Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1988-6-29
pubmed:abstractText
Two patients are described with hyperammonemia due to ornithine transcarbamylase (OTC) deficiency who suffered severe shrinkage and collapse of the brain. The cerebral cortex was spongy and cavitated, containing only a few residual neurons, and was markedly gliosed. In one patient the basal ganglia were affected and harbored Alzheimer type II astrocytes. These lesions resemble those of acquired hepatocerebral degeneration and occur especially in female children with the milder form of the disease, who have a potential to survive. Strict observance of dietary restrictions is mandatory to avoid catastrophic damage to the brain.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
0722-5091
pubmed:author
pubmed:issnType
Print
pubmed:volume
7
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
10-5
pubmed:dateRevised
2009-11-19
pubmed:meshHeading
pubmed:articleTitle
Severe cerebral damage in ornithine transcarbamylase deficiency.
pubmed:affiliation
Pathology Laboratories, Vancouver General Hospital, B.C., Canada.
pubmed:publicationType
Journal Article, Case Reports