天津及周边地区胆道闭锁的流行病学调查

目的通过对本院近年来收治的胆道闭锁患儿临床资料的流行病学调查,分析天津及周边地区胆道闭锁的发病趋势。方法收集1995年1月至2011年12月作者收治的经术中胆道造影确诊的168例胆道闭锁患儿临床资料,通过检索病历、电话随访及门诊复查相结合的方式获得研究资料,进行流行病学调查。应用SPSS16.0统计学软件进行数据分析,以P〈0.05为差异有统计学意义。结果 ①天津及周边地区胆道闭锁的发病率呈现逐年上升趋势。 ②天津市各郊县基层医院发现或怀疑胆道闭锁并转诊的患儿数量呈现明显差异。 ③首次就诊于基层医院的患儿平均手术年龄明显高于首次就诊于本院的患儿（P≤0.05）,平均手术年龄呈现逐年降低趋势。结论近年来胆道闭锁的发病率明显升高,手术年龄明显降低,分析原因可能与迅速上升的经济水平、不断增强的健康意识及广大家长和医师对胆道闭锁认识的提高有密切关系。     

上海地区胆道闭锁筛查报告

目的探讨基于婴儿大便色卡的筛查方案在上海地区进行胆道闭锁筛查的可行性和有效性。方法于2015年1月1日至2016年10月31日向在上海四家产科医院出生的新生儿家长发放新生儿大便色卡并宣教使用方法。新生儿回家后,家长若发现存在黄疸且大便颜色异常,可直接联系胆道闭锁筛查中心进行进一步检查。若未联系,产科医院会于新生儿42 d回访时回收色卡,并按照筛查方案向本筛查中心传报疑似病例,由筛查中心追踪至诊断或排除胆道闭锁。所有确诊胆道闭锁患儿均需经胆道造影和/或胆道探查明确诊断。以家长在42 d回访前联系胆道闭锁筛查中心并被识别或在42 d回访时被产科医院识别并传报为胆道闭锁患儿被成功筛查的标准。统计上海地区胆道闭锁发病情况,并通过计算本次筛查敏感度、特异性、手术日龄及早期手术率,分析其对胆道闭锁早期诊断及治疗的影响。结果上海市四家胆道闭锁诊治中心数据汇总显示,本次研究期间上海地区总共出生41例胆道闭锁患儿,发病率为10.86/10万活婴(41/377 420,95%CI:7.80～17.74)。共有95例疑似患儿被筛查中心识别,11例患儿被确诊为胆道闭锁,其中9例在42 d回访前自行联系本中心;...     

GPC1在胆道闭锁患儿肝组织中表达水平及临床意义的初步探索

目的初步探讨GPC1在胆道闭锁患儿发病过程中的临床意义。方法选取深圳市儿童医院2016年7月至2018年2月收治的胆道闭锁患儿35例作为观察组,同期选取本院收治的先天性胆总管扩张症患儿42例作为对照组。通过免疫组化结果了解GPC1在胆道闭锁肝脏中的分布情况;通过qPCR及Western Blot对胆道闭锁(BA)及先天性胆总管扩张症(CC)患儿肝脏中的CGPC1蛋白水平、GPC1 mRNA水平进行检测。结果本研究结果显示GPC1主要分布在胆道闭锁肝脏胆管细胞顶部,观察组和对照组患儿GPC1 mRNA表达水平(0.65±0.05 vs. 2.54±0. 12,P0.05)、蛋白表达量(1.31±0.14 vs. 2.83±0.25,P0.05)比较,差异有统计学意义。结论 GPC1 mRNA和蛋白产物的低表达可能与胆道闭锁的发病密切相关。     

胆道闭锁免疫失调机制的研究进展

胆道闭锁是一类累及新生儿肝胆系统的危害严重的疾病,该病在东亚民族高发.目前认为可能与遗传易感、病毒感染及免疫失调等有关,其中免疫失调可能是胆道闭锁发病的中心环节.病毒感染而继发胆管上皮自身免疫炎症损伤的学说目前逐渐获得国内外同行的认可,现对胆道闭锁免疫损伤失调机制的研究进展进行综述.     

新生儿巨细胞病毒感染与胆道闭锁肝脏纤维化的相关研究

目的探讨新生儿巨细胞病毒感染与胆道闭锁肝脏损伤的关系。方法回顾我院2004年1月-2005年1月收治21例胆道闭锁患儿临床资料。对肝组织纤维化和肝细胞变性坏死程度分级，同时进行肝脏和肝门纤维块巨细胞病毒-pp65免疫荧光染色。根据血清及病毒学检查结果将病人分组，比较两组患儿肝功能，肝脏纤维化，肝细胞破坏程度。结果巨细胞病毒感染组13例，非感染组8例。两组肝功能各指标除总胆红素，γ-谷氨酰转肽酶外无明显差异。巨细胞病毒感染组肝脏纤维化程度重于非感染组（P〈0．05）。结论巨细胞病毒活动性感染加重胆道闭锁患儿胆汁淤积和肝脏的纤维化。     

Treg/Th17细胞失衡在胆道闭锁发病机制中的研究进展

胆道闭锁是引起婴儿期梗阻性黄疸的疾病之一,主要累及肝内外胆管,其发病原因尚不明确。免疫反应异常可能是胆道闭锁发病的原因之一,近年来发现调节性T细胞（regulatory T cell,Treg）/辅助性T细胞17 （helper T cell 17,Th17）比例失衡可加重胆管炎性反应,促进肝纤维化,与胆道闭锁发生、发...     

