Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is a metabolic disease of long chain fatty acid oxidation. The clinical manifestations are heterogeneous, mainly with heart, liver, skeletal muscle and brain damage, and the onset of which can be from newborn to adult. Cardiomyopathy type is more serious with high mortality. The liver failure type and myopathy type would be potentially lethal, but generally the prognosis is relatively good. Recurrent hypoglycemia, energy metabolism disorder, liver dysfunction, cardiomyopathy and serious arrhythmia are the main causes of death. Most patients can be identified through neonatal screening, and the prognosis is usually good in patients with early diagnosis and treatment. The purpose of this consensus is to standardize the diagnosis, treatment and management of VLCAD deficiency, so as to improve the prognosis of patients and reduce death and disability.

AIM: To investigate the effects of losartan on myocardial fibrosis and left ventricular hypertrophy in hypertensive patients. METHODS: 35 essential hypertensive patients with left ventricular hypertrophy were treated with losartan 50-100 mg for 6 mon. Before and after the treatment, serum procollagen typeⅢ (PCⅢ), laminin (LN), and hyaluronic acid (HA) were measured by radioimmunoassay; and left ventricular mass index (LVMI) was determined with ultrasonic cardiogram. RESULTS: After treatment with losartan: the serum levels of PCⅢ, LN, HA, and LVMI were all decreased (P

BACKGROUND: Cervical lymph nodes are draining region of cornea. It is believed that aqueous fluid goes through a minor pathway named uveoscleral drainage, which will allow passage of antigen-presenting cells (APC) directly to the draining lymph nodes and induce allograft rejection after keratoplasty.OBJECTIVE: To explore the inhibitory effects of cervical lymphadenectomy in alkali induced high-risk corneal transplantation.DESIGN: A randomized controlled animal experiment.SETTING: State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University.MATERIALS: The experiment was performed in the State Key Laboratory of Ophthalmology (No. 2006DA105054), Zhongshan Ophthalmic Center, Sun Yat-sen University from May 2005 to February 2007. 144 male animals (1-2 months old) including 104 SD rats and 40 Wistar rats were provided by the animal experimental center of Sun Yat-sen University. Sandwich enzyme-linked immunosorbent assay (ELISA) kits for interleukin-2 (IL-2) and interferon-γ (IFN-γ) were brought from BioSource International company (USA). The animal treatment in the experiment was accorded with the statement in Association for Research in Vision and Ophthalmology (ARVO) for animals.METHODS: With the SD rats as recipients, and Wistar rats as donors, all rats were subjected to corneal allografting. The recipient rats were randomly divided into 4 groups (n=20): group A (control group) which underwent corneal transplantation; group B which was subjected to bilateral cervical lymphadenectomy; group C, corneal transplantation 21 days after the alkali burn injury; group D, cervical lymphadenectomy following group C. The immune rejection of grafts was evaluated by detecting the expression of IFN-γ and IL-2 using ELISA. The time when allograft rejection occurred was recorded and mean survival time (MST) was compared among the groups. The development of corneal inflammation and new vessels was examined by slit lamp microscope and histopathological examination.MAIN OUTCOME MEASURES: ①The development of corneal inflammation after corneal alkaline burns. ②MST of rats in each group following transplant. ③The expression of IL-2 and IFN-γ in grafts of each group. RESULTS: ①Normal rat cornea was transparent without inflammation or neovascularization. There were many inflammatory cells invading to stroma on day 3 after burn. Then, the inflammation of cornea resolved gradually 3 weeks after the burn, but corneal neovascularization reached the peak at that time. Corneal blood vessels regressed completely at the end of 8 weeks after the burn. ②The MST of group A, B, C, and D was (10.40±1.14), (46.30±9.46), (7.00±1.58), and (15.00±3.39) days, respectively. Compared with the group A, the MST of group B was significantly longer (P < 0.05), and the MST of grafts in group D was also significantly longer than group C (P < 0.05). ③The expression of IFN-γ and IL-2 proteins was absent in group B. Compared with group C, the expression of IL-2 and IFN-γ proteins in group D significantly decreased on days 3, 7, 10, and 14 after keratoplasty (P < 0.05). CONCLUSION: Cervical lymphadenectomy therapy can effectively inhibit corneal allograft rejection in normal and high-risk corneal beds after alkali burn injury.

