Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
1985-12-24
pubmed:abstractText
Two autopsy cases of neonatal argininosuccinate synthetase (ASS) deficiency demonstrating the particular histological changes of the liver are presented. Case 1 was a female infant with elevated blood ammonia and citrulline. The patient died of sepsis at nineteen days after birth. Autopsy revealed hematomas in bilateral cerebral hemispheres and a yellow liver parenchyma. Histologically, the cerebrum showed diffuse astrogliosis with Alzheimer type II cell and swollen cytoplasm. Status spongiosus and gliosis were observed in the subthalamic and pontine nuclei. The liver demonstrated fatty degeneration and wide portal space with bile duct proliferation and inflammatory cell infiltration. The ASS activities in the liver and the kidneys were not detected. Case 2 was a female infant who died of respiratory distress twenty-four days after birth. Autopsy revealed hematomas in the bilateral cerebral ventricles and a liver with yellow parenchyma. Histologically, there was destruction of arrangement in the cerebrum and cerebellum together with marked decrease of nerve cells with gliosis and phagocytosis. The liver showed mild fatty degeneration and wide portal space accompanied by bile duct proliferation and inflammatory cell infiltration. The ASS activities in the liver and kidneys were not detected.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jul
pubmed:issn
0001-6632
pubmed:author
pubmed:issnType
Print
pubmed:volume
35
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
995-1006
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1985
pubmed:articleTitle
Neonatal type of argininosuccinate synthetase deficiency. Report of two cases with autopsy findings.
pubmed:publicationType
Journal Article, Case Reports