儿童动脉缺血性脑卒中研究进展

很多全身性疾病可导致儿童动脉缺血性脑卒中(AIS),如脑动脉病.轻微感染在短暂性脑动脉病(TCA)中起重要作用.由于儿童卒中表现的非特异性以及医师对儿童AIS认识不足常常延误诊断和治疗.目前儿童AIS的治疗国内外尚无统一标准,急性期处理以支持疗法为主.儿童AIS的二级预防大多参照成年人治疗方案,预后并不乐观,一旦患病往...     

不同年龄段儿童动脉缺血性脑卒中病因分析

目的 探讨不同年龄段儿童动脉缺血性脑卒中（AIS）的病因。方法 回顾性分析首都医科大学附属北京儿童医院神经内科病房2016年1月至2020年12月收治的AIS患儿资料，根据2019年美国心脏协会和美国卒中协会儿童脑卒中的管理指南将AIS病因分为心源性、易栓症、脑动脉病、其他、病因不详5大类。根据年龄分为婴幼儿（>28 d至<3岁）、学龄（前）儿童（3～<10岁）和青少年（10～<18岁）。分析不同病因患儿的临床特点。结果共纳入129例AIS患儿。脑动脉病是首要病因，共47例（36.43%），最常见的亚型为局灶脑动脉病（FCA,15例）和Moyamoya病（17例）。婴幼儿AIS共51例（39.53%），外伤后基底节脑梗死是首要病因（24/51,47.06%），这部分患儿中21例有基底节钙化。学龄（前）儿童AIS共54例（41.86%），青少年AIS共24例（18.60%），两者均以脑动脉病为首要病因。学龄（前）儿童AIS中存在11例病因不详的患儿，均为男性，影像学椎基底动脉供血区有多个梗死灶，不能除外椎动脉夹层，但均未行颈部CTA或MRA检查。青少年AIS中2例...     

儿童动脉缺血性脑卒中的研究进展

近年来,神经影像学的发展为脑卒中的诊断提供了可靠依据,脑卒中逐步被认为是儿童一个重要的致病、致死原因。儿童脑卒中的发病率要比以往想象的要多,且一半以上的儿童动脉缺血性脑卒中(AIS)幸存者中有认知或者运动残疾[1],危害性极大,需要引起临床医生的足够重视。本文就儿童AIS的流行病学、危险因素、病因和发病机制、诊断、治疗、预后及复发危险性进行较全面阐述,并指出了相关研究方向。1流行病学儿童脑卒中的流行病学与成人明显不同。儿童群体的缺血性和出血性脑卒中大约各占50%的病例,但成年群体的出血性脑卒中只占20%。儿童脑卒中的…     

儿童动脉缺血性卒中的起病方式、梗死部位与病因诊断舷

目的 探讨儿童动脉缺血性卒中(AIS)的起病方式、梗死部位与卒中病因的关系.方法 回顾性分析1997年1月-2007年10月在武汉市儿章医院及华中科技大学同济医学院附属同济医院儿科住院、经头颅影像学证实并诊断为AIS的病例97例.年龄6个月～18岁.按病因分为炎性反应性动脉病组(32例)与非炎性反应性动脉病组(65例)(脑血管畸形、心源性栓塞、血液系统疾病及代谢疾病).记录二组患儿年龄、起病方式及梗死部位,并将起病方式分为急性与非急性,梗死部位分为基底核区与大脑中动脉主干供血区.采用t检验比较二组AIS患儿平均年龄差异,X2检验比较二组患儿起病方式与梗死部位差异.结果炎性反应性动脉病组与非炎性反应性动脉病组平均年龄比较差异无统计学意义(t=1.573 P＞0.05).炎性反应性动脉病组中急性起病者10例(31.2%),非炎性反应性动脉病组中急性起病者38例(58.5%),二组比较差异有统计学意义(X2=6.352 P＜O.05).炎性反应性动脉病组梗死灶位于基底核区21例(65.6%),非炎性反应性动脉病组梗死灶位于基底核区28例(43.1%),二组比较差异有统计学意义(X2=4.361 P＜0.05).结论起病方式与梗死部位可以提示儿童AIS的病因诊断.非急性起病、梗死灶位于基底核区的AIS,其病因多为炎性反应性动脉病.     

