Objective To observe the delayed brain myelination of children with phenylketonuria(PKU)combined with epilepsia,and explore effectiveness of the treatment and provide an objective criteria for patient recovering evaluation.Methods There were 42 PKU patients,aged 3 to 72 months were selected.The concentration of phenylalanine tested by high pressure liquid chromatography was greater than 1.2 mmol/L in blood,diagnosed as PKU.According to electroencephalogram and clinical symptom,21 cases were diagnosed as epilepsy,the other 21 cases were used as control group.All patients were taken MRI before treatment.Myelination in 10 sections(cerebellum,pons,mesencephalon,internal capsule posterior limb,corpus callosum,internal capsule anterior limb,occipital lobe,parietal lobe,temporal lobe,frontal lobe)were evaluated.Results Delayed myelinations were located mainly in the cerebral lobes and corpus callosum,average delayed incidence of the 10 region was 44.8% in epilepsy group and 30.9% in control group.The incidence of the corpus callsum was 80.9% in epilepsy group,52.4% in control group,the number of sections of delayed myelination showed statistically significant between 2 groups(P

Objective To explore the high risk factors of metabolic syndrome(MS) in obese children with acanthosis nigricans.Me-thods Body mass index(BMI),blood lipid including triglyeride(TG) and cholesterol(CHO),low density lipoprotein-cholesterol(LDL-C),blood pressure,the level of fasting blood glucose(FBG) and 2 h after oral glucose tolerance test blood glucose(OGTT 2h BG) and the level of fasting insulin(FINS) and 2 h after oral glucose tolerance test insulin (OGTT 2h INS) and homeostasis model appraisal insulin resis-tance index(HOMA-IR) were measured and compared between 25 obese children with acanthosis nigricans[male 15,female 10;aged 8.4-16.0,mean 10.6 years old,weight (72.11?17.66) kg;height (155?14) cm]and 32 normal healthy children[male 18,female 14;aged 7.6-15.8,mean 9.8 years old]in department of pediatric during 1 year.HOMA-IR were also analyzed.Ultrasonic inspections for liver were performed in those children.Results BMI,TG,LDL-C and blood pressure in the obese group were significantly higher than those in the normal control group(Pa0.05).Eighty-four percent of patients in obese children with acanthosis nigricans were diagnosed adiposis hepatica by ultrasonograph.Conclusions The increasing BMI,insulin resistance,blood lipid disorder and blood pressure increase in obese children with acanthosis nigricans are the high risk factors of MS,the close followed-up and treating this kind of obese children can acquire MS early and be helpful to postpone the progress of diabetesⅡ and cardiovascular diseases.

OBJECTIVETo explore the efficacy and the reproductive endocrinal mechanism of herbal-partitioned moxibustion in the treatment of primary dysmenorrhea.

METHODSOne hundred and seventy-one cases of primary dysmenorrhea were randomized into an herbal-partitioned moxibustion group (group A), an starch-partitioned moxibustion group (group B) and an acupuncture group (group C), 57 cases in each one. In the group A, moxibustion isolated with herbal medicine was applied to Shenque (CV 8). In the group B, moxibustion isolated with starch was used at Shenque (CV 8). In the group C, acupuncture was given at Sanyinjiao (SP 6). The changes of estradiol (E2), progesterone (P) and prostaglandin levels (PGF2alpha) were observed before and after treatment, and the therapeutic effects were compared among the 3 groups.

RESULTSThe therapeutic effect in the group A was better than those in the other two groups [compared the cured rate: 89.8% (44/49) vs 60.0% (30/50), 60.4% (32/53), both P < 0.05]. In the group A, E2 level [(110.99 +/- 12.90) pg/mL vs (83.94 +/- 8.91) pg/mL, P < 0.05] and PGF2alpha level [(24.58 +/- 3.01) pg/mL vs (14.34 +/- 1.48) pg/mL, P < 0.01] were decreased and P level was increased [(4.65 +/- 0.68) ng/mL vs (6.68 +/- 0.95) pg/mL, P < 0.05]. In the group B and C, PGF2alpha level were reduced. Concerning to the regulating of E2 and PGF2alpha levels, the results in the group A were better than those in the group B and C [(-30.16 +/- 10.20) pg/mL vs (10.79 +/- 15.01) pg/mL, (22.81 +/- 12.22) pg/mL; (-13.10 +/- 2.40) pg/mL vs (-6.52 +/- 1.88) pg/mL, (-3.14 +/- 1.19) pg/mL, (see text) P < 0.05]. Concerning to the regulation of P level, the results in the group A and B were better than that in the group C (all P < 0.05).

