Pediatric cardiac surgery and safety,in the past and in the future

This article is about safety in treatment, mostly surgical, for congenital heart disease, with special reference to the Nordic countries: Denmark, Finland, Sweden and Norway. The different factors that influence the safety of doing a procedure for congenital heart disease (CHD) are discussed. In the past, early post procedural mortality was the measuring stick, but nowadays, the mortality is so low, that one needs a very large number to differentiate between groups of treatment. However, the safety of the procedures is still very important, but post procedural morbidity is now more in focus. More complex organizations and increasing demands of efficacy, together with regulations of working hours, will probably change the ways things will be done. It is also underlined that the passive factors (not human) are of increasing importance, and will require health administrators with a deep understanding of the complexity of CHD-treatment, to provide the tools needed. Some changes in education of both cardiologists and surgeons are needed, and also a more close cooperation between them is necessary, in the interest of our patients.     

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1免疫缺陷病原发性免疫缺陷病(PID)的临床筛查工作已在我国部分地区开展,但全国范围内新发病例仍很少,提示儿科医师尚应加强警觉,逐步在相对发达地区基层医院建立免疫缺陷病初筛手段。PID是免疫学研究的天然模型,疾病相关基因的突变与表现型的关系对阐明靶基因的功能十分重要。随着PID病例逐渐累积,将可能在今后数年提供中国大陆PID基因突变特点与疾病表现的初步数据。重庆医科大学儿童医院分析了9例Wiskott Aldrich综合征患儿,发现其中4例患儿存在3种新型突变,但所有患儿均具有典型临床表现,扫描电镜检测其淋巴细胞异常亦相似[1]。其…     

小儿过敏性和免疫性疾病诊治进展

1　原发性免疫缺陷病病因是单个基因突变导致所编码的蛋白质缺失,引起相应的临床表型,即1个基因突变→1个蛋白质缺失→1种疾病的模式。许多单基因遗传病的突变或缺失基因已被定位克隆,突变形式已明确。DNA序列分析是这类疾病最有效的确诊试验。我国发现两种新型Wiskott Aldrich综合征基因突变类型(984delC ,H317fsX4 4 4 )和(1388G→T ,E4 5 2X) ,并被国际疾病基因库收录[1] 。甘露糖结合凝集素(MBL)具有补体早期成分C2 、C3 、C4的功能,在婴儿期补体成分低下时,于感染初期能代替补体发挥非特异性抗感染功能。已发现MBL基因外显子1…     

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1原发性免疫缺陷病儿科医生对原发性免疫缺陷病(PID)的认识较前有了提高,各地报道的病例较以往增多,但关于PID统一疾病登记工作未能按计划进行。7例典型X连锁无丙种球蛋白血症(XLA)BTK基因突变中,4例为未见报道的新突变(546插入ACACTGATCT,1900A→T,472缺失TTCTCCCC832C,1904A→C)[1]。甘露糖结合凝集素(MBL)的功能类似IgM或IgG,发挥调理素作用。2岁以前IgG2发育不成熟,此时若有MBL-54密码子突变,导致低MBL血症,可能会出现反复呼吸道感染[2]。2新生儿免疫新生儿缺氧缺血性脑病患儿血清TNF-α、IL-1β、IL-6、IL-8、…     

Muslim patients and health disparities in the UK and the US

This article provides a framework for understanding how Muslim identity, and the current social and political contexts in which it is shaped, affects the health of Muslims in the UK and the US, and the quality of health care they receive. Key medical and public health literature that addresses health concerns related to Muslim communities in the UK and the US is reviewed. Few data exist specific to health disparities for Muslim minorities. However, the article focuses on emerging studies concerning the consequences of "Islamophobia" for the physical and mental health and health care of Muslim families and children. We argue that, despite substantive structural differences in the health care systems of the UK and the US, social structural and political forces play similar roles in the health of Muslim children in both countries. Finally, we call for significant cultural and institutional adjustments in health care settings and further research studies to provide specific data to address health disparities for these growing and diverse populations.     

Physiologic variations in plasma osteocalcin in the child and the adolescent

Osteocalcin, the most abundant of non-collagen proteins in bone, is produced by osteoblasts and incorporated into the bone matrix. A small amount is circulated in the bloodstream where it can be detected by radioimmunologic assay. Serum assays in 128 children and adolescents showed that serum osteocalcin levels accurately reflect the various stages of statural growth: peak levels were found at three different ages, i.e. infants (21.8 +/- 4.9 ng/ml), children over seven (19.4 +/- 5.2 ng/ml), and adolescents undergoing the early stages of puberty (22.5 +/- 4.2 ng/ml). Although significant differences were found between each age group and the preceding or following group, these results can be applied to individual patients only for longitudinal studies.     

Changes in the neonatal and infant mortality rate and the causes of death in Korea

Neonatal mortality rate (NMR) or infant mortality rate (IMR) are the rate of deaths per 1,000 live births at which babies of either less than four weeks or of one year of age die, respectively. The NMR and IMR are commonly accepted as a measure of the general health and wellbeing of a population. Korea's NMR and IMR fell significantly between 1993 and 2009 from 6.6 and 9.9 to 1.7 and 3.2, respectively. Common causes of infantile death in 2008 had decreased compared with those in 1996 such as other disorders originating in the perinatal period, congenital malformation of the heart, bacterial sepsis of newborns, disorders related to length of gestation and fetal growth, intra-uterine hypoxia, birth asphyxia. However, some other causes are on the increase, such as respiratory distress of newborn, other respiratory conditions originating in the perinatal period, other congenital malformation, diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism. In this study, we provide basic data about changes of NMR and IMR and the causes of neonatal and infantile death from 1983 to 2009 in Korea.     

Ovarian cysts in the fetus and neonate—changes in sonographic pattern in the follow-up and their management

In a multicenter trial we retrospectively evaluated the clinical and sonographic data of 49 neonatal ovarian cysts, 44 of which were detected prenatally and 5 on the first day after delivery. Of the 44 prenatally detected cysts 39 were purely cystic, 5 echogenic or had a mixed pattern. In 20 patients the cystic appearance changed during delivery from purely cystic to a mixed pattern being independent on the size of the cyst. 26 of the 44 cysts were treated surgically. Salpingotorsion was found in 8 and was independent on the size of the cyst. In 15 a salpingo-oophorectomy or oophorectomy was performed, in 11 the ovary was saved. 23 patients were followed sonographically: 15 cysts showed complete resolution within 14 months without correlation to the sonographic pattern. The volume of these cysts varied between 5 and 71 ml. Neonatal ovarian cysts disappear spontaneously frequently and rarely cause severe symptoms. The authors recommend follow-up by ultrasound as the primary modality. Surgical intervention is recommended only if the cyst is space-occupying and percutaneous puncture can not be performed or in the case of emergency.