Background: Glucose transporter 1 deficiency syndrome (Glut1DS) was initially reported by De Vivo and colleagues in 1991. This disease arises from mutations in the SLC2A1 and presents with a broad clinical spectrum. It is a treatable neuro-metabolic condition, where prompt diagnosis and initiation of ketogenic dietary therapy can markedly enhance the prognosis.
View Article and Find Full Text PDFEpidemiological data suggest a relationship between maternal infection and a high incidence of childhood epilepsy in offspring. However, there is little experimental evidence that links maternal infection with later seizure susceptibility in juvenile offspring. Here, we asked whether maternal immune challenge during pregnancy can alter seizure susceptibility and seizure-associated brain damage in adolescence.
View Article and Find Full Text PDFDysfunction of dopamine D4 receptor (D4R) is linked to attention-deficit/hyperactivity disorder (ADHD) as well as ADHD associated cognitive impairment. Here, we tested the possible therapeutic benefit of the D4R-selective agonist ABT-724 in adolescent spontaneously hypertensive rats (SHRs). ABT-724-treated SHRs were administered ABT-724 (0.
View Article and Find Full Text PDFZhongguo Dang Dai Er Ke Za Zhi
June 2014
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a type of newly recognized autoimmune encephalitis which is commonly seen in children, but its precise etiology is still uncertain. To reveal the etiology of anti-NMDAR encephalitis is very necessary for understanding its pathology, and for starting immune-related therapy as early as possible to improve its prognosis. In the initial literature, tumor, especially teratoma is more related with the anti-NMDAR encephalitis.
View Article and Find Full Text PDFBrain-derived neurotropic factor (BDNF) is a member of the neurotropin family. High levels of BDNF are associated with more aggressive malignant behavior in human cancer. In the present study, we observed the effect of cisplatin on BDNF expression in SH-SY5Y cells and investigated the mechanism of cisplatin in inducing the apoptosis of SH-SY5Y cells.
View Article and Find Full Text PDFUnlabelled: We investigated the potential role of pentraxin 3 (PTX3) in Henoch-Schönlein purpura (HSP), a common multisystemic vasculitis affecting children, as a predictor of Henoch-Schönlein purpura nephritis (HSPN). A total of 108 cases consisting of 34 children with HSP, 37 children with HSPN, and 37 healthy control children were enrolled in this prospective study from March 2010 to February 2013. Blood and urine samples were collected to measure plasma PTX3, C-reactive protein (CRP), serum creatinine, blood urea nitrogen (BUN), urine microalbumin (MALB), and β2-microglobulin (β2-MG).
View Article and Find Full Text PDFObjective: To investigate the effect of ketogenic diet (KD) on the clinical and electroencephalogram features in children with pharmacoresistant epileptic encephalopathy.
Method: Thirty-one children (19 boys, 12 girls) aged 7 months to 7 years (mean 2 years 5 month) with epilepsy refractory to conventional antiepileptic drugs (AEDs) were included in this study. In addition to their original AED treatment, the children were assigned to different ketogenic diets based on their age.
Objective: To retrospectively analyze the clinical characteristics and experience of Chinese children with paraquat poisoning.
Methods: Twenty-two children with paraquat poisoning who presented to the hospital from October 2007 through September 2012 were enrolled into this study. The clinical indices of these cases were collected and analyzed.
Maternal infection during pregnancy is associated with an increased risk of neurodevelopmental injury. Our aim was to investigate whether prenatal immune challenge could alter susceptibility to seizure-induced brain injury in adulthood. Pregnant Wistar rats were injected intraperitoneally with lipopolysaccharide (LPS) or normal saline (NS) at days 15 and 16 of gestation.
View Article and Find Full Text PDFOur aim was to examine whether neonatal lipopolysaccharide (LPS) exposure is associated with changes in microglia and whether these alternations could influence later seizure-induced neurobehavioral outcomes. Male pups were first injected intraperitoneally with either LPS or saline on postnatal day 3 (P3) and postnatal day 5 (P5). Immunohistochemical analysis showed that LPS-treated animals exhibited increased microglia activation that persisted into adolescence.
View Article and Find Full Text PDFThe objective was to investigate the prevalence of attention-deficit hyperactivity disorder (ADHD) in children with frontal lobe epilepsy and related factors. The medical records of 190 children diagnosed with frontal lobe epilepsy at Qilu Hospital of Shandong University between 2006 and 2011 were retrospectively collected, and a follow-up analysis of the prevalence of ADHD in these children was conducted. Of the 161 children with an effective follow-up, 59.
