Objective To investigate the clinical features and the clinical effects of extracorporeal membrane oxygenation(ECMO) in children with fulminant myocarditis. Methods The data of one hundred and sixty one pediatric patients with fulminant myocarditis at Beijing Children′s Hospital from March 1,2007 to May 31,2018 were analyzed retrospectively,including clinical manifestations,electrocardiogram,echocar￣diography,myocardial enzyme,the application of ECMO,curative effect and prognosis. Results Among 161 cases of children with fulminant myocarditis,74 cases (46. 0%) had respiratory tract symptoms,54 cases (33. 5%) had digestive tract symptoms,24 cases (14. 9%) had nervous system symptoms,only 33 cases (20. 5%) had cardiovascular symptoms. One hundred and thirty￣two cases(82. 0%) had elevation of crea￣tine kinase isoenzymes( CK￣MB) and/or cardiac troponin I( cTnI),and 107 cases(66. 5%) had abnormal echocardiography. One hundred and fifty￣two cases ( 94. 4%) showed abnormal electrocardiogram, and the changes of ST￣T and atrioventricular block were most common. Five cases survived in 8 children treated with ECMO. They underwent ECMO with an median supporting time of 101. 5 (52. 75,142. 8) hours through a venous￣arterial ECMO model. After the application of ECMO,blood pressure,the heart rate and oxygen satura￣tion were obviously improved in the 8 children,and the level of blood lactic acid was statistically reduced com￣pared with that before the application of ECMO(P<0. 001). During the follow￣up period of 1 months,the 5 surviving children recovered with normal cardiac function,without abnormal function of other organs. There were a total of 17 cases(10. 6%) died,4 children(8. 9%) in 45 patients died since the application of ECMO and the other 13 children(11. 6%) in 116 patients died before that time. This proportion of death was reduced after the application of ECMO,whereas the difference was not statistically significant(P>0. 05). Conclusion Our study indicates the diverse symptoms in children with fulminant myocarditis. Comprehensive application of electrocardiogram,echocardiography,and myocardial enzymology markers may be of benefit to the timely diag￣nosis of fulminant myocarditis. The application of ECMO in fulminant myocarditis remains an effective approach in reduction of mortality rate,and can effectively support recovery of cardiopulmonary functions.

Objective To report the prenatal molecular diagnosis for two gravida in a family with spondylepiphyseal dysplasia congenita(SEDC)caused by G504S mutation of COL2A1 gene.Methods DNA of the two fetuses was extracted from amniotic fluid at the 19+3 and 18+6 weeks of gestation respectively.Direct sequencing of two samples were performed after amplifying exon 23 of COL2A1 containing the potential mutation.The femur length and biparietal diameter of the first fetus were measured by sonographic scans every two weeks from 17+3 weeks to 27+3 weeks of gestation,and for the second fetus these parameters were measured from 16+1 to 19+1 weeks of gestation.Results Sequncing analysis revealed the first fetus and his mother presented the same mutation which is specifically associated with SEDC,but the second fetus did not show the mutation of COL2A1 gene.Biparietal diameters of the both fetuses were appropriate for gestational age.Femur length of the second fetus was normal for gestational age but that of the first fetus was shortened evidently after the 23 week of gestation.The parents of the first fetus determined to terminate the pregnancy.A medical termination was carried out at 27+5 weeks of gestation and a male fetus with a relatively large head and short limbs was delivered.The radiological findings of the fetus were consistent with SEDC including generalized platy spondesand shortened long bones.Conclusions Prenatal molecular diagnosis is important for the fetus with risk of SEDC and useful for genetic counseling.Genotype of fetus with risk of SEDC can be identified before sonographic scan.Molecular genetic analysis in conjunction with sonographic monitoring was helpful in prenatal diagnosis of SEDC.

OBJECTIVETo observe enhanced effects of polypeptide extract from scorpion venom (PESV) combined Rapamycin on autophagy of H22 hepatoma cells in mice and to explore its possible mechanism.

