Clinical features analysis in 5 children of early onset epileptic encephalopathy with dyskinesia / 中国实用儿科杂志

OBJECTIVE: To investigate the clinical phenotype of children with early-onset epileptic encephalopathy and dyskinesia. METHODS: The patients with early onset epileptic encephalopathy and dyskinesia were enrolled from September 2013 to September 2017. The clinical data was retrospectively analyzed. RESULTS: A total of 5 children with early onset epileptic encephalopathy with dyskinesia were collected. Including 1 male and 4 females. 1 case of dyskinesia showed dancing-like movements,2 cases of dyslexia,ataxia mixed,1 case of limb tremor accompanied by wrist alternating twisting action,1 case of muscle tension associated with tremor,speech is unclear. 4 cases were positive for 1 genotype,1 case was mutation of SCN1A gene,2 cases were PRRT2 gene mutation,1 case was SLC2A1 gene mutation. After treatment,2 cases of seizure control effect is good,3 cases of poor results. CONCLUSION: Early onset epileptic encephalopathy with dyskinesia is easy misdiagnosed as epileptic seizures. Genetic associated with the pathogenic genes are mostly reported in the previous gene,PRRT2 gene,SLC2A1 gene mutation. Early onset of epilepsy with dyskinesia caused by SCN1A gene mutation has rarely been reported in the literature. Some children enjoy good results.

Effect of Hedgehog Signaling Pathway Abnormality on Chemothe-rapeutic Resistance of Multiple Myeloma / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To study the correlation between the excessive activation of Hedgehog signal and the drug resistance of multiple myeloma.</p><p><b>METHODS</b>The resistant cell line RPMI 8226/R of multiple myeloma was established by an ascending concentration gradient method. The experiment consisted of 4 groups: RPMI8226/R, RPMI8226/S, GANT61+RPMI8226/R and GANT61+RPMI8226/S. The CCK-8 (cell counting kit-8) assay was used to detect the cell proliferation inhibition rate in 4 groups; the RT-PCR was used to detect the expression of Gli1, Gli2, Shh, Ihh, Smo and Sufu in the RPMI8226/S and RPMI8226/R cells. The Western blot was used to detect the expression of the resistant protein Cyclin D1, P21 and BCL-2 and MDR-related signaling pathways protein p-Akt, p-MAPK and STAT3 in the RPMI8226/S and RPMI8226/R cells. After adding different concentration of GANT61, the Western blot was used to detect the expression of Gli2 in RPMI8226/R and RPMI8226/S cells.</p><p><b>RESULTS</b>The expression of Shh, Ihh, Smo Gli2 was enhanced significantly in the RPMI8226/R cells, but the expression of Sufu inhibitor was reduced, the expression level of related protein in Hedgehog signaling pathway was significanly higher in RPMI8226/R than that in RPMI8226/S. After theatment of GANT61 in vitro, the expression level of Gli2 in multiple myelom cells obviously decreased, the decreasing effect of GANT61 on Gli2 expression in RPMI8226/R cells was more significant than that in RPMI8226/S cells. The sensitivity of RPMI8226/R cells to DOX after treatment with GANT61 (IC) was risen from 7.11±0.061 µmol/L to 0.99±0.053 µmol/L, the corresponding cell resistance index decreased from 5.51 to 1.69.</p><p><b>CONCLUSION</b>the activation of Hedgehog signaling pathway is closely related with the resistance of multiple myeloma cells, and GANT61 can block the Hedgehog signaling pathway, thus Hedgehog signaling may be used as a new target for multiple myeloma treatment.</p>

An atypical case of mitochondrial acetoacetyl-CoA thiolase deficiency

Methylacetoacetyl-CoA thiolase deficiency (T2 deficiency) is a rare congenital and metabolic disease affecting the ketone body and isoleucine metabolism. The typical symptoms are refractory metabolic acidosis, in which large amounts of 2-methyl-3-hydroxybutyry1 carnitine, 2-methyl-3-hydroxybutyrate and tiglylglycine are often detected in the blood and urine. We herein describe an atypical case of T2 deficiency with a high level of 3-hydroxybutyrate and a low level of 2-methyl-3-hydroxybutyrate in the urine. Such a case was diagnosed by urinary organic analysis in combination with gene mutation evaluation. Organic acids in the urine were measured using a gas chromatography mass spectrometer and all exons were sequenced via deep sequencing. Molecular biology analysis confirmed the presence of a homozygous mutation in the acetyl-CoA acetyltransferase 1 (ACAT1) gene. The patient received a special diet of deeply hydrolyzed protein milk powder and raw corn starch. She was followed about 6 months. There were no ketoacidotic episodes and hypoglycemia even when she had fever. In conclusion, patients with atypical features of T2 deficiency should also be investigated early. Gas chromatography mass spectrometry and next-generation full exome sequencing may be helpful in diagnosis.

