Abstract
Prior to 1987 all pts with HT in Finland died in early years. In 1987 OLT on children was introduced in our country and since then 5 pts wirh HT have been diagnosed by high plasma tyrosine and urinary succinyl acetone (SAA) concentrations and enzyme analysis. In one pt, HT manifested acutely with bleeding, ascites and edema at 6 wks. Four presented with liver insufficiency and tubulopathy at 3 to 7 mos. Serurm α-fetoprotein (AFP) was 130-11000 times age matched median and liver CT showed tumor like nodules in most pts. The pts were treated by low tyrosine + low phenylalanine diet. In the pt with acute HT, blood exchanges and i.v. glutathione were used. One pt had several porphyria episodes which responded well to i.v. hemiarginate.
Reduced size OLT has been performed in 3 pts at the ages of 4, 12 and 27 mos. Serum AFP normalized within 6 wks. Four to 8 mos after OLT all pts are in excellent clinical condition, liver function tests and serum creatinine concentration are normal. All have low sustained SAA excretion. Two pts are waiting for OLT.
In conclusion: The short term results of reduced size OLT in our pts are excellent. High risk of hepatic malignoma makes early OLT the treatment of choice in HT.
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Salo, M., Holmberg, C., Heikinheimo, M. et al. 91 REDUCED SIZE ORTHOTOPIC LIVER TRANSPLANTATION (OLT) IN SMALL CHILDREN WITH HEREDITARY TYROSINEMIA (HT). Pediatr Res 28, 292 (1990). https://doi.org/10.1203/00006450-199009000-00115
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DOI: https://doi.org/10.1203/00006450-199009000-00115
