Purpose To compare the CT features of hepatic angiomyolipoma (HAML) and primary hepatocellular carcinoma (HCC) and improve diagnostic accuracy for HAML. Materials and Methods The CT findings of 9 patients with HAML confirmed pathologically and 20 patients with HCC were retrospectively analyzed in terms of lesion location, appearance, size, margin, CT value at each phase, cirrhosis and vessel presence inside tumor. Results Mean CT value on plain scan and at arterial phase on contrast enhanced scan of HAML was significantly lower than that of HCC (t=-2.885, P<0.05;t=-3.307, P<0.01). At portal vein phase, difference in CT value showed no statistic significance (t=-0.293, P>0.05). CT value on plain scan and at arterial phase of both HAML and HCC was not significantly different (t=1.289, P>0.05) whilst that at portal vein phase was significantly different (t=2.516, P<0.05). Central vessels were shown in 7 cases of HAML and 3 cases of HCC, pseudocapsule appeared in 1 case of HAML and 13 cases of HCC with statistical difference (χ2=10.828, 7.219;P<0.01). Conclusion CT scan of HAML reveals the component of fat and presentsfast in and slow outpattern, large shadow of vessel and absence of capsule on contrast enhanced scan, which can be helpful in the differential diagnosis from HCCs.

Objective To analyse the relationship between insulin resistance and the pancreatic beta cell dysfunction in critically ill children with hyperglycemia,to investigate the relationship between the pancreas injury and pancreatic beta cells dysfunction.Methods Seven hundred and thirty-six critically ill children admitted in PICU of Hunan Children's Hospital from Nov 2012 to Mar 2013 were reviewed and analyzed.According to the maximum intravenous blood glucose within 24 h after admission,they were divided into severely elevated group (blood glucose ≥11.1 mmol/L,n =67),slightly elevated group(blood glucose 6.1 ～ 11.1 mmol/L,n =361) and control group (blood glucose ≤ 6.1 mmo/L,n =308).Serum insulin,C peptide,serum amylase,lipase,urinary amylase,HOMA-β,HOMA-IR were compared among 3 groups.According to the severity of sepsis,they were divided into non-sepsis group (n =414),sepsis group (n =237),severe sepsis group (n =64),septic shock group (n =21).Blood glucose,serum insulin,C peptide,HOMA-βand HOMA-IR were compared among 4 groups.Results (l)The levels of insulin,C peptide,blood amylase,lipase and urine amylase were gradually increased with elevated blood sugar(rs =0.235,P ＜ 0.05;C =0.142,P ＜ 0.05 ; rs =0.142,P ＜ 0.05 ; rs =0.119,P ＜ 0.05 ; r.s =0.093,P ＜ 0.05).The differences among 3 groups were significant (P ＜ 0.05).The levels of serum amylase (IU/L) [102.81 (10.48-191.69)],lipase(U/L) [69.75(10.67-121.85)] were higher than upper limit of normal in severely elevated group.HOMA-β fell to 18.75％ in severely elevated group.The level of HOMA-β was decreased with the increase of blood sugar level (rs =-0.108,P ＜ 0.05).The level of HOMA-IR was increased with the elevated blood sugar level(rs =0.455,P ＜ 0.05).(2) The levels of blood glucose,insulin,C peptide and HOMA-β were significantly different among 4 groups of non-sepsis,sepsis,severe sepsis and septic shock group (P ＜ 0.05),HOMA-IR showed no significant difference among 4 groups (P ＞ 0.05).The levels of blood sugar increased to 9.21 (6.21-19.60) mmol/L,HOMA-β declined to 10.52％ in septic shock group,and blood glucose,insulin,C peptide,HOMA-β were significantly different compared with the other 3 groups (P ＜ 0.05).Conclusion Hyperglycemia is associated with insulin resistance and pancreatic β cells dysfunction in critically ill children,the cause of beta cell dysfunction is secondary to pancremic injury.Pancreatic beta cells dysfunction inducing hyperglycemia is more significant than insulin resistance in sepsis children.

Objective To investigate the clinicopathological features and prognostic factors in patients with low grade glioma. Methods Eighty- seven patients with low grade glioma confirmed by cytological examination were retrospectively analyzed. Results Among 87 patients, male accounted for 62.1%(54/87), and the average age was 36.2 years. The average followed- up time was 51.4 months (3-135 months), and 5 cases were lost in follow-up. The follow- up rate was 94.3% (82/87). The 3- year survival rate was 84.1%, and the 5- year survival rate was 66.7%. The 3- year survival rate was related to clinical symptoms, maximum diameter of tumor and the extent of resection (P<0.01 or<0.05);and the 5- year survival rate was related to years, Karnofsky score, clinical symptoms, maximum diameter of tumor, the pathology classification and extent of resection (P<0.05 or<0.01). Conclusions Low grade glioma is more common in male patients and patients under the age of 40 years. Age below 40 years, Karnofsky score ≥ 70 scores, only epilepsy symptom, tumor diameter below 6.0 cm, oligodendrogliomas, complete resection or subtotal resection are positive prognostic factors. Postoperative radiotherapy can reduce the rate of tumor progression.

