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06 April 2010, Volume 25 Issue 04 Previous Issue    Next Issue

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Clinical and electroencephalogram characteristics of children with late-onset epileptic spasms. 2010, 25(04): 266.  Abstract ( )   To analyze the clinical and electroencephalogram （EEG） characteristics， therapeutic response and long-term prognosis of the children with late-onset epileptic spasms. Methods　The clinical data， EEG features and response to different treatment protocols in59 children with late-onset epileptic spasms were retrospectively reviewed. Thirty-seven of them were followed up for 13～90 months and the age at last contacting ranged from 2 years 6 months to 12 years 2 months （median 60 months）. Results　The onset age of epileptic seizures ranged from 12 to 98 months （median 22 months）. The first seizure type was epileptic spasms in 36 cases （60.0%） and other seizure type in 23 children （39.0%）. Epileptic spasms were the only seizure type during entire period in 20 patients （33.9%）. There were also partial seizures in 17 cases （28.8%） and other generalized seizures in 22 patients （37.3%） during the course of disease. The EEG appeared classic hypsarrhythmia in 14（23.7%）， unilateral hypsarrhythmia in 2 （33.4%） and no hypsarrhythmia in 43 patients（72.9%）. Twenty-two patients （37.3%） presented with focal or unilateral clinical image and/or EEG abnormal. Forty-four patients were symptomatic epilepsies and the predominant diseases included perinatal brain injury， central nervous system infection and brain malformation. Fourteen patients （23.7%） met the criteria of late-onset West syndrome， 7 patients （11.9%） were diagnosed with Lennox-Gastaut syndrome. Fifty patients were responsible to ACTH therapy， including seizure-free in 32% of them. But recurrent rate was 61.5%. In 37 patients who were followed up， 46% maintained seizure free under taking the anti-epileptic drugs at the last follow up. Psychomotor development delay was observed in over 80% of patients who were followed up，16% of them had severe  egression. Multivariate regression with Cox analysis showed that etiologies， seizure types， phenotypes of epileptic spasms， and response to ACTH therapy all had no correlation with the long-term outcome of the seizure or psychomotor development. Conclusion　Epileptic spasms not only occur in West syndrome or infantile spasms， but also in the children beyond infantile period. Late-onset epileptic spasms may be caused by varied severe encephalopathy and it is usually accompanied with partial or other generalized seizures. Most cases do not present typical hypsarrhythmia on EEG. Similar to infantile spasms，most late-onset epileptic spasms are drug resistant and had a very poor outcome. ACTH therapy is still the first line therapeutic drug for such a catastrophic disease.

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Relationship between adipocytokines and adiposity in children and adolescents. 2010, 25(04): 271.  Abstract ( )   To explore the relationship between adipocytokines and the degree of obesity in children and adolescents. Methods　Based on the Beijing child and adolescent metabolic syndrome （BCAMS） study in 2004， 3511 children （1790 boys and 1721 girls） aged 6～18 were recruited. In this study， participants were categorized into three groups： 1626 cases in normal weight group（non-obese group）， 655 cases in overweight  group and 1230 cases in obese group， according to the sex， age and specific body mass index（BMI） . The levels of  adipocytokines  were measured by sensitive， specific double - antibody sandwich enzyme - linked immunosorbent assays （ELISA） . Analysis of covariance and multiple binary logistic regression analysis were performed in statistical analysis. Results　The levels of plasma insulin and serum leptin in overweight group and obese group were higher than those in non-obese group， and they increased with the increase of the degree of obesity. There were significant differences in sex.The level of serum adiponectin decreased as the degree of obesity increased. There was higher serum resistin level only in obese group than that in non- obese group.After adjustment for sex，age and pubertal development in multiple binary Logistic regression analysis ， the odds ratios  （OR ） and 95% confidence intervals of overweight and obesity were 4.38（3.37～5.70） and 12.75（10.11～16.08） for predicting hyperinsulinemia， respectively， 10.42（7.46～14.57） and 41.53 （30.23～57.07） for hyperleptinaemia， respectively， 1.88（1.50～2.35） and 2.81（2.33～3.39） for  ypoadiponectinaemia， respectively， 1.07（0.86～1.34） and 1.32（1.10～1.57） for hyperresistinaemia ， respectively. Conclusion　The levels of plasma insulin， serum leptin and adiponectin in children and adolescents may be intimately related to degree of obesity. The abnormal rates of their levels  increase as the weight of children increases. The prevention and management of obesity in children and adolescents should be of great importance to the prevention of  chronic diseases in manhood.

