Objective To investigate the clinical features of glucose transporter 1 deficiency syndrome(GLUT1-DS) and summarize the characteristics of GLUT1-DS through reviewing related references.Methods The clinical data including manifestation,cerebrospinal fluid (CSF) glucose,electroencephalogram,MRI and gene mutation of a patient with GLUT1-DS was collected and the related literatures were reviewed.Results The patient was a 6 years old boy.The patient,whose seizures occurred at the age of 9 month-old and prolonged to 6 year-old,attacked before breakfast.Physical examination showed microcephaly with head circumference 47.5 cm.Laboratory tests showed that CSF glucose decreased (1.87 mmol/L) and CSF-serum ratio was 0.36.And meantime the MRI was normal and electroencephalogram showed general spike and slow wave complex paroxysm.Mutation of SLC2A1 gene,c.350_385del,was found in the patient.There were 219 cases with GLUT1-DS had been reported and the age of onset was 15.69 months.In 219 patients,159 cases (72％) suffered seizures,105 cases (47％) had motor abnormalities,61 cases (27％) suffered intellectual disability.The CSF glucose values were (1.92±0.31) mmol/L,CSF-serum ratio was 0.36±0.07.SLC2A1 gene mutations were detected in 183 patients(96％)in which missense mutation was the most mutation.Conclusion A wide range of phenotypes of GLUT1-DS include seizures,motor abnormalities,mental retardation.The diagnosis is confirmed when CSF glucose and CSF-serum ratio are continuously decreased which in the absence of meningitis.The SLC2A1 gene should be detected in suspicion of GLUTI-DS patients.Early diagnosis and treatment may improve the prognosis of those GLUTI-DS patients.

AIM: To study the effect of cholecystokinin octapeptide(CCK-8) on lipopolysaccharide (LPS)-stimulated pulmonary interstitial macrophage(IM) in vitro. METHODS: Pulmonary IM were isolated and cultured in the presence of LPS, CCK-8, proglumide(the antagonist of CCK receptors) and vehicle alone or together. The expression of mCD14 protein was assayed by flow cytometry, and sCD14 in the supernatant was analyzed semi-quantitatively by Western blot, and TNF-? in the supernatant was detected with ELISA. RESULTS: CCK-8, at concentrations from 10 -7 mol/L to 10 -6 mol/L inhibited significantly the expression of mCD14, the release of sCD14 and TNF-? to the supernatant up-regulated by LPS(1 mg/L). The effect of CCK-8 was inhibited by proglumide. CONCLUSION: CCK-8 modulated negatively several functions of LPS-stimulated pulmonary IM through CCK receptors, which may be one of the mechanisms for CCK-8 to alleviate the inflammation in lung tissues during endotoxemia.

ObjectiveThe clinical features, bacteria distribution and antibiotic resistance proifle of blood stream infection(BSI) were investigated in the patients with decompensated liver cirrhosis for better management of such infections.MethodsThe clinical data of BSI were collected in the patients with decompensated liver cirrhosis between January, 2012 and December, 2014, and reviewed retrospectively in terms of risk factors, diagnosis and treatment, pathogen distribution and prognosis.ResultsOf the 1 071 patients with decompensated liver cirrhosis and suspected bacterial infection, 154 (14.4%) were diagnosed as BSI evidenced by blood culture. Of these patients, the leukocyte count in the peripheral blood was higher than 10×109/L in only 48 (31.2%) patients; neutrophil proportion>0.75 in 133 patients (86.4%); serum procalcitonin level>0.5 ng/mL in 74 patients (68.5%). A total of 155 bacterial strains were isolated, including 115 strains of gram-negative bacilli and 40 strains of gram-positive cocci. Most patients (68.8%) recovered and 31.2% died or discharged from hospital voluntarily. All these BSI patients had Child-Pugh grade C liver function. Some patients also had other serious systemic diseases or repeated hospitalization.ConclusionThe prevalence of BSI is high in the decompensated liver cirrhosis patients with poor prognosis. Gram-negative bacilli are the major pathogens of such septicemia. Early diagnosis and proper use of antibiotics based on antimicrobial susceptibility testing are important to improve patient outcome.

