According to the hemodynamic changes,circulatory failure is divided into warm shock and cold shock,and severe circulatory failure often co-exist in both conditions in children.Circulatory failure in children is characterized by rapid change on hemodynamic status,pathologic vasodilation,relative or absolute hypovolemia,myocardial depression,altered blood flow distribution,and metabolic disorders.Therapeutic strategies include improving cardiac output and tissue perfusion,achieving SVO2＞70％ and cardiac index to 3.3～6.0 L/( min· m2 ),ensuring the intravascular blood volume and cardiac load.

DNA polymerase γis the only known DNA polymerase in human mitochondria,and is essential for mitochondrial DNA replication and repair.DNA polymerase γ is encoded by POLG gene.POLG-related disorders resulted from mutations of POLG gene comprise a continuum of overlapping phenotypes including Alpers Huttenlocher syndrome and other five subtypes, with high prevalence rate at patients with intractable seizure.Genetic testing for POLG mutations in patients with intractable epilepsies is very important for clinical diagnostics, genetic counseling, and treatment decisions because of the increased risk for VPA-induced liver failure in patients with POLG mutations.

Salvia miltiorrhiza is dry root or rhizome of the labia-tae plant Salvia miltiorrhiza, whose water-soluble ingredients are recognized as its bioactive components in cardiovascular disea-ses. Based on researches at home and abroad in recent years, this article summarizes pharmacological effect resisting myocardi-al ischemia of Salvia miltiorrhiza ’ s water soluble compounds in multi-level from the prevention, treatment and anti-reperfusion damage, to provide theoretical basis for the research and devel-opment of the active monomers in Salvia miltiorrhiza and com-pound preparations.

OBJECTIVE:To observe the clinical efficacy and safety of ambroxol hydrochloride in the treatment of neonatal re-spiratory distress syndrome. METHODS:108 cases of neonatal respiratory distress syndrome were randomly divided into control group and observation group,54 cases in each group. Control group were treated with conventional treatment,such as oxygen inha-lation,anti-infection and nutritional support,observation group was additionally treated with 30 mg/kg Ambroxol hydrochloride in-jection,ivgtt,qd,for continuous treatment of 4-6 d. Clinical efficacy,blood gas indexes [oxygen partial pressure(PaO2),carbon dioxide partial pressure (PaCO2)],12,24 and 48 h after treatment remission time of clinical symptoms,hospitalization time, changes of X-ray film value,tidal volume(VT)and dynamic lung compliance(Cdyn)in 2 groups were compared,and the inci-dence of adverse reactions was observed. RESULTS:The total effective rate in observation group was 94.44%,which was signifi-cantly higher than control group (51.58%),the difference was statistically significant (P<0.05);there was no significant differ-ence in the blood gas indexes in observation group after 12 h(P>0.05),PaO2 was significantly higher than control group and Pa-CO2 was significantly lower than control group 24 and 48 h after treatment,there was significant difference between 2 groups(P<0.05). Remission time of cyanosis,dyspnea,lung moist rales and hospitalization time in observation group were shorter than con-trol group,the difference was statistically significant(P<0.05). X-ray film value,Cdyn and VT levels in observation group were significantly higher than control group,the difference was statistically significant(P<0.05). The incidence of adverse reactions in observation group was 3.70%,which was significantly lower than control group(18.52%),the difference was statistically signifi-cant(P<0.05). CONCLUSIONS:Ambroxol hydrochloride shows obvious efficacy in the treatment of neonatal respiratory distress syndrome,it can improve blood gas indexes and accelerate the recovery from disease,with good safety.