Association of mammalian target of rapamycin gene polymorphisms with the risk of pediatric epilepsy / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the association between two single nucleotide polymorphisms (SNP), rs2295080 and rs2536, in mammalian target of rapamycin (mTOR) gene and the susceptibility to pediatric epilepsy.</p><p><b>METHODS</b>A case- control study was performed on 480 children with epilepsy (116 cases of refractory epilepsy) and 503 healthy children. SNP rs2295080 and rs2536 in the mTOR gene were detected by polymerase chain reaction restriction and fragment length polymorphisms (PCR-RFLP). Genotype and allele frequencies of SNP rs2295080 and rs2536 were compared between the children with epilepsy and healthy controls.</p><p><b>RESULTS</b>There were no significant differences in the genotype and allele frequencies of SNP rs2295080 between the children with epilepsy and healthy controls. There were no significant differences in the genotype frequencies of SNP rs2536 between the two groups either, but the frequency of G allele of SNP rs2536 was higher in children with epilepsy than that in healthy controls (P=0.042, OR=1.344, 95%CI: 1.010-1.789).</p><p><b>CONCLUSIONS</b>SNP rs2536 of mTOR gene may be associated with the risk of pediatric epilepsy.</p>

Na (+) /Ca (2+) Exchanger 3 is Downregulated in the Hippocampus and Cerebrocortex of Rats with Hyperthermia-induced Convulsion / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Na + /Ca 2+ exchanger (NCX) plays a crucial role in pentylenetetrazol-induced convulsion. However, it is unclear whether NCX is critically involved in hyperthermia-induced convulsion. In this study, we examined the potential changes in NCX3 in the hippocampus and cerebrocortex of rats with hyperthermia-induced convulsion.</p><p><b>METHODS</b>Twenty-one Sprague Dawley rats were randomly assigned to control group, convulsion-prone group and convulsion-resistant group (n = 7 in each group). Whole-cell patch-clamp method was used to record NCX currents. Both the Western blotting analysis and immunofluorescence labeling techniques were used to examine the expression of NCX3.</p><p><b>RESULTS</b>NCX currents were decreased in rats after febrile convulsion. Compared to the control group, NCX3 expression was decreased by about 40% and 50% in the hippocampus and cerebrocortex of convulsion-prone rats, respectively. Furthermore, the extent of reduction in NCX3 expression seemed to correlate with the number of seizures.</p><p><b>CONCLUSIONS</b>There is a significant reduction in NCX3 expression in rats with febrile convulsions. Our findings also indicate a potential link between NCX3 expression, febrile convulsion in early childhood, and adult onset of epilepsy.</p>

Association of HLA-B*1502 and *1511 allele with antiepileptic drug-induced Stevens-Johnson syndrome in central China / 华中科技大学学报（医学）（英德文版）

