Acute liver failure is a pediatric clinical critical disease with complex etiology,rapid progress and medical treatment,contributing to a high fatality rate. Artificial liver support system can remove a variety of toxic substances through mechanical,physicochemical or biological device to replace part of liver function like metabolism,detoxification,or synthesis temporarily to win precious time for liver cell regeneration and further clinical therapy,which has become an important treatment of acute liver failure at present. Considered the limita-tions of different blood purification mode,combined mode of blood purification is mostly applied in clinical ther-apy. This article aims to review the effect of plasma exchange combined with continuous veno-venous hemodial-ysis/filtration for the treatment of children with acute liver failure.

BACKGROUND:Nuclear transcription factor(NTF) ?B is a transcription factor,which exists universally in eukaryocyte.It has been identified that its overacting is correlated to the ongoing and development of many diseases.Among the pathomechanism of rheumatoid arthritis,the abnormal activation of NTF ?B has a lot to do with the inflammatory hyperplasia of synovium and the erosion of tissue around articulation.OBJECTIVE:To review the recent progress of NTF ?B,pathogenesis and treatment of rheumatoid arthritis.RETRIEVAL STRATEGY:A computer-based online search of PubMed database was undertaken to identify the articles published in English from January 2000 to July 2007,with the Key words of "nuclear transcription factor ?B,rheumatoid arthritis,pathogenesis,treatment".Meanwhile,Wanfang database was searched for the related Chinese articles published between January 2000 and July 2007,with the same key words in Chinese.A total of 72 articles were finally selected for the first trial.Inclusive criteria:the articles focus on the NTF ?B,the pathogenesis and treatment of rheumatoid arthritis.Exclusive criteria:repeated experiments.LITERATURE EVALUATION:Among the 30 inclusive literatures,7 were related to reviews while the others were clinical or basic researches.DATA SYNTHESIS:①NTF ?B is a significant transcription factor,which participates in regulating many genes related to immune function and inflammation reaction.The promoters of many genes have the binding sites of NTF ?B.②It is indicated that the abnormal activation of NTF ?B plays an important role in the pathogenesis of rheumatoid arthritis.In recent years,many researches inhibiting its activation through different components of signaling pathways have been doing,which become very hot in anti-inflammatory and anti-rheumatism treatment.③In recent years,people have been making great progress in the therapy of rheumatoid arthritis aiming at cytokine and its receptor,however,interfering NTF ?B to treat rheumatoid arthritis is still in study stage.CONCLUSION:NTF ?B target therapy provides a new therapeutic strategy for the treatment of rheumatoid arthritis,which is a very exciting prospect indeed.

Objective To analyze the clinical characteristics and risk factors of prognosis in children with brain trauma.Methods We retrospectively analyzed the clinical data of 125 cases diagnosed as brain trauma in PICU of Shengjing Hospital affiliated to China Medical University from January 2009 to December 2014.The risk factors influencing prognosis were analyzed by using single factor analysis and multiple factors Logistic regression methods.The risk factors included Glasgow coma score (GCS) on admission,blood glucose,lactic acid,prothrombin time,international normalized ratio (INR),serum sodium,serum potassiumin,pulse within 24 hours after admission,gender,age,time for therapy,shock,respiratory failure,cerebral hermia and surgery.Results Eighty-four cases survived and 41 cases died.The fatality rate was 32.8％.T test and chi-square test of risk factors showed that GCS score,blood glucose,blood lactic acid,INR,respiratory failure,shock had a significant influence on the prognosis of brain trauma in children (P ＜ 0.05).Multviariable Logistic regression analysis showed that GCS score,blood glucose,blood lactic acid,respiratory failure were independent risk factors affecting the prognosis of brain trauma (OR =7.434,0.473,0.615,0.000,P ＜ 0.05).Conclusion Pediatric brain trauma has a rapid progress and poor prognosis with high mortality and disability rate.GCS score,blood sugar,blood lactic acid,respiratory failure are independent risk factors for prognosis of brain trauma in children.

