With the reform of the medical and health system entering a critical period,public hospitals have also exposed new risks and challenges in economic operation.As an important means of hospital standardized management,internal control can better prevent and resolve the risk of hospital economic operation and ensure the sustainable operation of the hospital.By interpreting the requirements of current national policies on hospital internal control,it analyzes the functional positioning of financial and accounting supervision in hospital internal control,shares the internal control implementation path of sample hospitals from the perspective of financial and accounting supervision,and puts forward suggestions on strengthening internal control construction of public hospitals in the new era,in order to lay a good foundation for the high-quality development of hospitals.

Objective To explore the effects of proteasome 20S subunit beta 8(PSMB8)on the prolif-eration,migration,and invasion of clear cell renal cell carcinoma(ccRCC)cells and whether PSMB8 promotes tumor progression by activating the mitogen-activated protein kinase kinase(MEK)/extracellular signal-regula-ted kinase(ERK)signaling pathway.Methods The Cancer Genome Atlas was employed to analyze the mRNA levels of PSMB8 in ccRCC and normal tissue,and the expression levels of PSMB8 in ccRCC tissue and cells were determined by real-time quantitative PCR,Western blotting,and immunohistochemistry.Furthermore,the cell lines with stable overexpression and knockdown of PSMB8 were constructed.The CCK-8 assay and colony forma-tion assay were employed to examine the cell proliferation,and the wound healing assay and Transwell assay were employed to examine the invasion and migration of cells.Kyoto Encyclopedia of Genes and Genomes pathway enrich-ment was performed to analyze the co-expressed genes of PSMB8.Western blotting was used to measure the phospho-rylation levels of the proteins in the MEK/ERK signaling pathway.Finally,the rescue experiment was carried out with the ERK agonist C16-PAF.Results Compared with the normal tissue,the ccRCC tissue showed up-regulated mRNA and protein levels of PSMB8(both P＜0.001),which were associated with the TNM stage of patients with ccRCC(P＜0.001).Compared with the negative control group,overexpression of PSMB8 promoted the prolifera-tion(P=0.021,P=0.039),migration and invasion(all P＜0.001)of 786-O and ACHN cells,and the knock-down of PSMB8 inhibited the proliferation(P=0.022,P=0.005),migration and invasion(all P＜0.001)of 786-O and ACHN cells.The pathway enrichment analysis of co-expressed genes of PSMB8 predicted the mitogen-ac-tivated protein kinase signaling pathway(P＜0.001).After the knockdown of PSMB8,786-O and ACHN cells showed lowered phosphorylation levels of MEK1/2(P=0.017,P=0.016)and ERK1/2(P=0.010,P=0.040)and down-regulated transcription levels of ERK downstream factors c-Myc(P=0.043,P=0.038),c-Fos(P=0.025,P=0.008),and CyclinD1(P=0.006,P=0.047).Compared with the ERK agonist C16-PAF group,the PSMB8 knockdown+C16-PAF group showed inhibited proliferation(P=0.003,P=0.002),migration and invasion(all P＜0.001)of 786-O and ACHN cells.Conclusion PSMB8 may promote the proliferation,migration,and invasion of ccRCC cells by activating the MEK/ERK signaling pathway.

OBJECTIVE: To analyze the clinical features and variants of ABCD1 gene in a Chinese pedigree affected with X-linked adrenoleukodystrophy. METHODS: Clinical data of the proband were collected and analyzed. Potential variant of the ABCD1 gene were analyzed by PCR and Sanger sequencing of the proband, his parents and 100 unrelated healthy individuals. RESULTS: The prominent features of the proband included cerebellar and brainstem lesions, along with increased serum level of very-long chain fatty acids. He was found to harbor a hemizygous c.1509delG (p.L504Sfs*54) variant of the ABCD1 gene, for which his mother was heterozygous. The same variant was not detected in his father and 100 healthy controls. CONCLUSION X-linked adrenoleukodystrophy has a variety of clinical manifestations. Discovery of the c.1509delG (p.L504Sfs*54), as a novel pathogenic variant of the ABCD1 gene, has enabled diagnosis and genetic counseling for this pedigree.
Adrenoleukodystrophy/genetics* ; Asians/genetics* ; China ; Female ; Genetic Testing ; Humans ; Male ; Mutation ; Pedigree