Objective To analyze the hospitalization expenses of cancer patients covered with byitem payment and quota payment packages,and probe into the impacts on such expenses for the two payment packages.Methods Inpatient records of 600 cancer patients were sampled by random from the medical insurance databases of Zhengzhou and Fuzhou to learn their hospitalization expenses and impact factors.Results Under the by-item payment package,the expenses of urban workers’ medical insurance were found higher than those of urban residents' medical insurance,with a per capita expense of RMB 32747.70 ± 32035.01 and 23035.83 ± 22875.65 respectively.Under the quota payment package however,there were no significant differences between expenses of the two kinds of inpatients,with a per capita expense of RMB 66043.41±47562.09 and 66576.54±73417.29 respectively.Conclusion There are gaps of reimbursement level between the two basic insurance schemes,which may not disappear in a short time.Under the by-item payment package,the gap exists in the difference of perreimbursement amount; under the quota payment package,the gap is negligible between the two populations under different insurance schemes.It is recommended to make reasonable use of these different payment schemes to minimize the relative gaps in medical service accessibility caused by the difference in reimbursement level.

Objective To investigate toil-like receptor 4 (TLR4) expression and activity in peripheral blood mononuclear cells(PBMCs) of obese patients.Methods PBMCs from 16 obese patients and 16 normal control subjects were collected.TLR4 and IκBα protein concentrations were measured in PBMCs by western blotting.TLR4 and interleukin-6 (IL-6) mRNA expression levels in PBMCs were measured by quantitative realtime PCR.The blood levels of glucose,insulin,free fatty acids (FFA),IL-6 and tmnor necrosis factor-α (TNF-α) were measured after an overnight fast.Results The levels of FFA,IL-6,TNF-α and homeostasis model assessment of insulin resistance index (HOMA-IR) in obese patients were higher than that of control group (FFA:(879 ± 64) μmol/L vs.(458 ± 48) μmol/L; IL-6:(2.20 ± 0.35) ng/L vs.(1.26 ± 0.25) ng/L; TNF-α:(1.96±0.32) ng/Lvs.(1.38 ±0.24) ng/L;HOMA-IR:(1.8±0.2) vs.(0.4±0.1) ;t=24.613,P=0.000;t =14.993,P =0.000;t =9.128,P =0.000;t =32.254,P =0.000).Increased TLR4 gene and protein expression were observed in PBMCs of obese patients compared with control group(TLR4 mRNA:(3.13±0.21) vs.(0.99 ± 0.03),t =54.758,P ＜ 0.05; TLR4 protein:(7.04 ± 0.42) vs.(2.53 ± 0.17),t =77.450,P ＜0.05).IκBα protein concentration in PBMCs of obese patients (2.52 ±0.16) was lower than in control group (4.00 ± 0.30,t =23.284,P ＜ 0.05),indicating elevated IKKβ/NF-κB signaling.The increase in TLR4 and NF-κB signaling was accompanied by elevated expression of the NFκB-regulated gene IL-6 ((2.55 ±0.15) vs.(1.03 ±0.11),t =53.981,P ＜0.05).Conclusion TLR4 expression and activity are increased in the PBMCs of patients with obesity and might involve in the development and progress of insulin resistance.

Objective To evaluate different expressions of high mobility group box 1(HMGB1)and receptor for advanced glycation end products(RAGE)in placentas and their relationship with preeclampsia.Methods Fifteen early-onset pre-eclaraptic women(early-onset pre-eclampsia group),22 late-onset pre-eclamptic women(late-onset pre-eclampsia group)and 12 normotensive women(control group)in the third trimester were recruited at the Shengjing Hospital of China Medical University from March 2006 to March 2007.The localization and levels of HMGB1 and RAGE in placentas of the three groups were detected by the strept avidin biotin-peroxidose method.Results (1)Immunoreactivities to HMGB1:positive immnnostaining for HMGB1 was observed in trophoblast,macrophages,decidual cells,vascular muscle cells,endothelial cells and placental mesenchymal cells in the placentas from the pre-eclamptic women,while a low level of immunoreactivities was observed in the placentas from healthy pregnancies;the staining was observed within both the nuclei and the cytoplasm,mainly in the cytoplasm.The cytotrophoblast,especially the nuclei was extensively positive for HMGB1 in early-onset pre-eclampsia. (2)Immunoreactivities to RAGE:positive immunostaining for HMGB1 was observed in syncytiotrophoblast,macrophages and endothelial cells in the placentas from the preeclamptic women,while a low level of immunoreactivities was observed in the placentas from healthy pregnancies:the staining was in the cytoplasm and(or)cell membrane.The trophoblast was extensively positive for RAGE in early-onset pre-eclampsia.(3)Positive rate of HMGB1 expression:the expression of HMGB1 in early-onset group(73%,11/15)and late-onset group(64%,14/22)was significantly higher than that in normal group(17%,2/12;P<0.05),but no significant difference was found in early-onset group and late-onset group(P>0.05).(4)Positive rate of RAGE expression:the expression of RAGE in early-onset group(80%,12/15)and late-onset group (82%,18/22)was significantly higher than that in normal group(25%,3/12;P<0.05),but no significant difference was found in early-onset group and late-onset group(P>0.05).Conclusions The increased expression of HMGB1 and RACE in the placenta may play an important role in the pathogenesis of pre-eclampsis.The different locations may be associated with the occurrence of different onset types of pre-eclampsia.