Objective To study the changes of serum procaleitonin(PCT) in the children with acute liver failure,and to investigate the relationship between PCT and severity and prognosis of acute liver failure.Methods A retrospective analysis of 24 children with acute liver failure admitted in Shengjing Hospital Affiliated to China Medical University from October 2010 to November 2013 was performed.The changes of serum PCT,blood routine,C-reactive protein,blood culture,virus,Mycoplasma pneumoniae antibody,blood ammonia,serum alaninetransaminase,serum glutamic oxaloacetic transaminase,international normalized ratio and prothrombin time level were observed.Results The serum PCT of children with acute liver failure originally increased at different degree.The serum PCT of 21 cases was more than 0.5 μg/L.The dynamic monitoring results of serum PCT in 6 cases on day 1,day 3,and day 8 were (12.55 ± 13.65) μg/L,(5.62 ±8.12) μg/L,(0.15 ± 0.26)μg/L,respectively,which showed decrease tendency.In 24 children with acute liver failure,serum PCT,international normalized ratio,blood ammonia of survival cases were significantly decreased compared with death cases[(28.37 ±60.22) μg/L vs(12.24 ± 14.76) μg/L;4.28 ± 2.50 vs 3.16 ±1.41 ; (213.30 ± 185.87) μmol/L vs (128.89 ± 102.17) μmol/L] (P ＜ 0.05).Conclusion Acute liver failure could increase the levels of serum PCT.Serum PCT may be an effective index to evaluate liver function,curative effect and prognosis of patients with acute liver failure.

A thermal desorption ( TD) device was developed and coupled to gas chromatography ( GC) or gas chromatography-mass spectrometry ( GC-MS ) for the qualitative and quantitative analysis of semi-volatile organic compounds on atmospheric particulate matters ( PM ) . The TD was operated by direct heating and placed on the GC injector, leading to high heating rate and easy transfer of analytes to GC without focusing of analytes by cold trap. For establishing the TD-GC method, the materials used for supporting PM samples, temperature and time of thermal desorption, and types of sample injection were investigated for detection of sixteen polycyclic aromatic hydrocarbons ( PAHs) and nine n-alkanes. The limits of detection of the proposed TD-GC method were in the range of 0. 014-0. 093 ng for PAHs, and 0. 016-0. 026 ng for n-alkanes, respectively, with the correlation coefficients of correlation above 0. 9975. The TD-GC method was applied to the determination of trace PAHs and n-alkanes on PM10 samples from three cities. The recoveries were in the range of 95%-135% ( PAHs) and 95%-115% ( n-alkanes) , respectively. Finally, the TD was coupled to GC-MS for comparison of the contents of PAHs and n-alkanes on PMx with different particulate size ( x=10 , 5, 2, 1, 0. 5, 0. 25, 0. 1).

Objective To explore the role of CD3 +CD8 +T cells in children with severe bacterial meningitis at early stage by studying the changes of CD3 +CD8 +T cells and its relationships with markers of inflammation and humoral immunity. Methods Thirty-nine cases aged ≤14 years old were enrolled in this retrospective study,who were admitted in our PICU from Aug 1. 2014 to Dec 31. 2015 with diagnosis of se-vere bacterial meningitis. The patients were divided into 2 groups according to the changes of CD3 +CD8 + T cells:group A:normal or elevated(≥190/mm3,n=22),group B:decreased( <190/mm3,n=17). Distribu-tion and differences of biomarkers of inflammation,humoral immunity and changes in cerebrospinal fluid be-tween the two groups were analyzed. Results The ratio of CD3 + CD8 + T cells were decreased in 17 (43. 6%)cases. Though there was no statisticly significant difference,the percentile of patients in group B (58. 8%) with Glasgow coma score lower than 8 was higher than that in group A(31. 8%). All the four death were in group B. The mean values of C-reactive protein and procalcitonin were 251. 0(26. 2,417. 0) mg/L,32. 7(0. 9,100. 0) ng/L in group B,which were higher than those in group A[106. 5(12. 0,458. 0) mg/L,4. 5(0. 1,200. 0)ng/L],respectively(P<0. 05). Six cases(35. 3%) in group B with WBC<4 × 109/L was more than 1 case(4. 6%) in group A,and 12 cases(70. 6%) in group B with ratio of neutrophils>80% was more than 7 cases(31. 8%) in group A,there were significant differences(P<0. 05). Fourteen cases(82. 3%) with glucose concentration lower than 2. 0 mmol/L in group B was more than 11 cases (50. 0%) in group A,the difference was significant(P<0. 05). Conclusion CD3 +CD8 +T cells might be suppressed in children with severe bacterial meningitis at early stage,and might associate to the damaging de-gree of brain,inflammation reaction and prognosis in those patients,which should be helpful in using of im-mune regulators for children with severe bacterial meningitis.

