ABSTRACT
Phenylketonuria (PKU) is the most frequent inherited disorder of amino acid metabolism. In our previous work, we investigated the role of NADPH oxidase (NOX) in a Pahenu2-BTBR PKU mouse model, and an in vitro cell culture model of PKU. In the current study, we evaluated various oxidative stress parameters, namely total antioxidant capacity (T-AOC), glutathione (GSH) and maleic dialdehyde (MDA) in the plasma of 40 PKU children, for further investigating the oxidative molecular regulation mechanism of NOX in PKU. It was observed that T-AOC and GSH markedly decreased in PKU as compared with the control group (P<0.01), and seemed to correlate negatively with Phe level. However, there was no statistical difference in MDA level among the three groups. And 8-isoprostane in the blood samples of PKU2 groups was slightly higher than control group (P<0.05). Additionally, mRNA levels of subunits of NOX included p47(phox) and p67(phox) significantly increased in PKU group (P<0.01). These results reflected that NOX is the important source of reactive oxygen species and is involved in the oxidative molecular regulation mechanism in PKU, which shows a new perspective toward understanding the biological underpinnings of PKU.
Subject(s)
Leukocytes, Mononuclear/metabolism , NADPH Oxidases/blood , Phenylketonurias/blood , Phenylketonurias/pathology , Aldehydes/metabolism , Analysis of Variance , Apoptosis , Child , Child, Preschool , Dinoprost/analogs & derivatives , Dinoprost/blood , Female , Flow Cytometry , Glutathione/genetics , Glutathione/metabolism , Humans , Infant , Infant, Newborn , Male , NADPH Oxidases/genetics , RNA, Messenger/metabolismABSTRACT
OBJECTIVE: To investigate the value of Glasgow Coma Scale (GCS) and Glasgow-Pittsburgh Coma Scale (GCS-P) scoring in predicting the prognosis of coma in children. METHODS: Clinical data of 17 comatose children were retrospectively reviewed. The results of GCS and GCS-P scoring, electroencephalogram (EEG) and cranial imaging were analyzed. Dynamic curves of GCS-P score were drawn. RESULTS: Seven patients received EEG examination and four showed low potential. The four patients had poor prognosis. Cranial CT and MRI were performed in 12 patients. Of these three showed cerebral hemorrhage and ischemia and had a poor prognosis. The accuracy rate for predicting the prognosis of GCS and GCS-P scoring was 85.71% and 88.57% respectively. A continuous GCS-P scoring was performed in 13 patients. A dynamic GCS-P curve showed an ascent in seven cases with good prognosis but a flat or declined tendency in six cases with poor prognosis. CONCLUSIONS: GCS-P scoring is valuable for predicting prognosis in children with coma. Combined with EEG and cranial imaging examinations, the accuracy for predicting prognosis of GCS-P scoring will increase.
