Objective: To analyze the short-time efficacy of empagliflozin in the treatment of glycogen storage disease type Ⅰb (GSD Ⅰb). Methods: In this prospective open-label single-arm study, the data of 4 patients were collected from the pediatric department in Peking Union Medical College Hospital from December 2020 to December 2022. All of them were diagnosed by gene sequencing and had neutropenia. These patients received empagliflozin treatment. Their clinical symptoms such as height and weight increase, abdominal pain, diarrhea, oral ulcer, infection times, and drug applications were recorded at 2 weeks, 1 month, 2 months, 3 months, 6 months, 9 months, 12 months, and 15 months after treatment to assess the therapeutic effect. The liquid chromatography-tandem mass spectrometry method was used to monitor the changes in 1, 5-anhydroglucitol (1, 5AG) concentration in plasma. At the same time, adverse reactions such as hypoglycemia and urinary tract infection were closely followed up and monitored. Results: The 4 patients with GSD Ⅰb were 15, 14, 4 and 14 years old, respectively at the beginning of empagliflozin treatment, and were followed up for 15, 15, 12 and 6 months, respectively. Maintenance dose range of empagliflozin was 0.24-0.39 mg/(kg·d). The frequency of diarrhea and abdominal pain decreased in cases 2, 3, and 4 at 1, 2 and 3 months of treatment, respectively. Their height and weight increased at different degrees.The absolute count of neutrophils increased from 0.84×10⁹, 0.50×10⁹, 0.48×10⁹, 0.48×10⁹/L to 1.48×10⁹, 3.04×10⁹, 1.10×10⁹, 0.73×10⁹/L, respectively. Granulocyte colony-stimulating factor was gradually reduced in 1 patients and stopped in 3 patient. Plasma 1, 5 AG levels in 2 children were significantly decreased after administration of empagliflozin (from 46.3 mg/L to 9.6 mg/L in case 2, and from 56.1 mg/L to 15.0 mg/L in case 3). All 4 patients had no adverse reactions such as hypoglycemia, abnormal liver or kidney function, or urinary system infection. Conclusion: In short-term observation, empagliflozin can improve the symptoms of GSD Ⅰb oral ulcers, abdominal pain, diarrhea, and recurrent infection, also can alleviate neutropenia and decrease 1, 5AG concentration in plasma, with favorable safety.
Humans ; Child ; Child, Preschool ; Adolescent ; Prospective Studies ; Glycogen Storage Disease Type I/drug therapy* ; Neutropenia ; Abdominal Pain ; Diarrhea/drug therapy* ; Hypoglycemia

Objective To investigate the expression and prognostic significance of mediator complex subunit 8 (MED8) in gastric cancer and its impact on the cell cycle.Methods The expression of MED8 in gastric cancer and adjacent tissues and its correlation with patients' prognosis were analyzed using public databases.A validation cohort of 104 patients who underwent radical resection for gastric cancer in the First Affiliated Hospital of Bengbu Medical College from June 2012 to July 2017 was included.The receiver operating characteristic curve was established to evaluate the predictive value of MED8 for postoperative 5-year survival.Bioinformatics tools were used to predict the biological roles of MED8 in gastric cancer.The effect of the MED8 level on the G1/S phase transition of gastric cancer cells (MGC-803) was analyzed via lentivirus transduction and flow cytometry.Western blotting was carried out to assess the impact of MED8 expression on the protein levels of cyclin-dependent kinase 4(Cdk4) and G1/S-specific cyclin-D1(CyclinD1) in MGC-803 cells.Results The high expression of MED8 in the gastric cancer tissue was associated with poor prognosis (P<0.001) and had prognostic significance (area under curve=0.733,P<0.001).Gene enrichment analysis suggested that MED8 may participate in the cell cycle process.Flow cytometry results revealed that the upregulation of MED8 expression promoted the transition of MGC-803 cells from the G1 phase to the S phase (P<0.001),while the downregulation of MED8 had the opposite effect (P<0.001).Western blotting showed increases in the protein levels of Cdk4 and CyclinD1 in MGC-803 cells with upregulated MED8 expression (all P<0.001),and decreases in the cells with downregulated MED8 expression (all P<0.001).Conclusion MED8 is highly expressed in gastric cancer and may affect its progression and prognosis by regulating the G1/S phase transition of gastric cancer cells.
Humans ; Stomach Neoplasms ; Prognosis ; Cell Proliferation ; Cell Cycle ; Mediator Complex/metabolism* ; Cell Line, Tumor

