Choreoacanthocytosis in a Chinese patient--a case report

A 50-year old Chinese woman with the rare neurological disorder of Choreoacanthocytosis is described. Her illness is characterised by seizures, buccolingual dyskinesia, choreiform movements, arreflexia and mild sensorimotor polyneuropathy. Acanthocytes were present in her peripheral blood in large numbers but the serum lipid profile was normal. Her features are consistent with those so far described in Caucasian and Japanese patients. The disease differs from Huntington's chorea in that there are acanthocytes, peripheral neuropathy, and metal function remains relatively intact.     

鼻咽癌常规放疗靶体积合理性的初步探讨

目的分析鼻咽癌常规放疗的局部控制情况和局部复发的剂量学模式,初步探讨照射靶体积确定的合理性.方法共476例初治鼻咽癌患者接受规范化单纯常规根治性放疗.全部病例均采用常规模拟定位,鼻咽靶体积为CT检查所见的原发病灶范围以及可能侵犯的亚临床病灶区域.利用Kaplan-Meier方法计算局部累积复发率;将鼻咽局部复发患者疗前和复发时局部病灶范围（Vnx和Vrecur）勾画于三维治疗计划系统,复制首程放疗的射野并按原处方剂量进行计算,根据剂量体积直方图进行剂量学评价：recurV95（95%处方剂量曲线包括的Vrecur）≥95%定义为野内复发,95%＞recurV95≥20%定义为野边缘复发,20%＞recurV95定义为野外复发.结果局部复发共52例,全组1、2、3、4年局部累积复发率分别为0.6%、3.9%、8.7%、11.5%.对42例局部复发的剂量学分析显示野内复发占多数（52%）,而大部分野边缘复发和全部野外复发均与射野设置不当、影像学阅片能力欠缺使首程放疗剂量不足有关.结论较好的局部控制率、复发的剂量学模式的分析结果提示该靶体积的设置比较合理.提高影像阅片能力,准确的射野设计,充分利用生物影像学工具,有针对性地提高照射剂量,有望进一步提高局部控制率.     

The remucosalizing alar cartilage flap: a reconstructive option for repairing nasal septal perforations

The objective of this study is to assess the results of repairing septal perforations with a vascularized pedicled alar cartilage island flap. Using the external rhinoplasty approach, a vascularized flap of alar cartilage, harvested as a cephalic trim and pedicled on the ascending columellar branches of the superior labial artery was raised. Bilateral mucoperichondrial septal flaps were elevated and the alar flap was transposed and secured within the defect and bilaterally overlaid with temporalis fascia. Silastic sheets were placed and remained in situ until the grafts were revascularized from the peripheries of the defect as well as centrally from the alar flap. The revascularized temporalis fascia acted as a scaffold for nasal remucosalization. The alar flap also increased the long-term structural robustness of the repair. Between 1999 and 2003, 14 patients with septal perforations ranging from 10 to 31 mm underwent septal reconstruction using this technique. There were nine males and five females. The flap was successfully raised in all cases and long-term closure was maintained in 12 patients (86%). The alar cartilage flap is an effective technique for repairing septal perforations in selected patients. It provides vascularized tissue which nourishes the grafts during remucosalization, and a cartilaginous framework, which affords long-term structural support to the repair. It also obviates the need to transpose nasal mucosa and create a secondary defect. The rhinoplasty approach furthermore permits additional nasal deformities to be corrected at the same time. Presented at the British Association of Plastic Surgeons Summer Scientific Meeting, Sheffield, UK (12 July 2006).     

The retinae of Prototherian mammals possess neuronal types that are characteristic of non-mammalian retinae

This study has shown that the retinae of Prototherian (egg-laying) mammals possess two neuronal types that are present in non-mammalian retinae, but absent or morphologically different in the retinae of Eutherian (placental) mammals. First, endogenous serotonin-like immunoreactivity has been localized in a population of presumptive amacrine cells in the platypus retina, the first such report in a mammalian retina. Second, the protein kinase C-immunoreactive (PKC-IR) bipolar cells in the echidna retina appear similar to the PKC-IR bipolars in the chicken retina, in that their dendrites give rise to a Landolt's club and their axons are multistratified. By contrast, the PKC-IR rod bipolar cells in the rabbit and in the brushtail possum, a Metatherian (marsupial) mammal, have no Landolt's clubs and their axons form terminal lobes in the innermost stratum of the inner plexiform layer.     

Transforming Growth Factor-β1 Induces Apoptosis through Down-Regulation of c-mycGene and Overexpression of p27Protein in Cervical Carcinoma

Transforming growth factor-β1 (TGF-β1) is known to be a potent growth inhibitor for many cell types, including most epithelial cells. In skin keratinocytes, TGF-β1 has been shown to inhibit growth and to rapidly reduce c-mycexpression. However, the molecular mechanism of TGF-β1 action on cell growth of cervical carcinoma has not yet been elucidated. We thus assessed the effect of TGF-β1 on the growth of cervical carcinoma cell lines. Two cervical squamous carcinoma cell lines, CUMC-3 and CUMC-6, were incubated with varying concentrations of TGF-β1, and growth inhibition was evaluated with tetrazolium-based colorimetric assay. After culture in TGF-β1 for 24 h, inhibition of growth was detected in a dose-dependent manner at concentrations of 0.1–10 ng/ml in both cell lines. This effect of TGF-β1 on cultured carcinoma cells was associated with apoptotic process including oligonucleosomal ladder DNA and apoptotic body formations. Northern blot analysis revealed c-mycmRNA expression was suppressed by 10 ng/ml of TGF-β1 following 3 h of treatment in both cell lines. Western blot analysis showed that the level of p27^Kip1protein was increased after TGF-β1 treatment in both cell lines. These results suggest that the mechanisms by which TGF-β1 inhibits the growth of cervical carcinoma are complex and may include effects on down-regulation of c-mycgene, and overexpression of p27^Kip1protein.