肝素和肾上腺皮质激素联合治疗过敏性紫癜31例报告

肝素已广泛应用于临床,文献记载对紫癜应禁用,故对过敏性紫癜的治疗国内报道甚少,我院儿科自1980年4月～1990年3月应用肝素治疗过敏性紫癜31例,疗效满意,现报告如下。临床资料病例随机分为观察组31例,对照组40例,两组患儿年龄、临床分型基本一致。治疗组男     

母乳性黄疸78例临床分析

近年来国内由于大力提倡母乳喂养,母乳性黄疸的患病率也明显增加。我院儿科于2004年7月~2005年7月收治的160例黄疸患儿中有78例为母乳性黄疸,几乎占50%。本研究对母乳性黄疸患儿的临床特点、诊断、治疗等进行讨论。1临床资料1·1一般资料78例患儿中男40例,女38例;早产儿10例,足     

中西医结合治疗36例小儿哮喘的临床疗效观察及护理

现将我院进行的中西医结合治疗小儿支气管哮喘的临床观察及护理报告如下。1资料与方法1.1一般资料从2002年1月~2004年6月我院收治的支气管哮喘患儿36例,男性17例,女性19例,年龄4~12岁。全部病例均符合全国儿科哮喘防治协作组制定的《儿童哮喘防治常规》诊断标准[1]。1.2方法将3     

维生素K缺乏性出血合并小儿巨细胞病毒感染20例临床分析

维生素K(VK)缺乏性出血是由于维生素K缺乏导致凝血因子下降而引起的出血,是儿科的急重症,常见于母乳喂养,营养不良,慢性腹泻,长期接受全静脉营养的患儿。临床工作中,我们发现,这些患儿中许多合并巨细胞病毒感染(CMV)。所以,我们将VK缺乏性出血合并巨细胞病毒感染的病例进行临床分析,报告如下。1临床资料1.1一般资料全部病例均系我科2005年6月~2006年6月住院患儿,年龄40d~3个月,男孩12例,女孩8例。发病后1~6d入院,住院天数10d~18d。上述病例均经血CMV-IgM及多次尿CMV-DNA检测符合CMV感染诊断标准[1],也符合VK缺乏性出血诊断标准[2]…     

氦—氖激光穴位照射佐治小儿肺炎48例疗效观察

1990年4月～1991年12月我们应用氦一氖激光穴位照射治疗48例小儿肺炎,现报告如下。临床资料一、一般资料全部病例均来自本院儿科门诊,病程2～7天,所有患儿均有咳嗽、肺部湿罗音及     

小儿左心房黏液瘤并发脑梗死和下肢动脉栓塞一例及文献复习

心脏黏液瘤占小儿原发性心脏肿瘤的第三位，在儿科临床上罕见，国内报告甚少。2005年3月我院收治1例左心房黏液瘤并发脑梗死和双下肢动脉多处栓塞的危重病例，已做黏液瘤切除术和下肢动脉取栓术，疗效满意，现报道如下，并复习文献，以期引起重视。     

中国科学引文数据库儿科期刊2010至2016年发表的病例报告质量评价

目的：评价中国儿科期刊发表的病例报告的报告质量,并分析其影响因素。方法：手工检索中国科学引文数据库（CSCD）收录的7本儿科期刊在2010年1月至2016年2月发表的病例报告,由2位研究者独立行文献筛选,资料提取,并采用2013年发表的病例报告规范条目表对所纳入文献进行评价,使用Stata12.0软件进行Meta分析。结果共纳入病例报告797篇,病例报告规范评分7～15 （11.1±1.5） 分,评分质量高（14～17分）36篇（6.0%）,评分质量中等（10～13分）638篇（62.7%）,评分质量差（～9分）123篇（31.2%）。报告率低于50%的病例报告规范条目有：题目、背景、时间轴、影响诊断的因素、影响预后的因素、疾病处理过程中值得借鉴的经验和存在的局限性。分层分析显示：病例报告整体报告质量,病例报告规范发表之后好于之前、有基金资助好于无基金资助;作者单位是否为三甲医院对病例报告整体报告质量影响不显著。结论：CSCD收录的7本儿科杂志中发表的病例报告质量有待提高,影响病例报告质量的因素主要有基金资助;合理利用病例报告规范将有助于提升病例报告的报告质量。     

