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In the light of their experience of surgical treatment of right ventricle muscular stenoses, the authors have gradually abandoned the classical approach (ventriculotomy) and now exclusively use a new one through the tricuspid and pulmonary rings. The new approach considerably reduces the number and extent of enlargement patches in the infundibulum and should result in better preservation of the right ventricular function.  相似文献   
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OBJECTIVE: To evaluate the hemodynamic effects of maternal hypo- and hyperoxygenation in normal term pregnancy. METHODS: Ten healthy women between 35-41 weeks' gestation were exposed to 10% oxygen in inspired air for 10 minutes and, after a 5-minute recovery period, to a stepwise increase in oxygenation with 50 and 100% oxygen for 10 minutes. Maternal ventilation, hemodynamics, and oxygenation were assessed noninvasively, and maternal and fetal vascular responses were assessed with pulsed-wave color Doppler velocimetry. Computerized cardiotocography was used for fetal heart rate (FHR) analysis. RESULTS: Substantial maternal hypoxia was achieved and accompanied by a statistically significant rise in the maternal heart rate (from 89 +/- 11 to 104 +/- 16 beats per minute) and systolic blood pressure (from 123 +/- 13 to 131 +/- 13 mmHg). Doppler measurements demonstrated a statistically significant decline in the pulsatility index (PI) of the maternal internal carotid artery (from 1.8 +/- 0.3 to 1.5 +/- 0.4) and an increase in the uterine artery PI (from 0.60 +/- 0.12 to 0.72 +/- 0.13). Baseline FHR, heart rate variability, and Doppler velocimetry in the umbilical artery and the middle cerebral artery showed no statistically significant changes. Hyperoxia did not cause changes in the maternal circulation, but the FHR decreased significantly (from 142 +/- 12 to 133 +/- 11 beats per minute). CONCLUSION: Acute short-term hypoxia modifies the maternal circulation, suggesting redistribution of maternal blood flow, but exerts no detectable effects on the healthy fetus. Maternal hyperoxygenation induces no apparent adverse effects.  相似文献   
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The fragile X mental retardation syndrome is caused by unstable expansion of a CGG repeat in the FMR-1 gene. Clinical expression is associated with a large expansion of the CGG repeat. The mutation in the FMR-1 gene and the cytogenetic expression of the fragile site at Xq27.3 have been studied in 52 fragile X male patients. The percentage of the cytogenetic expression of the fragile site at Xq27.3 positively correlates with the mean size of the full mutation in the FMR-1 gene (p < 0.0001) irrespective of the presence of additional premutation alleles. We noted a less frequent occurrence of additional premutation alleles in adult patients compared with juveniles, suggesting a continued mitotic instability in life. Additionally, the level of mental retardation has been ascertained in 35 patients using the Stanford-Binet or Terman-Merrill test of general intelligence. The presence of a full mutation in the FMR-1 gene seemed decisive for the occurrence of mental impairment in the patient. No correlation is observed between the degree of mental retardation and the size of the full mutation. The degree of mental retardation seemed not to be influenced by the presence of premutation alleles in part of the cells in addition to a full mutation. One patient is described with the 'Prader-Willi-like' subphenotype of the fragile X syndrome, showing a deletion in the FMR-1 gene in a part of his cells in addition to a full mutation.  相似文献   
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Fifteen years of clinical experience with porous-coated prostheses demonstrated the durability of this type of fixation. This experience was documented by clinical follow-up study of the 393 cases treated by the senior author before 1985. Only six of these femoral components have been revised: three for loosening, two for stem breakage, and one for infection. Thus, the revision rate for the porous-coated stems was 1.5%. Porous-coated acetabular components were used in 227 of the arthroplasties. Five of these porous-coated cups have been revised: four for malposition leading to dislocation and one for late loosening secondary to osteolysis. Thus, the revision rate for these porous-coated acetabular components was 2.2%. Twenty bipolar and 146 cemented acetabular components were used in the remaining 166 cases treated before 1985. Eleven (7.5%) of the cemented acetabular components were revised. Revisions of the porous-coated components were rare in the first ten postoperative years. The clinical data were supplemented with analysis of postmortem specimens from 15 patients. Mechanical testing of the femoral specimens showed the relative micromotion at the porous surface to be exceptionally small (less than 40 microns). Seven of these postmortem retrievals involved cases with unilateral arthroplasties. In these cases, the contralateral normal femur also was removed, and a prosthesis identical to that in the in vivo implanted side was inserted to simulate the immediate postoperative condition. Dual-energy X-ray absorptiometry (DEXA) of the seven paired femora demonstrated that bone remodeling can be expected to produce a 5%-52% loss of periprosthetic bone mineral content, with the greatest loss occurring in the more osteoporotic patients.(ABSTRACT TRUNCATED AT 250 WORDS)  相似文献   
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