Objective To explore the plasma ghrelin levels in children and adolescents with short stature and the role of ghrelin in growth hormone-releasing hormone-growth hormone(GHRH-GH) axis.Methods One hundred and fifty-seven children(115 male,42 female) with short stature were selected.Fasting plasma sample was extracted from 10 mL vemous blood of the children with short stature.Insulin tolerance test and arginine stimulation test was performed initially to differential diagnosis.And blood samples was divided into 3 ca-tegories:37 cases of complete growth hormone deficiency (CGHD),52 cases of partial growth hormone deficiency(PGHD) and 68 cases of idiopathic short stature(ISS) during these two growth hormone(GH)provocative tests.Controls consisted of age and gender-match 20 health children.Plasma ghrelin levels were measured by radioimmunoassay.Serum GH was detected by chemiluminescence method,and serum insulin-like growth factor-1 (IGF-1) was measured by using enzyme linked immunosorbent assay.Fasting glucose,insulin,testosterone,estra-diol,luteinizing hormone,follicle-stimulating hormone were measured.Statistical analysis were conducted by using SPSS 11.5 software.Results The fasting ghrelin levels of CGHD group were significantly lower than that of ISS group and control group(Pa0.05).The ghrelin levels were positive correlated with the stimulated GH peak(r=0.176 P0.05).Conclusion Ghrelin has an important role on GH secretion and abnormal secretion of ghrelin might be a reason of growth hormone deficiency which due to hypothalamic abnormality.

Objective Explore the conditions of the cloning,expression and purification of FMRP.Methods The plasmid pET22b(+)-FMR1,constructed by molecular cloning,was transformed into E.coli BL21(DE3) competent cells and induced to express FMRP by IPTG.Recombinant FMRP was purified by affinity chromatography,verified by Western-blot,and tested for its RNA binding ability.Results FMR1 cDNA was successfully cloned into pET22b(+) vector and expressed in E.coli BL21(DE3).A protein with Mr 79 000 was purified and confirmed to be FMRP.This protein retained the RNA binding ability of FMRP.Conclusion We successfully expressed recombinant hFMRP with high purity and activity in E.coli,which provided a reliable material to study the function of FMRP.

Adolescent ; Child ; Female ; Humans ; Inhibitor of Apoptosis Proteins ; Killer Cells, Natural ; pathology ; Leukocyte Common Antigens ; analysis ; Lymphoma, T-Cell ; genetics ; immunology ; Male ; Microtubule-Associated Proteins ; genetics

BACKGROUNDPremature ventricular contraction (PVC) is one of the most common kinds of arrhythmias for which the treatment falls into dilemma. Previous clinical application showed that the traditional Chinese Medicine Shensongyangxin (SSYX) capsule is efficacious for the treatment of PVCs. This randomized clinical trial aimed to further evaluate the efficacy and safety of SSYX capsule on treating PVC.

METHODSThe subjects who had frequent PVCs with or without organic heart disease and normal cardiac function were enrolled in the study. The primary endpoint was the change of PVC numbers after eight-week medication with SSYX capsule. The secondary endpoints included change of clinical symptoms related to PVCs and the safety evaluation of SSYX capsule. Totally 188 PVC patients were randomly enrolled in the non-organic heart disease PVCs trial and orally took either SSYX capsules or analogues (three times per day, 4 capsules one time). A total of 671 PVCs patients were randomly enrolled in the organic heart disease PVCs trial, and orally took either SSYX capsules (three times per day, 4 capsules one time) or mexiletine tablet (three times per day, 150 mg one time). The PVCs were monitored and calculated with 24-hour Holter electrocardiogram. Routine blood, liver and kidney function were tested before and after medication with SSYX capsule.

RESULTSSSYX capsules significantly decreased the PVCs numbers and alleviated the related symptoms in patients with or without organic heart disease. In non-organic heart disease group, SSYX capsules and the placebos decreased the PVCs from 12,561.34 ± 9,777.93 to 4,806.87 ± 6,507.17, and 12,605.69 ± 8,736.34 to 10,364.94 ± 9,903.41, respectively. The total effective rate was 74.2% and 28.9% in SSYX and placebo groups (P < 0.001). In organic heart disease group, SSYX capsule and mexiletine decreased the PVCs from 8,641.01 ± 8,923.57 to 3,853.68 ± 7,096.42, 8,621.61 ± 8,367.74 to 5,648.29 ± 8,667.38, respectively. The total effective rate was 65.8% and 50.7% in SSYX and mexiletine groups (P < 0.001). In addition, SSYX capsule significantly alleviated PVCs-related symptoms such as palpitations, chest tightness, insomnia, fatigue, and night sweats. No adverse cardiac events were observed except some slight gastrointestinal side effects during the study.

