Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UMLS:C0029713 (
immaturity
)
4,335
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
The activity, amount and protein composition of
pyruvate dehydrogenase
(
PDH
) and respiratory chain complexes were studied in muscle mitochondria obtained postmortally from 6 neonates with a gestational age of 23-29 weeks. The activities of
PDH
and respiratory chain complex III and IV and citrate synthase were significantly lower in comparison with control children aged 0.5-2 and 2-20 years. Protein analyses revealed a parallel decrease in the content of
PDH
, respiratory chain complexes and their subunits in the cases analyzed. The observed
immaturity
of the mitochondrial energy-providing system suggests that significant development of mitochondrial energy metabolism occurs during the last 3 months of prenatal development. The metabolic disturbances of mitochondrial energy conversion associated with the low functional capacity and content of
PDH
and respiratory chain complexes may play an important role in the morbidity of very premature neonates.
...
PMID:Mitochondrial energy metabolism in very premature neonates. 1201 66
Transient hyperinsulinism (HI) occurs in infants born to diabetic mothers, in infants experiencing perinatal asphyxia and in infants with intrauterine growth retardation. The precise mechanism of transient HI in these different aetiologies is not fully understood. Lactic acidosis is commonly seen in neonates as a secondary phenomenon due to hypoxia, hypovolaemia, anaemia and infection. The combination of transient HI and lactic acidosis is rare. We present the clinical and biochemical features of five infants presenting with transient HI associated with hyperlactataemia in the absence of markers of perinatal stress. This combination lasted for 3-4 weeks with complete resolution except in one patient in whom the hyperinsulinism lasted until 6 months before resolution. The precise mechanism of this association is not clear but may be related either to
immaturity
of the
pyruvate dehydrogenase complex
or to the accumulation of abnormal intramitochondrial intermediary metabolites. Infants presenting with HI should have a free flowing blood sample drawn for the measurement of plasma lactate levels.
...
PMID:Severe transient neonatal hyperinsulinism associated with hyperlactataemia in non-asphyxiated infants. 1505 55