儿童动脉缺血性卒中的起病方式、梗死部位与病因诊断

目的探讨儿童动脉缺血性卒中（AIS）的起病方式、梗死部位与卒中病因的关系。方法回顾性分析1997年1月--2007年10月在武汉市儿童医院及华中科技大学同济医学院附属同济医院儿科住院、经头颅影像学证实并诊断为AIS的病例97例。年龄6个月～18岁。按病因分为炎性反应性动脉病组（32例）与非炎性反应性动脉病组（65例）（脑血管畸形、心源性栓塞、血液系统疾病及代谢疾病）。记录二组患几年龄、起病方式及梗死部位，并将起病方式分为急性与非急性，梗死部位分为基底核区与大脑中动脉主干供血区。采用t检验比较二组AIS患儿平均年龄差异搿。检验比较二组患儿起病方式与梗死部位差异。结果炎性反应性动脉病组与非炎性反应性动脉病组平均年龄比较差异无统计学意义（t=1．573 P〉0．05）。炎性反应性动脉病组中急性起病者10例（31．2％），非炎性反应性动脉病组中急性起病者38例（58．5％），二组比较差异有统计学意义（χ^2=6．352 P〈0．05）。炎性反应性动脉病组梗死灶位于基底核区21例（65．6％），非炎性反应性动脉病组梗死灶位于基底核区28例（43．1％），二组比较差异有统计学意SL（χ^2=4．361 P〈0．05）。结论起病方式与梗死部位可以提示儿童AIS的病因诊断。非急性起病、梗死灶位于基底核区的AIS，其病因多为炎性反应性动脉病。     

儿童动脉缺血性与出血性脑卒中的危险因素及转归

目的总结儿童动脉缺血性卒中(AIS)和出血性卒中(HS)的临床特点、危险因素及转归。方法回顾性分析和比较2010年11月至2014年5月诊治的142例AIS和HS患儿的临床资料。结果 AIS 92例(64.8%),男60例、女32例,发病年龄(4.6±3.6)岁(1个月~16岁);HS 50例(35.2%),男34例、女16例,发病年龄(2.6±3.7)岁(1个月~13岁);两组患儿的年龄差异有统计学意义(P=0.007)。AIS最常表现为局灶神经功能受损,其中肢体瘫痪73例(79.3%)、中枢性面瘫30例(32.6%)、语言障碍19例(20.7%);HS最常表现为弥散神经功能受损,其中意识障碍29例(58.0%)、恶心/呕吐22例(44.0%)、头痛14例(28.0%)。AIS常见危险因素为脑动脉病49例(53.3%)、感染47例(51.1%)、头部轻微外伤史16例(17.4%);HS常见危险因素为维生素K缺乏22例(44.0%)、脑血管畸形8例(16.0%)、血液系统疾病6例(12.0%)。AIS患儿死亡5例(6.4%),致残48例(61.5%),复发9例(11.5%);HS患儿死亡15例(34.1%),致残19例(43.2%);HS病死率高于AIS,差异有统计学意义(P0.01)。结论儿童脑卒中HS所占比例并不低,且预后较AIS更差。     

儿童动脉缺血性卒中的病因

目的总结儿童动脉缺血性卒中(AIS)的临床特点、病因及危险因素。方法回顾性分析2003年1月-2011年3月在重庆医科大学附属儿童医院住院治疗的AIS患儿资料,采用SPSS 17.0软件进行分析,描述患儿年龄、性别分布等基本情况、相关病因及危险因素,并分析头部轻微外伤与基底核钙化是否存在相关性。结果本组患儿中,男31例,女27例;年龄2个月～13岁,平均3岁10个月。常见神经系统表现依次为肢体偏瘫52例(92.8%),中枢性面瘫34例(58.6%),运动性失语16例(27.6%),头痛、呕吐11例(19.0%),意识障碍8例(13.8%),惊厥7例(12.1%)。40例进行血管影像学检查,其中33例异常,左侧大脑中动脉受累最常见(19例,47.5%)。常见病因为轻微头部外伤(26例,44.8%)、烟雾病(8例,13.8%)和颅内感染(5例,8.6%),18例(31.0%)患儿为多病因共存,6例(10.3%)患儿未找到任何病因或危险因素,22例(37.9%)患儿有呼吸道感染。结论儿童AIS发病高峰人群为幼儿及学龄前期儿童,最常见的神经功能障碍为肢体偏瘫,神经影像学显示左侧大脑中动脉狭窄或中断最常见,常见病因为轻微头部外伤、烟雾病及颅内感染,较多患儿病前或病程中有上呼吸道感染。积极预防感染、减少头部外伤,对突发偏瘫患儿早期行头颅影像和血管检查将有利于AIS的预防和早期诊断。     

