Clinical application of flexible bronchoscopy and neck ultrasound in percutaneous dilatational tracheotomy in infants北大核心CSCD

目的探讨可弯曲支气管镜联合颈部超声在婴幼儿经皮扩张气管切开术中的应用价值。方法回顾性分析2018年12月至2021年5月山东大学附属儿童医院呼吸介入科收治的24例应用可弯曲支气管镜联合颈部超声经皮扩张气管切开患儿的临床资料。24例患儿中, 男12例, 女12例, 男女比例为1∶1;年龄21 d~2岁, 中位年龄5个月;中位体质量5.8 kg。在进行经皮扩张气管切开时, 先使用超声评估患儿甲状腺位置及血管走形, 选择合适穿刺入路并标记穿刺点, 然后在可弯曲支气管镜引导下完成经皮扩张气管切开术。结果术前接受持续气管插管机械通气支持治疗患儿19例(79.2%), 间断气管插管机械通气支持治疗患儿2例(8.3%), 无创呼吸机支持治疗3例(12.5%)。24例患儿中, 先天性上呼吸道发育畸形9例(37.5%);双侧声带麻痹8例(33.3%);上呼吸道占位性疾病3例(12.5%);外科术后撤机困难2例(8.3%);神经肌肉疾病2例(8.3%)。24例患儿均在30 min内成功完成手术, 出血量均少于5 mL, 术中无并发症发生。结论可弯曲支气管镜联合颈部超声辅助使婴幼儿经皮扩张气管切开术能够顺利完成, 并显著降低手术操作难度, 提高了手术的安全性。     

12例心脏病术后患儿体外膜肺的临床结果及经验

目的回顾性总结分析阜外心血管病医院12例儿童体外膜肺氧合(ECMO)支持治疗的临床结果和经验。方法2004年12月~2005年12月共实施儿童ECMO病例12例,所有患儿均使用Medtronic ECMO系统,管道、氧合器及离心泵内膜采用全肝素涂抹技术,行静脉-动脉ECMO辅助,激活凝血时间维持146~258 s,肝素用量5~20 U/(kg.h)。辅助期间平均流量在40~220 ml/(kg.min)。结果ECMO支持时间55~266 h,平均120 h;9例(75%)顺利撤离ECMO,其中6例康复出院(67%),3例术后死亡,3例不能撤离ECMO而放弃治疗;总出院率为50%(6/12)。存活出院患儿EC-MO前的动脉血乳酸水平明显低于死亡患儿(P=0.022),两组患儿体重也存在统计学差异(P=0.019)。结论ECMO支持在儿童复杂先天性心脏病术后循环呼吸衰竭的治疗中是一种有效的机械辅助方法,同时可以作为心脏移植患儿等待供体期间的过渡桥梁。手术畸形纠治满意、尽早对心肺衰竭的患儿使用ECMO支持、避免重要脏器的不可逆损伤依然是ECMO成功的关键。     