CONCLUSIONThe herbal-partitioned moxibustion achieves the significant efficacy on primary dysmenorrhea, which could be related to regulating the reproductive endocrinal level. It decreases E2 and PGF2alpha levels and increases P level.

Acupuncture Points ; Adult ; Dinoprost ; metabolism ; Dysmenorrhea ; metabolism ; therapy ; Estradiol ; metabolism ; Female ; Humans ; Moxibustion ; Progesterone ; metabolism ; Young Adult

OBJECTIVETo investigate the early clinical effects and radiological outcome of dynamic cervical implant (DCI) internal fixation in treating cervical spondylosis, and evaluate its safety and efficiency.

METHODSFrom June 2009 to December 2011, 19 patients with cervical spondylosis correspond to the indication of DCI internal fixation in the study, including 5 cases of cervical spondylotic myelopathy and 14 cases of cervical spondylotic radiculopathy. There were 8 males and 11 females, aged from 35 to 54 years with a mean of 43.2 years. Pathological segments included C3,4 in 1 case, C4,5 in 6, C5,6 in 6, C6,7 in 4, C3,4 and C5,6, C6,7 in 2. All patients were treated with anterior discectomy and decompression and DCI internal fixation, meanwhile, 2 cases of them with anterior cervical corpectomy and fusion plate fixation. Clinical evaluation included Modified Japanese orthopedics association (mJOA), neck disability index (NDI), visual analogue scale (VAS) score and patient satisfaction index (PSI) at pre-operation and final follow-up. Radiographic evaluation included flexion/extension lateral view at operative level and adjacent segment. The adjacent level degeneration was analyzed according to Miyazaki classification on MRI images.

RESULTSAll patients were followed up from 12 to 42 months with an average of 19.8 months. Preoperative mJOA score was 13.6±1.1 and at final follow-up was 16.3±1.2 with improvement rate of 85.0%. Preoperative VAS,NDI was 6.6± 1.4, 17.1±7.4 and at final follow-up was 1.4±0.8, 6.1±3.9, respectively; there was statistical significance in all above-mentioned results between preoperative and final follow-up (P<0.05). Preoperative ROM at operation level was (7.6±1.9)° and final follow-up was (7.8+2.1)°; preoperative ROM at C2-C7 was (38.6±7.2)° and final follow-up was (39.9±6.4)°; there was no statistical significance in all above-mentioned results between preoperative and final follow-up (P>0.05). Preoperative DHI at operation level was (6.3±1.1) mm and final follow-up was (7.1±0.8) mm, there was statistical significance in DHI between preoperative and final follow-up (P<0.05). No heterotopic ossification was found. All patients followed up MRI, degeneration of 3 segments aggravated 1 degree in 38 adjacent segments, without clinical symptom.

CONCLUSIONTreatment of cervical spondylosis with dynamic cervical implant can got satisfactory outcome in early follow-up. Activity of operative segment obtain reservation in some degree. The incidence of adjacent segment degeneration is lower and no adjacent segment disease occur. Nevertheless a longer follow-up time should be needed to assess the long term functionality of the DCI and the influence on adjacent levels.

Adult ; Cervical Vertebrae ; surgery ; Female ; Fracture Fixation, Internal ; methods ; Humans ; Male ; Middle Aged ; Prostheses and Implants ; Range of Motion, Articular ; Spondylosis ; physiopathology ; surgery

Acyl carrier protein is an essential component involved in the biosynthesis of DHA(Docosahexaenoic Acid) via PKS(Polyketide synthase) pathway,which takes the growing acyl chain from one enzyme to another.One cDNA clone,with high homology of ACP,was isolated from Schizochytrium sp.FJU-512 cDNA library.The deduced amino acid sequence contained 142 residues with isoelectric point of 5.04 and had the 4'-phosphopantetheine prosthetic(4'-PP) binding site.The target fragment was digested with BamHⅠ/HindⅢand inserted into the expression vector pET-30a resulting in the plasmid pET-30a/acp.The recombinant vector was transformed into E.coli BL21(DE3) and induced by IPTG.SDS-PAGE analysis demonstrated that ACP was effectively expressed.