胆道闭锁形态学研究及诊治进展北大核心CSCD

胆道闭锁是以炎症、纤维化及肝外胆道闭塞为特征的一种进行性的炎性胆道疾病.作为新生儿期的特发病,胆道闭锁的诊治有其特殊性,肝脏及肝内外胆道的组织形态学研究作为提高临床诊治水平的突破口,一直以来都是小儿外科领域的研究热点和难点.现从形态学角度加深对这一疾病的认识,并了解近年来国内外同道在胆道闭锁诊治方面的研究进展.     

胆汁酸在胆道闭锁疾病中的研究进展

胆道闭锁(biliary atresia)是以进行性肝内外胆管纤维化为特征的疾病,是导致婴幼儿终末期肝病的重要原因,目前病因及发病机制尚不明确.研究表明,胆汁酸可以用于胆道闭锁早期筛查、预后评估及胆管炎诊断.本文对近些年胆汁酸在胆道闭锁中的相关研究进展进行综述.     

单基因表达异常与胆道闭锁发病的研究进展

胆道闭锁是以肝内、外胆管进行性破坏和肝纤维性梗阻为特征的一种严重疾病, 手术是目前唯一的治疗方式。胆道闭锁的发生与地区及人种有关, 遗传易感因素是胆道闭锁病因之一。本文总结了近年来单基因表达异常与胆道闭锁关系的研究进展, 并分别从JAG1、PKHD1、ADD3、PROM1及GPC1等基因的表达异常与胆道闭锁发病的关系进行综述。     

胆道闭锁肝内毛细胆管HLA-DR表达免疫电镜研究

目的用免疫电镜技术检测胆道闭锁患儿肝内毛细胆管微绒毛人类白细胞抗原DR(HLA-DR)表达的变化。方法对14例胆道闭锁及12例对照组婴儿的肝活检标本,行肝内毛细胆管HLA-DR免疫电镜染色研究,并结合临床预后进行分析。结果14例胆道闭锁患儿中毛细胆管微绒毛表达HLA-DR阳性8例,对照组均不表达HLA-DR,差异有显著性(P<0.05),胆道闭锁肝内毛细胆管微绒毛HLA-DR表达与预后呈负相关(γ=-0.748,P<0.05)。结论免疫电镜发现,胆道闭锁患儿肝内毛细胆管微绒毛有HLA-DR异常表达,可能与局部的免疫应答和本病的发病机理有关。     

Pubertal growth and development and prenatal and lactational exposure to polychlorinated biphenyls and dichlorodiphenyl dichloroethene

OBJECTIVES: Polychlorinated biphenyls (PCBs) and dichlorodiphenyl dichloroethene (DDE) are ubiquitous toxic environmental contaminants. Prenatal and early life exposures affect pubertal events in experimental animals. We studied whether prenatal or lactational exposures to background levels of PCBs or DDE were associated with altered pubertal growth and development in humans.Study design: Follow-up of 594 children from an existing North Carolina cohort whose prenatal and lactational exposures had previously been measured. Height, weight, and stage of pubertal development were assessed through annual mail questionnaires. RESULTS: Height of boys at puberty increased with transplacental exposure to DDE, as did weight adjusted for height; adjusted means for those with the highest exposures (maternal concentration 4+ ppm fat) were 6.3 cm taller and 6.9 kg larger than those with the lowest (0 to 1 ppm). There was no effect on the ages at which pubertal stages were attained. Lactational exposures to DDE had no apparent effects; neither did transplacental or lactational exposure to PCBs. Girls with the highest transplacental PCB exposures were heavier for their heights than other girls by 5.4 kg, but differences were significant only if the analysis was restricted to white girls. CONCLUSIONS: Prenatal exposures at background levels may affect body size at puberty.     

Prevention and treatment of overweight and obesity in children and adolescents

Increasing numbers of obese children and adolescents all over the world demand an investment in the primary and secondary prevention of obesity and overweight in this age group. The goal of preventive measures in children is to avoid the negative short- and long-term health problems associated with obesity. Primary prevention aims at establishing a healthy, active lifestyle and keeping children and adolescents within a range of body weight which is considered to be healthy. Constant availability and affordability of palatable and energy-dense food in the affluent society of the western world demands preventive strategies. Universal or public health prevention seems to be the most suitable form because several other cofactors of morbidity and mortality of affluent societies can also be prevented. However, in most European countries there is a lack of awareness of the necessity of prevention programmes, not only among the general population but also among the medical society. More awareness and consciousness to the problem of obesity must be generated in order to lead to effective therapeutic programmes. For those children and adolescents who are already obese, secondary prevention is mandatory. Therapeutic intervention programmes for the obese aim at long-term weight maintenance and normalisation of body weight and body fat. They have to modify eating and exercise behaviour of the obese child and establish new, healthier behaviour and lifestyle. Treatments programmes must include behavioural components in order to permanently change nutrition and physical exercise of the obese children and adolescents. However, long-term results of treatment programmes in European countries are scarce and the reported results, even of multidisciplinary regimens, are not impressive. Conclusion In most European countries there is an urgent need not only for a growing awareness of the problem of obesity in children and adolescents but also for development of new comprehensive approaches in treating this group.     

Hypokalemia and Hyperkalemia in Infants and Children: Pathophysiology and Treatment

Potassium is the second most abundant cation in the body. About 98% of potassium is intracellular and that is particularly in the skeletal muscle. Electrical disturbances associated with disorders of potassium homeostasis are a function of both the extracellular and intracellular potassium concentrations. Clinical disorders of potassium homeostasis occur with some regularity, especially in hospitalized patients receiving many medications. This article will review the pathophysiology of potassium homeostasis, symptoms, causes, and treatment of hypo- and hyperkalemia.