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06 July 2012, Volume 27 Issue 07 Previous Issue    Next Issue

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Study on the results analyzed by liquid chromatography-tandem mass spectrometry of dry blood filter paper in 75 children with hepatic diseases.  2012, 27(07): 509-512.  Abstract ( )   Objective    To study the clinical significance of liquid chromatography-tandem mass spectrometry （LC-MS/MS） in the diagnosis of inherited metabolic liver disease in children with the main clinical manifestation of jaundice. Methods    A total of 75 cases of children（including outpatients and inpatients） with the clinical manifestations of jaundice，hepatomegaly and serum enymes increase were collected in Beijing Children’s Hospital  from Apirl 2010 to April 2011. The dry blood filter paper of all 75 cases were analyzed by LC-MS/MS and the data were analyzed by special software. Results The results of LC-MS/MS in 49 cases were normal. The levels of amino acids or acylcarnitines in 26 cases were abnormal，among whom 5 cases were troubled by inherited metabolic liver diseases，15 cases with other diseases，and 6 were with unknown diseases. Conclusion    If the children have the clinical manifestations of jaundice，hepatomegaly and high serum enymes，especially with high blood ammonia orlactic acid，the possibility of inherited metabolic liver disease should be considered. The levels of amino acids or acylcarnitines can be abnormal in some children without inherited metabolic liver disease because of liver secondary injury.

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Death analysis of Epstein-Barr virus associated hemophagocytic lymphohistiocytosis in children.  2012, 27(07): 513-516.  Abstract ( )   Objective    To explore the main causes of deceased cases and factors related with Epstein-Barr virus associated hemophagocytic lymphohistiocytosis（EBV-HLH） fatality. Methods    A retrospective study was performed on EBV-HLH cases admitted to Beijing Children’s Hospital between June 2003 and October 2010. All patients’ medical records were reviewed and analyzed. Follow-up was taken to get the prognosis information of all cases. Statistical evaluation was conducted using multivariate and univariate analysis. Results    In the 90 cases of follow-up results， 32 were alive while another 58 were deceased and the overall fatality rate was 64.4%. Most patients （33/36） without undergoing chemotherapy died in short time after diagnosis of HLH （usually less than two months）. The fatality rate was as high as 87.8% in non-chemotherapy group while it was 44.9% in chemotherapy group. Despite being given chemotherapy， 8 patients died within 2 months of diagnosis while 14 cases died 2 months after diagnosis. Seven of 8 patients suffered from severe infection， while 6 cases had severe coagulopathy and 7 cases had multiple organ failure. Causes of late deaths mainly included non-remission of the disease after chemotherapy （9 in 14） and relapse of HLH （4 in 14）. The fibrinogen was lower in deceased group than in survived group， while lactate dehydrogenase was higher. Logistic regression analysis showed that longer than 4 weeks of illness prior to diagnosis （OR=3.436）， non-chemotherapy （OR=11.09） and lower fibrinogen level （OR=1.866） were independent factors related with EBV-HLH fatality. Conclusion    EBV-HLH is a severe disease in pediatric patients with poor outcome and high fatality rate. Severe infection， coagulopathy and multiple organ failure are the main reasons which cause the early deaths， while non-remission of the disease after chemotherapy or relapse of HLH cause deaths after two months of diagnosis. Longer than 4 weeks of illness prior to diagnosis， non-chemotherapy and lower fibrinogen level are factors related to EBV-HLH fatality.