体外膜肺氧合在儿童呼吸衰竭中应用的多中心调查

目的 分析中国大陆地区PICU应用体外膜肺氧合(extracorporeal membrane oxygenation,ECMO)技术支持儿童呼吸衰竭的治疗效果.方法 采用问卷调查方法回顾2011年6月至2016年2月中国大陆地区三级儿童专科医院或综合医院PICU应用ECMO支持因重症肺炎、急性呼吸窘迫综合征等导致的呼吸衰竭,且常规呼吸支持方式效果不佳患儿的治疗情况及预后.结果 至2016年2月,共有来自4家医院的21例儿童呼吸衰竭患者接受ECMO治疗,平均年龄29.0(9.0,81.5)个月,平均体重12.0(9.0,20.8)kg.原发病为重症肺炎7例(33%),重症肺炎合并急性呼吸窘迫综合征6例(29%).21例患儿中成功撤离ECMO 12例(57%),出院存活率38%.患儿P/F比值均数为56.0(44.5,69.0)mmHg(1mmHg=0.133kPa).ECMO治疗前OI值31.5(19.2,41.0),ECMO治疗24h后OI值6.2(3.8,14.9);患儿经ECMO治疗24h后血气分析PO2、SaO2均有明显上升,PO2从ECMO前的49.5(40.4,61.9)mmHg升至65.0(42.6,120.5)mmHg,并且存活者上升水平显著高于死亡者[52.0(1.8,89.4) mmHg比8.2(-15.1,33.9) mmHg,P=0.036];SaO2从ECMO前的80%(70.35%,91.75%)升至98%(95.65%,100%),存活者SaO2上升水平显著大于死亡者[23.5%(11.4%,27.1%)比4.3%(2.4%,23.8%),P=0.039].ECMO的使用降低了呼吸机参数及平均气道压.ECMO平均治疗时间149(91.25,242)h,平均费用15.88(12.57,24.08)万元.VV置管模式存活率比VA置管模式高(3/3例 比 5/18例);ECMO前使用呼吸机时间越长,病死率越高,死亡组呼吸机治疗时间明显长于存活组[4.5(2.5,12.0)h比1.6(1.0,2.2)h,P=0.015].ECMO并发症主要为出血,本次调查出血发生率为38%,血流感染发生率为9.5%.结论 ECMO可以明显改善呼吸衰竭患儿的氧合情况.     

儿童暴发性心肌炎清醒体外膜肺氧合治疗三例报道

目的:探讨清醒体外膜肺氧合(ECMO)支持在儿童暴发性心肌炎患者中应用的有效性和可行性。方法:回顾分析2019年3月至2019年9月八一儿童医院PICU收治的3例清醒ECMO支持的暴发性心肌炎患儿资料,分析病例资料、管理及预后,对比治疗前后超声数据,总结呼吸机、ECMO使用时间,介绍清醒ECMO治疗在儿科应用的经验及可行性。结果:3例患儿行清醒ECMO,撤离呼吸机后,其中1例患儿因恶性心律失常再次行机械通气,最终3例患儿心功能恢复,其中1例遗留肾功能不全,3例患儿均存活出院。结论:静脉-动脉ECMO支持的儿童暴发性心肌炎,撤离呼吸机行清醒ECMO支持是可行的,有必要针对儿童清醒ECMO进行进一步研究。     

儿童上臂型植入式给药装置的临床应用

目的探讨儿童上臂型植入式给药装置(TIAP)植入技术应用及术后并发症的发生情况。方法 2018年4月—2018年12月期间诊治的6例白血病患儿接受上臂植入TIAP,男女各3例,年龄6岁～13岁。采用超声导引下经皮穿刺置管技术于上臂植入TIAP。结果 6例患儿均成功植入上臂TIAP,未发生气胸、血胸、误穿动脉、心血管问题等并发症。结论超声导引下上臂型TIAP植入技术在儿童临床应用中安全有效,创伤小,可作为儿童化疗的一个输液通路选择。     

复杂先天性心脏病患儿术后体外膜氧合支持治疗的研究

目的探讨复杂先天性心脏病患儿施行体外膜氧合(extracorporeal membrane oxygenation, ECMO)辅助支持治疗的情况。方法收集2017年1月至2020年12月于上海儿童医学中心心胸外科进行ECMO辅助支持治疗的133例复杂先天性心脏病患儿的相关资料。其中, 男76例, 女57例;年龄范围为0～18岁, ECMO辅助支持治疗时的中位年龄为4.2个月;身高为(71.33±28.53 )cm, 体重为(9.06±11.38) kg。需ECMO辅助支持治疗的复杂先天性心脏病占比为:大动脉转位占17.29%(23/133), 右心室双出口占9.77%(13/133), 肺动脉闭锁占9.02% (12/133)、瓣膜病变占8.27%(11/133)、主动脉弓中断和主动脉弓缩窄均占7.51%(10/133)。观察患儿的一般资料、诊疗情况及检查指标, 分析总结其治疗过程及预后。结果本研究患儿术中体外循环转流时间为(221.00± 161.78) min, 阻断时间为(100.31±68.09 )min, ECMO转流时间为(114.87±84.39 )h, ECMO整体撤...     

