OBJECTIVE: To analyze the clinical and molecular characteristics of a child with very long chain acyl-CoA dehydrogenase deficiency (VLCADD). METHODS: Peripheral blood sample of the patient was collected for the extraction of genomic DNA. Next generation sequencing (NGS) was carried out for the proband. Suspected mutations were validated by Sanger sequencing. RESULTS: The patient, a 12-month-old girl, was admitted for diarrhea, vomiting, fever, poor spirit and decreased blood pressure. During the course of the disease, she also manifested hypertrophic cardiomyopathy, cardiogenic shock, elevated myocardial enzyme kinase, fever and metabolic acidosis, and had died after three days due to ventricular tachycardia and respiratory failure. Genetic testing showed that she has carried heterozygous mutations of of the ACADVL gene, namely c.664G>A (exon 8) and c.1056_1057del (exon 10). Blood screening for metabolic genetic diseases showed increased C12, C14, C16, C18, C14:1, C14:2, C16:1, C4/C3 and C8/C3, accompanied with decreased C0, C0/C16 and C8/C10. VLCADD and secondary carnitine deficiency could not be excluded, which was in keeping with the result of genetic testing. CONCLUSION The child was diagnosed with VLCADD, which may be attributed to the compound heterozygous c.664G>A and c.1056_1057del variants of the ACADVL gene.

Objective:To evaluate the effect of parecoxib sodium on phenotypic transformation of alveolar macrophages in a mouse model of ventilator-associated lung injury (VALI).Methods:Forty-five SPF healthy adult male C57BL/6J mice, weighing 22-30 g, aged 8-12 weeks, were divided into 3 groups ( n=15 each) using a random number table method: sham operation group (S group), VALI group (V group) and parecoxib sodium group (P group). Lipopolysaccharide 20 ng was intraperitoneally injected, and 2 h later the animals were mechanically ventilated (tidal volume 30 ml/kg, respiratory rate 70 breaths/min, inspiratory/expiratory ratio 1∶2, fraction of inspired oxygen 21%, positive end-expiratory pressure 0) for 4 h to establish the model of VALI.Parecoxib sodium 30 mg/kg was intravenously injected at 1 h prior to mechanical ventilation in group P. The mice were sacrificed at 4 h of ventilation, the right lung was lavaged and the broncho-alveolar lavage fluid (BALF) was collected for determination of interleukin-6 (IL-6), IL-10 and tumor necrosis factor-alpha (TNF-α) concentrations (by enzyme-linked immunosorbent assay), expression of inducible nitric oxide synthase (iNOS) and arginase-1(Arg-1) in BALF and expression of phosphorylated Janus kinase 2 (p-JAK2) and phosphorylated signal transduction and transcription activator 3 (p-STAT-3) (by Western blot). The left lung was removed for determination of the wet/dry weight ratio (W/D ratio) and for examination of the pathological changes which were scored. Results:Compared with group S, the lung injury score, W/D ratio, concentrations of IL-6, IL-10 and TNF-α in BALF, and expression of iNOS, Arg-1, p-JAK2 and p-STAT-3 were significantly increased in V and P groups ( P<0.05). Compared with group V, the concentration of IL-10 in BALF and expression of Arg-1, p-JAK2 and p-STAT-3 were significantly increased, and the lung injury score, W/D ratio, concentrations of IL-6 and TNF-α in BALF and expression of iNOS were decreased in group P ( P<0.05). Conclusion:Parecoxib sodium promotes phenotypic transformation of alveolar macrophages from M1 subtype to M2 subtype and inhibits inflammatory responses, thus alleviating VALI, which may be related to activating JAK2/STAT-3 signaling pathway in mice.

