Model for end-stage liver disease (MELD) exception for unusual metabolic liver diseases

作者: Sue McDiarmid , Robert G. Gish , Simon Horslen , George V. Mazariegos

DOI: 10.1002/LT.20973

关键词: Central nervous systemContraindicationLiver transplantationLiver diseaseBioinformaticsMedicineDiseaseLiver function testsCirrhosisImmunologyModel for End-Stage Liver DiseaseHepatologySurgeryTransplantation

摘要: Metabolic liver disease is the underlying diagnosis in only a small proportion of patients who undergo transplantation (LT), but for these patients, LT lifesaving. Patients with metabolic often do not present typical findings end-stage and require special consideration scrutiny concerning appropriateness timing LT. Liverbased classified into 3 types: (1) that causes structural damage failure or cirrhosis, (2) without affects other organs (especially central nervous system), (3) systemic deficiencies are partially represented liver. There may be overlap presentation, some forms presenting either disease. General considerations affect review board decisions include relative contraindication use living-related donor unpredictable course cause severe system complications several states. Also, although many diseases mostly children, adolescents adults previously managed medically increasingly when medical management becomes more difficult complex as they mature.

参考文章(55)
Shao-Yin Chu, Huey-Ling Chen, Yin-Hsiu Chien, Po-Huang Lee, Frank Leigh Lu, Wuh-Liang Hwu, Ming-Je Ho, Jui-Yu Hsui, Living-related liver transplantation for methylmalonic acidemia: report of one case. Acta paediatrica Taiwanica. ,vol. 44, pp. 171- 173 ,(2003) , 10.7097/APT.200306.0171
E. Santos Silva, E. Martins, M. L. Cardoso, C. Barbot, L. Vilarinho, M. Medina, Liver transplantation in a case of argininaemia Journal of Inherited Metabolic Disease. ,vol. 24, pp. 885- 887 ,(2001) , 10.1023/A:1013960712516
Richard A. Marlar, Robert R. Montgomery, Andre W. Broekmans, the Working Party, Diagnosis and treatment of homozygous protein C deficiency The Journal of Pediatrics. ,vol. 114, pp. 528- 534 ,(1989) , 10.1016/S0022-3476(89)80688-2
L. Faivre, D. Houssin, J. Valayer, J. Brouard, M. Hadchouel, O. Bernard, Long-term outcome of liver transplantation in patients with glycogen storage disease type Ia. Journal of Inherited Metabolic Disease. ,vol. 22, pp. 723- 732 ,(1999) , 10.1023/A:1005544117285
Martin M. Burdelski, Atef F. Bassas, Dieter C. Broering, Sami H. Wali, Xavier G. Rogiers, Haider A. Al-Shurafa, Management of Crigler-Najjar Syndrome type I. Saudi Medical Journal. ,vol. 22, pp. 486- 489 ,(2001)
Akiko Tamamori, Yoshiyuki Okano, Hajime Ozaki, Akie Fujimoto, Masue Kajiwara, Kazuyoshi Fukuda, Keiko Kobayashi, Takeyori Saheki, Yasuko Tagami, Tsunekazu Yamano, Neonatal intrahepatic cholestasis caused by citrin deficiency: severe hepatic dysfunction in an infant requiring liver transplantation. European Journal of Pediatrics. ,vol. 161, pp. 609- 613 ,(2002) , 10.1007/S00431-002-1045-2
CARL G. GROTH, OLLE RINGDEN, Transplantation in relation to the treatment of inherited disease. Transplantation. ,vol. 38, pp. 319- 326 ,(1984) , 10.1097/00007890-198410000-00001
B. Gridelli, A. Lucianetti, S. Gatti, M. Colledan, R. Benti, A. Bruno, L.N. Rossi, L.R. Fassati, Orthotopic liver transplantation for Crigler-Najjar type I syndrome☆ International Congress of the Transplantation Society. ,vol. 29, pp. 440- 441 ,(1997) , 10.1016/S0041-1345(96)00179-0
Raili Kauppinen, Kaisa Timonen, Pertti Mustajoki, Treatment of the porphyrias. Annals of Medicine. ,vol. 26, pp. 31- 38 ,(1994) , 10.3109/07853899409147324