An investigation of the neuropsychological profile in adults with velo-cardio-facial syndrome (VCFS).

Velo-cardio-facial syndrome (VCFS) is associated with deletions on the long arm of chromosome 22, mild intellectual disability, poor social interaction and a high prevalence of psychosis. However, to date there have been no studies investigating the neuropsychological functioning of adults with VCFS. We compared 19 adults with VCFS with 19 age, gender and IQ matched controls using a comprehensive neuropsychological battery. Compared to controls, adults with VCFS had significant impairments in visuoperceptual ability (Visual Object and Space Perception Battery), problem solving and planning (Tower of London) and abstract and social thinking (Comprehension WAIS-R). It is likely that haploinsufficiency (reduced gene dosage) of a neurodevelopmental gene or genes mapping to chromosome 22q11 underlies the cognitive deficits observed in individuals with VCFS.     

Rheumatoid arthritis and membranoproliferative glomerulonephritis: an unusual case

SUMMARY: Renal involvement is not uncommon in rheumatoid arthritis (RA). Many RA patients have renal dysfunction either secondary to the drugs used to treat arthritis or because of the chronic inflammation. Renal pathologies have often included amyloidosis, drug-related renal disease and mesangial glomerulonephritis. However, membranoproliferative glomerulonephritis has only been rarely reported. We report a case of rheumatoid arthritis associated with membranoproliferative glomerulonephritis that rapidly progressed to end-stage renal disease.     

再论妊娠期糖尿病与胎儿生长发育

目的 研究妊娠期糖尿病与胎儿生长发育中糖代谢特点的关系。方法 选择妊娠期糖尿病25例（GDM组），正常孕妇20例（对照组），分别测孕妇空腹血糖、糖耐量、C肽、IGF－I、新生儿出生2h内空腹血糖，并依据新生儿出生体重分为大于胎龄儿组（LGA组，≥4000g），适于胎龄儿组（AGA组，2500－3999g)。结果 C肽及新生儿出生2h内空腹血糖GDM组与对照组差异有显著性；糖耐量各时点血糖值餐后2h血糖值LGA和AGA组差异有显著性。结论 血糖始终是影响胎儿生长发育的重要因素，孕妇餐后2h血糖水平与巨大儿的发生呈正相关。     

股骨近端带锁髓内钉在粗隆间骨折的应用

目的评价股骨近端带锁髓内钉(ProximalFemoralNail,PFN)治疗股骨粗隆周围骨折的临床疗效。方法对72例股骨粗隆周围骨折的治疗作回顾性总结分析。其中男29例,女性43例。年龄64～96岁,平均78岁。在本组粗隆周围骨折的分型中,EvenⅠ型16例、Ⅱ型31例、ⅢA型14例、ⅢB型6例、Ⅳ型3例。其余2例为粗隆下骨折。结果随访自出院以后超过6个月的患者65例,除一例患者因患有严重的骨质疏松而出现拉力螺钉脱出并髋内翻外,其余患者拍片显示骨折已愈合,生活能够自理,临床疗效满意,骨折平均愈合时间为8周。结论PFN是治疗股骨粗隆周围骨折的一种较好的内固定方法,为股骨粗隆部位的骨折创造了理想的愈合条件,由于其并发症低,手术操作简便,符合微创技术的治疗要求,值得临床推广应用。     

An open pilot study of nefazodone in depression with anger attacks: relationship between clinical response and receptor binding