Objective To evaluate the lung protective ventilation strategy on immune function in patients undergoing radical resection of lung cancer.Methods Sixty patients undergoing thoracoscopic radical resection of lung cancer, 47 males and 13 females, aged 35-64 years, BMI 18-29 kg/m2, falling into ASA physical statusⅠ orⅡ, were randomly divided into 2 groups with 30 cases in each:conventional mechanical ventilation (group C), protective mechanical ventilation group (group P).Volume-controlled ventilation was performed in the 2 groups.Protective mechanical ventilation mode was setted up as follows:tidal volume (VT) 8 ml/kg and respiratory rate (RR) 12-14 breaths/min during two-lung ventilation (TLV) ;VT 6 ml/kg, PEEP 5 cm H2O and RR 14-16 breaths/min during one-lung ventilation (OLV).Before induction of anesthesia (T0), at the end of surgery (T1), 24 hafter surgery (T2), 72 hafter surgery (T3), blood samples were taken from the central venous for determination of peripheral T lymphocyte subsets CD3+, CD4+, CD8+ and NK cell.The CD4+/CD8+ratio was also calculated.Results Compared with T0, the percentage of CD3+, CD4+, NK cell and the CD4+/CD8+ratio was significantly decreased at T1 and T2 in both groups (P<0.05).Compared with group P, the percentage of CD3+, CD4+, NK cell and the CD4+/CD8+ratio was significantly lower in the group C at T1 and T2 (P<0.05).Compared with T0, there was no significant difference at T3 with respect of the percentage of CD3+, CD4+, NK cell and the CD4+/CD8+ratio in the group P while those parameters still lower at T3 in the group C (P<0.05).Conclusion Perioperative use of lung protective ventilation strategy could not only alleviate the immune suppression but also make the immune function recover faster in patients undergoing thoracoscopic elective radical resection of lung cancer.

Objective To investigate the effects of phosphcreatine preconditioning on lung injury induced by renal ischemia-reperfusion (IR) in rats.Methods Forty-five SPF male Sprague-Dawley rats, aged 8-10 weeks, weighing 180-220 g, were randomly divided into 3 groups using a random number table:sham operation group (group S), renal IR group (group IR), and phosphcreatine preconditioning group (group PCr), 15 cases in each group.The rats in group S recieved dissoci ation of renal pedicles and right nephrectomy, on top of which renal IR model was prepared in group IR and group PCr.phosphcreatine 150 mg/kg was injected in group PCr for 30 minutes before ischemia, where as rats in group S and group I/R recieved the normal saline at the same time.The blood samples were obtained from left ventricle at 6 hours after reperfusion, the arterial blood gas analysis was performed in order to determined the oxygen partial pressure (PaO2).Serum levels of malondialdehyde (MDA) and the activity of superoxide dismutase (SOD) were also determined.Fluo 3-AM staining and flow cytometry were used to measure the concentration of alveolar macrophage calcium ions.The lung tissue was obtained with HE staining for determination of microscope examination of pathologic changes, and weight/dry (W/D) ratio were also determined.The lung tissue cell apoptotic rate was measured by Annexin V/PI apoptosis detection reagent staining and flow cytometry.Fluo 3-AM staining and flow cytometry were used to measure the concentration of alveolar macrophage calcium ions.Results Compared with group S, the histopathological demages, W/D ratio, lung tissue cell apoptotic rate, the serum levels of MDA and the concentration of alveolar macrophage calcium ions were signifcant increased (P<0.05), whereas the PaO2 and the activity of SOD were signifcantly decreased in group IR and group PCr (P<0.05).Compared with group IR, the histopathological demages, W/D ratio, lung tissue cell apoptotic rate, the serum levels of MDA and the concentration of alveolar macrophage calcium ions were signifcant decreased (P<0.05), whereas the PaO2 and the activity of SOD were signifcantly increased in group PCr (P<0.05).Conclusion Phosphcreatine preconditioning can attenuate lung injury induced by renal I/R, the mechanism is related to inhabit oxidative stress, and reduce cell apopotosis and calcium overload.

