Review
Copyright ©The Author(s) 2021.
World J Transplant. Jun 18, 2021; 11(6): 161-179
Published online Jun 18, 2021. doi: 10.5500/wjt.v11.i6.161
Table 1 Metabolic disorders can be classified into the following three broad groups based on a liver transplantation perspective
Category
Description
Examples
ADisorders with enzyme defect only in the liver and LT is done predominantly for ESLD and its related complicationsTyrosinemia (HT-1); A1AT deficiency; Galactosemia (type 1 Galactosemia); WD; Hereditary fructosemia; GSD 3 and 4; CDG; LSD
BDisorders with enzyme defects limited to the liver. These patients rarely have ESLD; LT is performed for extrahepatic organ involvement UCD; Porphyrias; GSD type 1: PH, FH; Crigler-Najjar syndrome
CDisorders having enzyme defect in the liver and extrahepatic tissue and LT only partially corrects underlying metabolic disease and alleviates symptoms of extrahepatic organ involvementMMA; PA; MSUD; MH
Table 2 The New Wilson (Dhawan) score for prediction of outcome in Wilson disease
Score
Bilirubin mg/dL
INR
AST (IU/L)
WBC (109/L)
Albumin (g/dL)
0< 5.85< 1.29< 100< 6.74.5
15.86-8.771.3-1.6101-1506.8-8.33.4-4.4
28.78-11.691.7-1.9151-2008.4-10.32.5-3.3
311.7-17.542.0-2.4201-30010.4-15.32.1-2.4
4> 17.55> 2.5> 300> 15.3< 2.1