Objective To investigate the image changes of proton magnetic resonance spectroscopy (1H-MRS) in children with epilepsy and their clinical significance. Methods Sixty-four patients with epilepsy,admitted to our hospital from March 2008 to March 2011,and 10 healthy children as control group were chosen in our study; the patients were divided into MR normal group and MR abnormal group according to the results of MR imaging. All of them received 1H-MRS examination on the hippocampal area.The ratio of NAA/Cho+Cr was compared between each 2 groups. Results No significant differences on the ratio of NAA/Cho+Cr were noted between the fight and left sides in all the groups (P＞0.05).The ratio of NAA/Cho+Cr was significantly different:MR normal group and control group enjoyed obvious difference as compared with MR abnormal group (P＜0.05); however,MR normal group and MR abnormal group existed no statistically significant differences (P＞0.05). Conclusion 1H-MRS is more sensitivity than MRI in children with epilepsy,therefore,1H-MRS can find the lesions earlier than MR imaging.

AIMTo investigate the toxic response in auditory cortex of guinea pigs caused by cis-platinum (DDP), and the protective role of melatonin in this effect.

METHODSCis-platinum and melatonin were injected peritoneally. LDH, MDA, NO in the auditory cortex were detected by spectrophotometeR.

RESULTSThe body weight of the guinea pigs was diminished by peritoneal injection of Cis-platinum for 7 days (P < 0.01). Peritoneal injection of Cis-platinum induced the increased leakage of LDH (P < 0.05 vs injection of normal saline). This effect was reduced by injection of MT (P < 0.05). The content of MDA in the auditory cortex was also increased because of injection of Cis-platinumv for 7 days (P < 0.01) and MT reduced this effect (P < 0.05). The change of NO in the auditory cortex was not statistically significant after injection of Cis-platinum or Cis-platinum with MT.

CONCLUSIONPeritoneal injection of Cis-platinum could destroy neurons in the auditory cortex. This effect could be reduced by melatonin by an anti-free radials mechanism.

Animals ; Auditory Cortex ; drug effects ; metabolism ; pathology ; Cisplatin ; antagonists & inhibitors ; toxicity ; Female ; Free Radical Scavengers ; pharmacology ; Guinea Pigs ; Male ; Malondialdehyde ; metabolism ; Melatonin ; pharmacology ; Neurons ; drug effects ; pathology ; Random Allocation

OBJECTIVETo assess the value of Tpeak-end interval (Tpe) in predicting myocardial infarction (MI).

METHODSTpe and Tpeak-end internal after correcting the heart rate (TpeRR) were measured and analyzed in 234 MI patients, who were followed-up for an average of 32 ± 10 months.

RESULTSClinical events occurred in 45 (19.2%) patients at the end TpeRR of the follow-up. Tpe and of the patients with clinical events were significantly higher than those in patients without the clinical events (P < 0.001). The incidence of clinical events in patients with Tpe > 140 ms were significantly higher than that in patients with Tpe ≤ 140 ms by Kaplan-Meier analysis (P < 0.001). With clinical event as the end point, the proportional hazards rate was 2.48 in univariate COX analysis (P < 0.01). After controlling for risk factors, the hazards rate was 2.66 by multvariate COX regression (P < 0.01).

CONCLUSIONTpe is positively correlated to the prognosis of MI and serves as an new index for predicting the clinical events in MI patients.

Aged ; Electrocardiography ; methods ; statistics & numerical data ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Myocardial Infarction ; diagnosis ; physiopathology ; Predictive Value of Tests ; Prognosis ; Risk Factors ; Survival Analysis

OBJECTIVETo explore the relationship between Tpeak-Tend interval (Tpe) and the extent and severity of coronary artery stenosis, and evaluate the effect of percutaneous transluminal coronary angioplasty and stent implantation (PCI) on Tpe in the patients with coronary heart disease (CHD).

METHODSThe ECG data were collected from 187 CHD patients undergoing coronary angiography and PCI to evaluate the extend and severity of coronary artery stenosis before and after the interventions.

RESULTSThe Tpe of patients with severe stenosis increased significantly as compared with that in patients with moderate stenosis (138.9-/+16.2 ms vs 116.5-/+13.7 ms, P<0.05), and a significant difference was also noted between the moderate stenosis and mild stenosis (86.4-/+12.9 ms) groups (P<0.05). The Tpe decreased significantly in the patients in the order of multi-vessel involvement (140.7-/+17.8 ms), double vessel involvement (118.6-/+14.9 ms), singly vessel involvement (100.5-/+13.2 ms), and stenosis-free (84.3-/+12.4 ms) groups (P<0.05). Tpe was correlated to the extent and severity of coronary artery stenosis (r>0.4). In patients with severe stenosis, the Tpe was significantly reduced at 1 h, 24 h, and 1 week after PCI (115.8-/+14.5, 92.7-/+12.9, and 88.2-/+11.3 ms, respectively, P<0.05).