威廉姆斯综合征3例报告

<正>威廉姆斯综合征(Williams syndrome,WS)是一种遗传性精神发育迟滞性疾病,该病的发病机制为7号染色体长臂近端(7q11.23)上弹性蛋白基因微缺失。本文就3例确诊的WS患者的病例资料进行报告如下,并总结了该病的临床特点及诊断方法。1病例资料病例1.患儿女,8岁。家长以"检查智商"为主诉于2014-12-27到中国医科大学附属盛京医院发育儿科门诊就诊。患儿系第2胎第2产,足月顺     

婴幼儿麻疹合并肺炎的护理

1临床资料2001年~2005年5月共收治12例患儿,其中男7例,女5例,年龄为6个月~7岁3个月。9例患儿来自农村,麻疹疫苗接种史不祥。另外3例均有明确的疫苗接种史。所有病例均符合《实用儿科学》临床诊断标准[1],其中7例病人有X线胸片检查的证据。临床表现发热、上呼吸道感染和麻疹黏膜斑。患儿体温高达39℃~40℃,同时伴有头痛、咳嗽、流泪等。多数患儿在第2d出现麻疹黏膜斑,多为白色斑点,周围红晕,1~2d后遍及全部颊黏膜。皮疹多见于耳后发际,渐延及面、颈、躯干、四肢及手足心底。为淡红色的丘疹,压之褪色。并发肺炎者多有咳嗽加剧,肺部可闻及少量…     

新生儿声嘶25例分析

小儿声嘶是儿科的常见疾病,但新生儿声嘶较儿童少见,发病无明显季节性,多伴有呼吸道感染。现就我科2003年5月至2005年10月25例新生儿声嘶分析如下。资料与方法一、资料来源25例新生儿声嘶的病例均来自我科2003年5月至2005年10月的住院病例。声嘶为家长的主诉之一,经检查25例患儿均有声嘶。二、方法25例新生儿声嘶的患儿均于入院后由喉科专科医生在直接或纤维喉镜下行喉部检查。结果25例患儿男15例,女10例,年龄1~28d,均有声嘶,均无产伤史,其中7例以单纯声嘶为主诉,无明显呼吸困难,诊断急性喉炎;18例声嘶伴有其他疾病,其中13例伴呼吸道感染症…     

先天性心脏病肺动脉高压手术前后右心功能变化和治疗

目的 了解先天性心脏病左向右分流导致的重度肺动脉高压对右心功能的影响以及手术矫治后右心功能的恢复状态。方法 对室间隔缺损(室缺)合并重度肺动脉高压(全肺循环阻力增加)的40例患儿，用心导管的方法进行术前、术后5～7年右心功能、肺循环的血流动力学随访测定。结果 术前右心心搏指数、作功指数、心排指数显著高于术后；术前右房压、右室收缩压及舒张压、肺动脉压力和阻力均增高，缺损修补后右室舒张压恢复正常，收缩压的降低与肺动脉压力下降有关；大型室缺左向右分流重度肺动脉高压右心功能不全系继发性右室高排出量心力衰竭，且伴有舒张功能障碍。结论 治疗心力衰竭不宜首选正性心肌收缩药物；降低肺动脉压力、减少左向右分流、根治心内畸形是合理的选择；术后右室收缩压持续不能恢复至正常水平，提示继发性肺血管梗阻性病变存在。     

Long term outcome of babies with pulmonary hypertension

Neonatal pulmonary hypertension (PH) is associated with many severe congenital abnormalities (congenital diaphragmatic hernia) or acquired cardiorespiratory diseases such as pneumonia, meconium aspiration and bronchopulmonary dysplasia (BPD). If no cause is found it may be labelled idiopathic persistent pulmonary hypertension of the newborn. Although PH may result in life threatening hypoxia and circulatory failure, in the majority of cases, it resolves in the neonatal period following treatment of the underlying cause. However, in some cases, neonatal PH progresses into infancy and childhood where symptoms include failure to thrive and eventually right heart failure or death if left untreated. This chronic condition is termed pulmonary vascular hypertensive disease (PHVD). Although classification and diagnostic criteria have only recently been proposed for pediatric PHVD, little is known about the pathophysiology of chronic neonatal PH, or why pulmonary vascular resistance may remain elevated well beyond infancy. This review explores the many factors involved in chronic PH and what implications this may have on long term outcome when the disease progresses beyond the neonatal period.     

