Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is a rare autosomal recessive neuromuscular disease.It is caused by mutations in the gene immunoglobulin μ-binding protein 2 which resides on chromosome 11 q13.3 and encodes the immunoglobulin μ-binding protein 2.This disorder is characterized by degeneration of anterior horn α-motoneurons and manifesting as irreversible diaphragmatic paralysis,respiratory distress associated with progressive symmetrical muscular weakness,distal lower limbs mainly involved,and muscle atrophy between the first 6 weeks and 6 months of life.Overall,SMARD1 is a poor-prognosis disease that artificial ventilation is needed for the whole life.

T was genotyped by polymerase chain reaction-restriction fragment length polymorphism(PCR-RFLP)in all the patients.Results The occurrence of CR in this population was 23.3%(70/300).There was CYP3A4 894C/T polymorphism in the study population.The frequencies of the three kinds of genotypes(CC,CT,TT)in CR group and non-CR(NCR)group were 45.7%,50.0%,4.3% and 63.5%,31.7%,4.8%,respectively.The frequency of TT genotype was significantly higher in NCR group than that in CR group(OR=2.06,95% CI:1.201-3.547,P=0.020).C allele carriers were more likely to develop clopidogrel resistance compared with that of T allele carriers(OR=1.59,95% CI:1.037-2.442,P=0.023).Conclusion CYP3A4 gene 894C/T polymorphism is associated with the risk of CR,and C allele carriers may be a possible genetic susceptibility factor for patients with CR.

Objective To investigate the risk factor profile of pneumonia caused by pandrug-resistant Acinetobacter baumannii (PDRAB)in intensive care unit for better distinction between PDRAB infection and colonization.Methods The clinical data of 99 patients diagnosed as A.baumannii pneumonia between February 2009 and March 2012 in Fangcun Hospital,a branch of Guangdong Provincial Hospital of Traditional Chinese Medicine,were reviewed and analyzed retrospectively.The risk factors associated with pandrug-resistance in A.baumannii were examined with multivariate logistic regression analysis.Results ICU stay,duration of invasive ventilation,longer duration of treatment with carbapenems,beta-lactam/beta-lactamase inhibitor,or quinolone antibiotics,use of ≥3 classes of antimicrobial agents,≥3 invasive procedures (including indwelling gastric canal, catheterization,central venous catheter,arterial sheath),and fiberoptic bronchoscopic treatment were associated with PDRAB pneumonia (P <0.05).Multivariate logistic regression analysis showed that use of ≥3 classes of antibiotics,fiberoptic bron-choscopic treatment,and longer duration of treatment with beta-lactam/beta-lactamase inhibitor were independent risk factors predicting the emergence of PDRAB pneumonia.Conclusions We should pay more attention to these risk factors in clinical prac-tice for better differentiation between PDRAB infection and colonization so that appropriate control measures can be taken pro-actively.

Acute Disease ; Adolescent ; Adult ; Bridged-Ring Compounds ; poisoning ; Cardiomyopathies ; etiology ; therapy ; Creatine Kinase ; blood ; Creatine Kinase, MB Form ; Electrocardiography ; Foodborne Diseases ; blood ; complications ; therapy ; Glutamyl Aminopeptidase ; blood ; Humans ; Isoenzymes ; blood ; Male ; Middle Aged ; Nausea ; etiology ; Seizures ; etiology ; Treatment Outcome

This paper analyzed the moral construction in the prenatal diagnosis of surgical nursing and nursing features, including:strictness and complexity , professional and comprehensive , collaborative, and independence , carry out from the patients with preoperative evaluation , operation and postoperative effect evaluation every link and detail, with the theory of nursing ethics to guide and standardize nursing , dealing with the relation between various ethic, improve patient satisfaction , based on the premise of mutual modern relationship between nurses and pa-tients.

OBJECTIVETo evaluate the outcomes of T3a prostate cancer with unfavorable prognostic factors treated with permanent interstitial brachytherapy combined with external radiotherapy and hormone therapy.