CONCLUSIONThe Tpe can reflect the severity and range of coronary artery stenosis, which can be reduced by PCI. Tpe can be a new index for evaluating myocardial ischemia in CHD patients.

Aged ; Angioplasty, Balloon, Coronary ; Coronary Stenosis ; physiopathology ; therapy ; Electrocardiography ; Female ; Humans ; Male ; Middle Aged ; Treatment Outcome

OBJECTIVELeukoencephalopathy with brain stem and spinal cord involvement and lactate elevation (LBSL) is a rare autosomal recessive disease. Affected individuals are invariably compound heterozygous for two mutations in DARS2. No reports of LBSL patients have been published in the mainland of China. The aim of this study was to explore the clinical and genetic features of a family with LBSL, which may contribute to definite diagnosis, genetic counseling and prenatal diagnosis of this rare disease in China.

METHODSClinical data of the proband and other family members as well as DNA samples were collected. Clinical features including symptoms, signs and cranial MRI were analyzed. All 17 exons and exon-intron boundaries of DARS2 gene were amplified with polymerase chain reaction (PCR) and directly sequenced for genomic DNA. The mutation was proved by DNA restriction enzyme digestion of PCR-amplified fragments.

RESULTS(1) The clinical features of patient with LBSL included slowly progressive cerebellar ataxia and spasticity, the neurologic dysfunction involving the legs more than the arms, and with characteristic abnormalities observed on brain and spinal cord MRI. (2) Two mutations were identified, one was a novel missense mutation [c.665 G > A(p.Gly222Asp)] in DARS2 gene exon 8, the other (c.228-16 C > G) was in DARS2 gene intron 3.

CONCLUSIONThis is the first report on LBSL patient and DARS2 mutation in China. p.Gly222Asp mutation is a novel mutation not reported around the world yet.

Adolescent ; Asian Continental Ancestry Group ; genetics ; Aspartate-tRNA Ligase ; genetics ; Brain Stem ; pathology ; DNA Mutational Analysis ; Exons ; Humans ; Lactic Acid ; metabolism ; Leukoencephalopathies ; genetics ; metabolism ; pathology ; Male ; Mutation ; Pedigree ; Spinal Cord ; pathology

OBJECTIVETo compare the effects of amlodipine, benidipine and nifedipine on myocardial hypertrophy and evaluate the underlying mechanism.

METHODSMyocardial hypertrophy model was created by transverse aortic constriction (TAC) in C57 BL/6 mice, and plasma catecholamine concentrations were measured 7 days after surgery to confirm the sympathetic activation. The 3 drugs were administered in TAC mice for 7 days and cardiac hypertrophy was evaluated according to the heart-to-body weight ratio (HW/BW). Effects of those drugs on the protein synthesis stimulated by phenylephrine in cultured neonatal cardiac myocytes were also examined.

RESULTSHW/BW and plasma concentrations of catecholamine were significantly increased in TAC mice one week after surgery in comparison with to sham-operated mice. One week after TAC, the HW/BW ratio was significantly lower in the amolodipine but not nifedipine-treated group than in the TAC group. Administration of nifedipine via minipump infusion for one week did not decrease HW/BW ratio. Treatment with amlodpine or benidipine, but not nifedipine, decreased the neonatal rat myocyte protein synthesis induced by phenylephrine stimulation.

CONCLUSIONAntihypertrophic effect of DHEs on myocardium is dependent on their potential of blocking N-type calcium channel, and the underlying mechanism involves the sympathetic inhibition.

Amlodipine ; pharmacology ; therapeutic use ; Animals ; Calcium Channel Blockers ; pharmacology ; therapeutic use ; Calcium Channels, N-Type ; drug effects ; Cardiomegaly ; drug therapy ; etiology ; Dihydropyridines ; pharmacology ; therapeutic use ; Disease Models, Animal ; Male ; Mice ; Mice, Inbred C57BL ; Nifedipine ; pharmacology ; therapeutic use

Leigh syndrome is a genetically heterogeneous disease caused by defects in enzymes involved in aerobic energy metabolism and the Krebs', cycle. Mitonchondrial complex I deficiency is a main cause of Leigh syndrome. In this study, a Chinese child with Leigh syndrome caused by 13513G>A mutation was reported. The proband was the first child of his parents. The previously healthy boy presented with generalized epilepsy at 12 years of age. When he visited Peking University First Hospital at 13 years of age, he had subacute loss of vision in two eyes and temporal defect of visual field in the left eye. He walked with a spastic gait. His blood lactate and pyruvate levels were elevated. Muscle biopsy showed mild lipid accumulation in muscle fiber. An electrocardiogram showed incomplete right bundle branch block. Brain magnetic resonance imaging showed bilateral, symmetrical lesions in the basal ganglia, supporting the diagnosis of Leigh syndrome. 13513G>A mutation was identified by gene analysis in the patient, which led to mitochondrial respiratory chain complex I deficiency. Multivitamins and L-carnitine were administered. At present, the patient is 16 years old and has progressive deterioration with significant muscle weakness and body weight loss. He is absent from school. He has no obvious retardation in intelligence. 13513G>A mutation was first identified by gene analysis in Chinese population with Leigh syndrome. This may be helpful in genetic counseling.
Adolescent ; DNA, Mitochondrial ; genetics ; Electron Transport Complex I ; deficiency ; Humans ; Leigh Disease ; genetics ; Male ; Mutation

