean | ||
BILI.D (mg/dl) | 0.46 ± 0.51 | 4.09 ± 8.30 | 0.0001 > |
ALT (U/L) | 79.85 ± 86.85 | 78.05 ± 81.49 | 0.547 |
AST (U/L) | 48.98 ± 97.46 | 83.49 ± 120.91 | 0.347 |
GGT (U/L) | 244.12 ± 553.36 | 550.39 ± 771.97 | 0.308 |
ALP (U/L) | 143.84 ± 319.78 | 387.52 ± 588.84 | 0.033 |
Albumin (g/dl) | 0.46 ± 3.18 | 0.64 ± 2.88 | 0.167 |
PT (sec.) | 0.33 ± 12.77 | 1.75 ± 14.07 | 0.002 |
INR | 0.00 ± 1.10 | 0.23 ± 1.29 | 0.370 |
Parameter | AUROC | Cutoff | P- value | Sensitivity % | Specificity% | PPV % | NPV % | Accuracy % |
BILI.T (mg/dl) | 0.724 | 6.2 | 0.01 | 60% | 82.2% | 52.9% | 86% | 80% |
BILI.D (mg/dl) | 0.746 | 4.6 | 0.005 | 66.7% | 75.6% | 47.6% | 87.2% | 73.3% |
Groups Survival | Successful outcome N = 15 | Failed outcome N = 45 | P1- value | Neglected BA N = 20 | -P2 Value | -P3 Value |
Mean | Mean | Mean | ||||
Means for Survival Time (Month) | 80.44 | 18.95 | 0.0001 > | 17.96 | 0.0001 > | 0.47 |
P1-value: successful outcome vs. failed outcome, P2-value: successful outcome vs. neglected BA, P3-value: failed outcome vs.
neglected BA.
Groups Survival | Successful outcome N = 15 | Failed outcome N = 45 | P value |
Mean | Mean | ||
Survival Time post KPE (Months) | 41.25 | 16.41 | 0.0001 > |
Cumulative Survival (Months) | Successful Outcome % | Failed Outcome % | Neglected BA % |
6 Months | 100% | 100% | 95% |
12 Months | 100% | 79.2% | 88.2% |
18 Months | 100% | 72% | 66.2% |
24 Months | 80% | 50.4% | - |
36 Months | 80% | 27.7% | - |
48 Months | 80% | 10.5% | - |
Cumulative Survival post-KPE (Months) |
Successful Outcome % | Failed Outcome % |
6 Months | 100% | 100% |
12 Months | 100% | 43% |
18 Months | 100% | 24.5% |
24 Months | 75% | 16.4% |
36 Months | 75% | 10.9% |
48 Months | 75% | - |
Cumulative Survival rate post-KPE (Months) |
Post-KPE % |
6 Months | 79.6% |
12 Months | 43.7% |
18 Months | 30.6% |
24 Months | 21.4% |
36 Months | 17.8% |
48 Months | 17.8% |


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