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    06 January 2011, Volume 26 Issue 01 Previous Issue    Next Issue

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    Effects of early therapeutic intervention with nerve growth factor on brain damage following severe asphyxia in neonates.
    ZHANG Zhe,ZHOU Wei,LU Wei-neng,RONG Xiao,CHEN Xiao-wen.
    2011, 26(01): 33. 
    Abstract ( )  

    Abstract: Objective To investigate the effects of early therapeutic intervention with nerve growth factor(NGF) on brain damage following severe asphyxia in neonates. Methods From Jan.2007 to Oct.2009,fifty-two cases of perinatal severe asphyxia in Guangzhou Children’s Hospital were randomly assigned to two groups: the control group (26 cases) received routine treatment(including cerebrolysin and citicoline); the study group (26 cases) was given NGF on the basis of routine treatment as early as possible (within twenty-four hours after birth). The incidence of the mortality were observed; the degree of hypoxic-ischemic encephalopathy (HIE) was evaluated; and the Neonatal Behavioral Neurological Assessment (NBNA) was done in both groups. Myelin basic protein(MBP) and S-100 protein were measured dynamically by ELISA in serum from all patients. Results There was no significant difference of the incidence of severe HIE and the mortality between the study group and the control group(23.08% vs 38.46%,χ2 = 1.4444,P > 0.05; 3.85% vs 7.69%,χ2 = 0.0000,P > 0.05). The level of blood serum MBP or S-100 protein in the asphyxiated newborns was higher than that of the normal term newborns. At 3d and 7~8d after birth,the level of blood serum MBP or S-100 protein in the study group was much lower than that in the control group. The percentage of the cases whose NBNA marks were less than 35 at 7~8 d and 14~16 d after birth in the study group was much lower than that in the control group(40.00% vs 70.83%,χ2 = 4.7055,P < 0.05;20.00% vs 50.00%,χ2 = 4.8640,P < 0.05). Conclusion Using NGF on the basis of the routine treatment as early as possible after resuscitation can reduce markedly the severity of asphyxia-induced brain damage in neonates.

    Electroclinical features and prognosis of epilepsy in children with methylmalonic acidemia.
    ZHAO Ying,ZHANG Yue-hua,YANG Yan-ling,MA Xiu-wei,LIU Xiao-yan,YANG Zhi-xian, BAO
    2011, 26(01): 37. 
    Abstract ( )  

    Abstract:Objective To summarize the clinical features and prognosis of epilepsy in children with MMA. Methods From Jan. 1997 to Dec. 2009 in Department of Pediatrics,Peking University First Hospital,the medical records of hospitalized MMA patients complicated with epilepsy were retrospectively reviewed. The clinical manifestations,laboratory examination results,and treatment modalities were analyzed. Results From 63 pediatric inpatients diagnosed with MMA,27 children (42.9%) complicated with epilepsy were enrolled in this study. These 27 patients were also accompanied with other neurological manifestations, including mental retardation or regression (n = 22),lethargy (n = 10),increased muscle tone (n = 8),muscle hypotonia (n = 8),recurrent vomiting (n = 4),tremor (n = 2),ataxia (n = 2),and abnormal posture (n = 1).The onset age of seizure ranged from 8 days to 11 years. The seizure types included partial seizure(n = 21),generalized tonic-clonic seizure (n = 5),tonic seizure (n = 3),myoclonic seizure (n = 3),and epileptic spasm (n = 2). Five patients had 2 or 3 seizure types. Nine patients (33.3%) had a history of status epilepticus. EEG showed slow background activity in 17 patients,focal or multifocal discharges in 16 patients,generalized discharges in 4 patients,hypsarrathmia in 2 patients,and suppression-burst pattern in 1 patient. Cranial MRI showed cerebral atrophy (n = 14),white matter changes (n = 12),agenesis of corpus callosum (n = 2),abnormal signal in basal ganglia (n = 2),and cerebellar atrophy (n = 1). Twenty-one patients were MMA combined with homocysteinemia.Seventeen patients were confirmed with cobalamin C disease and one with partial mutase deficiency (mut-). Vitamin B12-responsive patients had a better outcome compared with vitamin B12-unresponsive patients. Conclusion Epilepsy is a common manifestation of patients with MMA.Partial seizures is more common than other seizure types.Urine organic acid analysis should be performed for children with unknown cause of epilepsy combined with other neurological manifestations, so as to promptly identify the etiology and improve the prognosis.