OBJECTIVETo explore the recognition ability and abnormal processing characteristics to basic emotional faces in the early phase in children with autism spectrum disorders (ASD).

METHODSPhotos of Chinese static faces with four basic emotions (fearful, happy, angry and sad) were used as stimulus. Twenty-five ASD children and twenty-two age- and gender-matched typical developed children (normal controls) were asked to match the emotional faces with words. Event-related potential (ERP) data were recorded concurrently.

RESULTSN170 latencies for total emotion and fearful face in the left temporal region were faster than in the right one in normal controls (P<0.05), but the results were not noted in ASD children. Further, N170 latencies in the left temporal region of ASD children were slower than normal controls for total emotion, fearful and happy faces (P<0.05), and their N170 latencies in the right temporal region were prone to slower than normal controls for angry and fearful faces.

CONCLUSIONSThe holistic perception speed of emotional faces in the early cognitive processing phase in ASD children is slower than normal controls. The lateralized response in the early phase of recognizing emotional faces may be aberrant in children with ASD.

OBJECTIVEDespite the causes for melanin increase, the increased gene expression of TYR is a common pathological process. Based on this viewpoint, antisense-S-Oligo of TYR was designed and synthesized to regulate synthesis of melanin in order to explore the treatment for skin pigmentation.

METHODSThe cultured melanocytes were divided into 3 groups. The group 1 was treated with endothelin, group 2 treated with ultraviolet ray and group 3 was used as the control. In each group, the 5' antisense-S-Oligo, the 3' antisense-S-Oligo, the mixed antisense-S-Oligo of TYR or Dotap only was added. The melanin content and TYR gene expressions were examined.

RESULTSThe 5' antisense-S-Oligo, the 3' antisense-S-Oligo and the mixed antisense-S-Oligo significantly inhibited the increase of melanin content and TYR gene expression, which were caused by endothelin or ultraviolet ray treatment. Of the three treatments, the 3' antisense-S-Oligo showed the strongest effect.

CONCLUSIONAntisense-S-Oligo has significant regulating effects on TYR gene expression and melanin content. The 3' antisense-S-Oligo is more effective than the 5' antisense-S-Oligo.

3' Flanking Region ; genetics ; 5' Flanking Region ; genetics ; Endothelins ; pharmacology ; Gene Expression ; Melanins ; biosynthesis ; Melanocytes ; drug effects ; metabolism ; radiation effects ; Oligodeoxyribonucleotides, Antisense ; genetics ; pharmacology ; Tyrosine ; genetics ; metabolism ; Ultraviolet Rays

OBJECTIVETo analyze the postoperative short-term and long-term outcomes in the management of type I esophageal atresia, and to explore the ideal operative strategy.

METHODSClinical data of 22 patients with type I esophageal atresia treated from January 2005 to September 2012 were retrospectively reviewed. Of 22 patients, 6 patients gave up the treatment. Two underwent primary repair after birth. Of 14 patients undergoing cervical esophagostomy and gastrostomy, 8 patients received esophageal replacement. Postoperative short-term and long-term complications, nutritional state and neurodevelopment were studied on above 10 children with radical operations.

RESULTSOf 10 patients with radical operation, the short-term complications were hydrothorax in 1 case, anastomotic leakage in 4, dumping syndrome in 1, anastomotic stricture in 1. The long-term complications were esophageal stricture in 2 cases, and repeated respiratory infection in 3. These complications could be managed successfully. The postoperative follow-up duration ranged from 2 to 62 months. Two cases were lost during follow-up after 2 years. Weight-for-age was normal in 2 patients, mild malnutrition in 5 patients, and moderate malnutrition in 1 patients. Neurodevelopment is significantly delayed as compared to normal children.

CONCLUSIONSOperative strategy should be chosen according to the distance between proximal and distal esophagus in the treatment of type I esophageal atresia. The efficacy of radical operation is relative satisfactory in terms of short-term and long-term complications and the quality of life.

Child ; Esophageal Atresia ; surgery ; Female ; Follow-Up Studies ; Humans ; Male ; Postoperative Complications ; Quality of Life ; Retrospective Studies