Objective To detect HLA-DRB1(DR1-10) alleles in 5 families with multi-case rheumatic diseases, and to study the possible influence of DRB1 genes in the pathogenesis of rheumatic diseases.Methods Sequence-Specific Primer PCR (PCR-SSP)method was used to examine HLA-DRB1 alleles. Totally 36 members of 5 families and 166 healthy people were involved in this study. The results were assesed by Chi-square test.Results The HLA-DRB1 allele frequency in the patients and their relatives was similiar. No significant difference was found. But DR4 allele frequency in the patients (90.9%) and their relatives (68%) was much higher than that in normal controls (16.8%) and the difference was statistically significant (P<0.0001). In family 4, two RA patients have different DRB1 alleles, while in family 5, two patients have the same DRB1 alleles, one developed SLE and the other developed RA.Conclusions DR4 is closely related to rheumatoid arthritis. The nelatives of RA patients may be at greater risk to develop RA than individuals without family history. Some patients had the same DRB1 allele but developed different rheumatic diseases. This suggested that there might be some common pathways in genetic predisposing of rheumatic diseases. On the other hand, only a few patients with the same DRB1 allele developed rheumatic diseases during their life, so other factors besides DRB1 gene might also be involved in the pathogenesis of rheumatic diseases.

Objective To study the expression of tumor necrosis factor alpha (TNF-α) in the intestine of mice irradiated by neutron and γ rays.Methods 350 male BALB/c mice were irradiated with neutron and γ rays of different doses, and sacrificed at 6 and 12hours, 1, 2, 3, 4, 5, 7, 10, 14, 21 and 28 days after irradiation.The TNF-α in the mice intestinal tissue was detected by means of immunohistochemistry and image analysis.Results In normal control mice, TNF-α was expressed in the cytoplasm of macrophages in intestinal villus interstitium, submucosa and lymph tissue.After 2.5Gy neutron radiation, TNF-α was decreased progressively within 2 days, increased obviously in macrophages and crypt cells during 3rd～7th day, reached the peak at 5th day and recovered to normal level at 14th day and TNF-α was decreased progressively within 4 days after 4.0 and 5.5Gy neutron and 12Gy ray irradiation.TNF-α was increased obviously in 6～12 hours, decreased at 1st day, increased at 2nd～5th day, peaked at 3rd day and recovered at 10th day after 5.5Gy ray irradiation.Conclusion Neutron and ray radiation induce different expression profile of endogenous TNF-α in small intestine, which may be related with the pathologic courses of irradiation-induced damage and repair of intestine.

Objective To investigate the incidence of neonatal hyperphenylalaninemia (HPA) and analyze the characteristics of HPA gene mutations in the Yangzhou area. Methods From January 2013 to December 2022, 285, 549 newborns in Yangzhou were screened for HPA using either the ninhydrin fluorescence method or tandem mass spectrometry. Urinary pterin analysis, erythrocyte dihydrobiopterin reductase activity measurement and genetic diagnosis were performed to screen positive individuals. Results A total of 29 cases were confirmed as HPA, including 3 cases of tetrahydrobiopterin (BH₄) deficiency and 26 cases of phenylalanine hydroxylase (PAH) deficiency. The overall incidence of HPA in Yangzhou was 1/9, 847, with the incidence of PAH deficiency being 1/10, 983, which were slightly higher than the national average but lower than other regions in Jiangsu Province. Among the cases, 13 (44.83%) were classic phenylketonuria (PKU), 7(24.14%) were mild PKU, and 6(20.69%) were mild HPA. Sixteen patients with PAH gene mutations were all compound heterozygotes, with one case exhibiting three-site mutations. PAH gene mutations were predominantly missense mutations, primarily concentrated in exon 7, followed by exon 6, with E7 c.728G>A (21.21%) being the most frequent mutation. Three cases of BH₄ deficiency were detected with PTS gene mutations, including 1 homozygous mutation and 2 compound heterozygous mutations. E5 c.259C>T was the high-frequency PTS mutation gene in Yangzhou. Conclusion HPA has a certain incidence in Yangzhou, with classic PKU being the predominant type. This study clarified the characteristics of PAH and PTS gene mutations in Yangzhou, enriching the HPA gene database.