Objective:To investigate the effect of early postoperative enteral nutrition(EN)on postoperative rehabilitation and inflammation after laparoscopic radical gastrectomy for gastric cancer,in order to provide reference for postoperative rehabilitation of such patients.Methods:Patients who received laparoscopic assisted radical gastrectomy in Department of Gastrointestinal Surgery of The First Affiliated Hospital of Bengbu Medical College from January 2020 to December 2022 were included in the analysis.According to the different ways of postoperative nutritional treatment,patients were divided into the observation group(early postoperative EN group)and the control group(parenteral nutrition group),and indexes such as postoperative rehabilitation,abdominal drainage flow and the level of inflammatory mediators in drainage fluid were compared between the two groups.Results:A total of 81 patients were included,including 41 in the observation group and 40 in the control group.Interval of the first postoperative exhaust(t=3.806;P＜0.001)and resuming diet day(t=5.510;P＜0.001),and length of postoperative hospital stay(t=2.401;P=0.019)in the observation group were shorter than those in the control group.Levels of peripheral blood albumin(t=14.040;P＜0.001)and prealbumin(t=9.832;P＜0.001)of the observation group at postoperative day(POD)5 were significantly higher than those of the control group,but there was no significant difference in hemoglobin level(t=1.477;P=0.144).The level of CRP in peripheral blood of the observation group at POD 5(t=7.758;P＜0.001)and the incidence of postoperative SIRS[(12.2％,5/41)vs(32.5％,13/40),x2=4.830;P=0.028)]were significantly lower than those in the control group.The average drainage volume(t=6.858;P＜0.001),drainage removal time(t=5.016;P＜0.001),and TNF-α level(t=4.993;P＜0.001)and IL-6 level(t=20.640;P＜0.001)in postoperative drainage at POD 5 were significantly lower in the observation group than those in the control group.Conclusion:Early postoperative EN could accelerate the rehabilitation process after laparoscopic radical gastrectomy,improve postoperative nutritional status,and reduce abdominal inflammation.

OBJECTIVE: To assess changes of nutritional status by comprehensive nutrition assessment including nutritional risk screening, dietary assessment, blood biochemical index, and body composition in acute leukemia patients who had undergone chemotherapy. METHODS: A total of 169 patients with acute leukemia treated at The First Affiliated Hospital of Soochow University from June 2018 to August 2019 were recruited for this study. Before and after chemotherapy, the NRS-2002 and PG-SGA scales, dietary intake, blood biochemical index and body composition were evaluated to compare the changes of nutritional status. RESULTS: NRS-2002 score and PG-SGA score after chemotherapy were significantly increased than those before chemotherapy (P<0.001). Many patients had insufficient nutritional intake during chemotherapy, and the dietary intake score of patients with induction chemotherapy was significantly lower than that of patients with consolidation chemotherapy (P=0.043). The results of multivariate analysis showed that induction chemotherapy was the independent risk factor for the increase of PG-SGA scores and the decrease of dietary intake (all P<0.05). After chemotherapy, the white blood cell count, hemoglobin, and platelet count were significantly decreased (P<0.001), the prealbumin was significantly increased (P<0.001), and the blood glucose was increased (P=0.04), but albumin was not significantly changed. The weight, body mass index, fat-free mass, skeletal muscle mass and intracellular water were all significantly decreased (P<0.001), and visceral fat area was increased significantly after chemotherapy (P<0.05), especially in newly-diagnosed acute lymphoblastic leukemia patients after the induction of chemotherapy. CONCLUSION The nutritional status of patients with acute leukemia has undergone significant changes after chemotherapy. A single indicator has limited significance for nutritional status assessment. Comprehensive assessment of nutritional status by multiple tools is worthy of clinical application.
Acute Disease ; Humans ; Induction Chemotherapy/methods* ; Leukemia, Myeloid, Acute/drug therapy* ; Nutrition Assessment ; Nutritional Status ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy*

