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1.
Retention of restorations cemented with temporary cement varies. Some cements are adhesive and others are weak in retention. In addition, cement retention may vary over time. This study determined (1) the retentive properties of four temporary cements, and (2) the effects of aging on temporary cement retention. Cylindrical amalgam cores and mated stainless steel retainers with a 0.05 mm cement space were used in the study. Cores were cemented into the retainers and stored in 100% humidity at 37 degrees C until tested. Retention was measured by applying a compressive force to the cores through a rod in an Instron machine. Half the samples were tested after 1 week and half were tested after 6 weeks. The results indicate a significant difference in retentive value among the four cements, including a significant decrease in retention for one cement over the 6-week aging period.  相似文献   
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BACKGROUND—Hereditary spastic paraparesis is a genetically heterogeneous condition. Recently, mutations in the spastin gene were reported in families linked to the common SPG4 locus on chromosome 2p21-22.
OBJECTIVES—To study a population of patients with hereditary spastic paraparesis for mutations in the spastin gene (SPG4) on chromosome 2p21-22.
METHODS—DNA from 32 patients (12 from families known to be linked to SPG4) was analysed for mutations in the spastin gene by single strand conformational polymorphism analysis and sequencing. All patients were also examined clinically.
RESULTS—Thirteen SPG4 mutations were identified, 11 of which are novel. These mutations include missense, nonsense, frameshift, and splice site mutations, the majority of which affect the AAA cassette. We also describe a nucleotide substitution outside this conserved region which appears to behave as a recessive mutation.
CONCLUSIONS—Recurrent mutations in the spastin gene are uncommon. This reduces the ease of mutation detection as a part of the diagnostic work up of patients with hereditary spastic paraparesis. Our findings have important implications for the presumed function of spastin and schemes for mutation detection in HSP patients.


Keywords: spastin; hereditary spastic paraparesis; mutation; recessive  相似文献   
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Available data on clinical presentation and mortality of coronavirus disease-2019 (COVID-19) in heart transplant (HT) recipients remain limited. We report a case series of laboratory-confirmed COVID-19 in 39 HT recipients from 3 French heart transplant centres (mean age 54.4 ± 14.8 years; 66.7% males). Hospital admission was required for 35 (89.7%) cases including 14/39 (35.9%) cases being admitted in intensive care unit. Immunosuppressive medications were reduced or discontinued in 74.4% of the patients. After a median follow-up of 54 (19–80) days, death and death or need for mechanical ventilation occurred in 25.6% and 33.3% of patients, respectively. Elevated C-reactive protein and lung involvement ≥50% on chest computed tomography (CT) at admission were associated with an increased risk of death or need for mechanical ventilation. Mortality rate from March to June in the entire 3-centre HT recipient cohort was 56% higher in 2020 compared to the time-matched 2019 cohort (2% vs. 1.28%, P = 0.15). In a meta-analysis including 4 studies, pre-existing diabetes mellitus (OR 3.60, 95% CI 1.43–9.06, I2 = 0%, P = 0.006) and chronic kidney disease stage III or higher (OR 3.79, 95% CI 1.39–10.31, I2 = 0%, P = 0.009) were associated with increased mortality. These findings highlight the aggressive clinical course of COVID-19 in HT recipients.  相似文献   
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From May 1977 to August 1982 50 patients who were 1.5 to 44 months old underwent anatomic correction of transposition of the great arteries (TGA) and closure of ventricular septal defect (VSD) at our institution. Thirty-nine patients underwent preliminary pulmonary arterial banding. Hospital mortality was 32%: four patients died as a result of technical problems, seven as a result of associated lesions, three of pulmonary hypertension, and two of left ventricular failure. Three other patients died after the first postoperative month (one of mediastinitis, one at reoperation for a residual VSD, and one of pulmonary hypertension). All 31 survivors are in excellent clinical condition and are in sinus rhythm after a mean follow-up period of 31 +/- 14 months. Twenty-five patients were reinvestigated by echocardiography (M mode and two-dimensional) and/or catheterization. Parameters of left ventricular contractility were within normal limits, but systolic aortic diameter was larger than normal (p less than .01). Seven patients had stenosis of the right ventricular outflow tract and five of these required reoperation. The two persistent problems with the anatomic correction of TGA associated with VSD are a relatively high operative mortality and secondary right outflow tract stenosis. However, use of this procedure results in better left ventricular function and fewer arrhythmias than does use of atrial repair techniques and also results in the use of the anatomically left ventricle as the systemic ventricle.  相似文献   
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The case of a 22 months old child with no previous medical history hospitalised for an acute infection with pyrexia, arthritis, meningitis and leukocytosis with polynucleosis is reported. All bacteriological investigations were sterile; the search for soluble antigen and serological tests were negative. Antibiotic therapy (Ampicillin and Thiamphenicol) cured the meningitis and arthritis. On the 10th day of treatment the temperature rose, a systolic murmur was detected and echocardiography showed the presence of a large vegetation on the anterior mitral leaflet. Three weeks later (on Ampicillin and Amikacine), asymptomatic abolition of the femoral pulses and disappearance of the vegetation on echocardiography were observed. Angiography confirmed obstruction at the bifurcation of the aorta. Surgical removal of the embolism resulted in revascularisation of the femoral artery and was followed by apyrexia. This infant probably developed endocarditis on a healthy heart. It was complicated by systemic embolism and mitral regurgitation which at present is well tolerated.  相似文献   
8.
The au;hors report 12 cases of Fallot' tetralogy with stenosis or obstruction of one of the two branches of the pulmonary artery following palliative anterior anastomosis. The complications of correction by open heart surgery include the frequent incidence of pulmonary hypertension, which was responsible for 4 deaths. No satisfactory explanation could be found for these poor results. It is desirable to avoid the creation of asymetry in pulmonary blood supply in Fallot's tetralogy and, if such should be found, suggestions are made for its correction by open heart surgery in order to improve the prognosis.  相似文献   
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