Journal List > J Korean Soc Transplant > v.25(3) > 1034339

Kim and Choe: Indication of Pediatric Liver Transplantation

Abstract

Pediatric liver transplantation is a well-established and successful strategy. Survival after liver transplantation has improved significantly over the last decade with pediatric recipients. Biliary atresia is the most common indication of pediatric liver transplantation and needs adequate selection of patients and timing of transplantation in order to achieve best results. In fulminant hepatic failure, laboratory data and neurological signs help decide the need for transplantation and determine the prognosis. A judicious communication between physicians and parents is important for deciding appropriate time of liver transplantation.

References

1). Starzl TE, Marchioro TL, Vonkaulla KN, Hermann G, Brittain RS, Waddell WR. Homotransplantation of the liver in humans. Surg Gynecol Obstet. 1963; 117:659–76.
2). Kim ST, Park YH, Lee KU, Yoon YK, Kim SW, Yang HK, et al. An experience of liver transplantation in Korea. J Korean Soc Transplant. 1988; 2:27–35.
3). Lee SG, Lee YJ, Kwon TW, Kim KM, Choi KM, Park KM, et al. Liver transplantation from a living donor to his daughter. J Korean Soc Transplant. 1995; 9:187–92.
4). Jain A, Mazariegos G, Kashyap R, Kosmach-Park B, Starzl TE, Fung J, et al. Pediatric liver transplantation. A single center experience spanning 20 years. Transplantation. 2002; 73:941–7.
5). Oh SH, Kim KM, Kim DY, Lee YJ, Rhee KW, Jang JY, et al. Long-term outcomes of pediatric living donor liver transplantation at a single institution. Pediatr Transplant. 2010; 14:870–8.
crossref
6). Goss JA, Shackleton CR, Swenson K, Satou NL, Nuesse BJ, Imagawa DK, et al. Orthotopic liver transplantation for congenital biliary atresia. An 11-year, singlecenter experience. Ann Surg. 1996; 224:276–84. discussion 284–7.
7). Trey C, Davidson CS. The management of fulminant hepatic failure. Prog Liver Dis. 1970; 3:282–98.
8). Bernuau J, Rueff B, Benhamou JP. Fulminant and sub-fulminant liver failure: definitions and causes. Semin Liver Dis. 1986; 6:97–106.
crossref
9). Squires RH Jr, Shneider BL, Bucuvalas J, Alonso E, Sokol RJ, Narkewicz MR, et al. Acute liver failure in children: the first 348 patients in the pediatric acute liver failure study group. J Pediatr. 2006; 148:652–8.
crossref
10). Sternlieb I. Wilson' s disease: indications for liver transplants. Hepatology. 1984; 4(1 Suppl):15S–7S.
11). Kuang AA, Rosenthal P, Roberts JP, Renz JF, Stock P, Ascher NL, et al. Decreased mortality from technical failure improves results in pediatric liver transplantation. Arch Surg. 1996; 131:887–92. discussion 892–3.
crossref
12). McDiarmid SV, Merion RM, Dykstra DM, Harper AM. Selection of pediatric candidates under the PELD system. Liver Transpl. 2004; 10(10 Suppl 2):S23–30.
crossref
13). Shinkai M, Ohhama Y, Take H, Fukuzato Y, Fujita S, Nishi T. Evaluation of the PELD risk score as a severity index of biliary atresia. J Pediatr Surg. 2003; 38:1001–4.
crossref

Table 1.
Indications for liver transplantation at Children’ s Hospital of Pittsburgh from 1981 to 1998
Indications for liver transplantation n=808 (%)
Biliary atresia 417 (51.6)
Metabolic disease 123 (15.2)
Alpha 1 antitrypsin deficiency 63 (7.8)
Wilson disease 16 (2.0)
Tyrosinemia 13 (1.6)
    Glycogen storage disease 13 (1.6)
Oxalosis 7 (0.9)
Ornithine transcarbamyl deficiency 3 (0.4)
Fulminant hepatitis 50 (6.2)
NANB hepatitis 33 (4.1)
Cryptogenic cirrhosis 30 (3.7)
Familial cholestasis 27 (3.3)
Hepatic malignancy 23 (2.8)
Neonatal hepatitis 20 (2.5)
Secondary biliary cirrhosis 17 (2.1)
Congenital hepatic fibrosis 15 (1.9)
Autoimmune hepatitis 8 (1.0)
Primary sclerosing cholangitis 7 (0.9)
HCV 2 (0.2)
Table 2.
Underlying causes of 144 liver transplantation at Samsung Medical Center from 1996 to 2010
Underlying causes n=144 (%)
Biliary atresia 94 (65.3)
Fulminant hepatic failure 12 (8.3)
Metabolic disease 10 (6.9)
Wilson disease 6 (4.1)
Glycogen storage disease 3 (2.1)
Propionic acidemia 1 (0.7)
Alagille syndrome 8 (5.6)
Hepatic malignancy 7 (4.9)
Neonatal hepatitis 6 (4.2)
Cryptogenic cirrhosis 4 (2.8)
Congenital hepatic fibrosis 1 (0.7)
Autoimmune hepatitis 1 (0.7)
Liver abscess 1 (0.7)
Table 3.
Indications for liver transplantation at Asan Medical Center from 1994 to 2006
Indications for liver transplantation n=115 (%)
Biliary atresia 70 (60.9)
Fulminant hepatic failure 17 (14.8)
Metabolic disease 12 (10.4)
Wilson disease 8 (7.0)
Urea cycle disorder 3 (2.6)
Protein C deficiency 1 (0.9)
Cryptogenic cirrhosis 5 (4.3)
Alagille syndrome 4 (3.5)
Progressive familial intrahepatic cholestasis 2 (1.7)
Retransplantation 3 (2.6)
Congenital hepatic fibrosis 1 (0.9)
Bile duct obstruction 1 (0.9)
Table 4.
The study of Fulminant hepatic failure at Samsung Medical Center from 1996 to 2010
  Sex/Age Causes Duration from jaundice to coma (day) Duration from jaundice to LT (day) Duration from coma to LT (hr) Coma stage Survival
1 M/8 mo Rotavirus 0 8 192 3 Expired in 6 yr
2 F/14 yr Unknown 55 60 67 3 Alive
3 M/10 mo Herb medicine 0 3 72 3 Expired in 15 d
4 M/4 yr Unknown 5 7 48 3 Alive
5 M/26 mo Unknown 9 12 72 2 Alive
6 M/4 mo Unknown 15 16 19 2∼3 Expired in 17 d
7 M/9 mo Unknown 2 3 24 2∼3 Expired in 11 mo
8 F/14 mo Unknown 8 9 24 2∼3 Alive
9 F/12 yr Herb medicine 15 17 47 3 Alive
10 M/3 yr Unknown 11 12 27 3 Alive
11 M/30 mo Unknown 2 6 47 3 Alive

Abbreviation: LT, liver transplantation.

TOOLS
Similar articles