儿童肱动脉假性动脉瘤一例并文献复习

目的报道1例儿童肱动脉假性动脉瘤的诊疗,并回顾相关文献总结此类疾病的病因、诊治及预后。方法回顾性分析了2019年6月1例16天新生儿的左上肢肱动脉假性动脉瘤的临床资料。本例患儿出生后6d发现逐渐增大的左肘部包块。B型超声、左上肢MRI及CTA提示左肱动脉假性动脉瘤。经局部加压包扎治疗,包块持续增大并出现表面皮肤坏死渗液,遂在全麻下行假性动脉瘤切除+肱动脉端端吻合术。并通过PubMed、Springer Link、Google Scholar、Embase、Ovid、中国生物医学文献数据库(CBM)、中国知网(CNKI)、万方和维普数据库检索,总结儿童肱动脉假性动脉瘤的诊疗经验。结果术后左上肢远端皮肤红润,毛细血管充盈实验1~2 s。24 h后拔除引流片,伤口愈合良好,术后14 d拆线。肘关节及左手活动自如。术后超声检查示左上肢远端桡尺动脉血流灌注良好。随访3个月,左上肢循环良好,活动自如。检索文献发现22篇中英文文献,对其报道的24例患儿诊疗情况分析,发现肘部血管针刺采血是最常见的致伤机制,占62.5%(15/24)。其次为肱骨髁上骨折,肱动脉被骨折端或是克氏针刺伤,占20.8%(5/24)。主要治疗方法是手术切除假性动脉瘤,占75%(18/24),并重建血管。预后良好,文献报道病例无远端肢体坏死情况。结论儿童肱动脉假性动脉瘤较为少见,在婴幼儿多因肘部血管穿刺采血所致。较小的假性动脉瘤可尝试局部加压或瘤内凝血酶注射,无效则应切除假性动脉瘤并重建肱动脉。     

跟上免疫学进展的步伐

近年来免疫学的研究已经取得了令人瞩目的进展, 这些进展伴随着儿童免疫性疾病诊疗水平的不断提升。本文以免疫检查点的靶向治疗、中性粒细胞陷阱、辅助性T细胞17的发现及焦亡的机制为例, 展现这些进展对儿童免疫性疾病的疾病机制认识、精准诊疗的推动作用, 提示儿童免疫专业医生应当紧跟免疫学理论进展步伐, 不断促进临床诊疗的创新实践, 使患儿获益。     

关注新生儿心血管疾病诊疗

早在20世纪初,William Osler医生在他的医学著作中写道＂先天性心脏病具有有限的临床治疗价值＂,＂没有办法可以治疗这一缺陷或缓解症状＂。然而到了1938年,Robert Gross医生成功地为一名波士顿儿童医院的女孩结扎了动脉导管,从而开创了先天性心脏病诊疗的新篇章。从那时起,新生儿心血管疾病的诊疗状况有了巨大发展。     

The importance of different immunosuppressive regimens in the development of posttransplant diabetes mellitus