Objective To observe the clinical effect of plasma exchange(PE) combined with continuous veno-venous hemodialysis filtration(CVVHDF) in children with acute liver failure.Methods Thirty-nine cases with acute liver failure admitted to pediatric intensive care unit of Shengjing Hospital Affiliated to China Medical University from August 2008 to January 2014 were treated with PE combined with CVVHDF.CVVHDF was performed after PE immediately,each PE + CVVHDF lasted 24 ～ 72 hours.Prothrombin time,blood ammonia,alanine transaminase,aspartate transaminase,serum total bilirubin,conjugated bilirubin were detected before and after PE + CVVHDF treatment.Glasgow Coma Scale were assessed before and after PE +CVVHDF in the thirty-five cases with hepatic encephalopathy.Results After the treatment of PE + CVVHDF,20 children were clinical recover,7 died,12 abandoned the treatment and the results of follow-up were death.PE + CVVHDF was effective in the treatment by decreasing serum total bilirubin,conjugated bilirubin,alanine transaminase,aspartate transaminase [(128.8 ± 82.6) μmol/L vs (73.2 ± 92.4) μmol/L; (90.2 ±71.5) μmol/L vs (55.1 ± 42.6) μmol/L; (3 024.1 ± 2 457.0) U/L vs (1 256.8 ± 757.8) U/L; (3 420.6 ±2 216.3) U/L v s (579.4 ± 338.6) U/L] (P ＜ 0.05),increasing Glasgow Coma Scale (9.5 ± 3.1 vs 12.1 ±2.9) (P ＜0.01),shortening prothrombin time[(40.4 ± 23.0) s vs (22.8 ±9.4) s] (P ＜0.01),in association with the improvement of hemorrhage tendency.Conclusion PE + CVVHDF significantly improves clinical symptoms and biochemical abnormalities in children with acute liver failure.Security of PE + CVVHDF is good and with no obvious adverse reactions.

Objective To investigate the clinical manifestations ofi nfantile and late-onset glycogen sot rage diseaes type Ⅱ.Mte hods We analyzed the cliin calm anifestations and prognosiso f infantile and late-onset glcy ogen storage disease type Ⅱ with a retrospective analysis of five cases admitted in PICU of Shengjing Hospital of China Medical University from 2013 to 2014.Resulst Firsts ymptoms of three infan-tile cases were dyspnea,cardiac hypertrophy,hepatomegaly,skeletal muscle weakness and low concentration of α-glucosidase A.Two cases completed gene detection.One case had frameshift mutation and missense mu-tation,and the other had two missense mutatoi n.Three infantile csa es all showed arrhythmia performance. Two cases died of fat l arrhythmia.One caes received ne zyme replacement therapy and survived.The main symptoms of two al te-onset cases who had not get gene detection were dyspnea,low muscle strength,muscle hypotonia and low concentration of ca idα-gluco sidase.One case receivedm echanicla ventilation,complicated with multiple infections,severe pneumonia andv entilator dependence,finally gave up the treatment.The other died of cardiac arrhythmia.Concluis on Infantile cases have the major symptoms of myocardial hypert o-phy,hepatomegaly,low muscular tension with rapid progression,high mortality and fatal arrhythmia.Late-on-set cases have the clinical features of respiratory failure,proximal limb muscle weakness and be susceptible to ventilator dependence and multiple infections.Enzyme replacement therapy can improve the clinical symp-toms of infantile cases.

