Oral sodium phenylbutyrate for hyperammonemia associated with congenital portosystemic shunt: A case report

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Abstract

Objectives: The efficacy of sodium phenylbutyrate (SPB) for hyperammonemia associated with congenital portosystemic shunt (CPSS) remains unknown. We show the effectiveness of oral SPB. Case presentation: Our patient had CPSS with severe hypoplasia of extrahepatic portal veins. At 9 months of age, to assess the efficacy of oral SPB, we evaluated the 24 h fluctuations of venous ammonia levels. In the first two days without SPB, ammonia levels were above 80 μmol/L for half a day. On the third and fourth days, administration of oral SPB three times a day decreased ammonia to acceptable levels, except at midnight. On the fifth day, another oral SPB administration at 8 pm decreased ammonia at midnight. Low levels of branched-chain amino acids, as well as coagulation disturbances, were observed without apparent symptoms. At 12 months of age, he showed normal psychomotor development. Conclusions: Oral SPB may be effective for hyperammonemia associated with CPSS.

Original languageEnglish
Pages (from-to)407-410
Number of pages4
JournalJournal of Pediatric Endocrinology and Metabolism
Volume34
Issue number3
DOIs
Publication statusPublished - 2021 Mar 1

Keywords

  • Congenital portosystemic shunt
  • Hyperammonemia
  • Sodium phenylbutyrate

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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