体外膜肺氧合在儿童暴发型心肌炎应用的多中心调查

目的 探讨中国大陆PICU应用体外膜肺氧合(extracorporeal membrane oxygenation,ECMO)支持儿童暴发型心肌炎治疗的效果.方法 采用问卷调查方法回顾自2006年4月中国大陆PICU首次开展ECMO 治疗以来,国内三级儿童专科医院或综合医院的PICU应用ECMO支持暴发型心肌炎的治疗情况;总结分析临床特点及预后.结果 共有3家医院的23例儿童急性暴发型心肌炎患者接受ECMO治疗,男18例,女5例;平均年龄(86.3±48.8)个月,平均体重(25.8±12.1)kg,ECMO治疗前左室射血分数(39.5±15.6)%,ECMO平均治疗时间(119.1±57.3)h.18例患儿存活出院,5例死亡.所有患儿经ECMO治疗24h后平均动脉压均有上升,从ECMO前的(60.7±23.7)mmHg(1mmHg=0.133kPa)升至(72.1±9.8)mmHg,并且存活者上升水平显著高于死亡者(P=0.04);血清乳酸水平从ECMO前的(6.8±5.1) mmol/L降至(2.9±2.6)mmol/L,存活者血清乳酸水平降低幅度显著大于死亡者(P<0.001).23例患儿中,成功撤离ECMO 21例,成功撤离率为91.3%;3例患儿撤离ECMO后30d内死亡;18例好转出院,整体存活率为78.3%.死亡患儿ECMO支持时间长于存活患者,但两者比较差异无统计学意义(P=0.41).所有患儿平均医疗花费(16.4±4.9)万元,存活者与死亡者比较差异无统计学意义(P=0.24).18例存活患儿中,共有15例随访,发生神经系统后遗症2例,1例颈总动脉血栓形成,1例心功能不全.结论 ECMO可为儿童急性暴发型心肌炎患者提供有效的循环支持,促进血流动力学稳定,提高存活率.     

外周导入中心静脉置管术在极低出生体重儿中的应用

目的：介绍外周导入中心静脉置管术(PICC)应用于22例极低出生体重儿(VLBW)的经验。方法：选择贵要、肘正中、头静脉、腋静脉置管,持续生命体征及血氧饱和度监测(SaO2)。结果：20/22例(90.9%)患儿成功置管。17/20例(85%)顺利过度到完全胃肠道喂养,平均留置时间为(13.2±6.4) d。其中出现发绀3例、条索状静脉炎3例、塞管1例、导管末段细菌培养阳性3例。发绀患儿在穿刺次数、操作时间上有极显著性差异;条索状静脉炎与胎龄有关。结论： PICC使VLBW患儿顺利完成营养过度;穿刺技术,无菌操作及导管管理是成功施术的保证。     

超声引导在婴儿桡动脉穿刺置管中的应用

目的探讨超声引导技术在婴儿桡动脉穿刺置管中的应用价值。方法选择80例1岁以下需行桡动脉穿刺置管的手术患儿,随机分为超声引导组(n=40)和传统触摸组(n=40)。超声引导组采用超声引导法进行穿刺和置管,传统触摸组采用指尖触摸法进行定位和穿刺置管,分别记录每组患儿的穿刺时间、穿刺次数,比较两组的首次穿刺成功率、总成功率、首次穿刺成功时间、总穿刺时间、穿刺次数及套管针使用数量。结果超声引导组首次穿刺成功率和总成功率分别为72. 5%和97.5%,传统触摸组首次穿刺成功率和总成功率分别为50%和80%,差异均有统计学意义(P 0. 05)。超声引导组有1例穿刺失败,更换穿刺部位后穿刺成功。传统触摸组有8例穿刺失败,改超声引导后全部穿刺成功。超声引导组总穿刺时间(66. 6±56. 9) s,明显短于传统触摸组的(120±94. 9) s,差异有统计学意义(t=3. 052,P=0. 003)。两组首次穿刺成功所需时间分别为(36. 3±16. 2) s和(38. 3±19. 1) s,差异无统计学意义(P 0. 05)。超声引导组总穿刺次数少于传统触摸组[1(1～2) vs. 1. 5(1～3)](χ~2=3. 900,P 0. 05)。与传统触摸组相比,超声引导组使用穿刺针数量较少[1(1～1) vs. 1(1～2)],差异有统计学意义(χ~2=3. 464,P 0. 05)。传统触摸组有7例(17. 5%)出现动脉血肿及出血等并发症,而超声引导组仅1例(2. 5%)出现并发症,差异有统计学意义(χ~2=4. 507,P 0. 05)。结论围术期婴儿行桡动脉穿刺置管时使用超声引导技术可有效提高穿刺成功率,缩短穿刺时间,降低穿刺相关并发症,值得临床推广应用。     

