小儿遗尿症指南和专家共识质量评价

背景 小儿遗尿症是儿科常见病之一,有效的小儿遗尿症诊疗指南有助于症状的规范化控制和缓解,而制定高质量的临床实践指南是提高小儿遗尿症诊疗水平的重要方式。目的 基于AGREEⅡ和RIGHT工具,评价2010年以来国内外发布的小儿遗尿症指南和专家共识的质量,以期为临床实践及后续指南的制订提供参考。方法 检索中国知网、万方数据知识服务平台、维普网、中国生物医学文献数据库和PubMed(补充检索医脉通、世界卫生组织以及英国国家临床示范研究所等数据库)2010-01-01至2022-01-31公开发表的小儿遗尿症相关的诊疗指南和专家共识。采用AGREEⅡ和RIGHT工具评价纳入文献的方法学和报告质量。根据AGREE Ⅱ工具评价结果,将各指南/共识推荐等级评定为“推荐(A级)”“更新后推荐(B级)”和“不推荐(C级)”。使用组内相关系数(ICC)进行一致性评价。结果 纳入8部指南和5部专家共识。共形成推荐意见185条,其中44条为诊断评估方面的意见,140条为治疗方面的推荐意见,1条为随访推荐意见。AGREEⅡ工具评价结果显示,范围和目的、参与人员、严谨性、清晰性、应用性和独立性6个领域的总得分(报...     

Problem‐maintaining circles: Case illustrations of formulations that truly guide therapy

The cognitive behaviour therapy (CBT) emphasis on treatment relevance in assessment, and on evidence‐based intervention, has led to an increasing focus on problem maintenance factors (vs. precipitants) in both its models of psychopathology and in its individual case formulations. This article describes the reasons for this growing focus, and presents a generic CBT model based on the functional analysis of “problem‐maintaining circles” (PMCs) of causes. Some samples of the profuse literature implicating PMCs in many psychological disorders are presented, and the utility of PMC‐based functional analysis, case formulation, or modelling of psychopathology is advanced, not only as a guide to selection of therapeutic interventions, but as an alternative to standard psychiatric diagnosis. A sampling of a taxonomy of such PMCs is presented. And finally, the clinical application of PMC‐based functional analysis, case formulation, and treatment selection is illustrated in five case illustrations drawn from a recent clinical caseload.     

Undifferentiated Carcinoma of the Parotid Gland in a 10-Month-old Child

Malignant salivary gland tumors in children are very rare. This report describes the autopsy of a child with parotid gland cancer. The patient, a 10 month old girl, was admitted to the Nagoya First Red Cross Hospital with facial nerve palsy. lncisional biopsy of a post-auricular tumor was performed, and undifferentiated carcinoma was diagnosed. The patient died 6 months later of respiratory failure due to pulmonary lymphangitis carcinomatosis. Light and electron microscopic and immunohistochemical examinations of the tumor tissue were performed. The tumor cells were arranged in a medullary, sheet-like manner. Keratinization or mucus lakes were not observed. PAS-alcian blue staining demonstrated intracytoplasmic mucus as granules, and also small intercellular droplets of mucus that might otherwise have been unnoticed. Ultrastructurally, some of the tumor cells had tonofilament-like keratin filaments, and also small hollow spaces bounded by microvilli and containing secretory particles. These were stained by antisera against CEA and keratin. These findings are suggestive of differentiation to mucoepider-moid carcinoma. We also review and discuss malignant salivary tumors of epithelial origin in children. Acta Pathol Jpn 40: 149–152, 1990.     

30例麻疹暴发疫情的调查分析

目的调查本地某乡镇一次麻疹疫情的流行强度,描述其三间分布,分析暴发原因及影响因素。方法现场流行病学调查,酶联免疫捕获法检测麻疹IgM抗体,作免疫空白与麻疹发病关联的病例对照研究。结果2006年5月～7月,该乡镇发生30例麻疹局部暴发,其中29例儿童麻疹,1例成人麻疹,5例麻疹IgM抗体阳性。发病地点主要为小学和和幼儿园,罹患率分别为7.94%、7.87%。免疫空白与发病关系研究表明,初种免疫空白OR=7.2,X2=6.84,复种免疫空白OR=11.0,X2=9.48;影响免疫空白形成的社会因素调查,未接受麻疹疫苗初种或复种儿童的父母双亲常年在外务工占82.4%(28/34),有麻疹疫苗接种史儿童的父母常年在外务工占35.3%(12/34),二者有统计学差异(X2=15.54)。结论免疫空白是麻疹暴发形成的重要因素,农村儿童的父母常年在外务工形成隔代抚养教育对计免工作有负面影响。     