Solid-organ transplantation is the optimal long-term treatment for most patients with end-stage organ failure. After solid-organ transplantation, short-term graft survival significantly improved (1). However, due to chronic allograft nephropathy and death with functioning graft, long-term survival has not prolonged remarkably (2). Posttransplant immunosuppressive medications consist of one of the calcineurin inhibitors in combination with mycophenolate mofetil (MMF) or azathioprine (Aza) and steroids. All of them have different adverse effects, among which posttransplant diabetes mellitus (PTDM) is an independent risk factor for cardiovascular (CV) events and infections causing the death of many transplant patients and it may directly contribute to graft failure (3). According to the criteria of the American Diabetes Association (4), diabetes mellitus (DM) is defined by symptoms of diabetes (polyuria and polydipsia and weight loss) plus casual plasma glucose concentration ≥ 11.1 mmol/L or fasting plasma glucose (FPG) ≥ 7.0 mmol/L or 2-h plasma glucose level ≥ 11.1 mmol/L following oral glucose tolerance test (OGTT). This metabolic disorder occurring as a complication of organ transplantation has been recognized for many years. PTDM, which is a combination of decreased insulin secretion and increased insulin resistance, develops in 4.9/15.9% of liver transplant patients, in 4.7/11.5% of kidney recipients, and in 15/17.5% of heart and lung transplants [cyclosporine A (CyA)/tacrolimus (Tac)-based regimen, respectively] (5). Risk factors of PTDM can be divided into non-modifiable and modifiable ones (6), among which the most prominent is the immunosuppressive therapy being responsible for 74% of PTDM development (7). Emphasizing the importance of the PTDM, numerous studies have determined the long-term outcome. On the basis of these studies, graft and patient survival is tendentiously (8) or significantly (9, 10) decreased for those developing PTDM.     

GENETICS OF CARNEY COMPLEX AND RELATED FAMILIAL LENTIGINOSES, AND OTHER MULTIPLE TUMOR SYNDROMES

Carney complex is a multiple endocrine neoplasia (MEN) syndrome that affects the adrenal cortex, the pituitary and thyroid glands, and the gonads. The complex is also associated with skin and mucosa pigmentation abnormalities and myxoid and other neoplasms of mesenchymal and neural crest origin. Thus, this syndrome also belongs to another group of genetic disorders, the lentiginoses (or lentigenoses), which include the Peutz-Jeghers, LEOPARD, arterial dissections and lentiginosis, and Laugier-Hunziker syndromes, Cowden disease and Ruvalcaba-Myhre-Smith (Bannayan-Zonana) syndrome and the centrofacial, benign patterned and segmental lentiginoses, all of which can be associated with a variety of developmental defects. The inheritance of Carney complex, just like that of the other MENs and the lentiginoses, is autosomal dominant. Genetic loci or genes have been identified for Carney complex, Peutz-Jeghers and Ruvalcaba-Myhre-Smith syndromes, but not for other lentiginoses. Elucidation of the molecular defects responsible for these disorders is expected to shed light on aspects of early neural crest differentiation, the regulation of pigmentation, the development of autonomous endocrine function, and endocrine and nonendocrine tumorigenesis.     

Bibliometric data: a disaster for many non-American biomedical journals

Bibliometric data published by the Institute of Scientific Information in Philadelphia (ISI), and which was previously discussed in Acta Paediatrica , has increasingly been used despite all the relevant and severe criticism that has been raised against this method of evaluating individual research results and grading scientific journals. It is obvious that the present trend regarding the use of bibliometric data as a basis for priorities and funding of research and for the promotion of individual scientists favours American-oriented research projects at the expense of those that are based on concepts of predominantly European relevance.

Conclusion: For the future of non-American research, it is important that no single super-power, i.e. the USA, should dominate scientific priorities. The condition for efficient European competition is that European Centres with high levels of competence for creative research and training of scientists from all over the world are established. In addition, it is important that the results of European research are published in prestigious European journals, as was the situation before World War II.     

All you ever wanted to know about infant formulas (but were afraid to ask)

The World Health organisation recommends breast feeding infants for the first six months of life. When this breast feeding does not occur either through parental choice or medical need, infant formulas will be required. There is a bewildering array of formulas on the UK market for many different requirements. When faced with an unsettled infant many parents (and health care professionals) will experiment with the infant formula available and often attend the paediatric clinic looking for help and advice. It is therefore essential that paediatricians understand what milks are available and what the key differences between different products are. This review attempts to provide a simple guide through many of the formulations currently available in the UK; and offers advice for the dietary management of the child with extra calorie requirements, infants with cow's milk protein allergy, gastro oesophageal reflux disease, apparent unresolved hunger and infantile colic. Whatever the underlying condition, there is likely to be an infant formula that is suitable in this generation of ever expanding formulations.     