体外膜肺技术在9例危重症患儿中的应用

目的 探讨心肺功能衰竭患儿体外膜肺(ECMO)支持治疗的临床经验。方法 回顾性分析2008年12月至2012年5月收住复旦大学附属儿科医院重症监护病房及心脏监护病房的9例危重症患儿接受ECMO治疗的情况及转归。结果 9例患儿均采用颈内动、静脉置管,V-A ECMO模式,体外肝素抗凝,活化凝血时间(ACT)维持在180～220 s;体外辅助血流量为50~100 mL·kg-1·min-1。治疗期间进行心肺功能、血液指标和影像学等监测。9例患儿ECMO使用时间为5~280 h,中位数112 h。经治疗后8例病情好转,撤离ECMO治疗,其中7例痊愈出院,1例撤离ECMO治疗后3 d自动出院。1例因室间隔缺损修补术后合并严重脓毒症、脓毒症休克和心肺功能衰竭,ECMO治疗1周,撤机后死亡。发生各类并发症共14例次,其中机械系统并发症6例次,包括氧合器漏液3例次,管路血栓2例次,水箱加温器故障1例次;机体并发症7例次,其中6例发生贫血,1例右上肢功能障碍;意外事件1例,患儿在ECMO运行过程中因右腋下动脉置管意外滑脱而形成血肿。未发生颅内并发症、出血和感染等。结论 ECMO能有效对危重症患儿行心肺功能支持,使用安全。建立一支专业的技术团队能促进ECMO的更好开展。     

Allergic factors associated with the development of asthma and the influence of cetirizine in a double-blind, randomised, placebo-controlled trial: First results of ETA®

There is a common progression known as the allergic march from atopic dermatitis to allergic asthma. Cetirizine has several antiallergic properties that suggest a potential effect on the development of airway inflammation and asthma in infants with atopic dermatitis. Methods. Over a two year period, 817 infants aged one to two years who suffered from atopic dermatitis and with a history of atopic disease in a parent or sibling were included in the ETAC^® (Early Treatment of the Atopic Child) trial, a multi-country, double-blind, randomised, placebo-controlled trial. The infants were treated for 18 months with either cetirizine (0.25mg/ kg b.i.d.) or placebo. The number of infants who developed asthma was compared between the two groups. Clinical and biological assessments including analysis of total and specific IgE antibodies were performed. Results. In the placebo group, the relative risk (RR) for developing asthma was elevated in patients with a raised level of total IgE (≥ 30 kU/I) or specific IgE (≥ 0.35 kUA/I) for grass pollen, house dust mite or cat dander (RR between 1.4 and 1.7). Compared to placebo, cetirizine significantly reduced the incidence of asthma for patients sensitised to grass pollen (RR = 0.5) or to house dust mite (RR = 0.6). However, in the population that included all infants with normal and elevated total or specific IgE (intention-to-treat - ITT), there was no difference between the numbers of infants developing asthma while receiving cetirizine or placebo. The adverse events profile was similar in the two treatment groups. Discussion. Raised total IgE level and raised specific IgE levels to grass pollen, house dust mite or cat dander were predictive of subsequent asthma. Cetirizine halved the number of patients developing asthma in the subgroups sensitised to grass pollen or house dust mite (i.e. 20% of the study population). In view of the proven safety of the drug, we propose this treatment as a primary pharmacological intervention strategy to prevent the development of asthma in specifically sensitised infants with atopic dermatitis.     