    Clinical value of p-Hcy,hs-CRP,LDH and TSGF measurement in children with acute lymphocytic leukemia.
    ZHANG Yuan,XU Xue-ju,YANG Cheng-sheng,WANG Ying-chao,FANG Ying-qi,ZOU Xiang,LIU
    2011, 26(01): 41. 
    Abstract ( )  

    Abstract:Objective To detect the changes of the plasma homocysteine(p-Hcy),high-sensitivity C-reactive protein(hs-CRP),1actic dehydrogenase(LDH) and tumor specific growth factor(TSGF) determination in patients with acute lymphocytic leukemia(ALL) and its clinical significance. Methods p-Hcy(enzymatic cycling assay),hs-CRP(turbidimetry respectively),LDH(automatic analyzer) and TSGF (with biochemistry)before treatment and after remission were measured between ALL children and normal children in Department of Pediatrics,Zhengzhou university's First Hospital from Jan. 2009 to Jul. 2010. Results Before chemotherapy and after complete remission,p-Hcy,hs-CRP,TSGF and LDH levels in the ALL-CR patients were significantly higher than those in the controls(P < 0.01) .The level of p-Hcy,hs-CRP,TSGF and LDH after complete remission was lower than that before treatment(P < 0.05).The level of p-Hcy,hs-CRP,TSGF and LDH after cancer recurrence was higher than that of normal children and after complete remission of ALL-CR children(P < 0.05).p-Hcy level was significantly correlated with LDH,TSGF and hs-CRP level before chemotherapy of ALL-CR children(P < 0.01). Conclusion p-Hcy,hs-CRP,TSGF and LDH can be used as a reference marker in diagnosis,monitoring patients’condition and prognosis evaluation in acute lymphocytic leukemia of children.

    Characteristics and clinical significance of respiratory impedance in children with asthma.
    REN Li-xin, WAN Li-ya, ZHANG Qin, YUAN Yan, DONG Han-quan.
    2011, 26(01): 44. 
    Abstract ( )  

    Abstract:Objective To investigate the characteristics of respiratory impedance in children and adults with asthma and explore the clinical significance. Methods From Jul. 2005 to Jun. 2006 in Tianjin Children's Hospital, IOS (Impulse Oscillometry) was performed in 80 children and 62 adults with asthma and 112 healthy children to obtain respiratory impedance data; at the same time, the conventional pulmonary function test was also performed in some of the patients. Data coming from different groups were analyzed statistically. Results Based on IOS data, total resistance R5 increased(142.19±36.40)% and central resistance R20 was normal[(88.53±17.83)%]in child asthma group. Both R5 and R20 increased in adult asthma group[(190.03±49.97)%,(152.53±39.11)%]. In two groups, reactance X5 and Fres decreased or backed off in various degrees, more obviously for Fres. The conventional pulmonary test data also implied that the percentages of FVC and FEV1/FVC were (81.19±15.42)% and (55.57±12.93)% respectively in adult group,and (79.12±18.65)% and (64.81±9.78)% respectively in child group. Conclusion The characteristics of respiratory impedance between children and adults are different. The pathological changes of child asthma are mainly in the small airway where inhaled medicine should reach as far as possible.

    Clinical features of opsoclonus-myoclonus syndrome in children and the analysis of the treatment.
    LIN Xi,BAO Xin-hua,CHANG Xing-zhi,XIONG Hui,ZHANG Yue-hua,QIN Jiong.
    2011, 26(01): 47. 
    Abstract ( )  

    Abstract:Objective To identify the clinical features of opsoclonus-myoclonus syndrome and its response to adrenocorticotropic hormone (ACTH). Methods Fourteen OMS cases who were diagnosed during 2006~2010 in Peking University First Hospital were enrolled in this study. Data on medical history, neurological signs,laboratory tests,response to ACTH and the relapse were evaluated. Results Among 14 cases, eight were male and six were female. Age at presentation ranged from 12 to 44 months (average 20.7 months). Main symptoms at presentation were opsoclonus (14/14),myoclonus and ataxia (14/14),poor sleeping (14/14),irritability (14/14). Neuroblastoma was found in one of the fourteen cases. EEGs of all showed no epileptic discharges. Before the diagnosis of OMS,7 cases were ever misdiagnosed as acute cerebellar ataxia,4 were misdiagnosed as epilepsy, 4 were misdiagnosed as encephalitis and 1 was misdiagnosed as hereditary metabolic disease. All patients received ACTH therapy. Neurologic function improved in all children, but seven children had relapse within 3~12months (average 5.7 months). Conclusion OMS is a rare autoimmune neurological disorder which is often present in young chlidren.Neuroblastoma is common in children with OMS.OMS is characterized by rapid,involuntary and irregular conjugate eye movements (opsoclonus),myoclonic jerking of the limbs and trunk,ataxia,poor sleep and behavioral disturbances. Due to the possible immune-mediated mechanisms, treatment with ACTH can be successful, but relapse rate is high and the outcome is unfavourable. Neurological sequelae such as behavioral, language and cognitive problems occur in the majority.

    △GT MUTATION of NCF1 in children with autosomal recessive chronic granulomatous disease.
    HE Jian-xin,YIN Ju,LIU Xiu-yun,SHEN A-dong,SHEN Kun-ling,JIANG Zai-fang.
    2011, 26(01): 50. 
    Abstract ( )  

    Abstract:Objective To report and analyze the course of detection of △GT mutation of NCF1 in one case with autosomal recessive chronic granulomatous disease. Methods We evaluated hotspot △GT of NCF1 in one child with CGD who was admitted to Beijing Children's Hospital in Apr. 2009. Results We identified homogenous GT deletions in the start of exon 2 of NCF1. Conclusion Detection of △GT mutation of NCF1 must be emphasized in autosomal recessive CGD cases.