View Article and Find Full Text PDFBased on previous studies, we had made a try to administer sodium pyruvate to newborn Wistar rats suffering repetitive and profound hypoglycemia, which can induce brain injury. Fluoro-Jade B was used to marked degenerative neurons 1 day after the third hypoglycemic insult, and Morris water navigation task was performed to assess cognitive function when the rats were 6 weeks old. We found that administration of sodium pyruvate to those rats whose hypoglycemia was terminated by dextrose can reduce neurodegeneration induced by hypoglycemia and improve the cognitive function.
View Article and Find Full Text PDFObjective: Cleidocranial dysplasia (CCD) is a dominantly inherited skeletal dysplasia caused by mutations in the osteoblast-specific transcription factor-encoding gene, core binding factor α1 (CBFA1). Over 90 mutations in CBFA1 gene have been published to date in 500 independent cases of CCD, including missense mutations, deletions, insertions, frameshift, and splice mutations. However, mutational screening of the CBFA1 gene is still far from saturation, and more novel mutations will be identified to enrich the insights into the molecular basis for the pathogenesis of CCD.
View Article and Find Full Text PDFAstrocyte elevated gene 1 (AEG-1), a novel gene that was cloned in 2002, has emerged in recent years as a potentially crucial mediator of tumor malignancy and aberrant elevation of AEG-1 expression frequently occurs in several human cancers, including breast cancer, prostate cancer, gastric cancer. However, whether AEG-1 deregulation also occurs in neuroblastoma remains unclear. In previous study we reported that AEG-1 was over expressed in neuroblastoma cell lines and knockdown of AEG-1 inhibits proliferation and enhancing chemo-sensitivity to cisplatin or doxorubicin in neuroblastoma cells.
View Article and Find Full Text PDFZhonghua Er Ke Za Zhi
February 2010
Background: Islet beta-cells are almost completely destroyed when patients with type 1 diabete are diagnosed. To date, insulin substitute therapy is still one of the main treatments. The cure of type 1 diabetes requires beta-cell regeneration from islet cell precursors and prevention of recurring autoimmunity.
View Article and Find Full Text PDFEpilepsy is a common neurological disorder that occurs more frequently in childhood than in adulthood. Antiepileptic drugs (AEDs) which are used to treat seizures in pregnant women, infants, and young children may cause cognitive impairment or other uncertain injury. However, the exact mechanisms responsible for adverse effects of AEDs in the developing brain are still not clear.
View Article and Find Full Text PDFDehydroepiandrosterone (DHEA) is an abundant adrenal steroid in serum of humans, and has been reported to have anti-inflammatory, anti-proliferative, and certain immune-regulating properties. Experimental autoimmune neuritis (EAN) is a Th1 cell-mediated animal model of Guillain-Barré syndrome (GBS) in humans. In the present study, DHEA was administered subcutaneously to Lewis rats immunized with bovine peripheral myelin (BPM) in Freund's complete adjuvant.
View Article and Find Full Text PDFObjectives: The effect of vascular active peptides on the development of pulmonary remodeling and pulmonary hypertension due to left to right shunt congenital heart diseases is the focus of today's studies. The present study was conducted to investigate the roles of adrenomedullin (ADM) and adrenotensin (ADT) in pulmonary remodeling due to left to right shunt in rat lungs.
Methods: Twenty-one male Wistar rats were divided into two groups randomly.
Background: Decayed teeth are harmful to children's growth and development and can severely jeopardize their health. This study was set out to investigate and analyze the prevalence of dental caries in preschool children in Shanghe County in Shandong Province, China, and provide new insights into potential prevention and treatment strategies.
Methods: Based on the random sampling method, we performed dental examinations of children aged 2 to 6 years in kindergartens of Shanghe County.
Objective: To examine modulations caused by cyclooxygenase-2 (COX-2) inhibitors on altered microenvironments and overbalanced neurotransmitters in pilocarpine-induced epileptic status rats and to investigate possible mechanisms.
Methods: Celecoxib (a COX-2 inhibitor) was administered 45 min prior to pilocarpine administration. The effects of COX-2 inhibitors on mIPSCs (miniature GABAergic inhibitory postsynaptic currents) of CA3 pyramidal cells in the hippocampus were recorded.