Results and Conclusion:Articulation disorder for children is classified to Developmental Speech Sound Disorder (6A01.0). It involves in the eye, ear and related structures (s2), structures involved in voice and speech (s3), especially structure of external ear (s240), structure of middle ear (s250) and structure of inner ear (s260); voice and speech functions (b3), especially articulation functions (b320); communication (d3), learning and applying knowledge (d1), interpersonal interactions and relationships (d7), and community, social and civic life (d9), especially speaking (d330), conversation (d350), using communication devices and techniques (d360) and acquiring language (d133). The assessment of articulation disorder should be covered articulation accuracy and motor function of articulation. A holistic rehabilitation solution for children with articulation disorder has been developed, involving in body structure, body function, activities and participation, and environmental factors, including assessment, training and treatment, educational counseling, and psychological and social support. Objective:To explore the diagnosis of diseases and functioning of articulation function and conduct content analysis of assessment tools in Chinese, and to construct framework of rehabilitation solution based on International Classification of Diseases 11th Revision (ICD-11), International Classification of Functioning, Disability and Health (Children and Youth version) (ICF-CY) and International Classification of Health Interventions (ICHI). Methods:The diagnosis of disease and functioning was discussed with ICD-11 and ICF. The assessment tools were analyzed with ICF. A holistic rehabilitation solution was constructed with ICF and ICHI.

OBJECTIVESTo investigate the mechanism of Liuwei Dihuang Pill (, LDP) in treating postmenopausal osteoporosis (PMOP) with Shen (Kidney) yin deficiency.

METHODSIn this study, 205 cases of PMOP were divided into the PMOP Shen-yin deficiency group (Group A), PMOP Shen-yang deficiency group (Group B), PMOP without Shen deficiency group (Group C), and control group (Group N). Real-time polymerase chain reaction (RT-PCR) and Western blot techniques were used to observe the effects of LDP treatment on the cardiotrophin-like cytokine factor 1 (CLCF1), ankyrin repeat and SOCS box containing 1 (ASB1), and prokineticin 2 (PROK2) genes and the Janus kinase/signal transducer and activator of transcription (JAK/STAT) signaling pathway.

RESULTSThe mRNA (P<0.05) and protein (P<0.01) expression levels of the CLCF1 gene in Group A were significantly lower than the corresponding levels in Group N. After LDP treatment for 3 months, the mRNA expression levels of the CLCF1 gene were obviously up-regulated (P<0.01). After 6-month treatment, the expression levels of CLCF1 mRNA and protein were significantly up-regulated (both P<0.01), and the average bone density of the top femur had significantly increased (P<0.05). In vitro, CLCF1 overexpression resulted in a significant increase in the total protein and phosphorylated protein levels of JAK2 and STAT3.

CONCLUSIONSThe CLCF1 gene is an important gene associated with PMOP Shen-yin deficiency and the therapeutic effects of LDP may be mediated by up-regulation of CLCF1 gene expression and activation of the JAK/STAT signaling pathway.

Cytokines ; genetics ; metabolism ; Drugs, Chinese Herbal ; pharmacology ; therapeutic use ; Female ; Gene Expression Regulation ; Humans ; Janus Kinases ; metabolism ; Middle Aged ; Osteoporosis, Postmenopausal ; drug therapy ; genetics ; RNA, Messenger ; genetics ; metabolism ; STAT Transcription Factors ; metabolism ; Signal Transduction ; Up-Regulation ; Yin Deficiency ; drug therapy ; genetics