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Population pharmacokinetic analysis of levetiracetam in Chinese children with epilepsy.      WANG Ying-hui*，WANG Li，LU Wei，WEI Min-ji，WU Ye，SHANG De-wei，WANG Yun-xiu. *De 2012, 27(07): 517-52·.  Abstract ( )   Objective    To establish a population pharmacokinetic（PPK）model of levetiracetam in Chinese children with epilepsy.Methods    A total of 250 data from 220 epileptic children were analyzed.Levetiracetam concentrations were determined by the HPLC method.PPK model of levetiracetam was established using NONMEM，a population pharmacokinetic computer program，according to one-compartment model with first-order absorption and elimination.To validate a model，the normalized prediction distribution errors（NPDE） method was used.Results    Regression equation of the base model of levetiracetam was obtained，ie，clearance（CL/F）=1.56×EXP ［ETA（1）］，volume of distribution（V/F）was fixed 20，and Ka = EXP［ETA（3）］，and that of the final model was as follows：CL/F（L/h）=1.35×（WEIGHT/25.26）0.578，V/F（L）=20，KA（/h）=2.11×EXP［ETA（3）］.The final PPK model was demonstrated to be stable and effective in the prediction of serum levetiracetam concentrations by NPDE.Conclusion    A one-compartment mixture model with first-order absorption adequately describes the levetiracetam concentrations.Weight is identified as a significant covariate on levetiracetam clearance in this study.

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Treatment for a child with EBV-associated T/natural  killer-cell lymphoproliferative disorder by hematopoietic stem cell transplantation.     WU Nan-hai，GONG Xiao-jun，LUAN Zuo，WANG Kai，TANG Xiang-feng. Department of pediat 2012, 27(07): 522-525.  Abstract ( )   Objective   To explore the therapeutic effect of hematopoietic stem cell transplantation for treatment of EBV-associated T/Natural Killer-cell Lymphoproliferative disorder in children. Methods   A 13.5-year old boy，diagnosed with EBV-associated T/natural killer-cell lymphoproliferative disorder （EBV-T/NK-LPD），received HLA-matched sibling PBSCT from his younger sister， as his disease was persistent after treatment with anti-virus drugs，EBV-CTL，interleukin-2 and chemotherapy. The conditioning regimen was TBI/CY+VP16  （total-body irradiation，12 Gy in 8 fractions，-8d～-5d；cyclophosphamide，60mg/kg×two doses，-3d～-2d；a single dose of etoposide，30mg/kg，-4d）. The infused PBSCs contained 6.34×108/kg nucleated cells and 3.80×106/ kg CD34 positive cells. GVHD prophylaxis consisted of cyclosporine A （CsA） and a short course of methotrexate （sMTX）.  Results   The hematologic recovery was favorable. The patient achieved neutrophil engraftment at day +16；platelet engraftment was achieved at day +37；his hemogram completely recovered at day +60.  The patient had no severe complications，such as fatal infections，hemorrhagic cystitis，veno-occlusive disease （VOD），interstitial pneumonia （IP），cytomegalovirus （CMV） infection，et al.  He developed cutaneous Grade II acute graft-versus-host disease （GVHD） at day +62. The EBV-DNA was 1.2×105 copies/ml before transplant，and changed-over to negative at day +14； after this，the EBV-DNA was monitored weekly，persistently negative. After transplantation，the patient’s jaundice disappeared，and the liver function normalized. Rechecking type-B ultrasonic on the cervix and the abdomen，there was no signs of lymphadenectasis at pars cervicalis，hepatomegaly，or splenomegaly；the previous lymphadenectasis at porta hepatic and aside abdominal aorta disappeared. Computed tomography scan at thorax displayed no lymphadenectasis at portopulmonary，the mediastinum was normal. Up to now，and the patient has been followed up for 18 months after transplantation，he is still in continuing complete remission. Conclusion   This was the first case who was treated by hematopoietic stem cell transplantation for  EBV-T/NK-LPD in Mainland China. The transplantation was successful. The patient with  EBV-T/NK-LPD can reach remission by hematopoietic stem cell transplantation，and likely to be cured with this therapy.