Objective To discuss the frequent etiology of spontaneous sub-cortical hemorrhage and its diag- nosis.Methods The clinical materials of 79 cases of spontaneous sub-cortical hemorrhage were analyzed.Results 56% of the hemorrhage was caused by arterial-venous malformation.48% of the hemorrhage was caused by occult AVM.Conclusion AVM is the most frequent etiology of spontaneous sub-cortical hemorrhage.CTA plays a special role in its diagnosis.

This study was to investigate the effect of peoniflorin on the expressions of nuclear factor erythroid 2-related factor 2 (Nrf2) and its downstream signal molecules in the hippocampus of Alzheimer's disease (AD) rats for exploring the mechanism of peoniflorin protecting hippocampal neurons. AD model rats were established by bilateral intrahippocampal injection of beta-amyloid(1-42) (Abeta(1-42)) and divided randomly into 3 groups: AD model group, peoniflorin low-dose (15 mg x kg(-1)) group and peoniflorin high-dose (30 mg x kg(-1)) group. The vehicle control rats were given bilateral intrahippocampal injection of solvent with the same volume. After peoniflorin or saline was administered (ip) once daily for 14 days, the hippocampuses of all animals were taken out for measuring the expressions of Nrf2, heme oxygenase-1 (HO-1) and gamma-glutamylcysteine synthethase (gamma-GCS) mRNA by reverse transcription PCR, determining the contents of glutathione (GSH), malondialdehyde (MDA) and carbonyl protein (CP) using colorimetric method, and for assaying the expressions of neuronal apoptosis inhibitory protein (NAIP) and Caspase-3 by immunohistochemical staining method. The results showed that peoniflorin markedly increased the expressions of Nrf2, HO-1 and gamma-GCS mRNA, enhanced the level of GSH and decreased the contents of MDA and CP in the hippocampus, as compared with the model group. Peoniflorin also improved the NAIP expression and reduced the Caspase-3 expression in the hippocampus neurons. In conclusion, peoniflorin protects against the Abeta(1-42)-mediated oxidative stress and hippocampal neuron injury in AD rats by activating the Nrf2/ARE pathway.
Alzheimer Disease ; chemically induced ; metabolism ; physiopathology ; Amyloid beta-Peptides ; Animals ; Anti-Inflammatory Agents, Non-Steroidal ; pharmacology ; Caspase 3 ; metabolism ; Glucosides ; pharmacology ; Glutamate-Cysteine Ligase ; genetics ; metabolism ; Glutathione ; metabolism ; Heme Oxygenase (Decyclizing) ; genetics ; metabolism ; Hippocampus ; metabolism ; Male ; Malondialdehyde ; metabolism ; Monoterpenes ; pharmacology ; NF-E2-Related Factor 2 ; genetics ; metabolism ; Neuronal Apoptosis-Inhibitory Protein ; metabolism ; Neurons ; metabolism ; Oxidative Stress ; drug effects ; Peptide Fragments ; RNA, Messenger ; metabolism ; Random Allocation ; Rats ; Rats, Sprague-Dawley

OBJECTIVETo observe brain white matter changes in children with late-treated phenylketonuria (PKU) before and after receiving treatment.

METHODSThis study included 19 PKU patients (aged 34-410 weeks) who were administered a low-phenylalanine diet (< 15-50 mg/kg daily) for 8-16 months. The brain MR imaging with spin-echo T1-weighted and T2-weighted sequences in coronal and axial planes was taken before and after treatment. The white matter abnormalities (T2WI high signal intensity) were graded based on the Thompson grading system. Meanwhile the intelligence quotient (IQ) or developmental quotient (DQ) was tested by the Gesell's Intelligence Scale.

