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Two case reports are presented, both clearly demonstrating the diagnosis of oral-facial-digital syndrome, type I, but widely different in the expression of the condition. The first patient showed only mild expression of the syndrome. On examination at the age of 4 years there were no obvious extra oral signs, intraoral findings included the presence of supernumeraries in the primary dentition, spacing in two areas and the presence of an extra frenum. The second can be considered as a more severe case. This patient had many of the typical manifestations, including frontal bossing, a degree of zygomatic hypoplasia and clinodactyly. Orally, the most striking finding was a bilateral cleft palate which had not been diagnosed prior to examination at the age of 6 years. Other findings included multiple frena and a bifid tongue.  相似文献   
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Purpose

Unlike in the outpatient setting, delivery of aerosols to critically ill patients may be considered complex, particularly in ventilated patients, and benefits remain to be proven. Many factors influence aerosol delivery and recommendations exist, but little is known about knowledge translation into clinical practice.

Methods

Two-week cross-sectional study to assess the prevalence of aerosol therapy in 81 intensive and intermediate care units in 22 countries. All aerosols delivered to patients breathing spontaneously, ventilated invasively or noninvasively (NIV) were recorded, and drugs, devices, ventilator settings, circuit set-up, humidification and side effects were noted.

Results

A total of 9714 aerosols were administered to 678 of the 2808 admitted patients (24 %, CI95 22–26 %), whereas only 271 patients (10 %) were taking inhaled medication before admission. There were large variations among centers, from 0 to 57 %. Among intubated patients 22 % (n = 262) received aerosols, and 50 % (n = 149) of patients undergoing NIV, predominantly (75 %) inbetween NIV sessions. Bronchodilators (n = 7960) and corticosteroids (n = 1233) were the most frequently delivered drugs (88 % overall), predominantly but not exclusively (49 %) administered to patients with chronic airway disease. An anti-infectious drug was aerosolized 509 times (5 % of all aerosols) for nosocomial infections. Jet-nebulizers were the most frequently used device (56 %), followed by metered dose inhalers (23 %). Only 106 (<1 %) mild side effects were observed, despite frequent suboptimal set-ups such as an external gas supply of jet nebulizers for intubated patients.

Conclusions

Aerosol therapy concerns every fourth critically ill patient and one-fifth of ventilated patients.
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OBJECTIVE: Functional electrical stimulation (FES) improves exercise capacity and endothelial function in chronic heart failure (CHF) patients. This study evaluates the impact of FES on quality of life and emotional stress in patients with moderate to severe CHF. METHODS: Thirty patients with stable CHF (24 men; NYHA class II-III; left ventricular ejection fraction <35%) were randomly assigned (2:1) to a 6-week FES training program (n=20) or placebo (n=10). Questionnaires addressing quality of life [Kansas City Cardiomyopathy Questionnaire (KCCQ), functional and overall], and emotional stress [Zung self-rating depression scale (SDS), Beck Depression Inventory (BDI)], as well as plasma B-type natriuretic peptide (BNP) and 6-min walking distance test (6MWT) were assessed at baseline and after completion of training protocol. RESULTS: A significant improvement in KCCQ functional (F=76.666, p<0.001), KCCQ overall (F =41.508, p<0.001), BDI (F =17.768, p<0.001) and Zung SDS (F =27.098, p<0.001) was observed in the FES group compared to placebo. Patients in the FES group had also a significant increase in 6MWT (F =19.413, p<0.001) and a trend towards reduction in plasma BNP (F =4.252, p=0.053) compared to placebo. CONCLUSION: FES seems to have a beneficial effect on quality of life, exercise capacity and emotional stress in patients with moderate to severe CHF.  相似文献   
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Background : Transradial coronary catheterization has emerged over the last years as a favorable catheterization practice, based on evidence that it is associated with less vascular complications and shorter hospital stays. However, access site crossover appears to be more frequent when the initial route is the transradial one, one of the main reasons being arterial spasm. We hypothesized that radial flow‐mediated dilation (FMD) measurements could be used as a preprocedural method to assess the likelihood of arterial spasm. Methods : The study population consisted of patients scheduled for transradial diagnostic catheterization in whom ad hoc percutaneous coronary intervention (PCI) was performed. FMD was measured 1–2 days before PCI. The primary endpoint of the study was operator‐defined (operators were blinded as to the FMD results) radial artery spasm. Results : A total of 172 patients (110 male, age 65.3 ± 9) were included. Radial artery spasm was recorded in 13 patients (7.6%). FMD showed a very significant univariate association with the occurrence of spasm (P < 0.001) and was the most important predictor of spasm in the multivariate logistic regression analysis (beta ?3.15; P < 0.001), followed by baseline radial artery diameter (P = 0.04), the number of catheters used (P = 0.049) and the administered volume of contrast medium (P = 0.017). Conclusion : Preprocedural FMD is a significant predictor of arterial spasm before elective transradial PCI. It is a low cost, safe, and feasible noninvasive modality, whose results might be taken into account when deciding on the vascular access route for an elective procedure, the size of sheaths or catheters to be used or the intensity of antispasm medication.© 2010 Wiley‐Liss, Inc.  相似文献   
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Background  

Cardiac surgery is a major consumer of blood products, and hemodilution increases transfusion requirements during cardiac surgery under CPB. As intraoperative parenteral fluids contribute to hemodilution, we evaluated the hypothesis that intraoperative fluid restriction reduces packed red-cell (PRC) use, especially in transfusion-prone adults undergoing elective cardiac surgery.  相似文献   
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Purpose

The purpose of this randomized controlled study is to compare and evaluate the intraoperative and post-operative outcome of PLPS nerve block and that of femoral, obturator and sciatic (FOS) nerve block as a method of anaesthesia, in performing ACL reconstruction.

Methods

Patients referred for elective arthroscopic ACL reconstruction using hamstring autograft were divided in two groups. The first group received combined femoral–obturator–sciatic nerve block (FOS Group) under dual guidance, whereas the second group received posterior lumbar plexus block under neurostimulation and sciatic nerve block (PLPS Group) under dual guidance.

Results

The two groups were comparable in terms of age, sex, BMI and athletic activity. The time needed to perform the nerve blocks was significantly shorter for the FOS group (p < 0.005). Similarly, VAS scores during tourniquet inflation and autograft harvesting were significantly higher (p < 0.005) in the PLPS group and this is also reflected in the intraoperative fentanyl consumption and conversion to general anaesthesia. Finally, patients in this group also reported higher post-operative VAS scores and consumed more morphine.

Conclusions

Peripheral nerve blockade of FOS nerve block under dual guidance for arthroscopic ACL reconstructive surgery is a safe and tempting anaesthetic choice. The success rate of this technique is higher in comparison with PLPS and results in less peri- and post-operative pain with less opioid consumption. This study provides support for the use of peripheral nerve blocks as an exclusive method for ACL reconstructive surgery in an ambulatory setting with almost no complications.

Level of evidence

I.
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