Objective:Based on ear cartilage of congenital microtia, we tried to find the different metabolites and variational metabolic pathways on molecular level by gas chromatography time-of-flight mass spectrometry (GC-TOFMS).Methods:Patients with microtia were collected from June 2017 to January 2018. During operation, the ear cartilage tissue was collected and labeled, and the case group was residual ear cartilage tissue of the patients with microtia, with 18 cases. The control group was normal ear cartilage tissue, total 18 cases. After the sample pretreatment, based on the technique of GC-TOFMS and the software of XploreMET, the differential metabolites in the residual ear cartilage of microtia were analyzed by orthogonal partial least square discriminant analysis (OPLS-DA) and its variable importance projection (VIP) and U test statistical method. Compared with the metabolite database, the metabolic pathway enrichment analysis (MPEA) was used to screen the most related metabolic pathways. Results:According to the multidimensional statistical analysis, it is different in the metabolites of ear cartilage between the two groups. According to the verification of single dimensional statistical analysis, 14 specific biomarkers were identified, and their P value was less than 0.05. According to the metabolic pathway enrichment analysis (MPEA), 3 metabolic pathways had statistically significant difference, including arginine metabolism, taurine and the taurine metabolism, pantothenate and CoA metabolism, beta-alanine metabolism, glycerophospholipid metabolism, P <0.05. Conclusions:Arginine, taurine, L-cysteine and pantothenic acid may have an association with microtia, which proved that it is feasible to study the pathogenesis of microtia by metabonomics.

Objective:Based on ear cartilage of congenital microtia, we tried to find the different metabolites and variational metabolic pathways on molecular level by gas chromatography time-of-flight mass spectrometry (GC-TOFMS).Methods:Patients with microtia were collected from June 2017 to January 2018. During operation, the ear cartilage tissue was collected and labeled, and the case group was residual ear cartilage tissue of the patients with microtia, with 18 cases. The control group was normal ear cartilage tissue, total 18 cases. After the sample pretreatment, based on the technique of GC-TOFMS and the software of XploreMET, the differential metabolites in the residual ear cartilage of microtia were analyzed by orthogonal partial least square discriminant analysis (OPLS-DA) and its variable importance projection (VIP) and U test statistical method. Compared with the metabolite database, the metabolic pathway enrichment analysis (MPEA) was used to screen the most related metabolic pathways. Results:According to the multidimensional statistical analysis, it is different in the metabolites of ear cartilage between the two groups. According to the verification of single dimensional statistical analysis, 14 specific biomarkers were identified, and their P value was less than 0.05. According to the metabolic pathway enrichment analysis (MPEA), 3 metabolic pathways had statistically significant difference, including arginine metabolism, taurine and the taurine metabolism, pantothenate and CoA metabolism, beta-alanine metabolism, glycerophospholipid metabolism, P <0.05. Conclusions:Arginine, taurine, L-cysteine and pantothenic acid may have an association with microtia, which proved that it is feasible to study the pathogenesis of microtia by metabonomics.

Objective To investigate the effect of complement C3a on mouse podocytes phenotype transformation.Methods Purified C3a recombinant protein was used to stimulate mature mouse podocytes.The expression of the mature podocyte markers synaptopodin,podocin,nephrin,CD2-associated protein (CD2AP) and the mesenchymal cell markers fibroblast specific protein 1 (FSP-1),α-smooth muscle actin (α-SMA) were detected by RT-PCR,Western blotting,immunochemistry and immunofluorescence,respectively.Some podocytes were transfected with integrin-linked kinase (ILK) siRNA before the administration of C3a,the expression of nephrin and α-SMA were accessed by Western blotting,and the expression of Snail and α-actinin 4 were accessed by Western blotting and immunochemical method.The migration ability of podocytes was observed by scratch test.Results Immunocytochemistry and immunofluorescence analysis showed that synaptopodin,podocin,nephrin,CD2AP were highly expressed by mature mouse podocytes.The expression of these podocyte markers could be markedly inhibited after 24 h of C3a (0.1 μmol/L) treatment,and accompanied by the induction of mesenchymal markers FSP-1 and α-SMA.Compared with control group,the mRNA levels of synaptopodin,podocin,CD2AP and nephrin were significantly repressed by the administration of C3a in a dose-dependent manner,whereas the transcription of FSP-1 and α-SMA were remarkably up-regulated by C3a treatment (P ＜ 0.05,respectively).Western blotting analysis also confirmed the decrease of synaptopodin,podocin,nephrin and CD2AP protein and the increase of FSP-1 and α-SMA protein were closely depend on the C3a concentration (P ＜ 0.05,respectively).To further assess the downstream of C3a,some podocytes were transfected with ILK siRNA before the administration of C3a.Compared with C3a group,the protein levels of nephrin and α-SMA were significantly changed by the administration of ILK siRNA (P ＜ 0.05,respectively).The expression of α-actinin 4 and Snail induced by C3a were inhibited by ILK knockdown (P ＜ 0.05,respectively),accompanied by a decline of cell migration potency.Conclusion Complement fragment C3a can induce transformation of mouse podocytes to mesenehymal cells,and ILK signaling pathway is involved in this cell type transformation.