METHODSFrom January 2003 to December 2008, 38 patients classified as T3a prostate cancer with unfavorable prognostic factors were treated with trimodality therapy (brachytherapy + external radiotherapy + hormone therapy). The prescription dose of brachytherapy and external radiotherapy were 110 Gy and 45 Gy, respectively. The duration of hormone therapy was 2-3 years. The endpoints of this study included biochemical failure-free survival (BFFS), distant metastasis-free survival (DMFS), cancer-specific survival (CSS), and overall survival (OS). Survival curves were calculated using the Kaplan-Meier method. The Log-rank test was used to identify the prognostic predictors for univariate analysis.

RESULTSThe median follow-up was 71 months. The serum pre-treatment prostate-specific antigen (PSA) level ranged from 10.0 to 99.8 ng/ml (mean 56.3 ng/ml), the Gleason score ranged from 5 to 9 (median 8), and the percentage of positive biopsy cores ranged from 10% to 100% (mean 65%). The 5-year BFFS, DMFS, CSS, and OS rates were 44%, 69%, 82%, and 76%, respectively. All biochemical failures occurred within 40 months. The percentage of positive biopsy cores was significantly correlated with BFFS, DMFS, and OS (all P=0.000), and the Gleason score with DMFS (P=0.000) and OS (P=0.001).

CONCLUSIONST3a prostate cancer with unfavorable prognostic factors presents not so optimistic outcome. Hormone therapy should be applied to prolong the biochemical progression-free or metastasis-free survival. The percentage of positive biopsy cores and the Gleason score are significant prognostic factors.

Androgen Antagonists ; therapeutic use ; Brachytherapy ; Combined Modality Therapy ; Gonadotropin-Releasing Hormone ; agonists ; Humans ; Male ; Neoplasm Grading ; Prognosis ; Prostatic Neoplasms ; mortality ; pathology ; therapy ; Treatment Outcome

Objective To investigate the relation between Fas-mediated apoptosis and glucocorticoid treatment in myasthenia gravis (MG). Methods In 17 patients with MG, 6 patients received glucocorticoid treatment (glucocorticoid treatment group),and 11 patients were treated without glucocorticoid (nonglucocorticoid treatment group). Meanwhile, 13 healthy cases were selected as healthy control group. CD4,CD8 and Fas expressions in peripheral blood T lymphocyte were detected by flow cytometry in three groups and analyzed. Results The percentage of CD4-CD8+ cells in peripheral blood T lymphocyte in glucocorticoid treatment group was significantly higher than that in healthy control group[(36.75 ± 11.56)% vs. (26.31 ±9.00)%, P = 0.027], while the percentage of CD4-CD8- cells was significantly lower [(30.56 ± 9.72)% vs.(42.96 ± 11.54)%, P =0.018]. The percentage of CD4-CD8+ cells in peripheral blood T lymphocyte in glucocorticoid treatment group was significantly higher than that in non-glucocorticoid treatment group [(36.75 ± 11.56)% vs. (25.24 ±7.63)% ,P =0.019]. The percentages of Fas+ and CD8 +Fas+ cells in peripheral blood T lymphocyte in glucocorticoid treatment group were significantly higher than those in healthy control group[(46.10 ± 7.13)% vs. (31.22 ± 13.00)%, P=0.006; (62.86 ± 12.29)% vs. (45.59 ±11.50)%, P = 0.003]. The percentage of CD8+ Fas+ cells in peripheral blood T lymphocyte in glucocorticoid treatment group was significantly higher than that in non-glucocorticoid treatment group [(62.86 ± 12.29)%vs (50.84 ± 8.31 )%, P = 0.034]. Conclusions Glucocorticoid treatment may have influence on peripheral blood T lymphocyte subsets in patients with MG. Fas-mediated apoptosis may be involved in the mechanism of glucocorticoid treatment in MG.