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Clinical analysis of 168 neonates with respiratory distress syndrome treated by Calsurf and different mechanical ventilation modes. 2010, 25(04): 275.  Abstract ( )   To summarize our experience of Calsurf and mechanical ventilation （MV） to rescue 168 neonates with respiratory distress syndrome （RDS）. Methods　The study involved RDS patients who were admitted to Neonatal intensive care unit （NICU） of Hebei Provincial Children’s Hospital and received Calsurf and MV treatment from Sep. ， 2006 to Feb 29， 2008. The data were retrospectively collected from these cases in a standard form， which included general clinical characteristics， mechanical ventilation modes， complications， outcome， and so on. To evaluate the effect of Calsurf， blood gas and ventilator settings were recorded at 0， 1， 12， 72 hours of Calsurf treatment. Results　Mean （SD） gestational age （GA） of infants studied was 32.3±3.7weeks and 88.7% were preterm （GA < 37 weeks）. Mean birth weight （BW） was 1730±750 grams and 87.5% of them had BW < 2500 grams. The male/female ratio was 4.6（138/30）. At 0 ， 1， 12 and 72 hours of Calsurf treatment， FiO2 of RDS patients was 0.51±0.11， 0.32±0.1， 0.28±0.08 and 0.26±0.09， respectively. Significant decline in FiO2 and increase in PiO2/FiO2 and a/APO2 were observed at different time points， especially between 0 and 1 hour （P < 0.05），but there was no statistical difference. Different ventilation modes were applied to these patients. In 14.9%（25/168） of these infants， surfactant was administered during a brief intubation followed by immediate extubation （INtubation SURfactant Extubation， INSURE ）. Surfactant therapy was successfully given only during NCPAP without intubated ventilation in these infants. High Frequency Oscillation  Ventilation （HFOV） 7.7%（13/168）； SIPPV+VG was the most widely-used ventilation mode 60.11.% （101/168） .and 4.8%（8/168） received inhaled nitric oxide treatment. Ventilation associated pneumonia and pneumothorax were still two major complications. The mortality of the study population was 10.7% including hospital death （4.8%） and withdrawal treatment （5.9%） after parents’ decision. Conclusion　Calsurf can quickly improve oxygenation status of RDS patients. More and more clinicians are applying preventive pulmonary ventilation

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Clinical analysis of ten cases of community-acquired pseudomonas aeruginosa sepsis in previously healthy children. 2010, 25(04): 279.  Abstract ( )   To summarize the clinical features of community-acquired pseudomonas aeruginosa sepsis in previously healthy children.  Methods　We retrospectively reviewed the medical records of children with the diagnosis of pseudomonas aeruginosa sepsis between 2007 and 2008 in Shenzhen Children’s Hospital. Results　Among the ten previously healthy children with community-acquired pseudomonas aeruginosa infection， younger than one year，the attack happened from April to October. The common symptoms were fever,weakness,loss of appetite, abdominal distention, diarrhea, skin rash and tachypnea. The complications included septic shock, multiple organ dysfunction syndrome and DIC. Leucocytopenia was present in sixty percent cases during the disease，all with diverse anaemia. CRP showed elevated in all cases. CRP higher than 100mg/L was present in six cases. All the isolated samples of pseudomonas aeruginosa were susceptible to Piperacillin/Tazobactam ， Levofloxacin， Amikacin, Imipenem and Aztreonam， Others drug-resistance were Cefepime， Gentamicin， Ciprofloxacin， Tobramycin and Ceftazidime. Four children were not given susceptible antibiotics on admission， of whom three died， two died of septic shock and DIC （1 case combined with pulmonorrhage），and one died of acute renal failure.Conclusion　Community-acquired pseudomonas aeruginosa sepsis is usually found in infants and characterized with rapid onset, occuring in summer, high mortality, and complications and severe sequelae. If a child presents fever,weakness,diarrhea, rapid aggravation,sudden septic shock or leukocytopenia with significantly elevated CRP,pseudomonas aeruginosa sepsis should be suspected and anti-pseudomonas antibiotics should be included in the initial empiric antibiotic regimen.