Objective To explore the role and effectiveness of flexible fiberoptic brochoscopy ( FFB) in critically ill children with continuous invasive respiratory support. Methods From July 2014 to June 2017,135 critically ill children with severe pneumonia had received continuous invasive respiratory sup-port and undergone FFB in the pediatric intensive care unit( PICU) of our hospital. These patients were en-rolled into the therapy group. Another 104 severe pneumonia patients who had not received FFB were enrolled into the control group. Clinical characteristics of both groups were collected and compared. Results No sig-nificant differences were detected in patients′gender, age, PCIS, usage of antibiotics, and the levels of CRP and PCT assayed at the time of admission to PICU between the therapy group and control group(P>0. 05). The positive rate of bacteria culture of the bronchoscopic lavage was 78. 52％( 106 cases ) in the therapy group,and it was significantly higher than that in the control group(60. 58％,63 cases) (χ2 =5. 681,P <0. 005). For 106 cases in the therapy group,117 bacteria strains were identified,while there were 72 bacteria strains found in 63 cases with positive bacteria culture in the control group. Gram negative bacteria were the most common type,followed by Gram positive bacteria. The third most common pathogenic microbes were fungi,with a significantly higher frequency in the therapy group. No significant differences were found in PaO2 and oxygenation index between both groups before FFB. However,in a half hour after FFB,the PaO2 and oxygenation index significantly increased in the therapy group,and higher than those in the control group (P<0. 005). The levels of CRP and PCT assayed before and the first day after FFB were not significantly changed(P>0. 05). But the levels of CRP and PCT in the therapy group significantly decreased in the third day after FFB,and more than those in the control group. Furthermore,duration of invasive respiratory sup-port,and the stay of PICU were significantly shorter in the therapy group(P<0. 005). Conclusion FFB can play an important role in the collection of pathogenic microbes. It also achieves better results in the treatment of severe pneumonia for children in PICU. Therefore,it is worthwhile to be recommended as a safe and feasi-ble intervention in PICU.

Objective:To summary the mixed infection as well as clinical characteristics and analyze the risk factors for mixed infection of severe adenovirus pneumonia(SAP) in children.Methods:The clinical data of 114 children with SAP were retrospectively analyzed.Multivariate Logistic regression analysis was performed to assess the risk factors for mixed infection.Results:The incidence age was from 6 months to 2 years(62.5%). High fever(94.7%), cough(98.2%), dyspnea(86.8%) and lethargy(95.6%) were the main symptoms.Laboratory examination showed that children with SAP were prone to increased white blood cell count, C-reactive protein, procalcitonin, aspartate aminotransferase, alanine aminotransferase and CK-MB, as well as decreased proportion of CD3 + , CD4 + , CD8 + , CD4 + /CD8 + and NK cells.The main complications intrapulmonary organ were respiratory failure(80.7%). The main complications extrapulmonary organ were circulatory complications (55.3%). SAP was easily combined with other pathogenic infections.Streptococcus pneumoniae(22.9%)was the most common bacterial pathogen.Respiratory syncytial virus(10.0%)were the most common virus, in addition, mycoplasma pneumoniae(17.1%) was also common.Multivariable Logistic regression analysis showed that the decreasing ratio of CD4 + /CD8 + and NK cells, congenital heart disease and congenital airway dysplasia were the independent risk factors for mixed infection of SAP in children( P<0.05). Conclusion:The SAP patients could easily suffer from mixed infection and high fatality rate.Immune dysregulation is the important risk factors for mixed infection of SAP in children.So immunoregulatory treatment is very important.

AIM: To observe the effect of Dioscin treatment on NF-κB signaling pathway and cellular inflammatory injury and explore its potential mechanism in uric acid-induced mouse tubular epithelial cells (mTECs). METHODS: After 1.2 mol/L uric acid induced mTECs, Dioscin and NF-κB P65 inhibitor BAY11-7082 were given to intervene respectively. IκB-α, NF-κB P65, PP65, NLRP3, IL-1β and β-actin were detected by Western Blot, immunofluorescence staining and real-time PCR. RESULTS: Western Blot, immunofluorescence staining and real-time PCR analysis showed that expression levels of PP65, NLRP3 and IL-1β were significantly downregulated in the uric acid-induced mTECs with Dioscin and BAY11-7082 treatment. CONCLUSION: Dioscin attenuates uric acid-induced cellular inflammatory damage by suppression NF-κB signaling pathway.

Objective:The construction of public medical group is an important strategic measure to deepen the health reform in China. Taking Beijing Traditional Chinese Medicine Hospital as an example, the article introduces the measures, achievements, existing problems and possible development direction of the discipline co-construction in the entrusted district hospital.Methods:The article analyzes the measures of discipline co-construction and its implementation effect in detail.Results:It has effectively promoted the reasonable allocation of resources between " well-developed leading disciplines" and " disciplines to be nurtured" in the medical group, these disciplines entering a new rapid development phase through optimized organization and management, system construction, new research platform construction.Conclusions:" Disciplines to be nurtured" has been improved in medical service, scientific research capabilities, talent training, academic inheritance and quality of scientific research achievements, based on which, the goal of promoting the development of medical group through discipline co-construction was achieved.