A case of de novo interstitial deletion of chromosome 9(p12p13)

The present paper describes a girl with a small de novo deletion of chromosome 9(p12p13). This deletion has not been published previously. The deleted fragment is clearly outside the region involved in the so-called deletion 9p syndrome. The patient had mild dysmorphic features and feeding problems during the first weeks of life, but is now developing well. Because of the lack of severe clinical features in this patient, we speculate that the deletion may be prevalent in other patients who have no clinical indication for chromosome investigation.     

持续的躯体形式疼痛障碍患者抑郁症状的特征及治疗

目的：了解躯体形式疼痛障碍患者伴发的抑郁症状的特征，明确氟西汀对抑郁及焦虑症状的作用特点。方法：选取62例符合ICD－10中持续的躯体形式疼痛障碍诊断标准的患者，于治疗前及治疗第2，4，6周末评定HAMD，HAMA。疗效评定以减分率及有效率为评定指标。结果：氟西汀治疗第六周末，有效率为79．03％，治疗前及治疗第6周末的HAMD各项目分均有显著性差异（P值均＜0．01）；氟西汀治疗六周后，HAMD三个因子减分率分别为：迟滞（75．8％），焦虑／躯体性(焦虑（69．4％），体重（45．2％）；HAMA二个因子减分率分别为：精神性焦虑（70．97％），躯体性焦虑（72．58％）。结论：氟西汀对躯体形式疼痛障碍患者的抑郁及焦虑症状均有缓解作用。氟西汀对躯体疼痛障碍患者的动力缺乏亦有较好疗效。     

病例组合指数的内涵及应用探讨

国内普遍认为病例组合指数(CMI)属于医疗服务能力维度,体现收治疾病的疑难复杂程度和医疗服务的整体技术难度。通过结构方程模型分析发现,CMI与时间消耗指数、费用消耗指数等服务效率维度指标联系更紧密,与DRG组数、入组病例数及总权重等服务能力维度指标联系相对较弱,故应归属医疗服务效率维度。CMI的直接应用主要是对平均住院费用的预测控制及对科室绩效的评价;CMI的间接应用主要是对管理指标的调整/校正。医院管理者应准确把握CMI内涵,恰当应用CMI对不同层级及不同类别住院费用进行预测、评价与控制,并对其他管理指标进行调整后再评价。     

患者知情同意权代为行使的案例分析及启示

以医疗典型纠纷案例为例,通过厘清相关法律对患者知情同意权行使及代为行使的相关规定,探讨知情同意权代为行使行为的界定。启示如下:知情同意权代为行使应设前置条件进行限制和约束;代为行使主体包括近亲属或关系人、委托代理人;患者本人意见具有首要优先性,委托代理人意见应优先于近亲属意见。     

胎儿脑灰质异位合并FLNA基因突变病例报告

报告1例产前超声联合胎儿颅脑磁共振成像（magnetic resonance imaging,MRI）诊断胎儿脑灰质异位合并细丝蛋白A（filamin A,FLNA）基因突变的病例。患者超声检测结果显示侧脑室外壁凹凸不平,颅后窝池宽,透明隔间腔略窄,小脑延髓池轻度增宽。MRI诊断结果为胎儿脑灰质异位合并大枕大池。羊水穿刺获得脱落细胞,采用全外显子测序和基因拷贝数变异（copy number variations,CNV）分析,显示与脑灰质异位疾病相关的FLNA基因外显子出现缺失,其为可能导致脑灰质异位的致病基因。因此,超声结合胎儿颅脑MRI能有效提高脑灰质异位的诊断率,其中染色体检查可筛查出部分致病原因,并对下次妊娠提供帮助。该病例可丰富临床医生对胎儿大脑灰质异位的产前超声检查、合并畸形、临床表现等的认识。