This study was aimed to investigate the effects of hesperidin on the macrophage foam cell formation in RAW264.7 cells and the expression of ICAM-1.RAW264.7 cells were culture in vitro and induced by oxLDL (50 μg· mL-1).Furthermore,cells were separated into the control group,oxLDL model group and hesperidin treatment group.The effect of hesperidin on cell viability in RAW264.7 was assessed by MTF assay.Oil Red O staining was examined by foam cells formation.Effect of hesperidin on protein expression of ICAM-1 was analyzed by western blot.In addition,the effect of hesperidin on mRNA expression of ICAM-1 was assessed by RT-PCR.The results showed that the viability should been over 80％ after less than or equal to 5 μM hesperidin treatment.Hesperidin decreased the protein expression of ICAM-1 in RAW264.7 cells.Additionally,hesperidin suppressed the mRNA expression of ICAM-1 in RAW264.7 cells.It was concluded that hesperidin can inhibit foam cell formation and the expression of ICAM-1 in RAW264.7 cells.It suggested that hesperidin protect against atherosclerosis.

Objective To investigate the effects of nitric oxide (NO) and cholecystokinin (CCK) on the regulation of the motility of sphincter of Oddi (SO).Methods The basal pressure,action rate and contraction range of the SO were examined before and after the injection of CCK,sodium nitroprusside (SNP) and NG-nitroL-argininemethyl ester hydrochloride (L-NAME).The expression of neurons with positive expression of NO synthase was detected by immunohistochemical staining.The measurement data were analyzed by using the t test.Results The basal pressure,contraction rate and contraction range of the SO were (27 + 10)mm Hg (1 mm Hg =0.133 kPa),( 10 ± 3 ) times/minute and (32 + 8 ) mm Hg before injection of CCK,and (61 + 14) mm Hg,(64 +21 ) times/minute,(44 ± 15 ) mm Hg after injection of CCK of 20 ng/kg.After injection of CCK of 100 ng/kg,the basal pressure,contraction range and contraction rate of the SO were (77 ± 31 )mm Hg,(69 ± 18 ) times/minutes,(79 + 14) mm Hg when the inhibition effect of CCK reached peak,and were ( 140 ± 21 ) mm Hg,( 129 ± 25 )times/minutes,( 173 ± 63 ) mm Hg when the excitatory effect of CCK reached peak.After injection of SNP into the common bile duct,the basal pressure,contraction range and contraction rate of the SO decreased significantly ( t =3.706,5.183,P ＜ 0.05),while the 3 indexes increased significantly after injection of SNP (t =5.859,3.588,P ＜O.05).Conclusion Different from physiological dose (20 ng/kg) of CCK,large dose of CCK enhances motility of SO intensively.NO relaxes SO,which may play an important role in the inhibitor pathway of CCK.

Objective To explore the changes in serum concentrations of irisin,vaspin and reactive oxygen species (ROS) in patients with type 2 diabetes mellitus (T2DM) and metabolic syndrome (MS),and to investigated the correlation of irisin and vaspin with clinical parameters of MS.Methods A total of 260 T2DM patients were enrolled.Age and gender were recorded,anthropometrics,biochemical parameters,and levels of irisin,vaspin and ROS in fasting serum were measured,and homeostatic model assessment of insulin resistance (HOMA-IR) calculated.Wilcoxon rank sum test,correlation analysis,Logistic regression analysis,multiple linear regression analysis and receiver operating characteristic (ROC) curve analysis were performed.Results Compared to T2DM patients without MS,T2DM patients with MS had lower serum level ofirisin [male:112.81 (86.96-191.84) μg/Lvs.156.23 (110.61-225.97) μg/L,female:141.09 (77.52-175.55) μg/L vs.172.15 (95.69-240.37) μg/L,P ＜0.01],higher levels of vaspin and ROS [male:1.13 (0.95-1.38) μg/Lvs.0.36 (0.21-0.82) μg/L,1 540 (1 250-1 860) kU/Lvs.1 020 (920-1 350) kU/L;female:1.52 (1.13-1.80) μg/Lvs.0.51 (0.47-1.08) μg/L,1 650 (1 320-1 940) kU/Lvs.1 120 (980-1 420) kU/L,P ＜0.01].In the T2DM patients,serum irisin level was negatively correlated with vaspin (r =-0.382,P ＜ 0.01) and ROS (r =-0.410,P ＜ 0.01),while vaspin was positively correlated with ROS (r =0.400,P ＜ 0.01).Multiple linear regression analyses showed that irisin was significantly correlated with body mass index (BMI),waist circumference and triglyceride,while vaspin was correlated with gender,BMI,and waist circumference (all P ＜ 0.05).Logistic regression analysis revealed that irisin,vaspin and ROS were all associated with MS (OR =0.77,95 ％ CI 0.608-0.978;OR=1.39,95％ CI 1.252-1.539;OR=1.38,95％ CI1.112-1.718,all P＜0.05).ROC analysis demonstrated that irisin and vaspin had significant area under the curve (AUC =0.931,P ＜0.01;AUC =0.777,P ＜ 0.01) for the prediction of MS.Conclusions Serum irisin level was significantly decreased,while vaspin and ROS were significantly increased in T2DM patients with MS.Irisin and vaspin were associated with clinical presentations of MS,suggesting that irisin and vaspin might be valuable predictors of MS.