先天性心脏病肺高压患儿血浆肾上腺髓质素浓度的测定及其临床意义:附33例报告

用特异性放射免疫非平衡法测定左向右分流型先天性心脏病 (CHD)患儿血浆肾上腺髓质素 (AM )水平 ,并观察心脏手术后AM的动态变化。结果显示 :CHD肺高压组AM明显高于无肺高压组及正常对照组 (P<0.05 ,P<0.01)。且AM随着肺高压病情加重而升高 ;中、重度肺高压组AM高于轻度肺高压组 ,差异有显著性 (P<0.01)。肺高压组术后AM明显下降 ,与术前比较差异有显著性 (P<0.05 ,P<0.01)。CHD组患儿AM与肺动脉收缩压 (PASP)呈显著正相关 (r=0.77,P<0.05)。本资料表明 ,AM参与了左向右分流型CHD合并肺高压的病理生理过程 ,AM的升高是机体的一种防御性反应 ,对维持肺循环稳定 ,减缓肺高压的发展有积极作用     

不同病因急性心力衰竭患儿血清氨基末端脑钠素原水平变化及其与肺动脉高压的关系

目的探讨先天性心脏病、肺炎并心力衰竭患儿血清氨基末端脑钠素原(NT-proBNP)水平变化及其与肺动脉高压(PH)的关系。方法采用竞争性酶免疫法检测66例住院心力衰竭患儿(肺炎并心力衰竭25例,先天性心脏病并肺炎心力衰竭22例,先天性心脏病并中重度PH心力衰竭19例)及60例对照组患儿(肺炎、先天性心脏病、健康儿童各20例)外周血NT-proBNP水平。多普勒超声心动图检测心力衰竭患儿心室射血分数(LVEF)、缩短分数(FS),对先天性心脏病患儿估测肺动脉压力。结果1.肺炎心力衰竭组及先天性心脏病心力衰竭组NT-proBNP水平无显著差异(P>0.05),但二组均较相应对照组(肺炎对照组、先天性心脏病对照组)显著增高(Pa<0.001);先天性心脏病并中重度PH心力衰竭组较先天性心脏病无PH或轻度PH心力衰竭组水平亦明显增高(Pa<0.05)。2.三组心力衰竭患儿间LVEF、FS水平比较差异无显著性(Pa>0.05)。3.肺炎对照组、先天性心脏病对照组较正常对照组NT-proBNP水平略高,但无统计学意义(Pa>0.05)。结论血清NT-proBNP对肺炎心力衰竭、先天性心脏病并心力衰竭均有较敏感而特异的诊断价值,且随肺动脉压力增高而呈增高趋势。     

Review of Inhaled Nitric Oxide in the Pediatric Cardiac Surgery Setting

Surgical intervention for congenital heart disease (CHD) can be complicated by pulmonary hypertension (PH), which increases morbidity, mortality, and medical burden. Consequently, postoperative management of PH is an important clinical consideration to improve outcomes. Inhaled nitric oxide (iNO) is a widely accepted standard of care for PH and has been studied in the context of cardiac surgery for CHD. However, large randomized, double-blind, placebo-controlled, multicenter clinical trials in pediatric patients are limited. This review will provide an overview of the clinical studies in this setting and will discuss general treatment considerations to facilitate a better understanding of the clinical use of iNO for PH after pediatric cardiac surgery.     

Inhaled nitric oxide therapy via nasopharyngeal tube in an infant with end-stage pulmonary hypertension

Abstract The delivery of nitric oxide (NO) via a nasopharyngeal tube is an alternative to endotracheal intubation. A male infant with end-stage pulmonary hypertension (PH) due to a severe hypoplastic lung developed a PH crisis on day 145 and received NO inhalation via a nasopharyngeal tube. Clinical improvement was maintained for 7 days with18–22ppm NO inhalation. The patient remained in close physical contact with his parents without the use of sedation. Blood methemoglobin levels remained below 1%. The environmental NO levels were less than 0.06 ppm and NO₂ less than 0.3 ppm throughout the treatment, well within the safety margin. On day 152, the patient succumbed to hypoxemia and heart failure. The use of a nasopharyngeal NO delivery system without sedation, as an alternative to endotracheal intubation with sedation, was a practical method in treating a patient with PH while maintaining a certain quality of life for the patient and the family.     

