Document Detail


Clinical features and outcome of eight infants with intrahepatic porto-venous shunts detected in neonatal screening for galactosaemia.
MedLine Citation:
PMID:  9686654     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Of 18 newborn infants found to have persistent galactosaemia and without enzyme deficiencies, intrahepatic porto-venous (P-V) shunts were the cause in 8 cases. We retrospectively analysed the clinical and biochemical features of the eight infants. Four patients received prednisolone, one of whom with heart failure owing to arteriovenous shunts also underwent hepatic arterial embolization. The other four patients were merely observed without receiving drug therapy. Regardless of treatment, the P-V shunts disappeared in five infants before the age of 1 y and persisted in three others. All infants showed mild or moderate abnormalities in liver function tests. None exhibited hyperammonemia or neuropsychiatric symptoms related to the shunts. The data indicated that the prognosis of infants with intrahepatic P-V shunts is generally good. In the absence of complications related to the P-V shunts, no treatment other than galactose elimination diet is indicated.
Authors:
H Ono; H Mawatari; N Mizoguchi; T Eguchi; N Sakura
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Acta paediatrica (Oslo, Norway : 1992)     Volume:  87     ISSN:  0803-5253     ISO Abbreviation:  Acta Paediatr.     Publication Date:  1998 Jun 
Date Detail:
Created Date:  1998-10-06     Completed Date:  1998-10-06     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9205968     Medline TA:  Acta Paediatr     Country:  NORWAY    
Other Details:
Languages:  eng     Pagination:  631-4     Citation Subset:  IM    
Affiliation:
Department of Paediatrics, Hiroshima University School of Medicine, Japan.
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MeSH Terms
Descriptor/Qualifier:
Antineoplastic Agents, Hormonal / therapeutic use
Female
Galactosemias / diagnosis*,  etiology,  therapy
Hemangioma / congenital*,  diagnosis,  drug therapy,  etiology
Hepatic Veins / abnormalities*
Humans
Infant, Newborn
Liver Neoplasms / congenital*,  diagnosis,  drug therapy,  etiology
Male
Mass Screening*
Portal System / abnormalities
Portal Vein / abnormalities*
Prednisolone / therapeutic use
Remission, Spontaneous
Retrospective Studies
Risk Factors
Vascular Fistula / complications,  congenital*,  diagnosis
Chemical
Reg. No./Substance:
0/Antineoplastic Agents, Hormonal; 50-24-8/Prednisolone

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