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991.
INTRODUCTION: In patients with hypertrophic obstructive cardiomyopathy, obstruction in the left ventricular outflow tract may generate more hypertrophy. Our aim was to evaluate the impact of reducing ventricular outflow tract obstruction on left ventricular hypertrophy and remodeling after alcohol septal ablation. PATIENTS AND METHOD: 20 patients with hypertrophic obstructive cardiomyopathy who underwent alcohol septal ablation were included. Doppler echocardiography was performed in all patients at baseline, immediately after alcohol septal ablation, and at 3 and 12 months' follow-up. Left ventricular diameters and wall thickness and pressure gradients in the ventricular outflow tract were determined. RESULTS: Immediately after alcohol septal ablation, ventricular outflow tract pressure gradient decreased from 63.0 27.7 to 28.2 24.7 mmHg (p < 0.001), without significant changes in left ventricular dimensions. However, after 12 months we observed an increase in left ventricular end-diastolic (from 47.1 4.9 to 50.8 4.5 mm) and end-systolic diameter (from 27.1 3.0 to 33.7 4.6 mm), as well as a reduction in septal (from 19.5 4.0 to 15.5 2.7 mm) and posterior wall thickness (from 14.0 2.2 to 12.9 1.3 mm) (p < 0.01 in all cases). Left ventricular end-diastolic and end-systolic volumes increased (from 106.4 26.9 to 123.1 28.7 ml and from 50.2 17.3 to 56.7 18.3 ml, respectively, p < 0.01 in both cases), without changes in left ventricular ejection fraction. The reduction in ventricular outflow tract pressure gradient at 12 months' follow-up correlated significantly with the increase in left ventricular end-systolic diameter (r = 0.63; p < 0.01). CONCLUSIONS: In patients with hypertrophic obstructive cardiomyopathy who underwent alcohol septal ablation, relief of ventricular outflow tract obstruction is associated with an increase in left ventricular chamber diameters and volume. These findings suggest that middle- and long-term ventricular remodeling and regression of hypertrophy occur in these patients, which may contribute to their clinical improvement.  相似文献   
992.
993.
994.
Epidermolysis bullosa simplex in Israel: clinical and genetic features   总被引:4,自引:0,他引:4  
BACKGROUND: Epidermolysis bullosa simplex (EBS) is the most common form of epidermolysis bullosa. The disease is characterized by intraepidermal blistering due in most cases to mutations in cytokeratin genes 5 (K5) or 14 (K14). Extensive studies in the United States and Europe have shown that EBS is almost always inherited in an autosomal dominant fashion. OBJECTIVE: To assess the possibility that the molecular features of EBS may differ according to the type of population studied. DESIGN: We assessed 10 Israeli families diagnosed as having EBS and compared their clinical and genetic features with previous observations. Affected individuals underwent complete clinical evaluation. DNA from all family members was assessed for mutations in K5 or K14 using polymerase chain reaction amplification, direct sequencing, and subsequent mutation verification. In addition, specific cases were genotyped using a panel of microsatellite markers spanning the K14 locus. RESULTS: Eight distinct pathogenic mutations in K5 (3 mutations) and K14 (5 mutations) were identified. Six of these mutations are novel. The mutations included 2 nonsense mutations and 6 missense mutations. A third of the affected families inherited EBS in a recessive fashion, in contrast with previous observations in Europe and the United States. In addition, we identified a unique case that resulted from compound heterozygosity for a missense and a nonsense mutation in K14. Homozygous nonsense mutations were strongly associated with a severe phenotype. CONCLUSION: The present study demonstrates a unique mutation spectrum and a strikingly different pattern of inheritance for EBS in a series of Israeli families compared with families of European or US extraction.  相似文献   
995.
A phase II study was conducted to evaluate the safety and efficacy of the combination GIP (gemcitabine, ifosfamide, and cisplatin) for the treatment of patients with advanced non-small-cell lung cancer (NSCLC). Thirty patients with stage III B/IV NSCLC were treated with a combination of GIP. Patients received gemcitabine 1,000 mg/m2 administered intravenously on days 1 and 8, ifosfamide 3,500 mg/m2 on day 2, and cisplatin 80 mg/m2 on day 2, repeated every 21 days. Two of the 30 patients (7%) showed a complete response and 14 patients (46%) showed a partial response. The overall response rate was 53%. The estimated median survival for all patients was 60 weeks. All patients enrolled onto the study were eligible for toxicity assessment. Toxicities were treatable and included World Health Organization grade III or IV leukopenia (29%), thrombocytopenia (18%), anemia (7%) and nausea, and vomiting (6%). Febrile neutropenia occurred in 3 of 30 patients. There were no treatment-related deaths. The combination therapy of GIP is active, well tolerated, and easy to administer on an outpatient basis in advanced NSCLC.  相似文献   
996.
After spinal cord injury (SCI), the inability of supraspinal neurons to regenerate or reform functional connections is likely due to proteins in the surrounding microenvironment restricting regeneration. EphAs are a family of receptor tyrosine kinases that are involved in axonal guidance during development. These receptors and their ligands, the Ephrins, act via repulsive mechanisms to guide growing axons towards their appropriate targets and allow for the correct developmental connections to be made. In the present study, we investigated whether EphA receptor expression changed after a thoracic contusion SCI. Our results indicate that several EphA molecules are upregulated after SCI. Using semiquantitative RT-PCR to investigate mRNA expression after SCI, we found that EphA3, A4, and A7 mRNAs were upregulated. EphA3, A4, A6, and A8 receptor immunoreactivity increased in the ventrolateral white matter (VWM) at the injury epicenter. EphA7 had the highest level of immunoreactivity in both control and injured rat spinal cord. EphA receptor expression in the white matter originated from glial cells as coexpression in both astrocytes and oligodendrocytes was observed. In contrast, gray matter expression was localized to neurons of the ventral gray matter (motor neurons) and dorsal horn. After SCI, specific EphA receptor subtypes are upregulated and these increases may create an environment that is unfavorable for neurite outgrowth and functional regeneration.  相似文献   
997.
