Type II citrullinemia in an elderly patient treated with living related partial liver transplantation

Kiyoyuki Takenaka, Ichiro Yasuda*, Hiroshi Araki, Tomoo Naito, Yasushi Fukutomi, Hiroo Ohnishi, Noriyoshi Yamakita, Takeshi Hasegawa, Hirohide Sato, Yasunobu Shimizu, Hidetoshi Matsunami, Hisataka Moriwaki

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

30 Scopus citations

Abstract

A 60-year-old woman was admitted to our hospital for repeated consciousness disturbance. Blood examination showed hyperammonemia, and plasma amino acid analysis revealed a marked increase in the citrulline level. To establish a diagnosis, a percutaneous needle biopsy of the liver was performed. The determination of the urea cycle enzyme activities revealed a selective marked decrease in argininosuccinate synthetase activity, indicating the final diagnosis of type II citrullinemia. The mean survival period of this disease after the appearance of symptoms has been reported as 26.4 months, and most conservative treatments are not effective. We performed a living related partial liver transplantation. Over the subsequent 13-month follow-up, the patient's condition has remained fairly good.

Original languageEnglish
Pages (from-to)553-558
Number of pages6
JournalInternal Medicine
Volume39
Issue number7
DOIs
StatePublished - 2000/07

Keywords

  • Argininosuccinate synthetase
  • Consciousness disturbance
  • Hyperammonemia

ASJC Scopus subject areas

  • Internal Medicine

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