CONCLUSIONSCompared with placebo or mexiletine, SSYX capsules have significant therapeutic efficacy in reducing PVCs numbers and alleviate PVCs-related symptoms.

Capsules ; therapeutic use ; Double-Blind Method ; Drugs, Chinese Herbal ; therapeutic use ; Humans ; Medicine, Chinese Traditional ; Ventricular Premature Complexes ; drug therapy

Adolescent ; Adult ; Carcinoid Tumor ; pathology ; surgery ; Child ; Female ; Humans ; Male ; Middle Aged ; Pancreatic Neoplasms ; pathology ; surgery

OBJECTIVETo investigate the effect of glucosylceramide synthase (GCS) on P-glycoprotein (P-gp) expression via extracellular signal-regulated kinase (ERK) pathways in leukemia K562/A02 cell line.

METHODSK562/A02 multidrug resistance cells were treated with GCS siRNA and U0126, respectively. Expression of multidrug resistance protein 1 (MDR1) mRNA was analyzed with qRT-PCR. Phosphorylated ERK1/2, total ERK1/2 protein and P-gp in different groups were measured with Western blotting.

RESULTSAfter treated with U0126, P-ERK1/2 was decreased along with the increased U0126 concentration. P-ERK1/2 and P-gp were apparently down-regulated by U0126 at the concentrations of 20 μmol/L, 40 μmol/L and 60 μmol/L. After being transfected with GCS siRNA, GCS mRNA was inhibited by 70.50% (58.00%-76.00%) in K562/A02 cells. Compared with the negative control, both P-ERK1/2 and P-gp were inhibited significantly after RNAi for 72 hours (P<0.01 and P<0.05, respectively.

CONCLUSIONGCS may affect the expression of P-gp by ERK signal transduction pathway in leukemia cells.

ATP-Binding Cassette, Sub-Family B, Member 1 ; biosynthesis ; genetics ; metabolism ; Cell Line, Tumor ; Drug Resistance, Multiple ; Drug Resistance, Neoplasm ; Glucosyltransferases ; metabolism ; Humans ; K562 Cells ; Leukemia ; enzymology ; genetics ; metabolism ; MAP Kinase Signaling System

OBJECTIVETo study the application of abnormal electrophysiological substrate mapping for guiding ablation of ventricular tachycardias in arrhythmogenic right ventricular cardiomyopathy (ARVC-VTs) using a non-contact mapping system.

METHODSDynamic substrate mapping was performed in three male ARVC patients during sinus rhythm. The sites of the earliest activation, exit point and activation sequence were mapped for each induced VT.

RESULTSThree different patterns of substrates were determined in 3 patients, which located in right ventricular outflow tract, anterior right ventricular wall, and anterolateral right ventricular wall, respectively. Five different clinical VTs [mean CL (348 +/- 65) ms] were induced. Of 5 VTs, three were originated from substrate or boundary of substrate, and two had a remote origin. One VT conducted through the substrate. Linear ablations were created between the sites of the earliest ventricular activation and the VT exit point, or across the critical isthmus. The five clinical VTs were successfully ablated. There were no VT recurrences during 20 months of follow-up.

CONCLUSIONSDefining the abnormal electrophysiologic VT substrates is useful for understanding the mechanisms of ARVC-VTs and determining an ablation strategy. Linear ablation across a critical isthmus or between the earliest activation and the exit point can effectively cure these arrhythmias.

Adult ; Arrhythmogenic Right Ventricular Dysplasia ; etiology ; physiopathology ; therapy ; Catheter Ablation ; methods ; Electrophysiologic Techniques, Cardiac ; Humans ; Male ; Tachycardia, Ventricular ; complications ; physiopathology ; therapy

OBJECTIVEClinical observation of electrophysiological study and implantable cardioverter defibrillator (ICD) therapy in patients with Brugada syndrome.