Previous studies have demonstrated a strong association between carbamazepine (CBZ)-induced Stevens-Johnson syndrome (SJS) and HLA-B*1502 in Han Chinese. Here, we extended the study of HLA-B*1502 susceptibility to two different antiepileptic drugs, oxcarbazepine (OXC) and phenobarbital (PB). In addition, we genotyped HLA-B*1511 in a case of CBZ-induced SJS with genotype negative for HLA-B*1502. The presence of HLA-B*1502 was determined using polymerase chain reaction with sequence-specific primers (PCR-SSP). Moreover, we genotyped HLA-B*1502 in 17 cases of antiepileptic drugs (AEDs)-induced cutaneous adverse drug reactions (cADRs), in comparison with AEDs-tolerant (n=32) and normal controls (n=38) in the central region of China. The data showed that HLA-B*1502 was positive in 5 of 6 cases of AEDs-induced SJS (4 CBZ, 1 OXC and 1 PB), which was significantly more frequent than AEDs-tolerant (2/32, 18 CBZ, 6 PB and 8 OXC) and normal controls (3/38). Compared with AEDs-tolerant and normal controls, the OR for patients carrying the HLA-B*1502 with AEDs-induced SJS was 6.25 (95% CI: 1.06-36.74) and 4.86 (95% CI: 1.01-23.47). The sensitivity and specificity of HLA-B*1502 for prediction of AEDs-induced SJS were 71.4%. The sensitivity and specificity of HLA-B*1502 for prediction of CBZ-induced SJS were 60% and 94%. HLA-B*1502 was not found in 11 children with maculopapular exanthema (MPE) (n=9) and hypersensitivity syndrome (HSS) (n=2). However, we also found one case of CBZ-induced SJS who was negative for HLA-B*1502 but carried HLA-B*1511. It was suggested that the association between the CBZ-induced SJS and HLA-B*1502 allele in Han Chinese children can extend to other aromatic AEDs including OXC and PB related SJS. HLA-B*1511 may be a risk factor for some patients with CBZ-induced SJS negative for HLA-B*1502.

Association of HLA-B*1502 and *1511 allele with antiepileptic drug-induced Stevens-Johnson syndrome in central China / 华中科技大学学报（医学）（英德文版）

Previous studies have demonstrated a strong association between carbamazepine (CBZ)-induced Stevens-Johnson syndrome (SJS) and HLA-B*1502 in Han Chinese. Here, we extended the study of HLA-B*1502 susceptibility to two different antiepileptic drugs, oxcarbazepine (OXC) and phenobabital (PB). In addition, we genotyped HLA-B*1511 in a case of CBZ-induced SJS with genotype negative for HLA-B*1502. The presence of HLA-B*1502 was determined using polymerase chain reaction with sequence-specific primers (PCR-SSP). Moreover, we genotyped HLA-B*1502 in 17 cases of antiepileptic drugs (AEDs)-induced cutaneous adverse drug reactions (cADRs), in comparison with AEDs-tolerant (n=32) and normal controls (n=38) in the central region of China. The data showed that HLA-B*1502 was positive in 5 of 6 cases of AEDs-induced SJS (4 CBZ, 1 OXC and 1 PB), which was significantly more frequent than AEDs-tolerant (2/32, 18 CBZ, 6 PB and 8 OXC) and normal controls (3/38). Compared with AEDs-tolerant and normal controls, the OR for patients carrying the HLA-B*1502 with AEDs-induced SJS was 6.25 (95% CI: 1.06-36.74) and 4.86 (95% CI: 1.01-23.47). The sensitivity and specificity of HLA-B*1502 for prediction of AEDs-induced SJS were 71.4%. The sensitivity and specificity of HLA-B*1502 for prediction of CBZ-induced SJS were 60% and 94%. HLA-B*1502 was not found in 11 children with maculopapular exanthema (MPE) (n=9) and hypersensitivity syndrome (HSS) (n=2). However, we also found one case of CBZ-induced SJS who was negative for HLA-B*1502 but carried HLA-B*1511. It was suggested that the association between the CBZ-induced SJS and HLA-B*1502 allele in Han Chinese children can extend to other aromatic AEDs including OXC and PB related SJS. HLA-B*1511 may be a risk factor for some patients with CBZ-induced SJS negative for HLA-B*1502.

Molecular mechanism of Doxorubicin resistance in multiple myeloma cell line / 中国实验血液学杂志