Nefazodone has been widely used as an antidepressant, but it has not been tested for depression with anger attacks. In an open study, we administered nefazodone (maximum 600 mg/day) for 12 weeks to 16 outpatients who had major depression with anger attacks. Assessment instruments comprised the Structured Clinical Interview for DSM-IV (SCID), Anger Attacks Questionnaire (AAQ), 17-item Hamilton Rating Scale for Depression (HAM-D-17), Clinician Global Impression Scale (CGI), Symptom Questionnaire (SQ), Modified Overt Aggression Scale (MOAS), and MOAS-Self-Rated. Three subjects underwent positron emission tomography (PET) with [¹⁸F]-setoperone for 5-HT2 binding potential (BP) and [¹¹C]-SCH-23,390 for D1 BP, both at baseline and after 6 weeks of treatment. Eight subjects underwent PET with [¹⁸F]-setoperone and with [¹¹C]-SCH-23,390 at baseline only. In an examination of whether D1 and 5HT2 (data available in six subjects) receptor BP predicted treatment response, we found significant decreases in the HAM-D-17, CGI-S, weighted MOAS, MOAS verbal scale, OAS Self-Rated verbal, SQ Depression and Anger/Hostility scales after nefazodone; 50% responded to nefazodone (defined as ≥50% decrease in HAM-D-17 score), and 44% reported disappearance of anger attacks. A statistically significant percentage decrease in 5HT2 BP was observed for the right mesial frontal and left parietal regions after 6 weeks of treatment. No significant change was observed in D1 BP in any region. Although CGI-I scores correlated significantly with D1 BP in the left thalamic region, the correlation was not significant after Bonferroni correction. The effectiveness of nefazodone for depression with anger attacks may be related to widespread changes in 5HT2 receptor BP.     

MODS患儿血清磷酯酶A2的变化及意义

目的：探讨磷酯酶A2（PLA2）在多脏器功能障碍综合征（MODS）患儿中的作用及意义。方法：结合危重病例评分，采用ELISA法检测26例MODS患儿血清PLA2含量，并与健康体检儿童20例对照。结果：多脏器功能障碍综合征患儿血清PLA2含量明显高于正常对照组，P＜0．01，并与危重病例评分成正相关。结论：PLA2参与MODS的病理生理过程，与危重病的病情有关，并可作为早期预警多脏器功能障碍综合征的实验参数。     

Interactions Between Cytomegalovirus, Human Herpesvirus-6, and the Recurrence of Hepatitis C After Liver Transplantation

Recurrence of hepatitis C (HCV) following liver transplantation is common. Herpesvirus reactivation following transplant may have an immunomodulatory effect resulting in increased HCV replication. We studied whether cytomegalovirus (CMV) and human herpesvirus-6 (HHV-6) may be associated with HCV recurrence and viral load after transplant. We prospectively followed 66 HCV liver-transplant recipients with serial viral load testing for CMV and HHV-6. Infection and viral load were correlated with the development of biopsy-proven HCV recurrence and HCV viral loads. Histologic recurrence of HCV occurred in 41/66 (62.1%) patients. In the primary analysis, CMV infection and disease, and HHV-6 infection were not associated with HCV recurrence. Peak CMV and HHV-6 viral loads were not significantly different in patients with and without recurrence. No correlation was observed between HCV viral loads at 1 and 3 months post-transplant and peak HHV-6 or CMV viral loads. In a subgroup analysis, HHV-6 infection was associated with the development of more severe recurrence (hepatitis and/or fibrosis score > or = 2) (p = 0.01). Also, fibrosis scores at last follow up were higher in patients with CMV disease (1.67 vs. 0.56; p = 0.016) and in patients with HHV-6 infection (1.18 vs. 0.55; p = 0.031). In conclusion, HHV-6 and CMV infection and viral load were not associated with increased overall rates of HCV recurrence or HCV viral load after liver transplantation but may be associated with more severe forms of recurrence.     

PMMA/HA-GF医用复合材料制备及性能测试

本文主要研究了无机玻璃纤维增强聚甲基丙烯酸甲酯(PMMA)/羟基磷灰石(HA)复合材料的制备及相关的性能测试,证明此复合材料性能稳定、物理机械强度高以及工艺简单,有良好的应用前景。     

氚掺入法检测低分化鼻咽癌亚株的放射敏感性差异及放射增敏

目的 探讨人低分化鼻咽癌亚株之间的放射敏感性差异及对其放射增敏。方法 采用氚掺入法检测其亚株对射线敏感的差别；通过细胞周期同步化对其放射抵抗亚株S1增敏。结果氚掺入法结果表明放射后1-15受抑制程度大（P〈0．05）；TNF仅可以使细胞周期同步化达到了放射增敏，对放射抵抗亚株增敏后缩小了亚株间的放射敏感性差距。结论 低分化鼻咽癌亚株存在着放射敏感性差异；对其放射抵抗亚株增敏后差异不显著。