Objective To evaluate the clinical outcomes of different flaps for repairing the soft tissue defects of heels.Methods A total of 26 patients with soft tissue defects around the heel treated modified propeller perforator flap,medialis pedis flap,or anterolateral thigh flap from March 2012 to June 2016 were analyzed retrospectively.There included 19 males and 7 females,aged 4-64 years (mean,38.1 years).There were 9 patients with posterior heel defect,3 with weight-bearing defect,6 with posterior medial defect and 8 with posterolateral defect.The wound areas were from 6.0 cm × 4.0 cm to 12.0 cm × 9.5 cm,while the flap areas were from 7.0 cm × 5.0 cm to 13.5 cm × 10.5 cm.According to the principle of flap selection,the pedicled skin flap instead of free skin flap was selected in order to minimize damage to the donor site area.Modified propeller perforator flap was applied in 13 patients,medialis pedis flap in 3 patients and anterolateral thigh flap in 14 patients.The flap donor site was directly sutured in 23 patients and a simultaneous skin graft was applied in 3 patients.The survival rate,appearance,texture and feeling recovery of flaps,complications,walking ability,and the status of donor sites were compared.Besides,postoperative functions of all cases were estimated according to foot scoring scale of American Orthopaedic Foot and Ankle Society (AOFAS).Results All flaps survived well in 26 patients.The wounds of flaps and flap donor sites were healed at Ⅰ stage.A total of 24 patients were followed up for 12-36 months (average 16 months).The appearance,color and texture of the flaps were good.There was no ulcer in flaps or flap donor sites.The protective feeling of flaps was recovered and the feeling of distinguishing two points was 6-13 mm.Modified propeller perforator flap donor site was directly sutured,the wound of which showed a linear healing.There was no fat deformity or obvious scar formation around ankle.The skin graft of the medialis pedis flap donor site was healed well,without scar hyperplasia,rupture,or deformity of arch.The anterolateral thigh flap was healed linearly without scar,and the anterolateral skin felt slightly depressed.The muscle strength of the four biceps femoris muscle was 4.According to AOFAS score,the feet's functions were evaluated as excellent in 5,good in 16,fair in 4,and poor in 1,with excellence rate of 81％.Conclusions For different soft tissue defects of the heels,propeller perforators flap,medial plantar flap or anterolateral thigh flap can not only attain appearance reconstruction of the defects and good functional recovery,but also minimize the injury of flap donor site.