This study was aimed to investigate the molecular mechanism of doxorubicin resistance in multiple myeloma cell line and certify the effect of Notch signal over-expression on drug resistance of myeloma cells. The doxorubicin RPMI 8226 cell line (RPMI8226/DOX) was established by culturing 8226 cells with continuous low concentration and intermittent gradually-increasing-concentration of doxorubicin in vitro, the mRNA expression of Notch2,Jagged1, Jagged2, HES1 were measured by RT-PCR and the P-170 protein expression was detected by Western blot in RPMI 8226 cell line; the changes of IL-6 and VEGF were tested by ELISA. The results showed that the Notch mRNA expression (Notch2, Jagged1, Jagged2 increased gradually along with the increase of chemotherapeutic drug resistance, but the expression of HESI mRNA gradually decreased along with the increase of drug resistance. The expression level of P-170 protein was upregulated gradually along with the increase of drug resistance. The level of VEGF and IL-6 in culture supernatants of RPMI8226/DOX was higher than that in RPMI 8226. It is concluded that the establishment of RPMI 8226/DOX cell line is a useful model to analyze the mechanism of chemotherapeutic drug resistance in multiple myeloma, Notch activation is closely correlated with the drug resistance of multiple myeloma and Notch signaling may to be used as a new target for multiple myeloma treatment.

Clinical features and MECP2 mutations in children with Rett syndrome / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the clinical features and mutations in methyl-CpG-binding protein 2 (MECP2) gene among children with classical Rett syndrome in China.</p><p><b>METHODS</b>PCR and direct sequencing were employed to analyze the three exons of MECP2 gene in 9 children recently diagnosed with Rett syndrome and their parents.</p><p><b>RESULTS</b>Heterozygous mutations were identified in 5 out of 9 patients, with a mutation rate of over 50%; there was one case of insert mutation (c.913insT) and 4 cases of missense mutation (exon 3: c.316C>T (R106W); exon 4: c.502C>T (R168X), c.808C>T (R270X), and c.1126C>T (P376S). A new mutation (c.913insT) was found. No mutations were detected in their parents. Two patients had MECP2 mutations in the transcriptional repression domain (TRD). They had almost lost language functions and were found to have significantly delayed development compared with other patients.</p><p><b>CONCLUSIONS</b>Mutations in MECP2 gene were detected in 5 confirmed cases of Rett syndrome, and most of them were on exon 4. Mutations in the TRD of MECP2 protein may affect the language ability and development in children with Rett syndrome.</p>

A single-site retrospective study of pediatric arterial ischemic stroke etiology, clinical presentation, and radiologic features / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Stroke occurs upon obstruction of cerebral blood circulation and is clinically characterized by sudden onset symptoms. Advanced age is the main risk factor of stroke, but cases of pediatric stroke have been rarely reported. This study aimed to determine the etiology, clinical presentation, and radiologic features of neurological deficit for pediatric arterial ischemic stroke (PAIS).</p><p><b>METHODS</b>The medical records of 42 PAIS patients (age range: 9 months to 13 years) treated at Wuhan Children's Hospital between July 2007 and January 2011 were retrospectively reviewed. Infarction location was first determined by craniocerebral computed tomography and magnetic resonance (MR) imaging. The stenotic or occluded main cerebral arteries and/or branches were determined by MR angiography and digital subtraction angiography.</p><p><b>RESULTS</b>The majority of the 42 PAIS cases (66.7%, n = 28) were ≤ 3 years old (vs. >3 years old: 33.3%, n = 14; P<0.05), but the male: female ratio was similar in both groups (P > 0.05). The most frequently reported signs and symptoms for both age groups were limited physical activity followed by convulsions and delirium, but convulsions were more prevalent in children ≤ 3 years-old. Children > 3 years-old mainly experienced the limited physical activity symptoms, including hemiparalysis, aphasia, and ataxia. For all 42 cases, the most frequent etiologies were infections (38.1%, n = 16), iron deficiency anemia (16.7%, n = 7), and moyamoya syndrome (11.9%, n = 5). The predominant infarcts among all cases were middle cerebral artery (63.6%, n = 21) and basal ganglia (64.3%, n = 27).</p><p><b>CONCLUSIONS</b>PAIS occurs more frequently in younger children and this group most frequently presents with convulsion as the initial symptom. The overall etiologies of PAIS may be different from those of adult stroke and the involved regions may be distinguishing features of PAIS or its different forms, but more research is required.</p>

Gene therapy study on bladder cancer with recombinant adenoviral vector carrying LRIG1 gene driven by Survivin promoter / 中华外科杂志