Objective To compare the clinical characteristics of normal body mass index (BMI) polycystic ovary syndrome (PCOS) patients with or without insulin resistance (IR) , and analyze the related factors of IR. Methods From January 2013 to January 2017, 128 patients with normal BMI (18.5-23.9 kg/m2) PCOS patients were divided into A group (56 cases with IR) and B group (72 cases without IR) according to whether they had IR. The clinical indicators, sex hormones, glucose and lipid metabolism indexes of the two groups were statistically compared, and the related factors influencing insulin resistance index (HOMA-IR) were analyzed by Pearson. Results Compared with B group, the waist-to-hip ratio (0.91 ± 0.08 vs. 0.80 ± 0.07), Ferriman-Gallway scores [(4.87 ± 1.66) scores vs. (3.31 ± 1.29) scores], fasting blood glucose (FPG) [(5.19 ± 0.60) mmol/L vs. (4.77 ± 0.56) mmol/L], fasting insulin (FINS) [(17.89 ± 5.85) mU/L vs. (9.83 ± 3.87) mU/L], total cholesterol (TC)[(5.03 ± 1.42) mmol/L vs. (4.52 ± 0.62) mmol/L], triglyceride (TG)[(1.72 ± 1.05) mmol/L vs. (0.98 ± 0.26) mmol/L], low density lipoprotein cholesterol (LDL-C) [(3.10 ± 1.19) mmol/L vs. (2.55 ± 0.82) mmol/L] in A group were significantly increased, while high density lipoprotein cholesterol (HDL-C) [(1.28 ± 0.30) mmol/L vs. (1.52 ± 0.23) mmol/L] significantly reduced, the differences were statistically significant (P < 0.05). HOMA-IR was positively correlated with waist-to-hip ratio (r=0.397), FPG (r=0.461), FINS (r=0.992), TC (r=0.371), TG (r=0.354) and LDL-C (r=0.478), but negatively correlated with sex hormone binding globulin (r=-0.231) and HDL-C (r=-0.299). Conclusions The waist-to-hip ratio, Ferriman-Gallway scores, glucose and lipid metabolism in normal BMI PCOS patients with IR are significantly altered. The waist-to-hip ratio, FPG, FINS, TC, TG, LDL-C, HDL-C and sex hormone binding globulin are all related factors of HOMA-IR.

Objective To evaluate the clinical value of CT/MR-contrast enhanced ultrasound (CEUS) image fusion in immediate assessment for hepatocellular carcinoma (HCC)ablation.Methods Patients diagnosed with HCC to receive ablation therapy were randomly divided into two groups.The ablation for each patient was performed according to their pre-ablation plan.Immediate assessments for the ablation were performed using CT/MR-CEUS image fusion (IF group)and CEUS (CEUS group)separately and whether the need for additional ablations was decided and performed during the therapy.The therapy efficacy was evaluated with enhanced CT or MR 1 month after each complete ablation treatment.Results There were 101 patients with 131 nodules in IF group,and 15.1% of the nodules needed additional ablation according the immediate assessment during the therapy.The complete ablation rate of IF group was 99.2%(125/126).On the other hand,there were 93 patients with 122 nodules in CEUS group,and 4.2% of the nodules needed additional ablation according the immediate assessment during the therapy.The complete ablation rate of CEUS group was 94.2% (113/120).IF group has significantly higher additional ablation rate and complete ablation rate than CEUS group (P <0.05).Conclusions CT/MR-CEUS image fusion can further improve ablation efficacy with accurate intraoperative evaluation and guidance of additional ablation compared with CEUS.

