A model of corrective gene transfer in X-linked ichthyosis

Single gene recessive genetic skin disorders offer attractive prototypes for the development of therapeutic cutaneous gene delivery. We have utilized X-linked ichthyosis (XLI), characterized by loss of function of the steroid sulfatase arylsulfatase C (STS), to develop a model of corrective gene delivery to human skin in vivo. A new retroviral expression vector was produced and utilized to effect STS gene transfer to primary keratinocytes from XLI patients. Transduction was associated with restoration of full-length STS protein expression as well as steroid sulfatase enzymatic activity in proportion to the number of proviral integrations in XLI cells. Transduced and uncorrected XLI keratinocytes, along with normal controls, were then grafted onto immunodeficient mice to regenerate full thickness human epidermis. Unmodified XLI keratinocytes regenerated a hyperkeratotic epidermis lacking STS expression with defective skin barrier function, effectively recapitulating the human disease in vivo. Transduced XLI keratinocytes from the same patients, however, regenerated epidermis histologically indistinguishable from that formed by keratinocytes from patients with normal skin. Transduced XLI epidermis demonstrated STS expression in vivo by immunostaining as well as a normalization of histologic appearance at 5 weeks post-grafting. In addition, transduced XLI epidermis demonstrated a return of barrier function parameters to normal. These findings demonstrate corrective gene delivery in human XLI patient skin tissue at both molecular and functional levels and provide a model of human cutaneous gene therapy.      

Blood loss and replacement in total hip arthroplasty: a multicenter study. The Preoperative Autologous Blood Donation Study Group

To determine blood loss, the number of transfusions, and the hemoglobin levels achieved in patients via transfusion in the course of total hip arthroplasty, 324 patient records from 1987 through 1989 were reviewed at three university and three community hospitals. Calculated blood loss was 3.2 +/- 1.3 units in primary procedures and 4.0 +/- 2.1 units in revision procedures (mean +/- SD). Of 777 red cell units transfused, 455 (59%) were autologous units. Transfused patients received 2.0 +/- 1.8 units for primary procedures and 2.9 +/- 2.3 units for revision procedures (mean +/- SD). The maximum number of units given to 95 percent of the transfused patients was 4 for primary procedures and 6 for revision procedures. The mean postoperative hemoglobin level after all transfusions was 103 to 110 g per L, regardless of patient age group of physical status, autologous donor status, or hospital. No difference in length of hospital stay was observed for patients less than 65 years old with hemoglobin concentrations of 80 to 139 g per L at discharge.     

急性冠状动脉综合征置入支架患者使用替罗非班和阿司匹林时泮托拉唑对消化道保护作用的评价

目的:急性冠状动脉综合征(ACS)置入支架患者使用盐酸替罗非班、阿司匹林、氯吡格雷、低分子肝素(四联)时,评价泮托拉唑对消化道的保护作用.方法:选择ACS置入支架治疗的患者266例,随机分入观察组134例,对照组132例,所有患者均服用阿司匹林、氯吡格雷、低分子肝素和盐酸替罗非班.观察组患者静脉注射泮托拉唑40 mg/d 4～5天,之后改为泮托拉唑片剂40 mg/次,2次/天,服用30天.观察两组间30天全因死亡、再次心肌梗死、再次经皮冠状动脉介入治疗(PCI)、再次住院、颅内出血和消化道出血状况.结果:观察组134例患者30天内全因死亡5例、再次心肌梗死4例、再次PCI4例和再次住院8例;对照组132例患者分别为8例、6例、5例和13例,两组相比差异均无统计学意义(P>0.05);两组均无颅内出血发生.观察组消化道大出血0和总消化道出血事件3例,对照组分别为5例和15例,两组比较观察组消化道大出血和总消化道出血事件少于对照组(P<0.05),差异均有统计学意义.结论:ACS置入支架治疗的患者,盐酸替罗非班治疗是安全有效的,未见颅内出血发生.静脉注射和口服泮托拉唑并不增加30天全因死亡、再次心肌梗死、再次PCI和再次住院发生,同时可以减少30天内消化道出血发生率特别是消化道大出血事件的发生,具有良好的消化道保护作用和安全性.     

早产儿瘦素质量浓度与早期静脉营养及生长发育相关性探讨

目的探讨瘦素质量浓度与早期静脉营养及生长发育的关系。方法新疆医科大学第一 附属医院新生儿科于2005 01—2006 02，将收治的86例早产适于胎龄儿用随机数字表法分为观察组(早期微量喂养同时辅助胃肠外营养组)45 例和对照组(单纯早期微量喂养组)47例，分别测定脐血及第7天血清瘦素质量浓度，同时监测营养状况和生长发育指标，并作对比分析。结果 (1)观察组与对照组脐血瘦素质量浓度分别为(4.6±3.7)ng/mL、(4.8±2.2)ng/mL，生后第7天两组瘦素质量浓度分别为(4.3±2.2)ng/mL、(3.1 ±1.7)ng/mL。对照组第7天血清瘦素质量浓度明显低于脐血(P<0.05)，而观察组其差异无统计学意义(P>0.05)。(2)脐血瘦素质量浓度与出生体 重、胎龄成正相关(r=0.56、r=0.67)。(3)观察组第7天热卡及蛋白质摄入量、血清瘦素质量浓度、皮褶厚度变化值与对照组相比，差异有统计 学意义(P<0.05)。结论对早产儿应尽早喂养，同时需要胃肠外营养作为肠内营养的补充。瘦素可作为新生儿营养效果判定的实验室指标之一。     

The diagnostic dilemma of congenital unstable hemoglobinopathies

Unstable hemoglobin variants represent a rare etiology of congenital hemolytic anemia. Without a high index of suspicion, plus proper laboratory testing and interpretation, the correct diagnosis can be elusive. We report on five children who were initially thought to have other congenital disorders such as hereditary spherocytosis or thalassemia, before β‐globin gene sequencing led to the definitive diagnosis. Recognizing the variable clinical presentation and laboratory data reported will aid clinicians in diagnosis of unstable hemoglobins variants in children with atypical forms of hemolytic anemia, particularly those with low pulse oximetry values or whose hemoglobin electrophoresis suggest β‐thalassemia trait. Pediatr Blood Cancer. 2010;55:1393–1395. © 2010 Wiley‐Liss, Inc.     

大孔树脂吸附法富集野菊花总黄酮的工艺研究

目的研究大孔树脂吸附法富集野菊花总黄酮的工艺条件及参数。方法以野菊花总黄酮为考察指标,考察大孔树脂富集野菊花总黄酮的最佳工艺条件。结果野菊花提取液(50mg生药/mL)5mL上大孔树脂柱(150mm×10mm),吸附30min后,先用100mL蒸馏水洗脱除去杂质,然后用70%乙醇100mL洗脱,洗脱速度为2mg/mL,洗脱剂用量为9倍量树脂,树脂可重复使用3次,采用此条件为最佳工艺。结论AB-8型大孔树脂在所确定的工艺条件下,可较好的吸附分离野菊花总黄酮。其70%乙醇洗脱物中总黄酮质量分数达4.34%以上,总黄酮收率为84.47%以上。采用此法可以较好的富集野菊花中的有效成分。