Objective To assess the prevalence of Personality Diagnostic Questionnaire (PDQ)personality deviations in patients referred for functional dyspepsia (FD) with reliable and universal psychological measures, and to explore the relationship between co-occurring PDQ-personality deviations and functional dyspepsia. Methods The sample comprised 246 patients referred for functional dyspepsia. Four groups were divided according to their patterns of gastrointestinal symptoms: the FD group, FD with refluxlike symptom group(FD + RS group), FD with irritable bowel syndrome group( FD + IBS group), and FD with reflux-like symptom and irritable bowel syndrome group ( FD + RS + IBS group). Participants were assessed with the Personality Diagnostic Questionnaire for DSM-Ⅳ ( PDQ-4 ) to evaluate the presence of personality deviations. Results Overall 65% patients scored positive for any personality deviation, male and female alike. Cluster C (anxious/fearful) personality was most commonly found in FD patients (142 patients, 57.7% ). The FD + IBS group and the FD + RS +IBS group had significantly higher total PDQ scores than the FD group (23.39 ± 8. 77 and 24. 22 ± 10. 97 vs 18.98 ± 11.88, P ＜ 0. 05, respectively),indicating that FD patients with greater level of personality deviations tend to report other symptoms involving the esophagus and lower gastrointestinal tract. Reflux-like symptom without actual pathological acid regurgitation indicated cluster A (odd/eccentric) personality deviations. Conclusions The current study shows personality deviations are common in patients referred for functional dyspepsia. Negative emotions,maladaptive coping, and lack of social support, may strongly influence their healthcare-seeking behavior.There is no single personality type specific for some kind of gastrointestinal symptom. But FD patients with personality deviations tend to report other symptoms involving the esophagus and lower gastrointestinal tract.

Objective To observe effects of problem-based learning(PBL) combined with case-based study (CBS)teaching mode in clinical teaching in intensive care unit(ICU). Methods Students from ICU of Fangcun Branch of Guangdong Provincial Hospital of Traditional Chinese Medicine from March 2011 to February 2012 were divided into treatment group (31 cases, with PBL combined with CBS teaching mode) and control group (34 cases, with the traditional teaching mode). Scores of two groups were compared and analyzed and questionnaire survey was conducted in treatment group. Data were processed by SPSS 13.0 statistical software. Measurement data were compared by t-test and data of heterogeneous variance were compared by rank-sum test. Results Treatment group achieved better scores ((85.26 ±5.96) in theory examination and (80.59 ±7.33) in case-analyzing examination) compared with those of control group ((79.17 ±7.31) in theory examination and (76.02 ±9.27) in case-analyzing examination)(P<0.05). PBL combined with CBS teaching mode can stimulate learning interests but it was lack of systematization; most students did not volunteer to speak. Conclusion PBL combined with CBS teaching mode has certain advantages in ICU clinical teaching.

Objective To investigate effect of famitinib malate on adverse reactions by radiotherapy and chemotherapy in treatment of advanced nasopharyngeal carcinoma and the nursing strategies.Methods From November 2011 to December 2013, 20 cases of advanced nasopharyngeal carcinoma in our hospital were treated with famitinib malate combined with radiotherapy and chemotherapy.During the treatment,we observed the adverse reactions and gave the symptomatic treatment.Results The rates of adverse reactions such as hypertension,renal toxicity,oral mucositis,myelosuppression,gastrointestinal reactions,nasal bleeding, abnormal liver function,and hand-foot skin reaction were 60.0%,70.0%,90.0%,100.0%,85.0%,10.0%,45.0%and 25.0%respectively.After symptomatic treatment,all completed the treatment.Conclusions The incidence rate of adverse reactions of famitinib malate combined with radiotherapy and chemotherapy for treating advanced nasopharyngeal carcinoma is high.Close monitoring and observation during treatment courses can ensure the successful completion of treatment.