317 例儿童化脓性脑膜炎临床分析

目的 研究化脓性脑膜炎(PM)患儿的临床特点及治疗转归。方法 对317 例年龄在1 个月至15 岁的PM 患儿的病例资料进行回顾性分析。结果 PM 发病以婴儿(198 例,62.6%)居多,多有呼吸道前驱感染(171 例,53.9%)。临床以发热、惊厥、颅内高压为主要表现,惊厥在婴儿中发生率较高(152 例,93.6%)。主要并发症为硬膜下积液(95 例,29.9%),其中22 例头颅影像学未提示而直接通过硬膜下穿刺诊断;68 例行硬膜下穿刺,其中62 例于穿刺后3~5 d 内体温恢复正常。多因素logisic 回归分析显示,年龄、CSF蛋白≥ 1 g/L 是影响PM 患儿并发症和后遗症发生的主要因素(分别OR=0.518、1.524,均P<0.05)。治疗上初诊时以第三代头孢为首选用药,万古霉素、碳青霉烯类可作为替代。在对部分患儿出院后1~3 个月的随访发现,14.4%(13/90)发生延迟血管炎反应。结论 PM 多发于婴儿,惊厥在婴儿中易发生。小婴儿、CSF 蛋白≥ 1 g/L 时会增加PM 患儿并发症和后遗症的发生风险。硬膜下穿刺对硬膜下积液的诊断及治疗均有价值。部分治愈患儿存在延迟血管炎反应,应在出院后1~3 个月内进行随访。     

新生儿锁骨下静脉穿刺的临床解剖研究

目的:为提高新生儿锁骨下静脉穿刺术的成功率,避免其邻近结构损伤形成的并发症。方法:对50例新生儿尸体锁骨下静脉及其相关结构进行了观察和测量。结果:锁骨下静脉起始处外径4.6±0.8mm,末端外径6.2±0.8mm,长度为18.6±2.0mm。锁骨下静脉与锁骨下缘交点处皮肤至锁骨下静脉前壁的垂直距离为10.0±1.4mm。锁骨下静脉与锁骨下面交点的角度为38.2±11. 4°,交点指数为39.1％。结论:新生儿锁骨下静脉穿刺进针部位应以锁骨内、中1/3交点的稍外侧为宜。     

门静脉的体表定位及其临床意义

目的：为超声波检查门静脉或经皮经肝门静脉穿刺提供解剖学基础。方法：在４０例成人尸体标本上观测了门静脉的行程及其分叉位置的体表投影。结果：门静脉肝外段与身体的垂线呈约４０°角；门静脉分叉位置在经右半胸宽中点的垂线与右锁骨中线上肝高中点的水平线的交点附近；门静脉右支分为前、后支的位置在剑突尖平面下方约２ｃｍ，右锁骨中线上肝高的中点附近；门静脉左支分出第１外侧支的位置在剑突尖稍下方的右侧约２ｃｍ。结论：在右腋中线剑突尖平面下方约２ｃｍ经皮经肝穿刺至锁骨中线，导管即可进入门静脉右支内     

儿童中枢神经系统感染治疗疗程与腰椎穿刺检查系列建议之一——病毒性脑（膜）炎治疗疗程与腰椎穿刺检查建议

中枢神经系统感染是儿童神经系统常见疾病,也是临床住院的常见病种;中枢神经系统感染的病原以细菌和病毒感染较为常见,而结核菌、真菌、支原体感染相对少见;不同病原的中枢神经系统感染临床上有其共性表现又有不同病原感染疾病的特点;中枢神经系统感染的诊断包括病原学确诊或临床疑诊;不同的治疗转归其治疗疗程或腰穿检查与复查时间均缺乏研究性证据,困扰临床一线工作。为此,中华医学会儿科学分会神经学组组织的专家组制订了“儿童中枢神经系统感染治疗疗程与腰椎穿刺检查系列建议”,希望能为这一疾病的诊治提供参考。     

How to calculate human muscle fibre areas in biopsy samples—methodological considerations

Cross-sectional muscle fibre areas (type I, IIA and IIB) were determined in duplicate biopsies from the left vastus lateralis (n=11) and in biopsies from right and left vastus lateralis (n=8).The SD for the difference in means between duplicate biopsies was 510 μm² for type I, 1020 μm² for type II A and 860 μm² for type II B.Expressed as coefficient of variation (CV) these SD constituted 10, 15 and 15%, respectively. The variation in fibre size within a sample was considerably less than the variation between samples on the assumption that at least 15–20 areas of each fibre type were measured per sample. No difference in mean fibre area for type I, IIA and IIB fibres was obtained between the right and left muscle. Several artefacts due to the sampling and preparing procedures are discussed and a method for determining muscle fibre areas in biopsy samples is suggested.     

A BAFfling liver aspirate: Metastatic high grade SMARCA4 deficient undifferentiated gastroesophageal junction carcinoma masquerading as a hematolymphoid malignancy

Undifferentiated malignant SMARCA4‐deficient neoplasms are rare, recently characterized, high grade, potentially lethal malignancies. Such tumors are characterized by the loss of BRG1 encoded by SMARCA4, a key component of the Switch/Sucrose Non‐Fermenting (SWI/SNF) chromatin remodeling complex. As this complex, also referred as BAF (BRG1/BRM associated factors) complex, is involved in the epigenetic control of hundreds of genes, including those involved in lineage‐specific differentiation, BAF‐deficient tumors, show minimal or no differentiation and are difficult to classify. Their fine needle aspiration (FNA) cytologic features are still poorly defined. Here, we describe a 70‐year‐old man who presented with thickening of the wall of the distal esophagus and stomach and multiple liver and lung lesions. Liver FNA showed relatively uniform dispersed malignant cells with high nucleus: cytoplasm ratio, scant microvacuolated cytoplasm, eccentric nuclei and prominent nucleoli. Mitoses, necrotic debris, nuclear streak artifact, “ghost cells” and focal rhabdoid cytoplasmic inclusions were also present. The liver core biopsy and GI biopsies demonstrated sinusoidal and respectively submucosal involvement by a high grade undifferentiated malignant neoplasm. The tumor cells were negative for all applied markers on immunohistochemistry and flow cytometry, and only showed CD138 and weak PAX5 staining. After an initial diagnosis of hematolymphoid neoplasm, additional stains showed intact INI1 protein and loss of BRG1 protein immunoexpression, establishing the accurate diagnosis. This case highlights the difficulties and potential pitfalls encountered in the FNA diagnosis of BAF‐deficient tumors, the accurate diagnosis of which is important due to their lack of response to conventional therapy and potential response to targeted therapy.