第二鳃裂畸形的诊疗进展

鳃裂畸形是颈部第二常见的先天性肿块,其中以第二鳃裂畸形最为常见,临床可表现为囊肿、瘘管、窦道 3种类型,影像学检查可以帮助诊断及鉴别。目前,手术仍是治疗鳃裂畸形的主要方法。该研究就第二鳃裂畸形的发病机制、诊断、治疗进展作一综述。     

脑静脉畸形的MRI表现及其诊断价值

目的 探讨脑静脉畸形的MRI表现,评价各成像序列的诊断价值。资料与方法 搜集经临床手术证实的脑静脉畸形8例进行回顾性分析,所有患者均进行了常规MRI平扫及3D-MOTSA MRA检查。6例行Gd-DTPA增强T1WI,其中3例行3D-MOTSA增强MRA检查。结果 小脑4例,额叶、顶叶、枕叶共4例。5例MRI平扫引流静脉为长T1短T2流空信号,3例呈长T1低信号、长T2高信号。扩张的髓静脉为网状及条状长T1低信号、长T2高信号,增强扫描呈“海蛇头”样改变,即多条髓静脉呈辐射状汇入粗大的引流静脉。3D-MOTSA MRA检查显示部分引流静脉,髓静脉显示较少。3D-MOTSA增强MRA检查引流静脉全程显示,髓静脉显示数目多。结论 MRI能明确诊断脑静脉畸形的合并症,弥补脑血管造影的不足,3D-MOTSA增强MRA检查可取代脑血管造影。     

内耳骨迷路畸形的HRCT表现

目的 探讨先天性内耳骨迷路畸形的高分辨率CT（HRCT）特征。资料与方法 回顾分析28例（56耳）先天性感音性耳聋的内耳骨迷路畸形患者HRCT表现。结果 Michel畸形1耳,囊状耳蜗．前庭畸形（IP-1畸形）8耳,Mondinl畸形（IP-2畸形）17耳,耳蜗未发育4耳,耳蜗发育不全6耳,共腔畸形4耳,前庭．外半规管畸形8耳,前庭导水管扩大8耳。结论 HRCT可显示先天性内耳畸形表现,为内耳畸形的诊断和人工耳蜗植入术适应证的选择提供了依据。     

急诊手术治疗脑动静脉畸形破裂出血32例报告

我院自2002年1月至2005年12月共收治自发性脑出血135例,其中脑动静脉畸形破裂出血32例。全部病例经CTA、MRA、DSA及手术病理证实。现报告如下。1资料与方法1.1一般资料本组32例,男性22例,女性10例。年龄10~20岁5例,21~40岁25例,40岁以上2例。其中有不规则的头痛病史12例,有癫痫     

重复膀胱2例报告并文献复习

目的：提高对重复膀胱的认识及诊疗水平。方法：回顾性分析2例重复膀胱患者的临床资料。2均为女性,年龄21和27岁。分别以下腹部不适和尿频、尿痛就诊。术前诊断1例为左肾萎缩、左输尿管下段张;另1例为膀胱憩室,并发膀胱结石继发感染。结果：1例术中发现左输尿管下端连接一个肌性囊袋,此囊袋与一个盲管相连,将此囊袋和盲管与左肾和输尿管一并切除,病理检查诊断为重复膀胱尿道,左肾萎缩;另1例中见膀胱被一个肌性中隔分为左右两腔,借中隔中下部孔互相连通,切除的中隔病理检查为膀胱组织。2例均术后8天痊愈出院,随访无异常。结论：重复膀胱罕见,常并发其他器官发育异常。影像学检查可为诊断提供助,手术是唯一能根治的方法。     

后路半椎体切除短节段融合固定术治疗儿童腰骶段半椎体畸形及近端代偿侧凸自发矫正的影响因素

目的:评价后路半椎体切除、短节段融合固定术治疗儿童先天性腰骶部半椎体畸形的效果,探讨术后近端代偿侧凸(PCC)自发矫正的影响因素.方法:回顾性分析2012年1月～2018年12月我院诊治的25例腰骶部半椎体患儿的临床资料,其中男14例,女11例,年龄3.3～13.0岁(6.74±2.81岁),L4～S1半椎体10例,L...     

出生后采血时间对新生儿CAH筛查结果的影响分析

目的 探讨不同出生后采血时间对测定新生儿人群17-羟孕酮(17-OHP)筛查先天性肾上腺皮质增生症(CAH)的影响.方法 采用时间分辨荧光免疫分析方法,检测新生儿末梢血17-OHP浓度.分析不同出生后采血时间足月儿和早产儿中17-OHP水平.结果 出生后＜72hr和72～120hr,早产儿(＜37周)的17-OHP测定值均值都大于30nmol/L.出生后＜72hr、72-120hr,早产儿17-OHP测定值均高于足月儿.出生后72～120hr足月儿组的97th分位数为31.5nmol/L,早产儿组的97th分位数为62.9nmol/L.结论 采血时间对早产新生儿17-OHP测定筛查CAH有一定的影响,建议将出生后3～5天的早产儿的CAH筛查切割值设定为17-OHP＜60nmol/L较好,以减少假阳性率和召回率.     

Toward the elimination of lymphatic filariasis by 2020: treatment update and impact assessment for the endgame

Lymphatic filariasis (LF) is an important public health problem endemic in 73 countries, where it is a major cause of acute and chronic morbidity and a significant impediment to socioeconomic development. It is targeted for elimination by 2020, through preventive chemotherapy using albendazole in combination with either ivermectin or diethylcarbamazine citrate. Preventive chemotherapy enables the regular and coordinated administration of safe, single-dose medications delivered through mass drug administration (MDA). Many countries are now scaling down MDA activities after achieving 100% geographic coverage and instituting monitoring and evaluation procedures to establish the impact of several consecutive rounds of MDA and determine if transmission has been interrupted. At the same time, countries yet to initiate MDA for elimination of LF will adopt improved mapping and coverage assessment protocols to accelerate the efforts for achieving global elimination by 2020. This review provides an update on treatment for LF and describes the current global status of the elimination efforts, transmission control processes and strategies for measuring impact and continuing surveillance after MDA has ceased.     

Extracardiac Complications in Adults with Congenital Heart Disease

With the increasing number of adults living with repaired, or unrepaired, congenital heart disease, there is a growing incidence of extracardiac comorbidities. These comorbidities can affect various organ systems in complex ways, and may have a significant impact on a patient's quality of life and survival. Many of these potential complications may go undiagnosed until there is already a significant bearing on the patient's life. Therefore, it is important for physicians who care for the adult congenital patient to be mindful of these potential extracardiac complications, and actively assess for these complications in their adult congenital practice. Continued research to identify modifiable risk factors is needed so that both preventative and therapeutic management options for these extracardiac complications may be developed.