Objective To evaluate the effects of different doses of dexmedetomidine administered intranasally on the median effective concentration (EC50) of sevoflurane inhibiting responses to laryngeal mask airway (LMA) insertion in the pediatric patients.Methods American Society of Anesthesiologists physical status Ⅰ or Ⅱ pediatric patients of both sexes, aged 1-3 yr, with body mass index of 20-25 kg/m2, scheduled for elective surgery under general anesthesia, were randomly divided into 3 groups: control group (group C) , dexmedetomidine 1 μg/kg group (group D1) , and dexmedetomidine 2 μg/kg group (group D2).In D1 and D2 groups, dexmedetomidine 1 and 2 μg/kg (in 1 ml of normal saline) were administered intranasally at 1 h before induction of anesthesia, respectively.Group C received the equal volume of normal saline administered intranasally.The children were separated from their parents at 45-60 min after intranasal administration and admitted to the operating room.The children inhaled 8％ sevoflurane until they lost consciousness.After loss of consciousness, the end-tidal concentration of sevoflurane was determined using the modified Dixon's up-and-down method.The initial end-tidal concentration of sevoflurane was set at 2％.The concentration was increased/decreased by 0.2％ in the next patient according to the response to LMA insertion.The patient's sedation status and with LMA acceptance were evaluated, and the patient's satisfaction with sedation and with LMA acceptance was recorded.Probit analysis was used to calculate the EC50 and 95％ confidence interval of sevoflurane inhibiting responses to LMA insertion.Results The patient's satisfaction with sedation and with LMA acceptance was significantly higher in D1 and D2 groups than in group C, and in group D2 than in group D1 (P＜0.05).The EC50 (95％ confidence interval) of sevoflurane inhibiting responses to LMA insertion was 2.03％ (1.91％-2.15％), 1.76％ (1.65％-1.87％) and 1.63％ (1.53％-1.73％) in C, D1 and D2 groups, respectively.The EC50 was significantly lower in D1 and D2 groups than in group C, and in group D2 than in group D1 (P＜0.05).Conclusion Dexmedetomidine 1 and 2 μg/kg administered intranasally can decrease the EC50 of sevoflurane inhibiting responses to LMA insertion in the pediatric patients, and 2 μg/kg produces better efficacy.

Objective To investigate SLC25A13gene mutation in neonatal intrahepatic cholestasis caused by citrin defi-ciency (NICCD). Method A total of 17 children with NICCD were collected. PCR-RFLP method was used to analyze the most common eight mutations of SLC25A13 gene in Chinese populations and results were analyzed together with routine laboratory examinations. Results In the 17 NICCD patients, there were six cases of homozygous mutation, three cases of compound heterozy-gous mutation and eight cases of single heterozygous mutation in SLC25A13 gene. Three kinds of mutations detected were 851del4 (73.1%), 1638ins23 (11.5%) and IVS6+5G>A (15.4%). The seventeen cases showed classical NICCD symptoms of low birth weight, pathological jaundice. And laboratory data suggested liver dysfunction, hyperbilirubinemia, hyperbileacidemia, hy-poproteinemia, hypoglycemia, coagulation disorders, hyperlactacidemia and hyperammonemia. Conclusions 851del4, 1638ins23 and IVS6+5G>A are hot spots of SLC25A13 gene mutation in Chinese populations. PCR-RFLP is a rapid, convenient and reliable technology for NICCD molecular diagnosis.

Objective To investigate the expression of dopamine(DA),coactivator associated arginine methyltransferase 1(CARM1)and 25-hydroxyvitamin D3[25-(OH)-D3]in peripheral blood of diabetic pa-tients and their relationship with the occurrence of non-alcoholic fatty liver disease(NAFLD).Methods A to-tal of 70 patients with type 2 diabetes mellitus(T2DM)combined with NAFLD treated in this hospital from May 2021 to February 2023 were selected as T2DM with NAFLD group,66 patients with simple T2DM were selected as T2DM group,and 70 healthy people were selected as healthy group.The levels of DA,CARM1 and 25-(OH)-D3 in peripheral blood of each group were compared,and the levels of DA,CARM1 and 25-(OH)-D3 in peripheral blood of T2DM combined with NAFLD patients with different blood glucose control and severity were analyzed.Pearson correlation analysis was used to analyze the correlation between peripheral blood DA,CARM1,25-(OH)-D3 levels and glycosylated hemoglobin A1c(HbA1c),controlled attenuation parameter(CAP).Receiver operating characteristic curve was used to analyze the value of peripheral blood DA,CARM1,25-(OH)-D3 in the diagnosis of severe NAFLD.Results The levels of DA and 25-(OH)-D3 in T2DM with NAFLD group were significantly lower than those in T2DM group and healthy group(P＜0.05),while the level of CARM1 in T2DM with NAFLD group was significantly higher than that in T2DM group and healthy group(P＜0.05).The levels of DA and 25-(OH)-D3 in T2DM group were significantly lower than those in healthy group(P＜0.05),while the level of CARM1 in T2DM group was significantly higher than that in healthy group(P＜0.05).The levels of DA and 25-(OH)-D3 in T2DM with NAFLD patients with poor blood glucose control were significantly lower than those in patients with good blood glucose control(P＜0.05),while the level of CARM1 was significantly higher than that in patients with good blood glucose control(P＜0.05).The levels of DA and 25-(OH)-D3 in patients with severe were significantly lower than those in pa-tients with mild-to-moderate(P＜0.05),while the level of CARM1 was significantly higher than that in pa-tients with mild-to-moderate(P＜0.05).The levels of DA and 25-(OH)-D3in peripheral blood were negative-ly correlated with HbA1c and CAP,and the level of CARM1 was positively correlated with HbA1c and CAP(P＜0.05).The area under curve of CARM1,25-(OH)-D3 and DA were 0.858(95％CI 0.768-0.948),0.922(95％CI 0.856-0.989)and 0.571(95％CI 0.427-0.715),respectively.Conclusion The levels of DA and 25-(OH)-D3 are decreased in peripheral blood of T2DM patients,while the level of CARM1 is in-creased,especially in T2DM patients with NAFLD.DA,CARM1 and 25-(OH)-D3 levels are correlated with blood glucose control and the severity of NAFLD.CARM1 and 25-(OH)-D3 levels have certain application val-ue in the diagnosis of severe NAFLD.