Objective To explore the association of expression between the longevity gene SIRT1 and the forkhead transcription factor FOXO1 in the beta cell of islet in rat,and provide the basic prevention and cure rationale for the type 2 diabetes mellitus.Methods Eleven male SD rats of 18 months old were randomized into two groups:6 in calorie restriction(CR)group and 5 in the control group.After 6 months' breeding,the caudal end of islet was collected.The expression and location of SIRT1,FOXO1 and insulin were detected by immunohistochemistry;sections of pancreas were stained for senescence-associated ?-galactosidase(SA-?-Gal)to identify cell senescence of pancreatic islets.Results SIRT1 and FOXO1 were expressed in both the kytoplasm and nucleus,while insulin and ?-Gal were located in the kytoplasm.Compared with the control group rats,there was higher SIRT1 expression(P0.05)expression was detected in the beta cells of CR group rats.The lower positive rate of FOXO1 located in the nucleus the higher expression of SIRT1 in the rat beta cell of islet.Conclusion Calorie restriction could induce the high expression of SIRT1 in the rat beta cell of islet,and we think SIRT1 may delay the senescence process of rat beta cell through the repression of transcription factor FOXO1;it's beneficial to the prevention and cure for type 2 diabetes mellitus.

Objective To investigate the surgical outcome of cervical spondylotic myelopathy. Methods Twenty -five patients (14 males and 11 females) with cervical spondylotic myelopathy who underwent operative treatment between 2004 and 2007 in our hospital were included in this study. The mean duration of symptoms was 31 months (ranging from 8 days to 10 years) . The follow - up period ranged from 18 months to 4 years (mean 30 months). The study comprised 6 posterior decompression (laminoplasty) and 17 anterior cervical discectomy with fusion and anterior locking plate systems. 2 patients with severe cervical spondylotic myelopathy were treated by operation of the combining anterior and posterior. Results According to COA, the recovery (improving) rate among the tolal patients was classified as exellent in 20 cases, good in 3 cases, fair in 2 cases. The excellend and good rate was 92% . Conculusion Both anterior and posterior surgery were effective for cervical spondylotic myelopathy with effective decompression according to the location of abnormality.

Objective To investigta e the efficayc of bedside plasma exchange( PE) combined con-tinuous veno-venous hemodiafiltration ( CVVHDF ) in childer n with critical hemo lty ic uremic syndrome ( HUS) .Method s Eight patients with HUS from Pediatric Intensive Care Unit of Shanghai Ch ildren′s Hos-pitalw ere included in the present stuyd .The seveir ty of children was gar ded accordni g to peid atric critiac l ill-ness soc re and pediatric riks ofs core mortalityⅢ.Four of them received continuuo s blood purification treat-me nt.Meanwhile,the clinical manifestation and outcom e of HUS weer analyzed.Results Eight children with HUS weer ni itially trae ted with diuretic and blood transfusion for 12-24 hours.Four ac ses who deteriora-ted aggressively were ep rofr med PE and CVVHDF.Plasauto iQ21 and Prisma flex wereu sed with Pir sma TPE 2000 membrna e plasma separator and AN69 M60 membrane filter respectively.All the 4 patients with critical HUS survived after bedside continuous blood purification treatment.Clinical symptoms and serum bio-chemistry were improved sing ificantly as follows.The average levels of serum creatinine and lactate dehydro-genase decreased obviously(318μmol/L vs.162μmol/L;1 963 U/L vs.407 U/L,respectively).In addi-tion,platelet count increased significantly(40 ×109/L vs.97 ×109/L) .Eventually,symptoms disappeared in these 4 patients.Conclusion The combined therapy of PE and CVVHDF in HUS could stabilize fluid acid-base equilibrium,prevent hemolysis and improve the renal function.

Objective To investigate the effect of nCPAP as pre-emptive ventilatory support strategy in severe bronchiolitis. Methods A retrospective analysis was made on cases with nCPAP as pre-emptive ventilatory support strategy for severe bronchiolitis in PICU of our hospital from August 2012 to September 2014. Results Seventy-nine children received nCPAP therapy in our PICU. Overall, nCPAP was successful performed in 69 children, 10 patients required intubations. PaO2 and PaO2/FiO2 in patients received intubations were lower than those in patients received only nCPAP before treatment (P < 0.05). There were significant improvements in PaCO2, PaO2, PaO2/FiO2, respiratory rate and heart rate in the patients received only nCPAP at 12 hours and 24 hours post-treatment (P < 0.05). For the 69 patients with hypercapnia received only nCPAP, PaCO2 was significantly reduced at 2 hours, 12 hours and 24 hours post-treatment (P < 0.01). Conclusion Clinically, nCPAP could improve the oxygenation and hypercapnia of patients with severe bronchiolitis. However, these patients, who suffered from severe bronchiolitis with minor PaO2 or PaO2/FiO2, appeared to require intubation as soon as possible.

