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51.
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Central venous catheter (CVC) placement is a common bedside procedure that is frequently performed in critically ill patients. More than 5 million CVCs are inserted annually across the United States alone. Procedure-related complications may vary from minor bleeding to life-threatening complications such as arterial perforation and pneumothorax. Cardiac arrhythmias can often occur during the guidewire placement, and bundle-branch blocks have also been reported. We herewith report a case of complete heart block during CVC placement, which is a rare but serious complication of this procedure. Because the patient had a preexisting left bundle-branch block, complete heart block likely resulted from a probable trauma to the right bundle branch during the guidewire insertion.  相似文献   
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Aicardi–Goutières syndrome (AGS) is an encephalopathy of early childhood which is most commonly inherited as an autosomal recessive trait. The disorder demonstrates significant genetic heterogeneity with causative mutations in five genes identified to date. Although most patients with AGS experience a severe neonatal or infantile presentation, poor neurodevelopmental outcome and reduced survival, clinical variability in the onset and severity of the condition is being increasingly recognized. A later presentation with a more variable effect on development, morbidity and mortality has been particularly observed in association with mutations in SAMHD1 and RNASEH2B. In contrast, the recurrent c.205C > T (p.R69W) RNASEH2C Asian founder mutation has previously only been identified in children with a severe AGS phenotype. Here, to our knowledge, we present the first report of marked phenotypic variability in siblings both harboring this founder mutation in the homozygous state. In this family, one female child had a severe AGS phenotype with an onset in infancy and profound developmental delay, whilst an older sister was of completely normal intellect with a normal head circumference and was only diagnosed because of the presence of chilblains and a mild hemiplegia. An appreciation of intrafamilial phenotypic expression is important in the counseling of families considering prenatal diagnosis, and may also be relevant to the assessment of efficacy in future clinical trials. In addition, marked phenotypic variation raises the possibility that more mildly affected patients are not currently identified. © 2013 Wiley Periodicals, Inc.  相似文献   
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Background

Elective temporary clipping (ETC) is increasingly used in surgery for aneurysms. This study was to assess whether the impact of ETC on intraoperative aneurysmal rupture (IAR) translates into neurological outcome.

Methods

Patients who underwent surgery for ruptured anterior circulation aneurysms were prospectively studied for various factors related to ETC, IAR and neurological outcome at 3 months. Univariate and multivariate analyses were performed using SPSS20.

Results

Of the total 273 ruptured aneurysm surgeries studied, IAR was observed in only six out of 132 aneurysms (4.5 %) who had ETC, compared with 78 out of 141 (55.3 %) without ETC (p?<?0.001). Aneurysms complicated by IAR had significantly longer clipping time (8.3 min) compared with those without IAR (1.9 min) (p?<?0.001). IAR had significant association with unfavorable outcome (38 % vs. 24 %) (p?=?0.02). Patients with ETC had significantly shorter clipping time (2.9 min) compared with those without ETC (4.8 min) (p?=?0.02). Unfavorable outcome was noted in 30 out of 132 with ETC (23 %), compared with 48 out of 141 without ETC (34 %) (p?=?0.04). This beneficial effect was nonsignificantly greater in younger and good clinical grade patients. While episodes of ETC within clipping time of 20 min did not show significant difference in outcome, repeated rescue clipping (45 % unfavorable outcome, p?=?0.048) and total clipping time of at least 20 min (75 % unfavorable outcome, p?=?0.008) had significant impact on outcome. In multivariate analysis, the use of ETC (p?=?0.027) and total temporary clipping less than 20 min (p?=?0.049) were noted to result in significantly better outcome, independent of other factors.