Objective To compare gross tumor volume (GTV) of nasopharyngeal carcinoma (NPC) according to MRI and FDG PET/CT and to investigated four fixed threshold methods to delineate the GTV using FDG PET/CT.Methods Fifty patients with primary biopsy-proven NPC were prospectively were enrolled into the study.FDG PET/CT scans and MRI were carried out within one week prior to pretreatment,respectively.The GTV was named GTV-MRI (GTV were delineated according to MRI),GTV-PETvis,GTV-PET30,GTV-PET40,GTV-PET50 (GTV was delineated according to the PET-based GTVs obtained by visual interpretationor,by percentage of the SUVmax (30％,40％,50％) thresholds,respectively).The differences were compared among the GTV-MRI,GTV-PETvis,GTV-PET30,GTV-PET40 and GTV-PET50 in different by Wilcoxon test.Results Of 50 patients,the median of volume descending order were: GTV-MRI 27.8 cm3,GTV-PETvis 22.2 cm3,GTV-PET30 22.7 cm3,GTV-PET40 14.4 cm3 and GTV-PET50 9.0 cm3.However,there was no significant difference between GTV-PETvis and GTV-PET30 (Z=-0.05,P=0.958),as well as GTV-MRI and GTV-PETvis or GTV-PET30 in 25 patients who were T1-2 stage (Z =-0.93,-0.93,P=0.353,O.353),the other GTVs were all different in 50 patients' (Z=-5.74-2.09,P =0.000-0.037).Conclusions All the GTVs delineated by the different methods of using FDG PET/CT were less than the GTV delineated by MRI.The potential advantages with the GTV-PETvis or GTV-PET30 delineated by FDG PET/CT are reduction of biological metabolic tumor volume in GTV delineation and reduction of the size of the GTV in NPC patients.

Objective: To explore phenotypic and mutational characteristics of a pedigree affected with autosomal dominant Charcot-Marie-Tooth disease (CMT) and nephropathy. Methods: Clinical data of the proband and his family members was collected. Electrophysiology, renal biopsy and next-generation sequencing were carried out for the proband. Results: The proband presented with distal lower limb weakness and proteinuria in childhood. His mother and brother had similar symptoms. Electrophysiological test of the proband revealed demyelination and axonal changes in both motor and sensory nerves. Renal biopsy suggested focal segmental glomerulosclerosis. Genetic testing revealed a heterozygous c. 341G>A (p.G114D) mutation in exon 2 of the INF2 gene. Conclusion The phenotypic feature of the pedigree is autosomal dominant intermediate CMT and focal segmental glomerulosclerosis, which may be attributed to the c. 341G>A mutation of the INF2 gene.

OBJECTIVE: To explore phenotypic and mutational characteristics of a pedigree affected with autosomal dominant Charcot-Marie-Tooth disease (CMT) and nephropathy. METHODS: Clinical data of the proband and his family members was collected. Electrophysiology, renal biopsy and next-generation sequencing were carried out for the proband. RESULTS: The proband presented with distal lower limb weakness and proteinuria in childhood. His mother and brother had similar symptoms. Electrophysiological test of the proband revealed demyelination and axonal changes in both motor and sensory nerves. Renal biopsy suggested focal segmental glomerulosclerosis. Genetic testing revealed a heterozygous c.341G>A (p.G114D) mutation in exon 2 of the INF2 gene. CONCLUSION The phenotypic feature of the pedigree is autosomal dominant intermediate CMT and focal segmental glomerulosclerosis, which may be attributed to the c.341G>A mutation of the INF2 gene.
Charcot-Marie-Tooth Disease ; complications ; genetics ; Child ; Female ; Glomerulosclerosis, Focal Segmental ; complications ; genetics ; Heterozygote ; Humans ; Male ; Microfilament Proteins ; genetics ; Mutation ; Pedigree

Objective:To observe the effect of simulation combined with problem-based learning (PBL) in the clinical practice teaching of internal medicine.Methods:A total of 41 internal medicine interns accepted from January 2019 to August 2019 in The Third Hospital of Hubei Medical University were selected as the control group, and the traditional clinical teaching was adopted. Another 43 internal medicine interns received from September 2019 to August 2020 were selected as the experimental group, and the simulation combined with PBL teaching was adopted. The clinical skill knowledge and practical operation skills were compared between the two groups in the examination results, the changes in clinical ability before and after the internship, and the recognition of the teaching. SPSS 25.0 was used for t-test, Chi-square test, and rank sum test. Results:The clinical skill knowledge and practical operation skills of interns in the experimental group [(94.51±4.49), (91.05±7.66)] were higher than those in the control group [(89.20±9.23), (85.63±11.57)] ( P<0.05). There was no significant difference in various scores and total scores of clinical competences (including medical interview skills, physical examination skills, humanistic care skills, clinical judgment skills, communication skills, organizational effectiveness, and overall clinical competence) between the two groups before internship. After the internship, the above scores and total scores of the two groups were higher than those before the internship, and the experimental group was higher than the control group ( P < 0.05). The total recognition of the teaching by interns in the experimental group was 95.35% (41/43), which was significantly higher than 78.05% (32/41) in the control group. Conclusion:Simulation combined with PBL teaching can significantly improve the clinical skill knowledge, practical operation level, clinical ability, and recognition of teaching of medical interns.