Objective To investigate the effect of flurbiprofcn axetil combined with lung-protective ventilation on postoperative cellular immune function in the patients undergoing thoracoscopic radical resection of lung cancer.Methods Eighty American Society of Anesthesiologists physical status Ⅰ or Ⅱ patients of both sexes,with no abnormal lung function during the preoperative examination,aged 35-64 yr,with body mass index of 18-28 kg/m2,scheduled for elective thoracoscopic radical resection of lung cancer under general anesthesia,were divided into 4 groups (n =20 each) using a random number table method:conventional mechanical ventilation group (group C),flurbiprofen axetil combined with conventional mechanical ventilation group (group F+C),lung-protective ventilation group (group P) and flurbiprofen axetil combined with lung-protective ventilation group (group F+P).Flurbiprofen axetil 2 mg/kg was intravenously injected at 5 min before induction of anaesthesia in F+C and F+P groups.Patients were mechanically ventilated in volume-controlled mode in four groups.Conventional ventilator settings were adjusted with tidal volume (VT) 10 ml/kg and respiratory rate 10-20 breaths/min during two-lung ventilation and with VT 8 ml/kg and respiratory rate 13-16 breaths/min during one-lung ventilation.Lung-protective ventilator settings were adjusted with VT 8 ml/kg and respiratory rate 12-14 breaths/min during two-lung ventilation and with positive end-expiratory pressure 5 cmH2O,VT 6 ml/kg and respiratory rate 14-16 breaths/min during onelung ventilation.All patients received patient-controlled intravenous analgesia (PCIA) at the end of surgery until 24 h after surgery.PCIA solution contained sufentanil 100 μg and ondansetron 16 mg in 100 ml of normal saline in group C and group P.PCIA solution contained sufentanil 100 μg,flurbiprofen axetil 2 mg/kg and ondansetron 16 mg in 100 ml of normal saline in group F+C and group F+P.The PCIA pump was set up with a 0.5 ml bolus dose,a 15-min lockout interval and background infusion at a rate of 2 ml/h.Visual analog scale score was maintained ≤3.When visual analog scale score ＞3,tramadol 2 mg/kg was intravenously injected.Before induction of anesthesia (T0),at the end of surgery (T1),at 24 and 72 h after surgery (T2,3) and at 1 week after surgery (T4),blood samples were collected from the central vein for measurement of the levels of T lymphocyte subsets CD3+,CD4+,CD8+ and NK cells.The CD4+/CD8+ ratio was calculated.Results Compared with the baseline at T0,the levels of CD3+,CD4+ and NK cells and CD4+/CD8+ratio were significantly decreased at T1-3 in C,F+C and P groups and at T1,2 in group F+P (P＜0.05).Compared with group C,the levels of CD3+,CD4+ and NK cells and CD4+/CD8+ratio were significantly increased at T1-3 in the other three groups (P＜0.05).Compared with group F+C or group P,the levels of CD3+,CD4+ and NK cells and CD4+/CD8+ratio were significantly increased at T1-3 in group F+ P (P＜0.05).Conclusion Flurbiprofen axetil combined with lung-protective ventilation improves postoperative cellular immune function and provides better efficacy than either alone in the patients undergoing thoracoscopic radical resection of lung cancer.

Objective To evaluate the optimized efficacy of transversus abdominis plane (TAP) block combined with oxycodone in patients undergoing kidney transplantation.Methods Ninety patients of both sexes,aged 22-45 yr,weighing 45-75 kg,of American Society of Anesthesiologists physical status Ⅲ,scheduled for elective living donor kidney transplantation,were divided into 3 groups (n =30 each) using a random number table:blank control group (group C),TAP block group (group TAP) and TAP block combined with oxycodone group (group TAP+O).Ultrasound-guided lateral approach to TAP block was performed on the operated side with the mixture (20 ml) of 0.375％ ropivacaine and 5 rng dexamethasone at 30 min before induction of anesthesia in TAP and TAP+O groups.Anesthesia was induced by injecting midazolam,target-controlled infusion of remifentanil and propofol and injecting cisatracurium.The patients were mechanically ventilated after placement of the laryngeal mask airway,and the end-tidal pressure of carbon dioxide was maintained at 35-45 mmHg.Propofol and remifentanil were intravenously injected and sevoflurane was inhaled to maintain anesthesia,and cis-atracurium was intermittently injected to maintain muscle relaxation.Narcotrend value was maintained at 37-46 during operation.Oxycodone 0.1 mg/kg was intravenously injected at 20 min before the end of surgery in group