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Analysis of prognosis of pediatric liver failure in 76 cases.  2012, 27(07): 526-529.  Abstract ( )   Objective    To summarize the clinical data of 76 cases pediatric liver failure and analyze  the prognostic factors. Methods    All of the 76 cases were selected in Children’s Hospital Affiliated to Chongqing Medical University from Jun.2001 to Nov.2010，which were analyzed by using EXCEL and SPSS18.0 sofewares;one-factor analysis was done by ordinal regression model.Multifactors were  analyzed by logistic regression model. Results    （1）The survival rate of liver failure in children was  only 17.1%. （2）Etiology was not clear in 32 children（42.1%）with liver failure；clear in 44（57.9%） ； 72.7% were caused by congenital biliary malformation，CMV，HBV and EBV infection.（3）Twelve children received non-biological artificial liver treatment，3 of which survived while 9 had no effect.（4） Univariate analysis  showed  that the factors significantly correlated with prognosis were  age，prothrombin activity，albumin，hepatic encephalopathy，hospital stay and complications. Multivariate analysis showed that the factors significantly correlated with prognosis were  hepatic encephalopathy and hospital stay. Conclusion    Liver failure in children progresses quickly，whose mortality rate is high and most of which has unknown aetiology. Factors significantly correlated with prognosis are hepatic encephalopathy and hospital stay. It is meaningful for  diagnosis and treatment to analyze these prognostic factors.

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Clinical retrospective analysis of hemoptysis in 104 children.  2012, 27(07): 530-532.  Abstract ( )   Objective    To analyze the clinical characters ，etiology constituent and bronchoscopy manifestation of children with hemoptysis.  Methods    The study was performed in 104 children diagnosed with hemoptysis during 2002.1~2011.3 in Beijing Children’s Hospital. According to their clinical characters， radiologic manifestation and bronchoscopy outcome， sum up the etiology constituent of children with hemoptysis. Results    In 104 cases， male： female=49：55. Idiopathic pulmonary hemosiderosis （IPH） 24 cases，bronchiolitis 29 cases，pneumonia 11 cases，bronchiectasis 7 cases，endobronchial tuberculosis 3 cases，foreign body in bronchus 5 cases，bronchial arterial and pulmonary arterial fistula 6 cases，pulmonary vein obstruction or agenesis 3 cases，suspicious of bronchial pulmonary vessel dysplasia 10 cases（requiring angiogram），congenital cystic adenomatoid malformation（CCAM） of lung 1 case， bronchial inflammatory pseudotumor 1case，tumor of bronchus 1 case，Wegener granulomatosis 1 case，undifferentiated connective tissue disease 1 case，resistant ANCA small vessel vasculitis 1case. Conclusion    In conclusion， acute or chronic low airway infections， idiopathic pulmonary hemosiderosis （IPH） and congenital branch pulmonary vessels dysplasia are the first three etiology of hemoptysis in children.

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A study of antipyretic effect of ChaiHuang Keli with different doses on children with acute upper respiratory tract infection. 2012, 27(07): 533-536.  Abstract ( )   Objective    To Find the best dose of ChaiHuang Keli in clinical treatment of children with acute upper respiratory infection.Methods    Clinical randomized controlled trial was carried out for this research.Totally 200 patients were chosen based on the inclusion criteria，and they  were divided into five groups:positive control group，high，middle，and low dose of ChaiHuang Keli treatment group and placebo group.The patients were divided by age into three levels: below three years old，between 3 and 5，and beyond six；different treatments were given respectively as designed.The control group（Kanggan Keli，10 g each time，three times a day，tid）；treatment group A （ChaiHuang Keli， 6-10g，tid）；placebo group（placebo，10 g，tid）.All patients had the same three-day course of treatment and the effects were compared among the groups.Results    Effect ratio （FAS）of the control group，three treatment groups and placebo group were respectively 98.00%，93.17%，97.37%，73.68% and 40.54%.According to the comparison among groups， the effect of group B was nearly the same as group A and the positive control group，while it was better than group C；all treatment groups had quite statistical significance compared with the placebo group in this research.There was no significance among group A、B and C（P＞0.05），but there was statistical significance between treatment group and the placebo group（P＜0.05）.The statistical result t of FAS and PPS analysis was nearly the same.No adverse effects were observed in this research.Conclusion    The middle dose group of ChaiHuang Keli in this research shows the best effect in curing children with acute upper respiratory infection（wind-heat syndrome）， and can be recommended in clinical areas.