先天性心脏病合并感染性心内膜炎的诊断及治疗

目的：由于先天性心脏病（简称先心病）合并感染性心内膜炎（IE）的临床表现因疾病所处阶段的不同而呈多样必,这给早期诊断带来一定的困难。探讨IE的早期诊断和治疗的方法。方法：回顾性总结近10年收治的52例先心病合并IE的临床资料，就其诊断、抗生素选择、手术指征及影响预后的因素等作一分析。结果：发热、进行性心功能不全、心脏杂音改变、皮肤瘀点斑是常见的临床症状；实验室检查常半数以上有贫血、血沉（ESR）和C－反应蛋白（CRP）升高。血培养阳性28例（54％），其中葡萄球菌和链球菌占82％，二维超声（2DE）检查发现螯生物34例（65％）；26例（50％）因瓣膜受累造成顽固性心力衰竭，神经系统症状和周围血管栓塞并发症；死亡14例（27％）；7例经抗生素联合治疗后接受外科手术，均获痊愈。结论：先心病患儿包括已经外科手术纠治者，如存在不明显原因持续1周以上的发热，无明显心外病灶时，应仔细检查心脏杂音的变化，观察末梢血管栓塞表现，并及时及连续做规范的血培养检查及2DE检查，以明确IE的诊断。     

肺动脉高压的治疗现状与进展

肺动脉高压是一种威胁儿童生命的严重疾病.近10年来有关肺动脉高压的治疗取得了很大进展.目前有3类药物(前列环素类药物、内皮素受体拮抗剂及5型磷酸二酯酶抑制剂)在肺动脉高压患者治疗研究中取得很大进步,但多中心的随机对照研究尚待加强.房间隔切开术和心肺移植对于内科治疗无效的肺动脉高压患儿有一定疗效.     

Pulmonary hypertension in newborn infants: pathophysiology,clinical assessment and management

Clinically significant pulmonary hypertension (PH) affects up to 1 in 2000 infants after birth and is a major contributor to morbidity and mortality globally in newborn populations. Pulmonary hypertension is a component of many different neonatal diseases and can present a significant treatment challenge in both acute and chronic stages. PH is most commonly due to elevated pulmonary vascular resistance (PVR). This may be caused by structural and functional changes in the pulmonary vasculature, which may be combined with left, right or bi-ventricular dysfunction. Elevated PVR is frequently present from birth due to failure of the normal transition from fetal to extra-uterine environments, giving rise to the classic presentation of persistent pulmonary hypertension of the newborn (PPHN). Regardless of aetiology, the hallmarks of clinically significant PH are hypoxaemic right-to-left shunting, and cardiac dysfunction resulting in systemic cyanosis, hypotension and acidosis. Assessment of these informs targeted treatment aimed at optimising PVR and a supporting cardiac function. In this review we aim to provide an overview of the underlying pathophysiological concepts in PH, and use this to guide practical clinical management in all infants with PH. Based on the latest available evidence we discuss assessment of the severity of PH, and a structured, targeted approach to clinical management, acknowledging areas of uncertainty and controversy.     

Children with chronic organ failure possibly ending in organ transplantation: a survey in an Italian region of 5,000,000 inhabitants

Aim: The Italian Piedmont region sponsored in 2005 a population-based registry to assess the epidemiology of childhood chronic organ failure involving kidneys, liver, heart or lungs.
Methods: Patients in chronic organ failure who were younger than 18 years were selected, and entered the registry when accomplishing the standard failure criteria for each organ. The cases were reported by the general paediatricians of the region and integrated with the data gathered by the Children University Hospital, a tertiary care centre.
Results: In Piedmont (647 727 inhabitants < 18 years), a total of 146 children (217 cases per million of paediatric population) were found to be affected by chronic organ failure (mean age 10 years; range 0–17). The organ failure involved kidneys in 68 subjects (48%), liver in 24 (17%), heart in 21 (15%) and lungs in 28 (20%), and was severe in 32 subjects (6 on transplantation waiting list). The most represented disease leading to chronic renal failure was renal hypodysplasia (79%). Chronic liver failure was mostly caused by biliary atresia (30%), autoimmune hepatitis (25%) and Wilson's disease (21%). Dilated cardiomyopathy (62%) and surgically treated congenital cardiopathy were the two leading causes of chronic heart failure. The most represented disease leading to chronic lung failure was cystic fibrosis (89%).
Conclusion: This is the first report of the literature focusing on the epidemiology of chronic organ failure in children encompassing a region of 4 000 000 inhabitants. This clinical condition is rare, but medically and socially very demanding not only in childhood but the life along, as most of these patients will need solid organ transplantation decades later.