Aim To investigate the effect of Salvianol-ic acid A (Sal A)on mice with isoproterenol (ISO)-induced myocardial infraction and its possible mecha-nisms.Methods The mice were subcutaneously in-jected with ISO (8 mg·kg-1 )to induce myocardial in-farction.The myocardial protective effect of Salvianolic acid A was evaluated from mortality rate,electrocardio-gram (ECG),heart function,myocardial infarction in-dex,serum myocardial enzymes and its action mecha-nisms were explored from inflammation,anti-oxidation and cells apoptosis.Results Salvianolic acid A dose-dependently enhanced the heart function of myocardial infarction mice,reduced the heart index,inhibited the myocardial enzyme leakage,showed obvious myocardi-al protection effects.ELISA results showed that Salvi-
anolic acid A could reduce the expression of myocardial inflammatory cytokines such as IL-6(interleukin-6,IL-6),TNF-α(tumornecrosis factor-α,TNF-α).West-ern-blotting confirmed that Salvianolic acid A could in-crease the expression of anti-apoptotic proteins Bcl-2, reduce the expression of apoptosis protein Bax,and raise the phosphorylation level of PI3K and Akt.Con-clusion Salvianolic acid A displays a significant pro-tective effect against isoproterenol-induced myocardial infarction and its mechanism may be related to the in-crease of PI3K/Akt signal pathway and the inhibition of cell apoptosis and inflammatory reaction.

Objective To evaluate the value of noninvasive monitoring of pulmonary arterial pressure in the children with severe pneumonia and respiratory failure.Methods A prospective study was adopted to investigate 69 patients who suffered from severe pneumonia and respiratory failure in Pediatric Intensive Care Unit in Shanghai Children's Hospital from June 2013 to December 2013 were involved in this study,except for heart disease.The pulmonary arterial pressure (PAP) and cardiac function were monitored by using bedside color doppler ultrasound cardiogram,such as PAP,cardiac index (CI),left ventricle ejection fraction(LEFT),and heart early diastolic filling velocity maximum/heart late diastolic filling velocity maximum (E/A ratio).They were divided into 2 groups according to PAP,one group as pulmonary arterial pressure normal group,the other group as pulmonary arterial hypertension(PAH) group,and the impact of the PAP on the prognosis and mechanical ventilation was assessed.Milrinone[0.5 μg/(kg · min)] were given the patients who were combined with pulmonary hypertension,and the PAP and cardiac function before using Milrinone and 24 h,48 h and 72 h after giving medicine was observed.Results Among 69 cases,40 cases were male and 29 cases were female,age ranging from 2 months to 12 years old,and the weight range was (14.3 ± 8.9) kg.The pediatric critical illness score(PICS) was 70.5 ± 9.6,and the pediatric risk of score m ortality Ⅲ was 13.5 ± 5.0.Among 69 cases,46 cases had pulmonary arterial hypertension,38 cases of them experienced mechanical ventilation,and 9 cases died.Among 23 cases who had no pulmonary arterial hypertension,only 8 cases experienced mechanical ventilation.There was a significant difference in the mechanical ventilation rate and mortality between two groups(x2 =15.78,P ＜0.0l ; x2 =5.18,P ＜ 0.05).The mechanical ventilation time was longer in pulmonary arterial hypertension group (t =3.89,P ＜0.01).PAP was (58.23 ±5.44) mmHg(1 mmHg =0.133 kPa),(49.10 ±4.69) mmHg,(42.53 ±4.54)mmHg and(35.63 ±4.78) mmHg respectively before and after using Milrinone 24 h,48 h and 72 h in 46 cases with pulmonary arterial hypertension,and the pressure decreased significantly after using medicine (F =67.11,P ＜ 0.01).There was no significant difference in CI,LVEF and E/A(all P ＞0.05).However,9 cases of them did not show any response to Milrinone,and in the end they couldn't live without mechanical ventilation,they died.Conclusions Noninvasive pulmonary arterial pressure monitoring could be beneficial in judging patient's condition and assessing prognosis of children with severe pneumonia and respiratory failure,and milrinone could decrease PAP.