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Clinical significance of stem cell or myeliod antigen expression in children with T-cell acute lymphoblastic leukemia. 2010, 25(04): 283.  Abstract ( )   To investigate the prognosis of children T-cell acute lymphoblastic leukemia（T-ALL） with stem cell or myeloid antigen. Methods　A total of 57 children  with T-cell ALL or lymphoblastic non-Hodgkin’s lymphoma metastasized to bone marrow were  ubclassified into stem cell or myeliod antigen positive T-ALL（T/My+ALL） and stem cell or myeloid antigen negative T-ALL（T/My-ALL）. Results　Totally 31 （54.6%） cases were T/My+ALL， and 26 （45.4%） T/My-ALL. There was no relationship between stem cell or myeloid antigen expression and cyto-morphology，genetics and clinical characters in T-ALL（P > 0.05）; T/My+ALL was not different from T/My-ALL in 7 days prednisone inducement （P > 0.05）. But compared with T/My-ALL， T/My+ALL patients had a poor result on day 19 induction therapy （P=0.002）， and a low complete remission rate on day 35 induction therapy （P = 0.006）. The complete remission rates of T/My+ALL and T/My-ALL were 90.3% and 96.2% ； 5-year event-free survival （EFS） was 35.4% and 65.7% respectively， though it indicated T/My+ALL patients had a poor prognosis；there was no significance（P > 0.05）. Conclusion　Stem cell or myeloid antigen expression in T-ALL/NHL can not be considered as a poor prognosis factor by now.

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Study on intestinal mucosal barrier in children with Henoch-Schonlein purpura. 2010, 25(04): 286.  Abstract ( )   To study the changes of intestinal mucosal barrier in children with Henoch-Schonlein purpura（HSP）. Methods　The plasma diamine oxidase ， D-lactic acid and endotoxin were detected in 60 children with HSP and 30 normal children by the biochemistry methods. Results　The level of plasma diamine oxidase ， D-lactic acid and endotoxin before treatment in HSP group was higher than that in the normal control group， there being statistically significant difference （P < 0.01）. The level of plasma diamine oxidase ， D-lactic acid and endotoxin before treatment in HSP group with gastrointestinal symptoms were higher than that in HSP group without gastrointestinal symptoms and control group and there was also a significant difference between than（P < 0.01）. Comparing the measured level of plasma diamine oxidase ， D-lactic acid and endotoxin before and after treatment， with the exception of HSP group without gastrointestinal symptoms ，there was no significant difference （t ≤ 1.753， P > 0.05）；however，there was significant in other groups （t ≥ 2.556 ， P < 0.01）. Conclusion　There is damage to intestinal mucosal barrier and its function are damaged in children with HSP ，especially in HSP group with gastrointestinal symptoms. The repair of the intestinal mucosal barrier occurs during treatment.

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Gene mutation analysis in a pseudoachondroplasia（PSACH） family. 2010, 25(04): 289.  Abstract ( )   To perform mutation analysis in a pseudoachondroplasia （PSACH） family. Methods　Mutation screening was carried out by DNA-PCR and direct sequencing the exons 8 to 19 of COMP gene. Results　A c.815C > T substitution was identified in exon 8 of COMP gene in the proband and his affected father and brother. Conclusion　The disease in the family is caused by mutation of COMP gene.