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Metabolic improvements in intrahepatic porto-systemic venous shunt presenting various metabolic abnormalities by 4-phenylacetate.
MedLine Citation:
PMID:  23399721     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
BACKGROUND: Intrahepatic congenital portosysstemic venous shunt (CPSVS) presents hyperammonemia, cholestasis, hypergalactosemia and imbalanced vasomediators. Especially, fluctuating plasma ammonia often causing neurological signs and symptoms is a serious problem in the daily life. 4-phenylacetate (4-PA) has effects to eliminate blood ammonia, bile acids and bilirubin. 4-PA might be expected to improve the metabolic abnormalities in intrahepatic CPSVS. . METHODS: Three intrahepatic CPSVS children often receiving 4-PA from early life were enrolled. We analyzed biological and clinical changes by intravenous administration of 4-PA, RESULTS: 4-PA improved hyperammonemia enough to subside the clinical presentations: headache, cognition dysfunction and attention deficit. Concurrently, this drug decreased serum total bilirubin and total bile acids levels. In their neonatal ages, 4-PA also decreased galactose and galactose-1-phosphate levels. In their preschool or school ages, 4-PA increased nitric oxide (NO) prompting vasodilation, but not changed amino acids controlling NO production and endothelin-1 prompting vasoconstriction. Plasma ammonia level returned to the pre-administration level within one day of the discontinuation, and serum total bilirubin and total bile acid levels were maintained to be reduced a few days after the discontinuation. CONCLUSION: 4-PA improves galactosemia and imbalanced vasomediators, together with liver functions, in CPSVS, although such effects retract after the discontinuation.
Authors:
Hironori Nagasaka; Takashi Miida; Tohru Yorifuji; Ken-Ichi Hirano; Ayano Inui; Tomoo Fujisawa; Hirokazu Tsukahara; Hisamitsu Hayashi; Yukihiro Inomata
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-2-8
Journal Detail:
Title:  Clinica chimica acta; international journal of clinical chemistry     Volume:  -     ISSN:  1873-3492     ISO Abbreviation:  Clin. Chim. Acta     Publication Date:  2013 Feb 
Date Detail:
Created Date:  2013-2-12     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  1302422     Medline TA:  Clin Chim Acta     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 Elsevier B.V. All rights reserved.
Affiliation:
Department of Pediatrics, Takarazuka City Hospital, Takarazuka, Japan. Electronic address: nagasa-hirono@k2.dion.ne.jp.
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