<p><b>OBJECTIVE</b>To investigate the treatment efficiency and mechanism of recombinant adenoviral vector carrying LRIG1 gene driven by Survivin promoter for bladder cancer.</p><p><b>METHODS</b>Human bladder cancer cell line BIU87 and immortalized human bladder epithelial cells SV-HUC-1 were infected with Ad-Surp-LRIG1 and Ad-LRIG, respectively. The selective infection efficiency of Ad-Surp-LRIG1 and Ad-LRIG were evaluated by checking the expression of epidermal growth factor receptor (EGFR). The MTT method was used to test cell growth inhibition ratio of Ad-Surp-LRIG1 and Ad-LRIG. Heterotransplanted models of human bladder cancer cell line BIU87 cells in nude mice were established. The mice were randomly divided into 3 groups during the experiment: Ad-Surp-LRIG1 group received viral supernatant solution of Ad-Surp-LRIG1 by tail vein injection; Ad-LRIG group received viral supernatant solution of Ad-LRIG by tail vein injection; and PBS group received phosphate buffer solution (PBS). The growth of tumors were observed and the growth curve was mapped. The expression of LRIG1 and EGFR were examined by reverse transcription PCR (RT-PCR).</p><p><b>RESULTS</b>When Multiplicity of infection was 25, the transfection efficiency of Ad-Surp-LRIG1 was 74.56% in BIU87 cells and 0 in SV-HUC-1 cells (χ² = 58.640, P = 0.000), while the transfection efficiency of Ad-LRIG was 68.27% in BIU87 cells and 72.52% in SV-HUC-1 cells (χ² = 0.075, P = 0.784). The transfection efficiency difference of Ad-Surp-LRIG1 and Ad-LRIG in BIU87 cells was not statistically significant (χ² = 0.016, P = 0.898). Compared with PBS, Ad-Surp-LRIG1 and Ad-LRIG1 could inhibit BIU87 cell growth, the difference was significant in 4 days after transfection (F = 15.960, P = 0.000). There was not significant difference in cell growth rate of Ad-Surp-LRIG1 group and Ad-LRIG1 group. The tumor growth rate in Ad-Surp-LRIG1 group was slower than that in the other 2 groups. The tumor quality in Ad-Surp-LRIG1 was lighter than that in the other two groups, the differences were statistically significant (F = 97.860, P = 0.000), the quality difference in Ad-LRIG1 group and PBS group was not statistically significant difference (t = 1.73, P = 0.06). Compared with Ad-LRIG1 group and PBS group, the mRNA expression of LRIG1 was obviously up-regulated and that of EGFR was down-regulated in Ad-Surp-LRIG1 group (P < 0.01).</p><p><b>CONCLUSIONS</b>The recombinant adenoviral vector of Ad-Surp-LRIG1 could selectively transfected BIU87 cells, which could inhibit significantly the growth of bladder cancer in vivo and in vitro, the mechanism may be partly LRIG1 can downgrade the expression of EGFR.</p>

Effects of TNF-α on ICAM-1 and LFA-1 expression in peripheral blood mononuclear cells of children with febrile seizures / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the effects of TNF-α on ICAM-1 and LFA-1 expression in peripheral blood mononuclear cells (PBMC) of children with febrile seizures (FS).</p><p><b>METHODS</b>Sixteen children with FS and 16 age- and gender-matched healthy children were enrolled. The samples of PBMC from FS children were randomized into two groups with or without TNF-α treatment (TNF-α concentration 1.0 ng/mL). PBMC were purified and cultured with a conventional method in vitro. The expression of ICAM-1 and LFA-1 in PBMC was determined by flow cytometry (FCM).</p><p><b>RESULTS</b>ICAM-1［(20±9)% vs (14±7)%)］and LFA-1［(43±16)% vs (30±16)%］expression in PBMC in the untreated FS group was significantly higher than that in the normal control group (P<0.05). Compared with the untreated FS group, the treatment with TNF-α remarkably increased the ICAM-1 expression［(27±11)%］(P<0.05). PBMC LFA-1 expression［(52±21)%］in the TNF-α-treated group was higher than that in the untreated FS group, although there were no statistical differences between the two groups.</p><p><b>CONCLUSIONS</b>TNF-α treatment may increase LFA-1 and ICAM-1 expression in PBMC of children with FS.</p>