METHODSTen patients (all male) with Brugada wave (spontaneous or propafenone test positive in ECG) underwent electrophysiological study (EPS). The mean age was (41 +/- 10) years. They had no structural heart disease with echocardiogram and the angiogram work-up. The ICD implanted in the patients with EPS-induced ventricular fibrillation in those who were available.

RESULTSThree patients had the history of familial sudden cardiac death (SCD). Four patients had repeated syncope episodes, two of them had documented ventricular fibrillation during syncope episodes. The AH and HV intervals were 50 - 124 (86 +/- 21) ms and 41 - 84 (58 +/- 15) ms. The ventricular fibrillation was induced in four patients with syncope and atrioventricular reentry tachycardia in one patient with palpitation. Three patients had spontaneous or inducible atrial fibrillation. The ICD implanted in three patients with inducible ventricular fibrillation. Due to economic issue, one patient without ICD implantation had got SCD during follow-up. The patient with atrioventricular reentry tachycardia underwent a successful left atrioventricular accessory pathway ablation.

CONCLUSIONThe Brugada patients with syncope and high rate of inducible ventricular fibrillation in EPS are the high risk population for SCD, in whom ICD should implant promptly to prevent SCD.

Adult ; Brugada Syndrome ; physiopathology ; therapy ; Death, Sudden, Cardiac ; prevention & control ; Defibrillators, Implantable ; Electrophysiology ; Humans ; Male ; Middle Aged ; Ventricular Fibrillation ; therapy

OBJECTIVEBrugada syndrome is linked to sodium channel mutations and could induce arrhythmias that even lead to sudden death. The purpose of this study was to detect if there was gene mutation of SCN5A in 7 patients with Brugada syndrome and explore the molecular genetic characteristics of this disease.

METHODGenomic DNA was extracted from peripheral blood of all 7 patients with Brugada syndrome and 41 pairs of PCR primers were designed to amplify all the 28 exons of SCN5A.

RESULTThere was no novel mutation in exons of Gene SCN5A in these patients with Brugada syndrome.

CONCLUSIONBrugada syndrome might associated gene mutation or other mechanisms independent of SCN5A gene mutation.

Adult ; Brugada Syndrome ; genetics ; DNA Mutational Analysis ; Exons ; Humans ; Male ; Middle Aged ; Muscle Proteins ; genetics ; Mutation ; NAV1.5 Voltage-Gated Sodium Channel ; Sodium Channels ; genetics

OBJECTIVETo evaluate the efficacy of left atrium linear lesion encircling pulmonary veins (PV) guided by EnSite-NavX and double-Lasso technique for paroxysmal atrial fibrillation (PAF).

METHODSTwenty-two patients (male 19, mean age of 48.5 years +/- 11.4 years) with symptomatic PAF were enrolled. After a geometry of the left atrium was reconstructed by EnSite-NavX system, PV ostia were marked on the map based on venography. Two Lasso catheters were placed within the ipsilateral superior and inferior PVs. Irrigated radiofrequency energy was applied at 0.5-1.0 cm of distance from the PV ostia. Continuous linear lesion was done to obtain the disappearance of pulmonary vein potentials. Patients were on propafenone and perindopril for three months after the procedure.

RESULTSThe endpoint for ablation was reached in 21 Patients and 1 patient was not successful because of cardiac tamponade. The mean procedure time was 6.6 h +/- 1.3 h and the mean X-ray exposure time was 56.1 min +/- 18.0 min. After a mean 5.3 months +/- 2.7 months of follow-up, 10 patients were free of symptoms. Two patients had no PAF recurrence after the second procedure. Three patients had clinical recurrence of PAF in the first month. The total success rate in this study was 81% (17/21). Mortality was 0% and the overall complication rate was about 9% (2/22).

CONCLUSIONLeft atrium circumferential linear ablation surrounding PV ostia guided by EnSite-NavX and double-Lasso technique is effective in PAF, but some patients will need more than one procedure in order to achieve a success.

Adult ; Aged ; Atrial Fibrillation ; surgery ; Catheter Ablation ; methods ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Pulmonary Veins ; surgery