[Surgical and metabolic aspects of liver transplantation for tyrosinemia].
Tyrosinemia represents a very small percentage of patients undergoing liver transplantation world-wide. This disease is endemic within our referral area however, one-third of the liver transplantations at our institution are done for this disease. Since 1986, 16 patients with tyrosinemia and 34 patients with various other indications (non-tyrosinemic) have undergone a total of 55 liver transplantation. The survival rate for tyrosinemic patients is 87%, compared to 75% for non-tyrosinemic patients. Liver transplantation for hereditary tyrosinemia and other metabolic disorders without portal hypertension or previous portohepatic operations is notably easier to perform. Intraoperative blood loss was less, length of hospital stay was shorter and incidence of infections was lower in tyrosinemic than in non-tyrosinemic patients. Less than 10% of tyrosinemic patients had foci of hepatocellular carcinoma at the time of transplantation. For this reason, and while most patients with tyrosinemia will eventually require liver transplantation, our results do not support systematic early transplantation.
Bruneau
N
N
Hôpital Sainte-Justine, Montréal, Québec.
St-Vil
D
D
Luks
F I
FI
LaBerge
J M
JM
Bensoussan
A L
AL
Blanchard
H
H
D000293:Adolescent; D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications / Q000401:mortality / Q000601:surgery* / Q000652:urine; D000622:Aminolevulinic Acid / Q000652:urine; D002493:Central Nervous System Diseases / Q000209:etiology / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006537:Heptanoates / Q000652:urine; D006801:Humans; D007223:Infant; D007677:Kidney Function Tests; D016030:Kidney Transplantation; D017093:Liver Failure / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods* / Q000401:mortality; D008297:Male; D011182:Postoperative Care; D011300:Preoperative Care; D011792:Quebec; D051437:Renal Insufficiency / Q000209:etiology / Q000601:surgery; D014443:Tyrosine / Q000097:blood*
D006537:Heptanoates; D014443:Tyrosine; C020804:succinylacetone; D000622:Aminolevulinic Acid
Les aspects chirurgicaux et métaboliques de la transplantation hépatique pour la tyrosinémie.