济宁地区21-三体综合征患儿致家庭负担情况调查

目的 调查济宁地区21-三体综合征患儿致家庭经济负担及精神负担情况。方法 2013年1-8月对济宁市78例21-三体综合征患儿的170名陪护人（试验组）及同期济宁市年龄与患儿相仿的、近期无任何重大疾病的100例健康儿童的190名陪护人（对照组）进行问卷调查。采用生活满意度评定量表（LSR）及生活满意度指数A（LSIA）评定两组陪护人生活满意度,采用症状自评量表（SCL-90）评定两组家庭负担情况。结果 试验组农村地区和城镇地区患儿治疗支出占家庭收入的68.56%、55.91%。试验组陪护人LSR、LSIA评分均低于对照组（P＜0.05）|试验组农村地区陪护人LSIA评分低于城镇地区,差异有统计学意义（P＜0.05）|试验组陪护人SCL-90中躯体化、人际关系、抑郁、焦虑及精神病性5个因子评分均低于对照组,差异均有统计学意义（P＜0.05）|试验组农村地区陪护人SCL-90中仅人际关系因子评分高于城镇地区,差异有统计学意义（P＜0.05）。结论 21-三体综合征患儿不仅会给家庭带来经济负担,更影响陪护人的生活质量及精神健康状况,应该引起足够的重视。     

Distichiasis: An update on etiology,treatment and outcomes

Distichiasis, an extra row of eyelashes emerging from meibomian gland orifices, occurs due to the metaplastic transition of sebaceous glands into the pilosebaceous unit. It can present congenitally, such as in lymphedema distichiasis syndrome, or secondary to acquired conditions, such as cicatrizing conjunctivitis, trachoma. This review summarizes the etiology of distichiasis, its presentation, the evolution of various surgical techniques, and their outcomes in human and animal eyes. The published literature has focused on the different treatment modalities and their outcomes; the etiopathogenesis of this condition remains elusive. Truncating mutations (missense, frameshift, and nonsense) in the Forkhead family gene FOXC2 are involved in the distichiasis–lymphedema syndrome. The treatment options are no different for congenital versus acquired distichiasis, with no specific available algorithms. Acquired distichiasis in cicatrizing ocular surface diseases is difficult to manage, and existing treatment options offer success rates of 50%–60%. The outcomes of electroepilation or direct cryotherapy are not as good as surgical excision of distichiatic lashes after splitting the anterior and posterior lamella under direct visualization. The marginal tarsectomy with or without free tarsoconjunctival graft has shown good results in eyes with congenital and acquired distichiasis. The details of differences between normal and distichiatic lash, depth, or course of distichiatic eyelashes remain largely unknown. Studies exploring the distichiatic eyelash depth might improve the outcomes of blind procedures such as cryotherapy or radiofrequency-assisted epilation.     

小儿先天性心脏病行64排螺旋CT心脏检查的观察与护理

目的探讨64排螺旋CT在小儿先天性心脏病检查中的有效护理措施。方法分析36例先天性心脏病患儿接受64螺旋CT检查扫描的资料,并分析其检查前后护理过程。结果36例患儿均成功完成心脏64排CT扫描,无不良反应发生。结论检查前的准备,检查中及检查后细致有效的护理是成功完成小儿心脏CT扫描检查的关键。     

未闭动脉导管外科治疗300例报告

本文总结300例未闭动脉导管的手术治疗经验。300例中，合并其它先心病11例；风心病二尖瓣狭窄1例；另1例为他院手术后并发假性动脉瘤和再通。所有病例除4例为切断缝合外，均采用双重结扎法处理。全组无术中术后大出血和假性动脉瘤发生。手术死亡3例，术后再通4例。作者认为，粗大导管伴肺高压者，术中降温、降压以及操作慎重细致并结扎紧密牢固可避免术中大出血和减少术后再通。     

先天性颅面裂隙畸形的诊断和分类

目的 对先天性颅面裂隙畸形进行分类和诊断，有利于区分畸形的部位和程度，为手术修复提供指导。方法 用Tessier颅面裂分类的基本方法，对81例先天性颅面裂隙畸形进行分析，再根据畸形发生的部位、性状、CT扫描等临床检查结果，按照受累的范围，选用S(皮肤)、T(皮下组织)、O(颅面骨)等字母组合表示畸形部位和性状，选用阿拉伯数字的大小表示畸形的严重程度，作为Tessier分类的补充。结果 以STO分类法分析81例病例，眶下方畸形以Ⅲ、Ⅳ号裂多见(24．70％)，眶上方畸形以IX、X号裂多见(38．27％)，各种组织的受累程度并未表现出一定的规律性。结论STO分类法是Tessier分类法的补充，可为颅面裂隙畸形的修复提供依据。     

Congenital biliary dilatation may consist of 2 disease entities

Background/Purpose

This study aims to establish the possible mechanisms of pathogenesis of congenital biliary dilatation and to classify the disease accordingly.

Methods

Radiologic features of congenital biliary dilatation and pancreaticobiliary malunion in 107 affected children were examined and correlated with laboratory results. Relative lengths/diameters were calculated to provide comparison between children of different ages. Intraluminal pressures of common bile duct (CBD) were measured intraoperatively.

Results

The minimal relative diameters of distal CBD negatively correlated with the maximal relative diameters/lengths of dilated CBD, the maximal relative diameters of common hepatic duct, and left/right hepatic ducts. The intraluminal pressure in patients with a stenotic distal CBD (stenotic group) was significantly higher than that in patients with a nonstenotic distal CBD (nonstenotic group). The narrower the distal CBD, the more deranged the liver function. Conversely, serum/bile amylase levels were more elevated in the nonstenotic group. Common channel protein plugs were only found in the nonstenotic group, whereas common hepatic duct strictures, intrahepatic duct dilatations, and calculi were detected more frequently in the stenotic group.

Conclusion

We propose to categorize congenital biliary dilatation into 2 subgroups: (1) cystic type with stenotic distal CBD associated with deranged liver function and common hepatic duct stricture and (2) fusiform type with nonstenotic distal CBD associated with pancreatitis and common channel protein plugs. Different underlying pathologies of each group require different operative strategies.     

Intracardiac Eustachian Valve Cyst in an Adult Detected with Other Cardiac Anomalies: Usefulness of Multidetector CT in Diagnosis

We present an unusual case of an intracardiac Eustachian valve cyst observed concurrently with atresia of the coronary sinus ostium, a persistent left superior vena cava (LSVC) and a bicuspid aortic valve. There have been several echocardiographic reports of Eustachian valve cysts; however, there is no report of multidetector computed tomography (MDCT) findings related to a Eustachian valve cyst. Recently, we observed a Eustachian valve cyst diagnosed on MDCT showing a hypodense cyst at the characteristic location of the Eustachian valve (the junction of the right atrium and inferior vena cava). MDCT also demonstrated additional cardiovascular anomalies including atresia of the coronary sinus ostium and a persistent LSVC and bicuspid aortic valve.     

老年人肝内胆汁瘤6例治疗体会

目的 探讨老年人胆汁瘤的治疗方法及其效果.方法 患者共6例,术中均将相通胆管关闭后,行囊肿开窗引流术.结果 6例患者均痊愈出院,随访无复发.结论 将相通胆管关闭后行囊肿开窗引流术是治疗部分老年人胆汁瘤的一种安全有效的方法.