Trisomy 16鼠结肠和人先天性巨结肠蛋白基因产物9.5的表达

目的：研究Ｄｏｗｎ’ｓ 综合征动物模型ｔｒｉｓｏｍｙ １６ 结肠神经系发育和先天性巨结肠（ＨＤ） 病变肠管蛋白基因产物９－５（ｐｒｏｔｅｉｎ ｇｅｎｅ ｐｒｏｄｕｃｔ９ ．５ ,ＰＧＰ９－５） 的神经表达。方法：Ｔｒｉｓｏｍｙ １６ 鼠培育;细胞遗传学分析;Ｔｒｉｓｏｍｙ １６ 鼠结肠和ＨＤＰＧＰ９－５ 免疫组织化学。结果：（１）Ｔｒｉｓｏｍｙ １６ 鼠结肠神经系发育异常,肌间神经丛发育迟缓,粘膜下神经丛缺失,结肠末端有５ ｍｍ 的无神经节区,但结肠系膜神经发育良好;（２）ＨＤ狭窄段肠管ＰＧＰ９－５ 阳性神经纤维大量增生,神经节细胞缺如。结论：（１）Ｔｒｉｓｏｍｙ １６ 鼠具有稳定的遗传学特征,可能伴先天性巨结肠。（２） 由于ＨＤ 狭窄段肠管神经节细胞缺失,增生的ＰＧＰ９－５ 阳性神经纤维是肠道外源性神经的代偿,对其神经元的性质尚有待确定。（３）ＨＤ有遗传倾向     

剖宫产术后晚期产后出血11例分析

1临床资料1991-01/2004-10我院发生剖宫产术后晚期产后出血7例,外院转入4例,患者年龄25~34(平均26.6)岁.初产妇8例,经产妇3例,均为子宫下段剖宫产.出血发生在产后8~35(18±6)d(产后2~3wk多见),表现为突然发生阴道出血,鲜红色,出血量500~2500(平均1000)mL.患者均有头晕、心慌,合并出血性休克者6例.B超检查10例提示子宫下段切口有暗区,1例提示有胎盘残留.本组11例均用宫缩剂和广谱抗生素治疗,出血多或伴有休克者予输血,最多者输血2500mL,1例在B超监测下行清宫术,刮出物经病理证实为胎盘胎膜残留,7例行子宫次全切除术,3例行全子宫切除术.行子…     

A subtraction pipeline for automatic detection of new appearing multiple sclerosis lesions in longitudinal studies

Introduction

Time-series analysis of magnetic resonance images (MRI) is of great value for multiple sclerosis (MS) diagnosis and follow-up. In this paper, we present an unsupervised subtraction approach which incorporates multisequence information to deal with the detection of new MS lesions in longitudinal studies.

Methods

The proposed pipeline for detecting new lesions consists of the following steps: skull stripping, bias field correction, histogram matching, registration, white matter masking, image subtraction, automated thresholding, and postprocessing. We also combine the results of PD-w and T2-w images to reduce false positive detections.

Results

Experimental tests are performed in 20 MS patients with two temporal studies separated 12 (12M) or 48 (48M) months in time. The pipeline achieves very good performance obtaining an overall sensitivity of 0.83 and 0.77 with a false discovery rate (FDR) of 0.14 and 0.18 for the 12M and 48M datasets, respectively. The most difficult situation for the pipeline is the detection of very small lesions where the obtained sensitivity is lower and the FDR higher.

Conclusion

Our fully automated approach is robust and accurate, allowing detection of new appearing MS lesions. We believe that the pipeline can be applied to large collections of images and also be easily adapted to monitor other brain pathologies.     

Prolonged migraine aura without headache arrested by sumatriptan. A case report with further considerations

The case of a 42-year-old woman with prolonged migraine visual aura without headache, whose long-lasting episodes of visual aura were successfully controlled by oral sumatriptan, is reported. Effectiveness of sumatriptan was unequivocal, since, after taking sumatriptan, duration of aura would drop from 1.5 h to approximately 20 min. This case suggests that sumatriptan may cross the blood-brain barrier and block spreading depression.     

Thymus and mediastinal node involvement in childhood langerhans cell histiocytosis: Long‐term follow‐up from the French national cohort

Background

Mediastinal involvement (MI) in Langerhans cell histiocytosis (LCH) has been rarely reported. Here, we describe the clinical, radiological, and biological presentation, and the outcome of childhood LCH with MI.

Method

From the French LCH register, which includes 1,423 patients aged less than 18 years, we retrieved the medical charts of patients with mediastinal enlargement detected on chest X‐rays.

Results

Thirty‐seven patients were retrieved, including 18 males; median age of diagnosis was 0.7 years, and median follow‐up time was 6.2 years. The prevalence of MI varied with the age at diagnosis, ranging from 7% below 1 year old to less than 1% at >5 years. Thirteen cases (35%) were diagnosed because of MI‐related symptoms, including respiratory distress (N = 4), superior venous cava syndrome (N = 2), and/or cough and polypnea (N = 10). CT scans performed in 32 cases at diagnosis showed tracheal compression (N = 5), cava thrombosis (N = 2), and/or calcification (N = 16). All patients presented multi‐system disease at LCH diagnosis, and 35/37 were initially treated with vinblastine and corticosteroids. Death occurred in five cases, due to MI (N = 1) or hematological refractory involvement (N = 4). The overall 5‐year survival was 87.1%, and immunodeficiency was not detected as a sequel.

Conclusions

MI in LCH mainly occurs in young children, and diagnosis was based on CT showing thymus enlargement and calcifications. Pediatr Blood Cancer 2013;60:1759–1765. © 2013 Wiley Periodicals, Inc.     

Detection of clinical interactions between methadone and anti-retroviral compounds using an enantioselective capillary electrophoresis for methadone analysis

A capillary electrophoresis method was developed to detect interactions between methadone and anti-retroviral compounds. Eight subjects, who underwent methadone maintenance treatment in the Province of Alicante (Spain), consented to participate in the present study. Of those, one subject was followed up for 123 days to detect drug-drug interactions. The enantiomers of methadone and those of its main metabolite were conveniently resolved within 4 min using a chiral electrophoresis buffer mixture which consisted of phosphate buffer, pH 5, plus 0.2% highly sulphated-(beta)-cyclodextrin. The effective mobility of the analytes was in the 0.061-0.140 cm(2)/(kV s) range at pH 5. The R-methadone plasma concentration range for seven patients was 91-318 ng/mL, it decreased from 186 to 46 ng/mL in a patient followed-up on commencement of the anti-retroviral therapy, returning to the previous higher levels after progressive dose increases. We conclude that monitoring R-methadone plasma levels can be a useful tool for the dose adjustment of methadone.