RESULTSAll 19 PKU patients presented with the brain white matter lesions, manifesting abnormally high T2-signal intensity in the periventricular region around anterior and posterior horns of both lateral ventricles. Different extents of mental retardation were also observed in the 19 patients. The low phenylalanine diet treatment decreased the average grade of abnormal T2-signal intensity from 2.59 to 1.76 (P < 0.05). The mean IQ or DQ improved from 44.8 to 61.6 after treatment (P < 0.05). There was some correlation between the amelioration of brain white matter lesions and IQ or DQ.

CONCLUSIONSThe patients with late-treated PKU have a higher occurrence of the brain white matter lesions and mental retardation. A low-phenylalanine diet treatment can partly improve the abnormalities. Brain white matter lesions may play a part in mental retardation.

Brain ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Intelligence ; Magnetic Resonance Imaging ; Male ; Phenylalanine ; blood ; Phenylketonurias ; blood ; pathology ; psychology ; therapy

By reverse transcription-polymerase chain reaction (RT-PCR), an open reading frame of pathogenesis-related protein 1 (PR1) was isolated from Panax notoginseng and named as PnPR1. Molecular and bioinformatic analyses of PnPR1 revealed that an open reading frame of 501 bp was predicted to encode a 166-amino acid protein with a deduced molecular mass of 18.1 kD. Homology analysis showed that the deduced amino acid sequence of PR1 protein of Panax notoginseng had a high similarity with other higher plants had the same conservative structure domain of cysteine-rich secretory protein (CAP). The recombinant expressed plasmid pET28a(+)-PnPR1 was expressed in Escherichia coli BL21. The expression conditions were optimized by induction at different times, different temperatures, different IPTG concentrations and different giving times. The optimum expression condition was 0.4 mmol.L-1 IPTG at 28 degrees C for 20 h. The successful expression of PnPR1 provides some basis for protein purification and preparation of the monoclonal antibody.
Amino Acid Sequence ; Cloning, Molecular ; Escherichia coli ; metabolism ; Molecular Weight ; Open Reading Frames ; genetics ; Panax notoginseng ; chemistry ; Phylogeny ; Plant Proteins ; genetics ; metabolism ; Plants, Medicinal ; chemistry ; Reverse Transcriptase Polymerase Chain Reaction ; Sequence Alignment

Objective To investigate plasma glutathione S-transferase(GSTs) activity and GSTP1 gene Ile105Val polymorphism in Bijie City, Guizhou Province, a coal-burning fluorosis endemic area. Methods One hundred and sixty villagers from Yachi Twon using non-improved cooking stoves were selected as the non-intervened group in Bijie City, Guizhou Province where coal-burning fluorosis was prevailing; 153 villagers as the intervented group were chosen from Changchun Twon, where cooking stoves were improved; 151 villagers were served as the control group from Baiyunshan Twon, Changshun County without endemic fluorosis. The activity of GSTs was tested by colorimetric analysis with spectrophotometer. The genotype of the GSTP1 gene Ile105Val polymorphism, presenting as either homozygous wild-type (AA), or heterozygous mutation type (AG), or homozygous mutation type (GG), was detected through the PCR-RFLP procedure. Results The activity of GSTs in plasma of non-intervened group [(12.44±4.97) kU/L]was significantly lower than that of intervened group (P < 0.05), and that of intervened group[(20.78±6.20)kU/L]was significantly lower than that of control group[(24.30±6.27)kU/L, P< 0.05]. The difference of the enzyme activity of three groups were statistically significant (F = 51.71, P < 0.05), but this enzyme activity did not vary significantly in each sex of each grnup(P > 0.05). Compared intervened group [AA:67.3%(103/153), AG:29.4%(45/153),GG:3.3%(5/153)]and non-intervened group[AA:66.9%(107/160), AG:30%(48/160), GG:3.1%(5/160)]with control group[AA:74.8%(113/151), AG:25.2%(38/151), GG:0 (0/151)], the Ile105Val polymorphism site of GSTP1 gene had significant difference(χ2= 6.04,6.07, both P< 0.05), but not significant between intervened and non-intervened groups(χ2 = 0.02, P>0.05). Conclusions Fluorosis can decrease the activity of GSTs and introduce the GSTP1 gene Ile105Val polymorphism, intervention with the fluorine intake will improve the effect of fluoride on the body.