Objective:To investigate the clinical and genetic features of Pompe disease, and analyze the effect of enzyme replacement therapy on it.Methods:A retrospective study was performed. The clinical data and genetic results of 14 patients with Pompe disease from 12 families, admitted to our hospital from January 2017 to June 2021, were collected. Some patients were followed up after therapies.Results:Twelve of the 14 patients were late onset, with onset age ranged from 1.5 to 37.0 years (mean 15.2 years), and the other 2 patients were infantile onset. The predominant manifestations included proximal lower limb weakness, accompanied by easy fatigue and myalgia; 8 patients presented with dyspnea, of which one had dyspnea as initial presentation. Serum creatine kinase ranged from 172 to 1397 IU/L (mean 878 IU/L). Electromyography revealed myogenic pattern in 6 patients and myotonic discharge in 4 patients. Forced vital capacity decreased in 10 patients, and scoliosis was detected in 5 patients; 13 patients had decreased acid-alpha-glucosidase (GAA) activity; muscle pathology indicated vacuolar myopathy in 8 patients. Genetic test revealed 17 variants in GAA gene, among which c.2331G>C, c.1622C>T, c.1585T>C, and c.1837T>C were 4 novel likely pathogenic variants. The c.2238G>C and c.2662G>T were found in 5 and 3 families, respectively. Muscle strength and lung function got improvement in 1 patient who received enzyme replacement therapy and had regular follow-up, while muscle strength and lung function were worsened in those who did not receive enzyme replacement therapy. Conclusions:Pompe disease is characterized by skeletal muscle weakness and pulmonary dysfunction, and may be associated with spinal deformity; creatine kinase is mildly to moderately elevated, and myotonic discharge can be detected. GAA c.2238G>C and c.2662G>T are hotspot mutations in China; the 4 novel variants enrich the GAA mutational spectrum. Enzyme replacement therapy may improve motor and pulmonary function.

Objective:To investigate the clinical, pathological and genetic characteristics of myopathy-type very long chain acyl-coenzyme A dehydrogenase deficiency (VLCADD).Methods:The detailed clinical data, muscle biopsy pathology and molecular results of 4 patients with genetically confirmed myopathy-type VLCADD admitted to Henan Provincial People′s Hospital and Xuanwu Hospital, Capital Medical University from June 2014 to November 2019 were retrospectively analyzed.Results:All of the 4 patients were late-onset myopathy-type VLCADD. The onset age ranged from 13 to 16 years, with a mean age of 14.5 years. The age at diagnosis ranged from 21 to 54 years, with a mean age of 42.5 years. The main clinical manifestation was repeated rhabdomyolysis, including myalgia, weakness and dark urine. Obvious somnolence was observerd in 1 patient. Muscle biopsy pathology revealed mild lipid accumulation, without vacuoles. Six ACADVL variations were detected in the 4 patients, including c.1283G>A (p.R428H), c.1532G>A (p.R511Q), c.833_835delAGA (p.K278del), c.1843C>T (p.R615 *), c.1748C>T (p.S583L) and c.1391C>T (p.T464I),among which c.1391C>T (p.T464I) was a novel variation, predicted to be likely pathogenic. Other 5 variations were reported pathogenic variations. Conclusions:Myopathy-type VLCADD is characterized by paroxysmal rhabdomyolysis and can be associated with somnolence. There is no specificity in muscle pathology. There are ACADVL variations, among which c.1391C>T is a novel variation.