Objective To investigate the diagnostic value of plasma B-type natriuretic peptide(BNP) in left to right shunt congenital heart disease accompanied by heart failure in PICU.Methods We retrospectively reviewed the clinical data of 52 cases diagnosed left to right shunt congenital heart disease in the PICU of Shengjing Hospital of China Medical University from January 2012 to June 2014.The cases were divided into negative control group(n=18) and heart failure group(n=34) according to the criteria for the diagnosis of pediatric heart failure.We respectively compared plasma BNP,size of heart defects,left ventricular end-diastolic volume index(LVEDVI),ratio of left ventricular early diastolic filling blood flow velocity and left ventricular late diastolic filling blood flow velocity(E/A),left ventricular ejection fraction(LVEF),pulmonary artery systolic pressure(PASP),and cardiothoracic ratio between the two groups.We analyzed the correlation between plasma BNP and the size of heart defects,LVEDVI,E/A,cardiothoracic ratio,LVEF,PASP.The receiver operating characteristic curve was used to determine the optimal cut-off value of plasma BNP to diagnose heart failure.Results Plasma BNP were 87.7(22.7,165.7)pg/ml in negative control group and 716.5(326.8,1813.0)pg/ml in heart failure group.The plasma BNP level of heart failure group was significantly higher than that of negative control group(Z=5.3,P<0.01).Size of heart defects were 5.0(3.0,6.8) mm in negative control group and 7.4(5.5,9.0)mm in heart failure group.Size of heart defects of heart failure group was significantly higher than that of negative control group(Z=3.5,P<0.01).LVEDVI were (44.6±18.3)ml/m3 in negative control group and (70.8±38.4)ml/m3 in heart failure group.LVEDVI of heart failure group was significantly higher than that of negative control group(t=2.7,P=0.01).E/A were 1.3±0.3 in negative control group and 1.1±0.3 in heart failure group.E/A of negative control group was significantly higher than that of heart failure group(t=2.2,P=0.04).Plasma BNP had a positive relation with cardiothoracic ratio(r=0.49,P=0.01) and a negative correlation with E/A(r=-0.28,P=0.04).The optimal cut-off value of plasma BNP was 181.8 pg/ml.The sensitivity of diagnosis of heart failure was 94% and the specificity was 88%.The area under the receiver operating characteristic curve was 0.951.Conclusion Plasma BNP may comprise a sensitive marker for heart failure of left to right shunt congenital heart disease.It is recommended that 181.8 pg/ml is the optimal cut-off value to diagnose heart failure of left to right shunt congenital heart disease.

Objective To investigate the clinical features of eleven cases of acute liver failure as the initial presentation of hemophagocytic syndrome(HPS),in order to improve the early diagnosis.Methods Eleven cases of acute liver failure as the initial presentation of HPS admitted in PICU of Shengjing Hospital affiliated to China Medical University from September 201 1 to February 2015 were investigated,the clinical manifestations,laboratory findings,therapy methods and prognosis were analyzed.Results Eleven cases of HPS had the initial symptom of acute liver failure accompanied by severe coagulation abnormalities,increase of alanine aminotransferase and aspartate aminotransferase,decrease of fibrinogen.All 1 1 cases with speno-megaly had more than 1 week thermal process.Glucocorticoid and gamma globulin were used to inhibit the activation of monocyte-macrophage cell system.Chemotherapy such as Etoposide were used as the basic treat-ment in the early stage.Plasma exchange and continuous hemodialysis and filtration were used in severe cases with bleeding tendency.One of these 11 children survived,4 cases died of multiple organ dysfunction syn-drome,and discharged six cases were followed up for mortality.Conclusion Unexplained acute liver failure, fever and cytopenias may suggest HPS,the mortality rate can be reduced by early diagnosis and treatment.