METHODSThe H22 hepatocarcinoma cell suspension was subcutaneously inoculated into 40 Kunming mice. Then tumor-bearing mice were randomly divided into four groups, i.e., the control group,the high dose PESV group, the low dose PESV group, and the combination group (high dose PESV + Rapamycin), 10 in each group. Mice in high and dose PESV groups were administered with 20 mg/kg and 10 mg/kg PESV respectively by gastrogavage. Mice in the combination group were administered with 2 mg/kg rapamycin and 20 mg/kg PESV by gastrogavage. The intervention lasted for 14 successive days. The tumor volume was measured once every other day, the tumor growth curve was drawn, and then the tumor inhibitory rate calculated. Pathological changes of the tumor tissue were observed by HE staining. Protein expression levels of mammal target of rapamycin (mTOR), UNC-51-like kinase-1 (ULK1), microtubule-associated protein1 light chain3 (MAPILC3A), and Beclin1 were detected by immunohistochemical assay.

RESULTSThe growth of H22 hepatoma transplantation tumor was inhibited in high and low dose PESV groups and the combination group (P < 0.05). And there was statistical difference in tumor weight and tumor volume between the combination group and high and low dose PESV groups (P < 0.05). There was no statistical difference in tumor weight or tumor volume between the high dose PESV group and the low dose PESV group (P > 0.05). lmmunohistochemical assay showed that the protein expression of mTOR was higher, but protein expressions of ULK1, MAP1LC3A, Beclin1 were lower in the control group than in the rest 3 groups (P < 0.05, P < 0.01). Compared with the high dose PESV group, protein expressions of ULK1, MAP1LC3A, and Beclin1 were obviously lower (P < 0.05).

CONCLUSIONPESV combined Rapamycin might inhibit the development of H22 hepatoma transplantation tumor in mice possibly through inhibiting the activity of mTOR, enhancing expressions of ULK1, MAP1LC3A, and Beclin1.

Animals ; Antineoplastic Combined Chemotherapy Protocols ; pharmacology ; therapeutic use ; Autophagy ; drug effects ; Carcinoma, Hepatocellular ; Cell Line, Tumor ; Liver Neoplasms ; Mice ; Neoplasm Transplantation ; Peptides ; Scorpion Venoms ; pharmacology ; therapeutic use ; Sirolimus ; pharmacology ; therapeutic use

OBJECTIVETo give some theory support of Cistanche tubulosa cultivation by searching dry matter accumulation and echinacoside content of C. tubulosa.

METHODDry matter accumulation content of C. tubulosa culturing in Huabei plain was analysed in different growth season of C. tubulosa. Echinacoside content was determined by HPLC.

RESULTDry matter accumulation of C. tubulosa showed "S" variation. Dry matter accumulation increased fastest in September among growing seasons. Dry matter amount was 138.58 g after C. tubulosa grew a year. Dry matter amount decreased significantly along with inoculation time retarded. Echinacoside content was 30.59% when C. tubulosa grew in 5 months, decreased guadully after that, and 9.76% in annual.

CONCLUSIONVariation rule of dry matter accumulation and echinacoside content was found in C. tubulosa that grew one year in Huabei plain.

Biomass ; China ; Cistanche ; chemistry ; growth & development ; Glycosides ; metabolism ; Plants, Medicinal ; chemistry ; growth & development ; Seasons

The aim of this study was to investigate the expression of wt1 gene and the changes of gene expression in minimal residual disease (MRD) models (K562, HL-60 cell lines) and acute leukemia (AL) patients through inhibiting the expression of wt1 gene by antisense oligonucleotides (ASO). The bone marrow (BM) of 56 AL patients with complete remission (CR) was collected, then the BM samples with positive expression of wt1 gene were screened by RT-PCR. The cells of MRD model and screened wt1 gene positive samples were cultured and treated by ASO, then the changes of wt1 gene expression were detected. The results indicated that the sensitivity of wt1 gene was 10(-3)-10(-4), and the positive rate of BM wt1 gene expression in 56 AL patients with CR was 16%. After BM of 9 AL CR patients with MRD and MRD model (K562, HL-60 cells) expressing wt1 gene were treated by ASO, it was found that the wt1 expression in ASO group was blocked, while wt1 gene could be still detected in both sense oligonucleotides (SO) and control groups. It is concluded that ASO can obstruct the expression of wt1 gene on the residual leukemia cells in vitro.
Gene Expression ; HL-60 Cells ; Humans ; K562 Cells ; Neoplasm, Residual ; genetics ; Oligonucleotides, Antisense ; genetics ; WT1 Proteins ; genetics

OBJECTIVETo investigate the effects of pethidine on electrophysiological properties of the isolated ventricular myocytes and the underlying mechanism.