Objective To explore the value of double contrast enhanced ultrasound (D-CEUS) in detection of periampullary lesions. Methods Clinical data of 43 patients with periampullary lesions who received examinations of D-CEUS and contrast-enhanced magnetic resonance imaging (MRI) both in the Third Affiliated Hospital of Sun Yat-sen University from July 2006 to December 2012 were analyzed retrospectively. The informed consents of all patients were obstained and the ethical committee approval was received. There were 29 males and 14 females with a mean age of (56±2) years old. There were totally 45 lesions in 43 patients, including 30 malignant lesions (13 duodenal papillary carcinomas, 7 distal common bile duct carcinomas, 6 pancreatic uncinate process carcinomas, 4 ampullary carcinomas) and 15 benign lesions which all were distal common bile duct stones. All the lesions were conifrmed by surgical pathological biopsy or choledochoscope. The detection rates of periampullary lesions and the display rates of relation between periampullary malignant lesions and surrounding tissues by D-CEUS and contrast-enhanced MRI were compared. The rates of 2 groups were compared by Chi-square test. Results Periampullary lesions could be displayed clearly both by 2 examination methods. The detection rate of periampullary lesions was 98%(44/45) by D-CEUS and was 96%(43/45) by contrast-enhanced MRI, where no signiifcant difference was observed (χ2=0.345, P>0.05). For the display of relation between periampullary malignant lesions and surrounding tissues, 77%(23/30) was well-deifned, 20%(6/30) was poorly-deifned, and 3%(1/30) was ill-deifned by D-CEUS, while it was 86%(26/30), 7%(2/30), 7%(2/30) by contrast-enhanced MRI accordingly, where no significant difference was observed (χ2=2.517, P>0.05). Conclusions D-CEUS has a high detection rate of periampullary lesions. Its detection ability is similar to contrast-enhanced MRI, and it can be an important supplement to, even a replacement for contrast-enhanced MRI.

Objective To evaluate the clinical value of intraoperative image fusion assisted contrastenhanced ultrasound (CEUS) in real-time assessing the curative effect on radiofrequency ablation of hepatocellular carcinoma(HCC).Methods From November 2010 to August 2011,the patients with HCC which would accept radiofrequency ablation (RFA) in our hospital were divided into image fusion assisted CEUS group and conventional CEUS group.The HCC lesion in each group was named as difficult lesion if it was invisible in B-mode ultrasound,larger than 50 mm in diameter or the puncuture route was affected by lung,ribs or blood vessels.Ten minutes after RFA,two groups of patients were performed intraoperative image fusion assisted CEUS examination and conventional CEUS examination respectively.All the patients received CT/MRI one month after ablation to decide whether the HCC had been completely ablated.Difference of complete ablation rate of HCC was compared between the two groups as well as between the difficult lesions in each group.Results Forty-eight lesions in 39 patients were enrolled in image fusion assisted CEUS group including 19 difficult lesions.Sixty-eight lesions in 53 patients were enrolled in conventional CEUS group including 18 difficult lesions.No statistical difference was found between the two groups in lesion size,proportion of difficult lesions,proportion of lesions received TACE or RFA plus PEIT (P =0.052,P =0.136,P =0.185,P =1.000).Postoperation following-up results demonstrated that complete ablation rate of HCC in navigation assisted CEUS group(100 ％,48/48) was not statistically higher than that in conventional CEUS group(92.6％,63/68) (P =0.145).But the difficult lesions in navigation assisted CEUS group (100％,19/19) had statistically higher complete ablation rate than that in conventional CEUS group(72.2％,13/18) (P =0.020).Conclusions Intraoperative navigation assisted CEUS could real-time assess the curative effect on RFA of HCC,especially in the difficult lesions,and could be used as the beneficial supplement of the conventional CEUS.