Conclusions

The use of ETC decreased the occurrence of IAR and the total clipping time, thereby leading to significantly better outcome, independent of other factors. While repeated elective clipping within total clipping time of 20 min did not influence outcome, repeated rescue clipping and total clipping time of at least 20 min had significant impact on outcome.  相似文献   
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An innovative simultaneous triple point-of-care (STPOC) screening strategy for syphilis, hepatitis B and HIV with Determine(?) tests was offered to pregnant women presenting for antenatal care and evaluated for feasibility and preference in rural India. Of 1066 participants approached, 1046 consented, of which 1002 (96.0%) completed the strategy. Only 9% reported any history of testing in their current pregnancy. With STPOC screening, 989 women (98.7%) tested negative and 13 had preliminary positive results for infection. The total time taken was 45 minutes per participant. Mothers and infants were provided prophylaxis/treatment for HIV, syphilis and hepatitis B, with interventions initiated within 3-5 days. STPOC was preferred by 99.3% (95%CI: 98.8-99.8%) of participants, facilitated early simultaneous screening for the three infections, timely initiation of prophylaxis/treatment and was feasible in this rural setting. These data suggest that multiplexed STPOC screening for syphilis, hepatitis B and HIV in pregnancy would be desirable for women in rural India.  相似文献   
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Background: Childhood maltreatment has been linked to adolescent substance use in cross-sectional studies but the studies were unable to test the associations between childhood maltreatment and changes in substance use patterns during adolescence. The present study investigated the linkages between exposure to childhood maltreatment and developmental trends of alcohol, cannabis, cocaine, opioid, and hallucinogen use among high-risk adolescents. Methods: We used a sample of 937 adolescents (mean age: 15.9 years; range: 13-18), who were selected from five publicly-funded service systems, to examine the extent to which childhood maltreatment may influence changes in patterns of adolescent substance use over time. Results: The present study identified a 3-class model of adolescent substance use. Mover-stayer latent transition analyses (LTA) indicated that progression toward heavy polysubstance use increased with experience of childhood maltreatment. Findings also suggested that older male adolescents (ages 15-18) who are involved with public service systems are at high risk for developing and maintaining multiple-substance use in adolescence. Conclusions: Experience of childhood maltreatment is associated with problematic patterns of adolescent substance use and may shape the longitudinal course of substance use during adolescence. (Am J Addict 2012;21:453-461).  相似文献   
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Background

Health Related Quality of Life (HRQOL) instruments need disease and country specific validation. In Arab countries, there is no specific validated questionnaire for assessment of HRQOL in chronic kidney disease (CKD) patients. The aim of this study was to present an Arabic translation, adaptation, and the subsequent validation of the kidney disease quality of life-short form (KDQOL-SFTM) version 1.3 questionnaire in a representative series of Egyptian CKD patients.

Methods

KDQOL-SFTM version 1.3 was translated into Arabic by two independent translators, and then subsequently translated back into English. After translation disparities were reconciled, the final Arabic questionnaire was tested by interviewing 100 pre-dialysis CKD (stage 1-4) patients randomly selected from outpatients attending the Nephrology clinic at the Main Alexandria University Hospital. Test re-test reliability was performed, with a subsample of 50 consecutive CKD patients, by two interviews 7 days apart and internal consistency estimated by Cronbach’s α. Discriminant, concept, and construct validity were assessed.

Results

All items of SF-36 met the criterion for internal consistency and were reproducible. Of the 10 kidney disease targeted scales, only three had Cronbach’s α <0.7: quality of social interaction (0.23), work status (0.28), and cognitive function (0.60). All disease specific scales were reproducible. Results from discriminant validity showed that the study questionnaire could discriminate between patients’ subgroups. As for concept validity, the correlation between all domains of the questionnaire with overall health ratewas significant for all domains except for the work status, sexual function, emotional wellbeing, and role emotional. Furthermore, the correlation between the disease specific domains and the two composite summaries of SF-36 (physical and mental composite summaries) was significant for all domains except for sexual function with mental composite summary. Construct validity was indicated by the observation that the majority of the domains of the kidney disease targeted scale of KDQOL-SFTM 1.3 were significantly inter-correlated. Finally, principal component analysis of the kidney disease targeted scale indicated that this part of the questionnaire could be summarized into 10 factors that together explained 70.9% of the variance.

Conclusion

The results suggest that this Arabic version of the KDQOL-SFTM 1.3 questionnaire is a valid and reliable tool for use in Egyptian patients with CKD.
  相似文献   
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