Expression of pituitary tumor transforming gene in patients with AML / 中国实验血液学杂志

The aim of study was to explore the expression of pituitary tumor-transforming gene (pttg) in acute myeloid leukemia (AML) and its relationship with the pathogenesis of AML, simultaneously to investigate the difference of the pttg expression among AML different subtypes. The expressions of pttg mRNA were quantitatively detected by real-time fluorescence quantitative polymerase chain reaction (real-time PCR) in bone marrow from 47 patients with AML and 28 normal controls. The results indicated that the expression of pttg mRNA was significantly higher in AML patients [(1.1323 ± 1.3934) × 10(5)] than that in normal controls [(4.5766 ± 1.1817) × 10(3)] (p < 0.05). The expression of pttg mRNA was higher in M(3) patients than that in other AML subtypes, such as M(1), M(2), M(4), M(5). It is concluded that the overexpression of pttg may be related to the pathogenesis and progression of AML, in which the overexpression of pttg may be more intimately related to the pathogenesis and progression of M(3). This study provides a new idea to research the pathogenesis and targeted gene therapy of AML.

A recommendable standard protocol of adult male circumcision with the Chinese Shang Ring: outcomes of 328 cases in China / 中华男科学杂志

<p><b>OBJECTIVE</b>Standardization of and training in adult male circumcision can significantly reduce its complication rate. Currently no such program exists for its standardization and training, making it difficult to guarantee the quality of male circumcision services. We therefore established a standardized surgical protocol for adult male circumcision in China using the Shang Ring, and applied it to a clinical study examining the performance of the Shang Ring in adult male circumcision.</p><p><b>METHODS</b>A total of 328 adult men aged 18-58 (mean 27.8) years, 25 with phimosis and 303 with redundant prepuce, underwent circumcision with the Shang Ring, and evaluation of the operation time, pain scores (using the visual analog scale), postoperative complications, time for wound healing, and their satisfaction with the postoperative appearance.</p><p><b>RESULTS</b>The operation time was 4.7 +/- 1.3 minutes. The pain scores were 0. 2 +/- 0.6 during the surgery, 1.6 +/- 1.0 twenty hours postoperatively, 1.7 +/- 1.1 twenty hours prior to the ring removal, and 2.7 +/- 1.4 during the ring removal. Complications included infection in 2 (0.6%), bleeding in 2 (0.6%), and wound dehiscence in 2 (0.6%) of the patients. None of the patients with wound dehiscence required postoperative suturing and all were managed conservatively instead. Sixteen of the patients (4.9%) experienced penile edema. The time for complete wound healing after circumcision was 20.3 +/- 6.7 days. The rate of the patients'satisfaction was 99.7% (327/328).</p><p><b>CONCLUSION</b>The standard protocol of adult male circumcision with the Shang Ring has the advantages of short operation time, slight pain, low rate of complications, and high satisfaction and acceptance of the patients. Strict standardization of the surgical protocol can maximize its clinical advantages for adult male circumcision.</p>

Efficacy and Safety of Levetiracetam Monotherapy on Children with Epilepsy / 实用儿科临床杂志

Objective To evaluate the efficacy and safety of levetiracetam (LEV) monotherapy on children with epilepsy.Methods Forty-one children (26 cases were male,15 cases were female) with epilepsy aged 7 months to 13 years were treated with LEV as monotherapy.These patients were selected from Department of Neurology ,Wuhan Children′s Hospital, from Aug.2007 to Aug.2009.The starting do-sage of LEV was (13.6?4.7) mg?kg-1?d-1,twice daily,and its objective dosage was (25.7?7.5) mg?kg-1?d-1,twice daily.LEV monotherapy was investigated by a self-controlled and open-label research,and the follow-up period ranged from 6 months to 2 years.Results The effective rate was 68.3% (28 cases),with 39.0% (16 cases) achieving seizure freedom in LEV monotherapy of children with epilepsy.Thirteen patients (31.7%) had poor efficacy in reduction of seizures,7 patients (17.1%) discontinued LEV monotherapy due to either an inadequate seizure control or aggravated seizures.Fifteen patients (36.6%) had the therapy-related adverse events in LEV monotherapy,including gastrointestinal dysfunction (5 cases),irritability (5 cases),dizziness (2 cases) and somnolence (2 cases).The adverse effects appeared in 2-4 weeks of early LEV therapy and were spontaneously disappeared in 1 week to 1 month of continuing therapy.Conclusions The LEV monotherapy is effective and safe for the control of partial and generalized seizures in children with epilepsy.LEV appears to be a broad-spectrum,first-line anti-epileptic drug in treatment of children with epilepsy.