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.     

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孤独症谱系障碍(autistic-spectrum disorders,ASDs)近年来患病率逐年攀升至1%左右,其症状往往伴随终生,成为严重威胁儿童健康和发展的神经发育性疾患;注意缺陷多动障碍(attention deficit hyperactivity disorder,ADHD)是儿童期最常见的精神障碍,国内报道患病率为4.13%～5.83%,其症状可延续至青少年期,甚至到成年期[1]。这两类精神障碍在成年期的临床表现、共患病、治疗策略和预后与儿童期有哪些不同呢?本文通过回顾相     

Infiltrations of Epstein-Barr virus-carrying cells in granular lymphocyte-proliferative disorders corresponding to chronic active Epstein-Barr virus infection

A 21-year-old man with granular lymphocyte-proliferative disorders (GLPD) associated with chronic active Epstein-Barr virus (EBV) infection is described. Chromosomal analyses revealed several clonal abnormalities and two of them were mainly repetitious. High copy numbers of monoclonal EBV genome were also detected in the proliferative large granular lymphocytes (LGLs), indicating the monoclonal expansion of EBV-infected LGLs. The patient had an indolent course for several years, and there was no evidence of infiltrations of his bone marrow until the end stage. At autopsy, microscopic studies revealed marked infiltrations of LGL in the liver and spleen, and the infiltrating cells were NK-cell immunophenotype. The infiltrated LGLs showed latency I.     

LUTEINIZING HORMONE RECEPTOR MUTATIONS IN DISORDERS OF SEXUAL DEVELOPMENT AND CANCER

Human male sexual development is regulated by chorionic gonadotropin (CG) and luteinizing hormone (LH). Aberrant sexual development caused by both activating and inactivating mutations of the human luteinizing hormone receptor (LHR) have been described. All known activating mutations of the LHR are missense mutations caused by single base substitution. The most common activating mutation is the replacement of Asp-578 by Gly due to the substitution of A by G at nucleotide position 1733. All activating mutations are present in exon 11 which encodes the transmembrane domain of the receptor. Constitutive activity of the LHR causes LH releasing hormone-independent precocious puberty in boys and the autosomal dominant disorder familial male-limited precocious puberty (FMPP). Both germline and somatic activating mutations of the LHR have been found in patients with testicular tumors. Activating mutations have no effect on females. The molecular genetics of the inactivating mutations of the LHR are more variable and include single base substitution, partial gene deletion, and insertion. These mutations are not localized and are present in both the extracellular and transmembrane domain of the receptor. Inactivation of the LHR gives rise to the autosomal recessive disorder Leydig cell hypoplasia (LCH) and male hypogonadism or male pseudohermaphroditism. Severity of the clinical phenotype in LCH patients correlates with the amount of residual activity of the mutated receptor. Females are less affected by inactivating mutation of the LHR. Symptoms caused by homozygous inactivating mutation of the LHR include polycystic ovaries and primary amenorrhea.     

EXCITING NEW TREATMENT APPROACHES FOR PATHYPHYSIOLOGIC MECHANISMS OF SICKLE CELL DISEASE

During the past several decades, our understanding of the complex pathophysiology of vasoocclusion associated with sickle cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vaso occlusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sickle cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for younger children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects of mutant genes.     

Personality profiles and heart rate variability (vagal tone) in children with recurrent abdominal pain

The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7-15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7-13 y) were also investigated, non-invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)-scale for most of the patients and 15 patients had scores above or close to the clinical cut-off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal. Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L-scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children.     

Understanding infant formula

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 healthcare professionals) will experiment with the infant formula available and then 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.     

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.