以肺动脉高压为首发表现的先天性中枢性低通气综合征临床特征和基因分析

目的探讨先天性中枢性低通气综合征(CCHS)的临床和基因变异特征。方法分析1例首发表现为不明原因肺动脉高压的CCHS患儿的临床资料,并总结国内外文献中CCHS病例的临床特点、致病机制和基因变异情况。结果11月龄女婴,主要表现为浮肿、尿少、低血压、嗜睡、发绀、抽搐及颅内压增高。B型脑利钠肽、丙氨酸氨基转移酶升高,凝血酶原时间延长。颅脑磁共振示右侧额叶出血;超声心动图示中重度肺动脉高压。靶向捕获二代测序未发现可能的致病基因。采用Sanger法验证示患儿PHOX 2 B基因第3外显子存在多聚丙氨酸重复扩展变异,基因型为20/25。患儿入院后采用无创通气,睡眠时呼吸浅慢、微弱,伴血氧下降;血气分析提示二氧化碳潴留。随后改用夜间无创通气、降肺压药物治疗。复查肺动脉压力明显下降,生命体征稳定。随访至24月龄,夜间只需较低压力水平的无创通气,生长发育无异常。结论对于不明原因的肺动脉高压伴撤机困难患儿,需警惕CCHS。疑诊者应尽早针对CCHS相关基因进行靶向捕获二代测序及PHOX 2 B基因Sanger法验证。早期给予无创通气有望改善预后。     

手术室新护士三年阶梯式培训模式探讨

目的探讨手术室新护士三年培训模式和方法。方法采用三年阶梯式培养模式,建立完整的系统培训架构,包括培训对象的界定、培训原则及目的、培训目标师资队伍的培养、阶段培养计划、使用培训手册、培训考核体系的建立、培训评价体系的建立等。结果新护士培训系统、科学。结论新护士三年阶梯式培养值得深入研究和推广应用。     

Sex‐related Disparity in Surgical Mortality among Pediatric Patients

Background. It has been reported that gender differences in cardiovascular outcomes found in adults also are present in children who undergo surgical repair for congenital heart disease. Methods. California statewide hospital discharge data 1989–99 were used to study outcomes in children <18 years undergoing cardiac surgery. Hospital discharge data were linked to death registry data to study postdischarge death within 30 days of discharge. We used logistic regression to evaluate the effect of gender on mortality controlling for age, race and ethnicity, type of insurance, household income, date and month of surgery, type of admission, hospital case volume, and various types of procedures. Results. There were 25 402 cardiac surgery cases with 1505 in‐hospital deaths (mortality rate of 5.92%). An additional 37 deaths occurred within 30 days after hospital discharge. Crude mortality rates for males (5.99%) and females (5.84%) were not significantly different. However, fewer neonates were female and females underwent a higher proportion of low‐risk procedures than males. Logistic regression revealed that females, compared with males, had a significantly higher odds ratio (OR) for in‐hospital mortality (OR = 1.18, P < .01) and overall (up to 30 days post discharge) mortality (OR = 1.18, P < .01). The risk‐adjusted length of hospital stay was similar between females and males while charges per hospital day were slightly higher in females than males. The prevalence of Down syndrome, pulmonary hypertension, and failure to thrive were higher in females. Conclusions. Female gender is associated with an 18% higher in‐hospital and 30‐day postdischarge mortality as compared with male gender. There was no difference in length of hospital stay between males and females. The mechanism by which female gender acts as a risk factor requires further investigation.     

