This paper introduces the airway care of a patient with motor neuron disease treated with mechanical ventilation for eight years. The airway care for the patient focused on individualized suctioning,measures to prevent atelectasis such as lung hyperinflation,adjustments of ventilator parameters,regular weaning exercise,as well as measures to prevent ventilator-associated pneumonia such as prevention of aspiration,strict disinfection and isolation,wound care of tracheotomy. The ventilator-associated pneumonia was effectively prevented. Effective nursing care for patients with long-term mechanical ventilation can prevent atelectasis and ventilator-associated pneumonia.

Glucocorticoids ; therapeutic use ; Humans ; Muscular Dystrophy, Duchenne ; drug therapy

A total of 88 patients with chronic heart failure were divided into 2 groups: the carvedilol group( n =40) and the control group( n =48). Patients in 2 groups were all treated with routine regime,the experimental group also received carvedilol treatment.Before and 7 months after treatment, their left ventricular ejection fractions (EF%) and the rate of left ventricular drawing back on short axis contration were recorded to evaluate the effects of carvedilol. The values of EF% and short axis were higher in both groups after 7 months follow up ( P

Objective To investigate the effects of Yangxueqingnao Granule on the proliferation and differentiation of endogenous neural stem cells and their neuroprotective mechanism after brain ischemia. Methods Cerebral ischemia was induced in rats by temporary middle cerebral artery occlusion. Ischemic animals received Yangxue Qingnao Granule at high and low doses at days 3,7, 14,and 21 after MCAO. Neurological score and TTC staining were used to assess the neurological deficits and the volume of cerebral infarction, respectively. Immunohistochemistry was used to detect Brdu and Nestin expression in the subventricular zone(SVZ)and hippocampal dentate gyrus subgranular zone(SGZ)region. Results The rats in MCAO control group, low dose group and high dose group showed varying degrees of neurological def-icits and the volume of cerebral infarctions. The neurological defect scores and volume of cerebral infarction were signifi-cantly different at different time points in each group ( P<0.01). 2. The mean fluorescence intensity values of Brdu posi-tive cells or the Nestin positive cells in SVZ and SGZ regions were significantly different ( P< 0.01) at different time points after treatment for 3,7,14 or 21 days. The mean fluorescence intensity values of Brdu positive cells or the Nestin positive cells in SVZ and SGZ regions were significant different among low dose group, high dose group and MCAO con-trol group. The mean fluorescence intensity values of Brdu positive cells or the Nestin positive cells in SVZ and SGZ re-gions were significantly different among low dose group, High dose group and control group. High dose group significantly increased the mean fluorescence intensity values of Brdu positive cells or the Nestin positive cells in SVZ and SGZ re-gions. Conculsions Yangxue Qingnao Granule is able to promote the functional recovery and reduce the volume of cere-bral infarction in ischemic rats. Yangxue Qingnao Granule can enhance the proliferation and differentiation of endoge-nous neural stem cells in ischemic rats.

Objective To study the efficacy and safety magnesium sulfate for the treatment of adult severe tetanus.MethodsTwenty-seven inpatients with adult severe tetanus during April 2005 - October 2010 were retrospectively analysed.ResultsThe total serum magnesium more than 4 mmol/L was found in 2 patients ( 2/27,7.40％ ).The tracheotomy was performed in 25 Patients (25/27,92.6％),22 of which (22/25,88.0％) run mechanical ventilation with the mean mechanical ventilation time (7.5 ± 3.7 ) days,(4 to 16 days).Pulmonary infection occurred in 25 cases (25/27,92.6％).Twenty-three of 27 (23/27,85.2％ ) cases were cured except 4 cases died,and the mean hospital stay was (32.22 ± 18.78) days,(25to 48) days.Conclusions20％ magnesium sulfate is safe for treatment of the patients of adult severe tetanus.It can control not only muscle rigidity and spasms but also autonomic instability efficaciously,and lower the dosage of sedative such as diazepam and chlorpromazine.This method is worth popularizing in clinical practice for its easy operation and low cost.

Objective To construct the eukaryotic expression plasmids of human Fas and TNFR1 gene(pcDNA3.0-hFas and pcDNA3.0-hTNFR1)and microRNA(miRNA)expression plasmid of hFas and hTNFR1 named p-hFasmiRNA and p-hTNFR1miRNA,and to investigate their inhibitory effects in vitro.Methods The eukaryotic expression plasmids of human Fas and TNFR1 gene were constructed(pcDNA3.0-hFas and pcDNA3.0-hTNFR1)and have been shown successfully to express hFas and hTNFR1 protein.miRNA expression plasmids of hFas and hTNFR1 named p-hFasmiRNA and p-hTNFR1miRNA complimentary to the sequence responsible for hFas and hTNFR1 respective were constructed,meanwhile irrelevant miRNA plasmid was used as a control.By respective co-transfection of p-hFasmiRNA and pcDNA3.0-hFas,p-hTNFR1 miRNA and pcDNA3.0-hTNFR1 expression construct into 293T cells,the inhibition of hFas and hTNFR1 expression was analyzed by real-time PCR and Western blot.Results The experiments showed the significant inhibitory effect of p-hFasmiRNA on hFas and p-hTNFR1miRNA on hTNFR1 expression at 48 h post-transfection both at RNA level and protein level as well in 293T cell lines with the inhibitory efficiency being as high as 87% for hFas and 80% for hTNFR1,respectively.Conclusion The p-hFasmiRNA and p-hTNFR1miRNA were constructed successfully,and it was verified that they could specifically inhibit the hFas and hTNFR1 expression at the cellular level.