Objective To study the antibacterial constituents of Senecio cannabifolius.Methods The antibacterial effect of all the extracts was tested in vitro with Staphylococcus aureus,Bacillus subtilis(Gram-positive bacilli),and Escherichia coli(Gram-negative bacillus).Chemical constituents were isolated by chromatography.Physicochemical characters and spectroscopic analyses were employed for their structural identification.Results Five compounds were isolated and their structures were elucidated by chemical and spectral methods as follows: methyl-5-hydroxy-2-pyridinecarboxylate(E-13),6-hydroxy-7,7a-dihydro-2(6H)-benzofuranone(E-14),2-(1,4-dihydroxycyclohexanyl)-acetic acid(E-16),3-hydroxycyclohexanecarboxylic acid(E-17),and 4-hydroxy benzaldehyde(B-14).Conclusion All of them are isolated from the plants of Senecio L.for the first time.

[Objective] To explore the effect of early treatment of Chinese herbal medicine on the long-term prognosis of acute myocardial infarction (AMI) . [Methods] One hundred and fifty-seven AMI patients were divided into two groups according to the treatment during the hospitalization: 129 patients treated with Chinese herbal medicine and western medicine were in group A, and 28 patients treated with western medicine only were in group B. Statistical analysis of age, sex, infarction location, complications and medical history was made in all of the patients. A follow-up survey was made to investigate the subsistence of the patients and the incidences of all the events (including death and severe cardio-cerebrovascular events) . [Results] With the death as the end event, the survival graph of group A was higher than group B (P=0.1166); when with all the severe events as the end event, the survival graph of group A was still higher ( P=0.048) .[Conclusion] The probability of incidences of severe events including death in group A is lower than that in group B.

OBJECTIVE To study the relativity among PreS1-Antigen,HBV markers and HBV-DNA. METHODS The HBV markers,PreS1-antigen and HBV-DNA were determined by ELISA and PCR in 102 patients with chronic hepatitis B and 73 healthy persons. RESULTS Among 102 patients with chronic hepatitis B,the concordance rate of PreS1-antigen and HBeAg with HBV-DNA was 70.6% and 75.5%.The sensitivity of PreS1 was better than HBeAg but the specificity was contrary.It represented some patients with HBeAg(-) still had viral replication.On the increase in the level of HBV-DNA,the positive rates of PreS1-antigen and HBV markers increased. CONCLUSIONS The detection of PreS1-antigen can well reflect the HBV replication.The synchronous dynamic detection of PreS1-Antigen,HBV markers and HBV-DNA has its important clinical meaning.

Objective To examine the effects of IL-10 on cardiac fibroblasts ( CFBs) proliferation and phenotype transformation to myofibroblasts (MyoFbs) induced by transforming growth factor-β1 (TGF-β1);and to investigate the regulating pathways .Methods Cardiac fibroblasts were isolated from cardiac ventricles of neonatal SD rats . The passage 2~4 were used and divided into the following groups for treatment:1) control group, 2) IL-10 reac-tion group, 3) TGF-β1 reaction group, and 4) IL-10 plus TGF-β1 reaction group (TGF-β1 treatment followed with IL-10 pretreatment ) .Cells proliferation was assessed by MTT assay and immunocytochemistry staining for prolifera-ting cell nuclear antigen (PCNA);the phenotype transformation into MyoFbs was assessed by immunocytochemistry of α-smooth muscle actin (α-SMA);extracellular signal related kinase ( ERK1/2) and P38 kinase pathways were assessed by western-blot.Results TGF-β1 (10 μg/L) treatment boosted the proliferation and the expression ofα-SMA significantly (P<0.01), while IL-10 (10, 50 or 100 μg/L) plus TGF-β1 co-treatment induced lower cell proliferation and expression of α-SMA than treating with TGF-β1 alone ( P<0.05 ) , with the inhibitory effect of IL-10 being concentration dependent .TGF-β1 could significantly stimulate the ERK 1/2 and P38 kinase phospho-rylation ( P<0.01 ) , however IL-10 (100 μg/L) plus TGF-β1 co-treatment failed to down-regulated the phospho-rylation of ERK1/2 and P38 kinase compared with TGF-β1 alone ( ERK1/2:P<0.05;P38:P<0.01 ) .Conclu-sions IL-10 can attenuate TGF-β1-induced CFBs proliferation and phenotype transformation to MyoFbs .The in-hibitory effects may explained by a mechanism of inhibiting the activation of ERK 1/2 and P38 kinase .