Differential effects of inhaled budesonide on serum osteocalcin in children and adolescents with asthma

In recent years, measurement of serum osteocalcin has been introduced for assessment of bone turnover in patients treated with exogeneous glucocorticoids. Studies in children with asthma on inhaled glucocorticoids, however, have shown inconsistent results. The aim of the present study is to assess bone turnover in prepubertal children and in adolescents with asthma treated with inhaled budesonide using three different osteocalcin assays: the Pharmacia Osteocalcin CAP FEIA, the CIS OSTK-PR and CIS IRMA ELSA-OSTEO assays. Two studies were conducted: 1) a randomised double blind two-period crossover study of 22 prepubertal children aged 5-12 years. In one period 800 μg budesonide was given once in the morning, in the other 400 μg was given twice daily; 2) a randomised double blind placebo controlled two period crossover study of inhaled budesonide 400 μg twice daily in fourteen 13-16 year old adolescents with pubertal stages II-V. In both studies, treatment periods were of four weeks duration, and blood samples were collected at the last day of each period. In the prepubertal children none of the osteocalcin assays detected any statistically significant differences between any of the periods. In the adolescent group reduced levels of osteocalcin were seen during budesonide treatment. The suppression reached statistical significance with the CAP FEIA (P = 0.03) and the OSTK-PR (P =0.01) assays, but not with the ELSA-OSTEO assay (P = 0.06). Correlation analyses showed statistically significant correlation coefficients varying between 0.58 and 0.91 (P=0.03 and P < 0.0001, respectively). The effect of inhaled glucocorticoids on serum osteocalcin may depend on the assay applied, and inhaled glucocorticoids have differential effects in children and adolescents.     

alpha -SMOOTH MUSCLE ACTIN DISTRIBUTION IN THE PULMONARY VASCULATURE COMPARING HYPOPLASTIC AND NORMAL FETAL LUNGS

We investigated the intra-acinar pulmonary vascular muscularization in the developing human fetal lung between the 17th and 24th gestational weeks, that is, during the canalicular phase of lung development. Fifteen hypoplastic and 25 normal developed lungs were included in this study using monoclonal alpha -smooth muscle (sm) actin antibodies for smooth muscle detection. Computer-aided image analysis was performed for morphometrical measurements and statistical evaluation. Alphasm-actin-immunoreactive intra-acinar vessels down to a luminal diameter of less than 10 mu m were detected in hypoplastic as well as in normally developed lungs. Crucial differences presented as follows: significantly higher density of intra-acinar vessels, especially due to alpha -sm-actin-negative vessels less than 30 mu m in luminal diameter, in the control group; significantly higher alpha -sm-actin immunoreactivity per section unit as well as per vessel in the hypoplastic lung group. As suggested by others, alpha-sm-actin-positive cells of the intra-acinar vessel wall in the developing human lung were demonstrated to be smooth muscle cells, their immediate precursors, and pericytes. We conclude that the increased alpha -sm-actin immunoreactivity represents muscularization of the vessel wall in functional terms and may be regarded as one structural cause among others for the establishment of persistent fetal circulation in hypoplastic lungs.     

Représentations émotionnelles de la maladie chez 22 enfants drépanocytaires

Background

The emotional and psychological impact of chronical disease among children is considerable. The aim of this study was to explore the emotional representations of sickle cell children followed up at Bordeaux University Hospital in both qualitative and quantitative ways.

Methods

Prospective observational study, conducted from February to May 2010 among 22 sickle cell children (SS, SC and Sβ) followed at Bordeaux University Hospital and among their parents. A questionnaire evaluating depressive symptoms and emotional representations was proposed to children and to their parents separately, measuring their child's emotions. Children were asked to draw themselves during sickle cell crisis and without any painful episode, in order to illustrate their perception of their disease.

Results

Emotional and psychological impact on sickle cell children was important in this study. Eighty-six percent of children have commonly had negative feelings such as sadness, anger or fear. Thirty-six percent of them had depressive symptoms. Parents largely underestimated this impact. Drawings and answers to the questionnaire emphasized an important lack of disease understanding, social consequences, and depressive affects.

Conclusion

Psychological and emotional difficulties in sickle cell children should be identified and supported. Resources for psychological and educational support are necessary to improve the quality of life of sickle cell patients in France.     