TAP+O.Patient-controlled intravenous analgesia (PCIA) was performed with sufentanil 1.5 μg/kg in 100 ml of normal saline at the end of surgery,the PCIA pump was set up to deliver a 2 ml bolus dose,a 20 min lockout interval and background infusion at a rate of 1.5 ml/h,and visual analog scale score was maintained ≤4.The consumption of intraoperative remifentanil and effective pressing times of PCIA within 24 h after surgery were recorded.Blood samples were collected at 1 day before surgery and 2 and 5 days after surgery from the median cubital vein for determination of blood urea nitrogen and creatinine (Cr) concentrations in serum.The occurrence of nausea and vomiting,dizziness and headache,respiratory depression and TAP block-related complications (hematoma at puncture site,injury to abdominal organs,local anesthetic intoxication) was recorded within 24 h after surgery.Results Compared with group C,the consumption of intraoperative remifentanil was significantly reduced,the effective pressing times of PCIA within 24 h after surgery were decreased,and the concentrations of creatinine and blood urea nitrogen in serum were decreased at 2 days after surgery in TAP and TAP+O groups (P＜0.05).Compared with group TAP,the effective pressing times of PCIA within 24 h after surgery were significartly decreased,the concentrations of creatinine and blood urea nitrogen in serum were decreased at 2 days after surgery,and no significant change was found in the consumption of intraoperative remifentanil in group TAP+O (P＞0.05).TAP block-related complications were not found in TAP and TAP+O groups (P＞0.05).There was no significant difference in the incidence of nausea and vomiting or dizziness and headache among three groups (P＞0.05).Conclusion TAP block combined with oxycodone can reduce the intraoperative consumption of opioids,inhibit postoperative hyperalgesia and be helpful for early recovery of transplanted kidney function in patients undergoing kidney transplantation.

Objective To evaluate the effect of preoperative oral rehydration on postoperative recovery in patients undergoing laparoscopic radical resection of colorectal cancer.Methods Eighty patients of both sexes,aged 18-64 yr,with body mass index of 18-24 kg/m2,of American Society of Anesthesiologists physical status Ⅰ or Ⅱ,scheduled for elective radical resection of colorectal cancer,were randomly divided into either routine fluid replacement group (group R) or oral rehydration group (group O) with 40 patients in each group.Fasting for solid food was performed at 8 h before surgery and for water at 6 h before surgery,and fluid was replaced according to the volume expansion during induction and 4-2-1 formula in group R.In group O,multivitamin drinks containing sugar and electrolyte was given orally according to the fasting time:12-15 ml/kg at 8 h of fasting,15-20 ml/kg at 8-12 h of fasting,with the total volume not exceeding 1 200 ml,300 ml at 3 h before surgery,and the rest of fluid was given at different times the night before surgery,and fluid replacement 1 200 ml was performed according to the basic requirement of patients and intraoperative fluid loss.Fluid replacement was maintained at a rate of 1.5 ml · kg-1 · h-1 on the day of surgery in two groups.The volume of oral fluid intake,intraoperative net volume of fluid intake and volume of intravenously given fluid on the day of surgery were recorded.The time of surgery,emergence time,time to first flatus,time to first liquid diet,first ambulation time and length of postoperative hospital stay were recorded.The development of postoperative nausea and vomiting and hypotension was recorded.Peripheral venous blood samples were collected on the morning of day 2 after admission to hospital,the day of surgery and day 1 after surgery (T0-2) for determination of the fasting blood glucose and insulin concentrations,and insulin resistance index was calculated.Results Thirty-seven and 35 patients were included in R and O groups,respectively.Compared with group R,the intraoperative net volume of fluid intake and volume of intravenously given fluid on the day of surgery were significantly decreased,fasting blood glucose and insulin concentrations and insulin resistance index were decreased,and the time to first flatus,time to first liquid diet and length of postoperative hospital stay were shortened in group O (P＜0.05).Conclusion Preoperative oral rehydration can promote postoperative recovery,which may be related to mitigating insulin resistance in patients undergoing laparoscopic radical resection of colorectal cancer.