Prognosis of Children with Tourette Syndrome and Affective Factor / 实用儿科临床杂志

Objective To explore the risk factors affecting prognosis of children with Tourette syndrome(TS).Methods The follow-up visits were conducted on the clinical data of 98 cases with TS(85 male,13 female;aged 4-16 years old)from 1997 to 2005 in Wuhan children's hospital.All cases were consistent with the diagnostic criteria of TS in the 4th edition of Diagnostic and Statistical Manual of Mental Disorders(DSM-Ⅳ).The investigations were performed by the investigators who received special training using the unified questionnaire with the methods of direct inquiry or by telephone.The factors included sex,age,severity of TS,the primary symptoms,family history,coexisting diseases,basic diseases,perinatal abnormity and family-social relations.The prognosis of TS and these factors were analyzed by linear regression and stepwise regression with SPSS 12.0 software.Results About 16 cases lost follow-up and the other 82 cases with follow-up(72 male and 10 female)received retrospective review.They were 14 to 25 years old with complete data,and 50 cases healed,32 cases not healed.Results from non-conditional simple variant Logistic regression showed that such cases were associated with the following factors:age,family history of TS,severity of TS,coexisting diseases,basic diseases and perinatal abnormity(Pa0.05).Out of 6 suspicious factors,there were coexisting diseases(OR=84.088,95%CI 10.850-651.682),severity of TS(OR=13.956,95% CI 2.412-80.762),and family history of TS(OR=27.127,95% CI 1.047-702.831)of risk factors.Conclusion The long-term prognosis of children with TS may be related with coexisting disease,severity of TS and family history respectively.

Age-Dependent Feature of Damage of Hippocampus at Different Maturational Stages after Repeated Seizures in Rats / 实用儿科临床杂志

Objective To observe age-dependent feature of damage of hippocampus to different maturational stages rats after kindling repeated seizures.Methods The effects of 5 daily pentylenetetrazol-induced convulsions in different rats beginning at postnatal day 10,20,60(P10,P20,P60)were evaluated.In the 3 groups,Thionin staining method was utilized to observe morphological changes and cell counting of dentate granule cells,CA3,CA1,and hilar neurons.Timm's method of silver sulfide staining was adopted to observe the mossy fiber sprouting.Results 1.Cell counting of CA1,CA3 and hilar neurons in P10 and P20 groups demonstrated no differences from controls in rats,whereas P60 with daily seizures had a significant decrease in CA1,CA3 neurons(8.22?1.88,5.62?1.68 vs 6.31?1.50,3.62?1.40)(t=2.246,2.587 Pa

Clinical Characteristics and Diagnosis of Children with Poliomyelitis-Like Syndrome / 实用儿科临床杂志

Objective To investigate the clinical characteristics and diagnostic main points of poliomyelitis-like syndrome(PS).Met-hods The clinical data of 15 children with PS were analyzed retrospectively including nervous system manifestation,results of lab andauxiliary examination,diagnosis and reason of misdiagnosis,treatment and progress.Results PS in all 15 children was characterized by mean age of onset(3.3 years),and forerunner infection(in 11 cases,73%)with acute upper respiratory infection of diarrhea 1-2 weeks pre-onset or during onset.All cases were acute flaccid paralysis,12 cases(80%) of which were only one limb.The positive outcome of serologic examination in PS consisted of 4 cases in coxsackie virus-IgM,1 case in EB virus-IgM,1 case in herpes simplex virus-IgM and 1 case in mycoplasma-IgM.All children showed the electromyologram changes in nerve damage.The muscle force of 4 cases increased one grade.Conclusions Children with PS are characterized by the age of onset under 5 years old,acute flaccid paralysis(mostly affected one limb),and the most pathogen being enterovirus.The electromyologram examination can help establish a definite diagnosis in PS.

Significance of Biological Rhythm on Selective Nocte Treatment on Autosomal Dominant Nocturnal Frontal Lobe Epilepsy / 实用儿科临床杂志

0.05).The adverse effect of treatment group was significantly less than control group(P