Limiting light-induced lipid peroxidation and vitamin loss in infant parenteral nutrition by adding multivitamin preparations to Intralipid

Parenteral lipids are susceptible to light-induced peroxidation, particularly under phototherapy. Ascorbic acid is protective. The aim of this study was to investigate whether dark delivery tubing and/or coadministration of multivitamin preparations could prevent peroxidation of Intralipid without undue vitamin loss. In experiments carried out on the benchtop, lipid peroxidation occurred in ambient light and was more extensive under phototherapy. Dark tubing decreased peroxide formation, but only by about 65%. In simulated clinical conditions in which solutions were pumped through standard clear or dark minibore plastic tubing, Intralipid accumulated lipid peroxides as measured by the FOX assay (280 µM) or as triglyceride hydroperoxides (52 µM). Multivitamin preparations (MVIP or completely, and were fully protective when used with dark tubing. There was loss of riboflavin (65% from Soluvit and 35% from MVIP) in clear tubing but this was decreased to 18% and 11%, respectively, in dark tubing. Ascorbate loss was 20% (MVIP) and 50% (Soluvit) and only slightly less in dark tubing. Ascorbate loss was also seen in the absence of Intralipid and is due to riboflavin-induced photo-oxidation.Conclusion: Multivitamin preparations protect Intralipid against light-induced formation of lipid hydroperoxides, and administering multivitamins with Intralipid via dark delivery tubing provides a practical way of preventing peroxidation of the lipid while limiting vitamin loss. This procedure should be considered for routine use as well as with phototherapy. Soluvit/Vitlipid) inhibited peroxide formation almost     

SociaBillyQuizz,un jeu pour l’entraînement aux habiletés sociales chez l’enfant et l’adolescent : étude exploratoire

Background

The SociaBillyQuizz is a therapeutic game designed for social skills training groups with children and adolescents. Using an entertaining method, this media requests several dimensions: exposure, cognition, communication skills, imagination, emotional expression and sign decoding. In this preliminary study, the setting includes two groups of six adolescents, one with social anxiety disorder and the other with Asperger syndrome.

Objective

To evaluate, in an exploratory study, the effects of a therapeutic device involving this game for these two clinically different groups of adolescents.

Methods

During 26 of 1 hour weekly sessions, two adolescents groups participate to a program including the SociaBillyQuizz and cognitive behavioral therapies. The groups are moderated by two therapists. The SociaBillyQuizz is a board game for two to six players; its goal is to collect points by answering instructions from the different thematic cards. There are four thematic cards: action cards (players have to do something), brainstorming cards (players have to use their imagination and demonstrate cognitive flexibility), interview cards (players have to express themselves about what they think or feel) and mystery cards (unexpected instructions). According to the groups’ clinical characteristics, some aims are specifically highlighted. In the anxiety disorder group, the cognitive dimension is privileged and in the Asperger syndrome group, we emphasise the pretend, cognitive flexibility and theory of mind. The effects are measured by the Rathus Assertiveness Schedule and the Fear Avoidance Hierarchy (FAH) for the social anxiety disorder group and by the Faux Pas Recognition Test and the Social Responsiveness Scale (parent version) for the Asperger group.

Results

These assessment tools indicate, for both groups, a significant increase of the scores corroborating the observed clinical effects. For eleven of the twelve adolescents, a clinical interview 6 months after the retest shows a continuity of therapeutic benefit.

Discussion

These early results suggest that a social skills training device featuring the SociaBillyQuizz produces clinical improvements in these two groups of adolescents. In future researches, with control group and more complete follow-up, nature and effectiveness of its contribution should be specified.

Conclusion

In this preliminary study, the SociaBillyQuizz appears to be an interesting therapeutic tool that can increase implication, motivation, participation and cohesiveness of the group. It also makes easier the cognitive-behavioural-strategies learning.     

Les effets de l’incarcération chez les mineurs

Aim of this study

In this article we wish to question the effects of incarceration on minors. The history of prison reveals that it is the work of a humanist and philanthropic discourse that ensued from the effects of revolutionary ideas. However, from the moment of its reform – that transformed it from a place of confinement to a penal institution – it has only demonstrated its dysfunctional aspect. Our objective is to initiate a reflection on the effects of incarceration, whether it be collective or individual and in particular when it involves adolescents.