Objective To assess the relationship between the serum IgG antibody titer of pregnant women and the hemolytic dis‐ease of newborn(HDN) .Methods Using microcolumn gel coombs card assay method to determine titer of 255 cases of couples an‐tenatal serum IgG antibody and ABO blood group .Results with 195 cases of ABO‐incompatible couples ,the positive rate of abnor‐mal serum IgG antibody(≥64)was 93 .8% .The titer of anti‐A/B IgG :in 12(6 .2% ) cases was <64;in 12(6 .2% )cases was 64;in 60(30 .8% )cases was 128 ;in 39(20% )cases was 256 ;in 45(23 .0% )cases was 512 ;in 27(13 .8% ) cases was 1 024 .There were no statistical differences between IgG anti‐A(B) titers distribution between A/O blood group matching and B/O blood group matching (χ2 =4 .361 ,P=0 .499) ,IgG anti‐A( B) titers was higher in AB/O blood group matching .Conclusion we can take early and effec‐tive prevention ,treatment ,reducing the incidence of HDN by the determination of prenatal serum IgG antibody titers in ABO‐in‐compatible couples ,which is important for the population of eugenics .

Objective To investigate the drug-resistance of Staphylococcus aureus induced by erythromycin in vitro and its changes in growth and susceptibility of antibiotics.Methods Erythromycin in vitro induction was conducted with the S.au-reus reference strain ATCC25923 on the serial of erythromycin agar plates.The growth of S.aureus,and the susceptibility a-gainst other antibiotics was compared after induced to the parent strain.Results Resistance to erythromycin was successful-ly,and themaximum MIC was over 256 mg/L.The Erythromycin-resistant S.aureus grew much slower than the susceptible parent,and the strains didn’t have cross-resistance to other antibiotics.Conclusion S.aureus could be induced resistance in vitro by erythromycin,and this resistance inherited stably.Some phenotype and biochemical characteristic features of the strain were changed after induced.

Objective To analyze the epidemiological and clinical characteristics of 403 local cases with dengue fever , and to provide guidance for dengue fever's prevention , diagnosis and treatment . Methods The epidemical data ,clinical characteristics ,laboratory results ,therapy and outcomes of 403 cases with dengue fever treated in Mengchao Hepatobiliary Hospital of Fujian Medical University ,Fuzhou from July to November 2016 were collected and analyzed retrospectively .Results Among the 403 cases , 56 cases (13 .89％ ) were younger than 20 years old ,160 cases (39 .71％ ) were 21 to 50 years old ,and 187 cases (46 .40％ ) were over 50 years old .The peak incidence period of dengue fever was from August to October ,accounting for 94 .29％ of the cases .The clinical classification was dominated by typical dengue fever (98 .01％ ) .Most of the cases had sudden onset .The main clinical symptoms included fever in 391 cases (97 .02％ ) , body aches in 355 cases (88 .09％ ) , and rash in 156 cases (38 .71％ ) . All patients were tested for serology and etiology of dengue virus at the early stage of disease .Among them , the positive rate of NS1 antigen was 95 .04％ ;the positive rate of IgM antibody was 13 .90％ ,and the positive rate of IgG antibody was 8 .19％ . Thrombocytopenia was founded in 332 cases (82 .38％ ) .Neutrophil count decreased in 293 cases (72 .70％ ) , w hite blood cell count decreased in 274 cases (67 .99％ ) .Aspartate aminotransferase increased in 256 cases (63 .52％ ) and alanine aminotransferase increased in 146 cases (36 .23％ ) .Creatine kinase elevated in 130 cases (32 .26％ ) and prothrombin time was prolonged in 40 cases (9 .93％ ) .A total of 289 cases (82 .75％ ) had bilateral lesions in both lungs and 25 cases (6 .20％ ) had pleural effusions . Color doppler ultrasound found 46 cases (11 .41％ ) of hepatomegaly ,84 cases (20 .84％ ) of splenomegaly ,8 cases (1 .99％ ) of a small amount of ascites . Conclusions Most of the 403 cases of dengue fever show typical clinical manifestations .Dengue fever mainly affects the blood system ,liver ,heart ,lung ,kidney ,skeletal muscle system ,and immune system . Clinicians should carefully observe the condition ,master the warning signs of severe dengue fever ,and achieve early identification and timely treatment of severe cases ,thereby reducing the mortality rate .