BACKGROUND: Postoperative nausea and vomiting after laparoscopic cholecystectomy remains a common problem despite routine antiemetic prophylaxis. Therefore, the authors investigated the effect of administering 4 mg intravenous dexamethasone as an adjunct to a 5-HT3 antagonist (12.5 mg intravenous dolasetron) with respect to patient outcome. METHODS: Outpatients (N = 140) were enrolled in this prospective, randomized, placebo-controlled, double-blind, institutional review board-approved protocol involving two antiemetic treatment groups. After induction of anesthesia, the control group received 1 ml intravenous saline, whereas the dexamethasone group received 4 mg intravenous dexamethasone. Both groups received 12.5 mg intravenous dolasetron at the time of gallbladder removal. A blinded observer recorded the recovery times, emetic episodes, rescue antiemetics, maximum nausea score, and time to achieve discharge criteria. Postdischarge side effects, as well as patient satisfaction and quality of recovery scores were assessed at 24 h after surgery. RESULTS: Although there was no difference in the incidence of postoperative nausea and vomiting in the early recovery period, the dexamethasone group had a shorter stay in the day-surgery unit (136 +/- 57 vs. 179 +/- 62 min) and more rapidly achieved discharge criteria (161 +/- 32 vs. 209 +/- 39 min). In addition, fewer patients in the dexamethasone group experienced nausea at home within 24 h after discharge (13 vs. 28%, P < 0.05). Finally, the dexamethasone group reported higher quality of recovery and patient satisfaction scores (P < 0.05). CONCLUSIONS: The authors conclude that the adjunctive use of 4 mg intravenous dexamethasone shortened the time to achieve discharge criteria and improved the quality of recovery and patient satisfaction scores after laparoscopic cholecystectomy procedures in outpatients receiving prophylaxis with 12.5 mg intravenous dolasetron.  相似文献   
998.
BACKGROUND: Immunization against hepatitis A and B has been available for some time, protecting against both infections. With a view to achieving further reduction in the number of interventions and increasing convenience of the vaccinee, we investigated the reactogenicity and immunogenicity of a combined hepatitis A and B vaccine in healthy 4- to 20-year-old subjects at a 0, 6-month schedule. METHODS: Two hundred forty-eight study subjects were allocated to two study groups and received either two doses of the combined hepatitis A and B vaccine (68% of subjects) or the corresponding monovalent hepatitis A and hepatitis B vaccines (32% of subjects) concomitantly in opposite arms. Reactogenicity was assessed via diary cards after each vaccination. Serum samples were analyzed at months 1, 2, 6, and 7. RESULTS: All vaccines were well tolerated and very few symptoms were scored as severe. All but one subject seroconverted for anti-hepatitis A virus (anti-HAV) antibodies (98.6%) and 100% of subjects seroconverted for anti-hepatitis B (HBs) antibodies, with respective seroprotection rates of 98.7% for the combined vaccine group and 95.9% for the concomitant vaccine group (p >0.05), respectively. Geometric mean titers were higher in the group receiving the combined vaccine: 6,635 mIU/mL vs. 2,728 mIU/mL (p = 0.0001) for anti-HAV and 3,362 mIU/mL vs. 1,724 mIU/mL (p = 0.0205) for anti-HBs, respectively. Younger subjects had a stronger immune response compared to older subjects. CONCLUSIONS: The combined hepatitis A and B vaccine was well tolerated at this two-dose schedule. The combined vaccine had higher immunogenicity, probably explained by a adjuvant effect of the antigens. Vaccination programs requiring fewer injections will most likely have a positive impact on compliance rate and comfort of the vaccinee.  相似文献   
999.
The present study is a report of an experience of nurses from the South of Brazil as technical proofreaders of books about nursing diagnosis. It aims at contributing with colleagues who might do similar task arousing some aspects for discussion. The development of nursing diagnosis in Brazil is presented. The main steps of a translated book production are identified and the attributions of technical proofreaders are cited. The positive aspects and the difficulties found in this activity are commented. Alternatives are given in order to maintain the final quality of the work and to enlarge the availability of specialized texts as faithful to the original as possible.  相似文献   
1000.
Peripheral resolution acuity for achromatic gratings is known to be limited by the density of the underlying ganglion cell sampling array. After confirming isolation of the short-wavelength sensitive (SWS) system using chromatic adaptation methods, we wished to determine if resolution is limited by blue/yellow ganglion cell sampling (evidenced by a superiority of detection over resolution acuity and the perception of aliasing) and thus directly related to SWS-driven ganglion cell density. We measured detection and resolution acuity between 0 degrees and 35 degrees, using blue sinusoidal gratings superimposed on a yellow adapting background which ranged in intensity from 2.5 to 4.7logTrolands. At all locations, a break could be observed in the acuity vs. illumination curves followed by a plateau. Detection and resolution acuity were the same for low background intensities, but resolution acuity was lower than detection at higher intensities, accompanied by observations of chromatic aliasing. SWS resolution is sampling-limited across the retina and agrees well with predicted performance based on anatomical estimates of small bistratified ganglion cell density.  相似文献   
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