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91.
We report a 16-year-old boy, born to consanguineous parents, with mental retardation, gait disturbances and dysarthria; brain magnetic resonance showed features consistent with rhombencephalosynapsis. This condition is characterised by a hypoplastic single-lobed cerebellum. The interest of this case is the presence of common ancestors, pointing to an autosomal recessive inheritance of the malformation.  相似文献   
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We evaluated the effect of halothane anesthesia on the motility of the sphincter of Oddi and simultaneous transsphincteric flow in Australian Brush-tailed possums (Trichosurus vulpecula). Halothane levels in the range of 0.25 to 2% were administered and decreased transsphincteric flow in a dose-dependent manner. Sphincter of Oddi basal pressure was higher than normal, but not in a dose-dependent manner. Additionally, halothane anesthesia influenced the sphincter of Oddi motility by decreasing the motility index (mean amplitude multiplied by frequency of contractions). This decrease was dose dependent. These findings indicate that sphincter of Oddi basal pressure is a major component of sphincter of Oddi motility responsible for regulating transsphincteric flow in this species. For studies of the sphincter of Oddi motility in anesthetized Australian Brush-tailed possums, we recommend anesthetic induction with ketamine (50 mg/kg, i.m.) and the inspired halothane level should not exceed 0.75% during the study period, as the effects we have demonstrated were most evident at levels greater than 0.75%. If higher halothane levels are required to maintain satisfactory anesthesia, an alternative anesthetic agent should be considered.  相似文献   
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Radical abdominopelvic lymphadenectomy for rectal cancer is based on the tenet that removal of all potentially involved lymphatic tissue will yield a lower rate of locoregional failure and improve survival. At centers with extensive experience with the procedure, the operating time is only modestly prolonged compared with conventional resection. Blood loss and postoperative hospitalization are not significantly increased. Urinary dysfunction and impotence associated with radical abdominopelvic lymphadenectomy (as high as 80 percent and 76 percent, respectively, in recent series) have been major deterrents to its more routine application. Preservation of the hypogastric plexus and even selective preservation of a unilateral S4 nerve root have been shown to reduce the occurrence of genitourinary complications. Improved five-year survival of 68 percent and local recurrence rates of 5 to 20 percent for TNM Stage III cancers have been achieved with radical abdominopelvic lymphadenectomy. These results compare favorably with recent trials of adjuvant chemoradiation after conventional resection in stage-matched patients. The rationale, evolution, and application of radical abdominopelvic lymphadenectomy to the surgical management of rectal cancer are critically examined. The potential benefits of radical abdominopelvic lymphadenectomy, which have been demonstrated in nonrandomized trials, should be evaluated in a prospective and properly randomized study to clearly establish or refute its efficacy.  相似文献   
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The IgE-mediated allergic response involves a series of physiological pathways that result in the array of symptoms commonly associated with allergy. This article describes the mediators that prompt specific clinical responses and identifies those at risk for latex hypersensitivity.  相似文献   
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