Objective: To study the expression and distribution of filaggrin (FLG) in oral submucous fibrosis (OSF) and to explore the significance of FLG in the occurrence and development of OSF. Methods : Ten cases with a normal oral mucosa (normal buccal mucosa group) and 30 cases of tissues with OSF lesions, including 10 cases each in the early (early OSF group), moderate (middle OSF group) and advanced stages (late OSF group), were selected. FLG was analyzed by immunohistochemistry. The FLG-positive cells were counted to calculate the percentages of cells with FLG-positive expression in each group. Results: FLG expression was negative in most of the normal buccal mucosa group specimens and was positive in the OSF buccal mucosal epithelial specimens. With aggravation of the OSF lesion, the number of FLG-positive cells increased. In the early OSF group, FLG-positive expression was mainly concentrated in the granular and keratinized epithelial layers. In the middle OSF group, the number of FLG-positive epithelial cells increased gradually. In the late OSF group, almost all epithelial cells were FLG-positive in the cytoplasmic nucleus. The percentages of FLG-positive cells in the early, middle and late OSF groups were (24.63 ± 9.06)%, (54.23 ± 10.63)% and (83.97 ± 8.72)%, respectively. The percentage of FLG-positive cells was significantly higher in the OSF group than in the normal mucosa group (P < 0.05). Conclusion FLG was expressed at a higher level in the OSF epithelium than in the normal oral mucosal epithelium and was upregulated in the OSF epithelium with aggravation of the OSF lesions. Abnormal FLG expression may be related to the terminal differentiation disorder of OSF epithelial keratinocytes.

Objective To discuss the impact of bundles of nutritional nursing interventions on nutritional status and quality of life of esophageal cancer patients who underwent concurrent radiotherapy and chemotherapy .Methods We selected eighty esophageal cancer patients admitted to our department for concurrent radio-chemotherapy during July 2012 to June 2014 .We divided them into experimental and control group according to random number table , and each of forty cases .After a course of treatment , hemoglobin (Hb), serum albumin (ALB), serum pre-albumin (PA), total lymphocyte count (TLC) and the score of QLQ-C3.0 were compared between two groups .Results When patients admitted hospital , there were no statistical differences in index of the patients between two groups (P>0.05).After one course of treatment,the data of Hb(110.0 ±6.1) g/L,ALB(30.8 ±5.4) g/L,PA(181.6 ±58.4) mg/L,TLC(2.8 ±0.4) ×109/L, score of whole healthy status ( 55.6 ±10 .2 ) in the experimental group which were higher than those in the control group (t=2.484 2,3.272 2,3.255 5,9.877 7,5.527 5,respectively;P<0.05).Conclusions Bundle of nutrition interventions can significantly improve the nutritional status and the quality of life of esophageal cancer patients who underwent concurrent radio-chemotherapy , and it is worthy to promote .

Objective:To explore the construction and effect of biobank in biological research in nursing field.Methods:Through the establishment of a biological research plan for nursing in Affiliated Jinhua Hospital, Zhejiang University School of Medicine, the biobank in biological research for nursing was constructed from aspects of biological samples, instruments and equipment, operating software, biobanking team formation, personnel training and standardized process formulation.Results:In August 2020, the Nursing Department and Central Laboratory of Affiliated Jinhua Hospital, Zhejiang University School of Medicine established a biobank for biological research in the nursing field, formed a biobank team, conducted personnel training and formulated standardized procedures. From December 1, 2020 to June 30, 2021, biological samples were collected from 264 gastric cancer patients and 56 healthy controls, mainly including whole blood, serum and urine.Conclusions:The construction of biobank for biological research in nursing field can collect abundant biological samples, which lays a foundation for subsequent independent biological research in nursing field.

Objective To clarify the clinical application effect of electronic medical record linkage time node reminder system(hereinafter referred to as reminder system)in trauma center.Methods A total of 90 patients with severe trauma admitted to the Trauma Center of Huzhou First People's Hospital from June 2022 to March 2023 were selected,and 45 patients admitted from June to October 2022 were included in control group for routine pre-hospital in-hospital treatment.Forty-five patients with severe trauma admitted from November 2022 to March 2023 were included in experimental group,and reminder system was implemented to guide the treatment process.The indexes of medical quality control,emergency black hole procedure time and patient outcome were compared between two groups.Results Bedside FAST ultrasound examination time,CT scan completion time,emergency room stay time,nursing waiting time,make diagnosis and treatment plan and surgical talk time,and travel transport time of patients in experimental group were significantly shorter than those in control group(P<0.05).There were no significant differences in the first tranexamic acid administration time,completion time of damage control treatment,length of stay in intensive care unit,duration of invasive mechanical ventilation and success rate of treatment between two groups(P>0.05).Conclusion The application of reminder system is helpful to strengthen the cooperation of medical care,shorten the unnecessary waiting time for treatment,and accelerate the time node transformation in the process of severe trauma treatment.