METHODSLangendorff was applied to perfuse rat heart model and whole-cell current clamp and voltage clamp techniques were used.

RESULTSPethidine decreased heart rate (HR) in a concentration dependent manner and caused severe atrioventricular block (AVB) at >or=250 micromol/L. Pethidine reduced action potential amplitude and maximal rate of depolarization, prolonged action potential duration. Pethidine at 100 micromol/L decreased sodium currents (I(Na)), transient outward potassium currents (I(to)), delayed rectifier potassium currents (I(k)) and L-type calcium currents (I(Ca.L)) to (60.7+/-6.9)%, (55.4+/-5.6)%, (65.1+/-8.0)% and (67.4+/-10.1)% of control levels,respectively. These effects could be recovered by washout. Naloxone, an opioid receptor antagonist, could not abolish the effects of pethidine on ionic currents.

CONCLUSIONPethidine decreased HR and induced AVB, which may be related to the inhibition of I(Na), I(to), I(k) and I(Ca-L) of heart. The depression of cardiac currents is not mediated by opioid receptor.

Action Potentials ; drug effects ; Animals ; Heart ; drug effects ; physiology ; Heart Block ; chemically induced ; Heart Rate ; drug effects ; In Vitro Techniques ; Ion Channels ; antagonists & inhibitors ; Male ; Meperidine ; pharmacology ; Rats ; Rats, Sprague-Dawley ; Receptors, Opioid ; physiology

OBJECTIVETo analyze the inoculation ratio and echinacoside content of Cistanche tubulosa and provide theoretical basis for Tamarix introduction, resource protection and screening of C. tubulosa.

METHOD8 Tamarix species were introduced in the North China Plain and inoculation of C. tubulosa was conducted on all species. Phenylethanoid glycosides fingerprinting and echinacoside content of C. tubulosa were analyzed by using HPLC.

RESULTThe adaptability of 8 Tamarix species were significantly different, phenylethanoid glycosides component of C. tubulosa on T. gansuensis and T. austromongolica were basically identical in contrast to T. chinensis, echinacoside content showed no obvious difference in C. tubulosa plant growing 4 months.

CONCLUSIONT. gansuensis and T. Austromongolica are suitable for the host introduction plant of C. tubulosa resource protection and screening in North China Plain.

China ; Cistanche ; chemistry ; growth & development ; Conservation of Natural Resources ; Ecosystem ; Glycosides ; analysis ; Phenols ; analysis ; Plants, Medicinal ; chemistry ; growth & development ; Rain ; Soil ; Tamaricaceae ; classification ; growth & development

Interleukin-2 (IL-2) therapy often results in potentially life-threatening side effects including hypotension. However, the mechanism has not been completely elucidated. In order to determine whether IL-2 modifies vascular tone, we investigated the effect of IL-2 on rat thoracic aorta rings and the underlying mechanisms. Effects of IL-2 on the contraction of high KCl and phenylephrine (PE) preconstricted rat thoracic aorta with or without endothelium were determined by organ bath technique. To explore the mechanism, nitric oxide synthase inhibitor L-N(G)-nitroarginine methyl ester (L-NAME), guanylyl cyclase inhibitor methylene blue, and cyclooxygenase inhibitor indomethacin were used. IL-2 (10-1000 U/ml) caused concentration-dependent relaxation of aorta rings preconstricted with PE (10 micromol/L) in endothelium-intact rings, but had no effect on KCl (120 mmol/L) preconstricted rings. Removal of the endothelium, or pretreatment with L-NAME (0.1 mmol/L) or methylene blue (10 micromol/L) or indomethacin (10 micromol/L), inhibited the relaxation of IL-2. The results indicate that the relaxation by IL-2 in rat aorta ring is endothelium-dependent and is possibly mediated by the NO-guanylyl cyclase pathway and cyclooxygenase-dependent pathway.
Animals ; Aorta, Thoracic ; drug effects ; physiology ; Endothelium, Vascular ; drug effects ; Endothelium-Dependent Relaxing Factors ; pharmacology ; Guanylate Cyclase ; metabolism ; In Vitro Techniques ; Interleukin-2 ; pharmacology ; Male ; NG-Nitroarginine Methyl Ester ; pharmacology ; Nitric Oxide ; metabolism ; Prostaglandin-Endoperoxide Synthases ; metabolism ; Rats ; Rats, Sprague-Dawley ; Signal Transduction ; drug effects ; Vasodilation ; drug effects ; Vasodilator Agents ; pharmacology