Objective:To investigate the optimal time of tracheotomy/arytenoidectomy and the improvement of dyspnoea, dysphonia and dysphagia after arytenoidectomy with CO₂ laser in iatrogenic bilateral vocal folds paralysis patients. Method:Thirty patients [29 females, 56 (49-60) years, one male, 49 years] with bilateral vocal cords paralysis resulted from neck surgery were retrospectively analyzed by case archived information and following-up questionnaire. The data included patients' dysponea time, degree and duration from tracheotomy/arytenoidectomy to neck surgery. Twenty sixty patients required unilateral partial/total arytenoidectomy. The results of treatment were evaluated by questionnaire including dyspnoea, dysphonia and dysphagia. Result:All patients whose bilateral vocal paralysis were resulted from thyroid gland surgery. Dysponea occurred immediately after thyroidectomy surgery in 14 cases (46.7%), and 2 years later after thyroidectomy in 13 cases (43.3%), 8 years later in 3 cases (10.0%). There was one (3.3%) patient without tracheotomy. The duration of tracheotomy/arytenoidectomy to neck surgery was significantly correlated with duration of tracheotomy/arytenoidectomy to dyspnoea appearance (r=0.879, P<0.05), not correlated with duration of thyroid surgery to dyspnoea appearance. There is significantly negative correlation between degree of dyspnoea and duration of tracheotomy/arytenoidectomy to neck surgery (r=0.452, P<0.05). Twenty six patients appeared dyspnoea and underwent CO₂ laser arytenoidectomy after thyoidectomy 0.5-23 years. Five patients did unilateral total arytenoidectomy and 21 patients did unilateral partial arytenoidectomy. After 12-96 months following up, dyspnoea improved in 24 patients, no improved in 2 patients. Dysphonia improved and remained in 17 patients, being worse mildly in 8 patients and obviously in one patient. Dysphagia improved and remained in 24 patients, being worse in 2 patients. There was no difference between total and partial arytenoidectomy in dyspnoea, dysphonia and dysphagia. Conclusion:The morbidity of dyspnoea was correlated with time after neck surgery. It was rarely necessary to take tracheotomy immediately in bilateral vocal fords paralysis patients after neck surgery. The severer degree of dyspnoea led to shorter duration between neck surgery and tracheotomy/arytenoidectomy. There was obvious improvement after arytenoidectomy in dyspnoea, no significant change in dysphonia and dysphagia. The effect of total arytenoidectomy on bilateral vocal paralysis was similar to partial arytenoidectomy.

Objective To evaluate the safety and efficacy of cefaze-done injection ( CZD) compared with cefazolin injection ( CZL) in the treatment of acute bacterial respiratory infections.Methods Eligible subjects were divided randomly to receive 2.0 g cefazedone injection or cefazolin injection twice a day for 7 to 14 days.Efficacy and safety evaluation were done in accordance with the clinical trial protocol.Results Two hundred and sixty patients in 11 hospitals were en-rolled, 126 in CZD group( trial) and 134 in CZL group( control).There were no statistical differences in basic conditions between two groups( P >0.05 ).Cure rates of CZD group and CZL group were 95.5% and 94.9% in PPS ( P>0.05 ).Bacteria clearance rates of CZD group and CZL group were100% and 91.7% in BPPS and the total cure rates of CZD group and CZL group were 94.4% and 91.7% in BPPS, respectively ( P>0.05).Ten out off 126 patients in CZD group and 14 out off 134 in CZL group developed adverse events( AE ).Six and eleven events in CZD group and CZL group
were evaluated to be related with study drugs.One case in CZL group developed severe AE , which was considered not related with study drug.Conclusion Cefazedone injection is safe and effective in the treatment of respiratory infections.

OBJECTIVETo investigate the dynamic changes of oxidative stress and nuclear factor-E2 related factor 2 (Nrf2) expression in the lung tissues of acute hydrogen sulfide (H2S) intoxicated rats and intervention effects of ulinastatin (UTI).

METHODSA total of 96 SD rats of clean grade were divided randomly into four groups: normal control group (n = 8), UTI control group (n = 8), H2S -intoxicated model group (n = 40), and UTI treatment group (n = 40). The H2S-intoxicated model group and UTI treatment group were exposed to H2S (283.515 mg/m3) by inhalation for 1h, then UTI treatment group was intraperitoneally exposed to UTI at the dose of 10(5) U/kg for 2 h. H2S-intoxicated model group and UTI treatment group were sacrificed at 2, 6, 12, 24 and 48 h after exposure, respectively. The levels of malondialdehyde (MDA), superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GSH-Px) and glutathione (GSH) in the rat lung tissues were measured. The expression levels of Nrf2 mRNA in the rat lung tissues were detected. Pathological changes of rat lung tissues were observed under a light microscope and the lung injury scores were evaluated.

