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991.
A new case of mitochondrial malonyl coenzyme A decarboxylase deficiency is described. The patient presented with an initial episode of metabolic acidosis, seizures, hypoglycemia, and cardiac failure at 2 months of age which slowly resolved. Subsequent evaluations at 4 years of age for developmental delay revealed a prominent elevation of malonic acid in urine. Malonyl carnitine was also elevated. The activity of malonyl CoA decarboxylase in cultured fibroblasts was 7% of normal. Conclusion Malonyl CoA decarboxylase deficiency may result in inhibition of fatty acid oxidation, which may account for the cardiomyopathy. Received: 12 April 1996 / Accepted: 24 September 1996  相似文献   
992.
A 4-week-old female infant presented with congestive heart failure, moderate mitral regurgitation, and an electrocardiographic pattern of anterolateral myocardial infarction. Angiography revealed normal coronary arteries and moderate mitral regurgitation. A single-catheter electrophysiology study confirmed the presence of an accessory atrioventricular conduction pathway.  相似文献   
993.
Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac lesion. The lesion includes ventricular septal defect, overriding aorta, and absence of the pulmonary valve, with resultant pulmonary incompetence. It has been suggested that the pulmonary incompetence induces intrauterine dilatation of the pulmonary artery, which leads to tracheobronchial compression. One of the presenting features in infants with APVS is severe airway obstruction, which may be difficult to manage. We report an infant who benefited from bilateral endobronchial endoscopic stent placement.  相似文献   
994.
A number of studies have reported that most individuals with autism have difficulty in attributing mental states. The primary aim of the present study was to compare the ability of children with autism to refer to knowledge states with that of mainstream school children and children with Down's syndrome. The second aim was to investigate the role of verbal mental age in referring to knowledge states. The third aim was to compare the ease with which the children referred to their own mental state and to that of others. The results suggest that some individuals with autism are able to attribute knowledge to themselves and others but that they need to have higher verbal skills than is necessary in normal individuals. The level of language skill predicted the performance of the individuals with autism, but not that of the children in the other groups. There was no good evidence that referring to one's own mental states was easier than referring to another person's, a finding which supports representational theory rather than the simulationist position.  相似文献   
995.
喉癌组织恶性度与颈淋巴结转移关系   总被引:2,自引:0,他引:2  
采用五因素评点法对40例喉鳞癌进行组织学和临床方面的研究。结果表明,组织恶性度与T分期无相关性,浸润方式弥散及恶性度高(评分≥11)的肿瘤发生颈淋巴结转移率显著高于有完整肿瘤边界者及恶性度低(评分≤10)者。提示喉癌组织恶性度在预测颈淋巴结转移上有实用价值,可临导临床治疗方案的选择。  相似文献   
996.
突发性聋与铁代谢障碍疾病关系的临床调查   总被引:6,自引:1,他引:6  
对1979年7月至1996年6月的突发性聋发病情况进行前瞻性研究与回顾性分析,探讨突聋与铁代谢障碍疾病的关系,前瞻性研究的对象分为铁代谢障碍疾病组218例,血液病组215例和正常对照组4850例;回顾性分析为17年间资料完整的429例突聋患者史的代谢障碍疾病及其他血液病的发病情况。结果表明,铁代谢障碍疾病组的突聋调整年发病率显著高于血液病组以及正常对照组,429例突聋患者的无贫血缺铁,缺铁性人同血  相似文献   
997.
报告下咽癌切除术后,应用会厌剖开延伸的粘软骨膜瓣修复下咽颈段食管的粘膜缺损8例,术中分别采用会厌瓣和胸骨舌骨肌筋股瓣、会厌瓣和残存下咽粘膜瓣作复合修复,术后恢复良好的吞咽功能,无咽颈瘘发生.临床上虽可应用胃、空肠、结肠和胸大肌肌皮瓣代下咽颈段食管,但手术费时、复杂、病人痛苦大。本术式优点:就近取材,血运丰富,操作简便,成功率高,并发症少。  相似文献   
998.
正常与病态下嗓音的客观指标的评价   总被引:3,自引:0,他引:3  
对158例正常人和136例各种喉疾病患者连续元音[i]发声时的平均气流率、声强、频率、频率微扰商和发声效率同步进行了测定.并对各测定指标间的相互关系进行了探讨.频率微扰商是表示连续元音发声时声带振动相邻周期间的微细变动量;发声效率反映平均气流率与声能的有效转换率.在低、中和高强度发声时.随发声强度的提高,平均气流率.频率和发声效率随之提高而频率微扰商则变小.中等声强发声时.平均气流率与声强,声强与频率均呈正相关;而平均气流率与发声效率.声强(男性)和频率(女性)与频率微扰商均呈负相关.多数喉疾病患者显示出较正常人高的平均气流率和频率微扰商,发声效率则降低.当声强和频率作为参考指标时,平均气流率,频率微扰商和发声效率可作为评价喉发声功能的客观、定量指标.  相似文献   
999.
输卵管妊娠保留输卵管手术后综合疗法的宫内妊娠探讨   总被引:3,自引:1,他引:3  
张建青  芦莉 《生殖与避孕》1997,17(4):241-244
对输卵管妊娠52例有生育要求者,其中28例行切开输卵管壶腹部取胚胎术,10例行输卵管峡部破裂口修补术,7例行输卵管伞部成形术,5例行输卵管峡部端端吻合术,2例行输卵管子宫角部植入术。术后给MTX与中药等综合治疗2~5个月,对其中45例行输卵管通畅检查,双侧输卵管通畅4O例,通畅率为88.9%;有41例宫内妊娠,妊娠率为78.9%,另有1例再次异位妊娠。提示:对有生育要求的输卵管妊娠尽量根据输卵管的不同情况采用相应的手术方法,并应用有效的术后综合措施,对保留患者的生育功能和提高妊娠率有重要意义。  相似文献   
1000.
Purpose: Our purpose was to characterize and describe anesthesia practice in programs performing IVF in the United States. Methods: We used a telephone survey requiring respondents to be either the program director, a physician, or a nurse familiar with the practice. Two hundred seven (78%) Society of Assisted Reproductive Technology (SART) registered programs agreed to participate. Programs were divided by geographic region and type of practice (academic versus private). Results: Ninety-one private (68%) and 41 academic (56%) programs used personnel provided by the Department of Anesthesiology. Conscious sedation was performed most commonly (95%). The remaining 5% used primarily either general, regional, or local anesthesia. Typical recovery times were 90 to 120 min. Average costs of anesthetic administration were $300–$400 and were similar among groups except for the Eastern academic programs, with a higher mean cost of $543. Programs using personnel from anesthesiology reported higher costs compared to programs utilizing their own staff ($391±15 vs $157±11; P<0.05). Complications were infrequent (<10%); no hospitalizations or serious life-threatening incidents were reported. Conclusions: A large number of programs safely used their own trained personnel to deliver anesthesia, and realized a significant reduction in cost.  相似文献   
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