OBJECTIVETo study the pathological and clinical characteristics of a patient with spontaneous platelet aggregation of his giant and morphologically abnormal platelets.

METHODSPlatelet size and structure were observed under light microscope and electron microscope. Platelet aggregation was measured turbidometrically. Platelet glycoproteins (GP) were analyzed using flow cytometry. PCR and DNA sequencing were performed to identify the gene abnormality.

RESULTSThe patient had spontaneous platelet aggregation of giant platelets with thickened plasma membrane and increased number of granules in various shapes. Aspirin and ticlopidine did not affect the spontaneous aggregation. The expression of GP I b, GP II b, GP III a and P-selectin in the platelet membrane were in normal range. Results of gene analyses for GP I balpha, GP I bbeta and GPIX were also normal.

CONCLUSIONBoth morphological and functional abnormalities of the platelets from the patient were clearly distinguishable from that of other hereditary giant platelet disorders. It would probably represent a novel platelet disorder which had not been reported to date.

Aspirin ; pharmacology ; Bernard-Soulier Syndrome ; metabolism ; pathology ; Blood Platelet Disorders ; metabolism ; pathology ; Cell Size ; physiology ; Child ; Cytoplasmic Granules ; pathology ; ultrastructure ; Female ; Humans ; Platelet Aggregation ; drug effects ; physiology ; Platelet Aggregation Inhibitors ; pharmacology ; Platelet Membrane Glycoproteins ; genetics ; metabolism ; Ticlopidine ; pharmacology

OBJECTIVETo study the role and effect of Schwann cells (SCs) remyelination in contused spinal cord.

METHODSGreen fluorescence protein expressing-SCs were transplanted into the epicenter, rostral and caudal tissues of the injury site at 1 week after the spinal cords were contused. At 6 weeks, the spinal cords were removed for cryosections, semithin sections and ultrathin sections, and then immunocytochemical staining of myelin basic protein (MBP), P0 protein (P0) and S100 protein (S100) was carried out on the cryosections. Qualitative and semiquantitative analyses were performed on the cryosections and semithin sections. Ultrastructure of myelinated fibers was observed on the ultrathin sections under electron microscope.

RESULTSTransplanted SCs and myelinated fibers immunocytochemically labeled by MBP, P0 as well as S100 distributed in whole injured area. The quantity of myelinated fibers labeled by the three myelin proteins showed no statistical difference, however, which was significantly larger than that of controls. On the semithin sections, the experimental group demonstrated more myelinated fibers in the injured area than the controls, but the fibers had smaller diameter and thinner myelin sheath under electron microscope.

CONCLUSIONSCs can promote regeneration of injured nerve fibers and enhance remyelination, which may be histological basis of SCs-mediated functional repair of injured spinal cords.

Animals ; Immunohistochemistry ; Microscopy, Electron ; Myelin Basic Protein ; metabolism ; Myelin P0 Protein ; metabolism ; Nerve Regeneration ; physiology ; Rats ; Rats, Sprague-Dawley ; S100 Proteins ; metabolism ; Schwann Cells ; physiology ; ultrastructure ; Spinal Cord Injuries ; metabolism ; physiopathology