Previous study on TNFRl-mediated hepatocyte apoptosis has been implicated in the development of fulminant viral hepatitis.To interfere with the potentially effective target,plasmid named p-mTNFR1shRNA complimentary to the sequence responsible for mTNFR1 was also constructed and further confirmed by sequence analysis.To investigate the effect of mTNFR1shRNA plasmid on mTNFR1 expression in vivo and the disease progress in MHV-3 induced fulminant hepatitis mice model.By hydrodynamic injection of mTNFRlshRNA plasmid,the survival rate of mice,hepatic pathological change were examined and compared between mice with/without mTNFR1 shRNA plasmid intervention.The expression of mTNFR1 was detected by Real-time PCR,immunohistochemistry staining.The mTNFR1 shRNA plasmid significantly reduced mTNFR1 expression in vivo,markedly ameliorates inflammatory infiltration,prolonged the survival time period and elevated the survival rate from 0 up to 13.3% in Balb/cJ mice with MHV-3 induced fulminant hepatitis.This study was designed to explore the opportunity of RNA interference technique in inhibiting TNFR1 expression,which has been reported to be involved in the development of a variety of diseases including fulminant viral hepatitis and severe chronic hepatitis B.

Objective To study the NR5A1 gene mutation in patients with idiopathic hypogonadotropic hypogonadism(IHH), and to find the new mutation point.Methods Sixty-one IHH patients and 100 normal control subjects were collected and genomic DNA was extracted from blood samples.These patients were with normal karyotype and no abnormality was discovered in magnetic resonance imaging (MRI) scan of the pituitary.Other endocrine diseases were also excluded.The 2-7 exons and splice-sites of NR5A1 gene were amplified with polymerase chain reaction.DNA of the coding sequence and splice-sites of NR5A 1 were sequenced by double deoxidizing terminal end sequencing method in 61 IHH and 100 normal control subjects.The results of sequencing were compared with their corresponding sequence data.61 IHH kindreds were investigated and the clinical data of these patients were collected.Finally, the phenotype and genotype positive cases were analyzed.Results Six patients carried NR5A1 gene mutational sites in 61 cases of IHH.Analysis of sequencing results from 100 age and ethnicity matched control subjects did not show any of these novel changes.Conclusions One mutation in NR5A1 gene may affect protein structure and function, which should be considered in male IHH patients with normal karyotype and without insufficiency of adrenal function.

Objective To discuss the clinical features and treatment of isovaleric academia (IVA) patients,and to gain more comprehensive understanding of isovaleryl-CoA dehydrogenase(IVD) mutation in 2 siblings in order to raise awareness to prevent the occurrence of IVA.Methods The clinical history and laboratory test of 2 cases of children with IVA were carried out.The exons and neighboring introns of IVD gene of the whole family were PCR-amplified for DNA sequencing.The literature review of IVA in China was also conducted.Results Organic acid analysis of urine by GC/MS for both siblings showed extremely elevated concentrations of isovaleric glycine.For the older sibling,an acute episode of IVA caused severe metabolic stress and eventually death in the neonatal period.However,the disease was well-controlled for the younger sibling due to timely treatment and follow-up care for 2 years.The DNA sequencing of the IVD gene in the family revealed a novel c.1016G ＞ A(C339Y) heterozygous mutation in mother and both of the siblings.No IVD mutation was detected in father or in any of the 50 cases of healthy controls.According to literature review,15 cases of IVA were reported in recent 15 years in China,including neonatal onset (11 cases),acute episode (12 cases),odor of sweaty feet (12 cases),pancytopenia (9 cases),hyperammonemia (5 cases),hypocalcemia (6 cases),and 6 cases of death were reported.Additionally,5 cases that received treatment of BCAA-free formula milk showed positive outcome.However,only 2 cases were followed up for more than 2 years.Conclusions Two new IVA patients carrying c.1016G ＞ A(C339Y) mutation were reported in China.The mutation may lead to conformational change and functional deficient of the IVD protein.It is also necessary to point out that using direct DNA sequencing can not identify all patients with IVA due to limitations of this technology,and thus clinicians should be aware of the possibility of genetic misdiagnosis.Moreover,there is a trend of increasing IVA in China in recent years.