RESULTSCompared with control group, the pulmonary SOD, CAT and GSH levels at 2,6 and 12 h after exposure and the pulmonary GSH-Px levels at 2, 6, 12 and 24 h after exposure in H2S-intoxicated model group significantly decreased (P < 0.05 or P < 0.01). The levels of pulmonary MDA at 2, 6, 12 and 24 h after exposure in H2S-intoxicated model group were significantly higher than those in normal control group (P < 0.01). As compared with H2S -intoxicated model group, the pulmonary GSH-Px activities at 6 and 12 h after exposure, the pulmonary CAT activities at 2, 6 and 12 h after exposure, the pulmonary GSH levels at 2, 6, 12 and 24 h after exposure and the pulmonary SOD activities at 2, 6, 12, 24 and 48 h after exposure in UTI treatment group significantly increased (P < 0.05 or P < 0.01), the pulmonary MDA levels at 2, 6 and 12 h after exposure in UTI treatment group significantly decreased (P < 0.01). The expression levels of Nrf2 mRNA at 2, 6, 12, 24 h after exposure in H2S-intoxicated model group were 0.314 +/- 0.011, 0.269 +/- 0.010, 0.246 +/- 0.011 and 0.221 +/- 0.018, respectively, which were significantly higher than those (0.149 +/- 0.012) in control group (P < 0.01). As compared with H2S-intoxicated model group, the expression levels (0.383 +/- 0.017, 0.377 +/- 0.014, 0.425 +/- 0.017, 0.407 +/- 0.011 and 0.381 +/- 0.010) of Nrf2 mRNA at 2, 6, 12, 24 and 48 h after exposure in UTI treatment group significantly increased (P < 0.01). The lung injury at 24 h after exposure in H2S-intoxicated model group was higher than that in UTI treatment group. Histopathological examination showed that the scores of lung injury at 12, 24 and 48 h after exposure in UTI treatment group was significantly lower than those in H2S-intoxicated model group (P < 0.01).

CONCLUSIONOxidative stress and Nrf2 activation may be the important factors in rat lung injury induced by H2S-intoxicated, UTI may reduce the rat lung injury and protect the rat lung from damage induced by H2S by inhibiting ROS, improving the imbalance in redox and up-regulating Nrf2 mRNA expression.

Acute Lung Injury ; chemically induced ; metabolism ; Animals ; Glycoproteins ; pharmacology ; Hydrogen Sulfide ; poisoning ; Lung ; metabolism ; Male ; NF-E2-Related Factor 2 ; metabolism ; Oxidative Stress ; drug effects ; Rats ; Rats, Sprague-Dawley

Objective To observe the variation of airline pilot's cognitive ability at age 60 by dual-task test and to analyze the results upon one year longitudinal study in order to investigate the dynamic changes on the cognitive function of civil airline pilots older than 60 years old. Methods Fifty-two airline pilots at age 60 were tested by computer generated tasks,including mental arithmetic of 4-figure consecutive addition and computer simulated flying attitude control task,as well as the dual-task which was composed by above two.The aging effect was explored by comparing the results with that of other age group airline pilots (31-39 age group,40-49 age group and 50-59 age group).Paired t test was used to compare twenty-six pilots' cognitive ability based on one year longitudinal study in order to obtain the dynamic changes. Results There was significant aging effect on every task indexes (F=2.98-13.47,P＜0.05).The cognitive ability of the pilots at age 60 had significant difference with that of tbe 31-39 years age group,(t=2.33-7.46,P＜0.01 or P＜0.05),but with that of other groups (P＞ 0.05). The one year longitudinal study results showed that there was no significant decline on cognitive ability as performing dual task. Conclusions The results of age comparison and longitudinal study indicate that 60 years old is not the break where the dual-task ability significantly decayed and imply the airline pilot's cognitive ability varied insignificantly at that age.Results of study would be suggestive to the retirement policy of airline pilots.