大鼠脑血肿周围组织半胱氨酸天冬氨酸蛋白酶3表达变化与尼莫地平的影响

目的：观察脑出血后血肿周围半胱氨酸天冬氨酸蛋白酶3的表达。及尼莫地平对其的影响。方法：实验于2004—07在大连医科大学中心实验室进行。取120只SD雄性大鼠随机分为3组：①尼莫地平组（n=50）：尾壳核注射自体股动脉血50μL复制脑出血模型，造模即刻腹腔注射尼莫地平1．6mg／kg（即8μL／g），以后每天1次。②模型组（n=50）：同前造模，术后腹腔注射等量生理盐水。③假手术组（n=20）：手术，但进针人尾壳核后不注血。各组分为术后6，24，48，72h、5d 5个时间点。造模动物醒后进行Bederson评分，评估其行为和神经功能缺陷（0—3分。评分越高，神经功能缺陷越重，评分≥2分为造模成功）。人组动物在以上5个时间点进行Bederson评分后，麻醉状态下处死取脑，经尾壳核行冠状切片，行免疫组化测定半胱氨酸天冬氨酸蛋白酶3表达。结果：80只大鼠进入结果分析。①Bederson评分：模型组、尼莫地平组大鼠醒后迅速出现偏瘫，24h后评分趋于稳定，至72h之间评分最高，然后逐渐下降，5d时仍有体征。模型组、尼莫地平组各时间点评分均高于对照组（P〈0．01），尼莫地平组术后48，72h评分低于模型组（P〈0.05）。②血肿周围组织半胱氨酸天冬氨酸蛋白酶3表达：假手术组进针侧脑组织表达很少，模型组6h后即有表达，24h达高峰，持续72h后逐渐下降，5d后仅有少量表达；尼莫地平组动态变化趋势与模型组相同，但各时间点的数值均较低，尤其是术后24～72h（P〈0．01）。结论：①脑出血后血肿周围组织中半胱氨酸天冬氨酸蛋白酶3表达升高，提示其与脑出血血肿周围组织损伤有一定关系。②尼莫地平降低脑出血后血肿周围组织中半胱氨酸天冬氨酸蛋白酶3表达，从而减轻细胞凋亡程度，对神经细胞起到保护作用，降低神经功能缺陷。     

Interdisciplinary approach to abdominal Burkitt’s lymphoma

Burkitt’s lymphoma is a high-grade, rapidly growing B-cell neoplasm. It is recognized by its aggressive course, brief median survival, and low rates of long-term survival. The authors discuss the case of a patient who acutely presented with intraabdominal complications from a new onset of Burkitt’s lymphoma. The clinical and pathological features, staging, treatment options, and survival data are reviewed. In addition, the role of surgical intervention is carefully analyzed.     

A case report: Severe bone marrow suppression caused by 6-mercaptopurin in Crohn's disease patient]

A 23-year-old man was admitted for treatment of acute exacerbation of ileitis and perianal abscess caused by Crohn's disease. After incision and drainage of the abscess, coupled with antibiotic therapy, 6-mercaptopurine (6-MP) was commenced. His white blood cell (WBC) count on day 12 after initiation of 6-MP was not decreased. However, on day 24 he was re-admitted because of severe myelosuppression (WBC: 300/microl), which was complicated by the recurrence of the perianal abscess. Myelosuppression was prolonged and required the administration of granulocyte colony stimulating factor (G-CSF). G-CSF was continued for 17 days to achieve recovery of his WBC count to a normal level.     

Evaluation of mandibular block using a standardized method.

The purpose of this prospective randomized study was to investigate the manifestations and efficiency of mandibular block (MB) using a standardized method. In this study, 123 patients requiring MB were randomly assigned to receive MB using a repeated measures design. Mandibular block was administered in each patient by using 2.1 mL of 2% lidocaine with 1:100 000 epinephrine for the inferior alveolar nerve, lingual nerve, and/or buccal nerve. Clinical parameters including the electric pulp test and test for soft tissue sensation using a sharp dental explorer were assessed on both the operated side and contralateral side before and 5, 10, and 15 minutes after injection, and at the end of surgical procedure. Results of the test of the contralateral tooth served as the control group. No response to the electric pulp test at a maximum output (80 readings) was used as the criterion for pulpal anesthesia. Results obtained with electric pulp test and sharp explorer test were not significantly different during various intervals on the contralateral side, indicating the reliability of this standardized method for assessing MB. The MB technique achieved a high rate of subjective numbness (100%) according to the perception of the patients of overall numbness and lip numbness. Overall anesthetic success rates of pulpal anesthesia were 5.7% for the central incisor, 38.2% for the canine, 55.3% for the first premolar, and 90.2% for the first molar. No significant difference was found in the efficiency of anesthesia related to sex or age of the patients. A slower onset of pulpal anesthesia after MB was found for the anterior teeth than for the posterior teeth. This study suggests that the use of a standardized method might produce a clearer and more comprehensive evaluation of the efficiency and manifestations of MB.     

医药企业对电子商务营销模式的科学选择

根据电子商务在我国快速发展的形势，分析我国医药企业可选择的电子商务应用模式，研究企业进行模式选择时所需要考虑的因素，提醒医药企业选择电子商务模式时应依据自身特点积极稳妥地决策，切忌盲目一哄而上。