Document Detail


A severely brain-damaged case of 3-hydroxyisobutyric aciduria.
MedLine Citation:
PMID:  11377004     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report a male case of 3-hydroxyisobutyric aciduria (3HiB-uria) with severe brain damage. He had mild asphyxia at birth. He needed tube feeding for a month. He showed mild dysmorphic features, including low set ears, a long philtrum and micrognathia. At 4 months of age he had acute encephalopathy. Thereafter, severe brain damage remained and mechanical ventilation care was needed all day. After he had been admitted to our hospital at 3 years of age, repeated organic acid analysis of urine confirmed the diagnosis of 3HiB-uria. This patient had been previously diagnosed as having cerebral palsy and sequelae of acute encephalopathy.
Authors:
M Sasaki; H Iwata; K Sugai; M Fukumizu; M Kimura; S Yamaguchi
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Brain & development     Volume:  23     ISSN:  0387-7604     ISO Abbreviation:  Brain Dev.     Publication Date:  2001 Jul 
Date Detail:
Created Date:  2001-05-29     Completed Date:  2001-08-02     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  7909235     Medline TA:  Brain Dev     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  243-5     Citation Subset:  IM    
Affiliation:
Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry (NCNP), 4-1-1 Ogawahigashi-cho, Kodaira, 187-8551, Tokyo, Japan.
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MeSH Terms
Descriptor/Qualifier:
Acidosis / drug therapy,  urine*
Brain Damage, Chronic / etiology*
Child
Humans
Hydroxybutyrates / urine*
Male
Metabolism, Inborn Errors / diagnosis,  drug therapy,  urine*
Chemical
Reg. No./Substance:
0/Hydroxybutyrates; 2068-83-9/3-hydroxyisobutyric acid

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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