Objective: To study the anti-inflammatory effect and mechanism of Gutong plasters in acute inflammatory model of rats. Methods:Totally 60 SD rats were randomly divided into the blank group,the model group,Gutong plasters at low,medium and high dose groups(0.594,1.188 and 2.376 g/patch containing crude drug 0.48,0.96 and 1.92 g,respectively) and prednisone ace-tate group(0.005 4 g·kg-1). The acute inflammation model was prepared by injecting 5% formaldehyde into right side of foot plan-tar. And then,the anti-inflammatory effect was evaluated by measuring the foot plantar volume. Enzyme-linked immunosorbent assay (ELISA) was used to detect the levels of nitric oxide(NO),histamine(HIS) and 5-hydroxytryptamine(5-HT) in serum and inflam-matory tissue,the levels of prostaglandin E2(PGE2),tumor necrosis factor alpha(TNF-α),interleukin 1 beta(IL-1β) and interleu-kin 6 (IL-6) in inflammatory tissue were also determined. Pathological changes were observed through the pathological sections pre-pared by HE staining. Results:When compared with the model group,Gutong plasters could significantly inhibit the swelling of foot plantar in inflammatory rats(P<0.01). At the same time,Gutong plasters could significantly reduce the levels of NO,5-HT and HIS in serum and inflammatory tissue in different degrees(P<0.05 or P<0.01),significantly reduce the content of PGE2in inflammatory tissue (P<0.05 or P<0.01),increase the content of IL-6 in inflammatory tissue and improve the pathological changes of inflammato-ry tissues (the pathological score was significantly reduced). In partical indictor changes,Gutong plasters and prednisone acetate showed an equal effect.In addition,Gutong plasters didn't show significant dose-dependent manner in inhibiting foot swelling,affect-ing inflammatory mediators and improving pathological changes(P>0.05). Conclusion:Gutong plasters have better anti-inflammato-ry effect in the acute inflammation model induced by formaldehyde in rats. The anti-inflammatory effect may be associated with the de-crease of the levels of NO,5-HT and HIS in serum and inflammatory tissue,the decrease of the content of PGE2and the increase of the content of IL-6 in inflammatory tissue. In addition,the anti-inflammatory effect may be relevant to improving the local inflammatory tis-sue subcutaneous edema and inflammatory cell infiltration.

Objective To investigate the effects of methylprednisolone on lung function and inflammation during one-lung ventilation in patients undergoing pulmonary lobectomy.Methods Sixty patients (46 males, 10 females, aged 18-60 years, ASA grade Ⅰ or Ⅱ) scheduled for pulmonary lobectomy were randomly divided into two groups (n=30 each) using a random number table: the control group (group C) and the methylprednisolone group (group M).Patients in group M were treated with methylprednisolone before induction of anesthesia within 30 min, whereas patients in group G received equal volume of normal saline at the same time.The heart rate (HR), mean arterial pressure (MAP), peak airway pressure (Ppeak), plateau airway pressure (Pplat), and dynamic lung compliance (Cdyn) were recorded at the momment before OLV (T1), 30 min after OLV (T2), 1 h after OLV (T3), 10 min after resuming two-lung ventilation (T4) and the end of the surgery (T5).The arterial blood gas analysis were performed at the above time points in order to determined the oxygen partial pressure (PaO2) and carbon dioxide partial pressure (PaCO2), then the oxygenation index (OI), alveolar to arterial difference of oxygen tension (A-aDO2) and respiratory index (RI) of each patient was evaluated.The concentration of serum TNF-α, IL-6 and IL-10 were determined by ELISA at T0, T4, 6 h (T5) and 24 h (T6) after surgery using venous blood samples.The incidence of pulmonary complications at 72 h after operation were also recorded.Results Compared with T0, the MAP and Cdyn was decreased, whereas the Ppeak and Pplat was increased significantly in both group at T1, T2 (P＜0.05), the OI was decreased and the A-aDO2, RI was increased significantly at T1-T4 (P＜0.05), and the concentration of serum TNF-α, IL-6 and IL-10 at T4-T6 was increased significantly in both group (P＜0.05).Compared with group C, the Cdyn was increased, the Ppeak and Pplat were decreased significantly in group G at T1, T2 (P＜0.05), the concentration of serum TNF-α and IL-6 were decreased, and IL-10 was increased significantly in group G at T4-T6 (P＜0.05).The incidence of postoperative pulmonary complications had no statistically signifcant differences between the two groups.Conclusion Methylprednisolone can significantly improve the pneumodynamics and lung compliance, alleviates the inflammatory responses, but have no significant effect on intrapulmonary oxygenation and gas exchange during one-lung ventilationin patients undergoing pulmonary lobectomy.