Clinical management of hereditary angio‐oedema in children

Hereditary angio‐oedema (HAE) results from the deficiency of C1‐esterase inhibitor (C1‐INH). The clinical picture of this autosomal dominant disorder is characterized by recurrent attacks of subcutaneous oedema and/or potentially life‐threatening swelling of the submucosa. This review discusses the authors' decade‐long experience obtained in the treatment and follow‐up of pediatric patients with HAE. Twenty‐six children with HAE were reviewed. Pedigree analysis was performed in all cases to identify afflicted relatives. C1‐INH concentrate was reserved for the emergency treatment of acute oedematous attacks, whereas tranexamic acid and danazol were administered for short‐ or long‐term prophylaxis. Follow‐up care included laboratory tests and abdominal ultrasound, which was repeated at regular intervals. Twenty‐one children had Type I HAE and five suffered from Type II HAE. Clinical manifestations of the disease first occured in children when 2.5–12 years of age. Oedema formation primarily afflicted subcutaneous tissues. Mechanical trauma was identified as a precipitating factor in 20 patients. Pedigree analysis revealed 24 patients with relatives who suffered from HAE. Long‐term prophylaxis with tranexamic acid or danazol was initiated in 11 patients; two children required short‐term prophylaxis. No drug‐related adverse effects were observed, except for one case of delayed menarche. Therapy improved serum complement parameters significantly and substantially reduced the frequency and severity of clinical episodes. Adequate prophylaxis and follow‐up care can spare pediatric patients from oedematous attacks caused by HAE. Undesirable adverse effects can be avoided and the patient's quality of life enhanced considerably by administering the lowest effective drug dose.     

血液灌流在儿科临床的应用

血液灌流(HP)是血液借助体外循环,通过血液灌流器中具有特殊吸附功能的吸附剂,将溶解在血液中的有毒物质吸附的一种治疗过程,以达到除去患者血液中内源性或外源性毒物或致病物质的目的.它是血液净化学的重要组成部分,不仅对重症毒物、药物中毒有良好的疗效,而且目前亦可用于许多慢性、顽固性和疑难性疾病的治疗.     

免疫吸附在儿科临床的应用

免疫吸附(immunoadsorption)是将高度特异性的抗原、抗体或有特定物理化学亲和力的物质(配基)与吸附材料(载体)结合制成吸附剂(柱),选择性或特异地清除血液中致病因子的血液净化方法,不同于一般非特异的血液灌流.免疫吸附疗法是在血浆置换的基础上发展起来的新技术,其优点是对血浆中致病因子清除的选择性更高,而血浆中有用成分的丢失范围与数量更小,同时避免了血浆输入所带来的各种不良影响.     

儿童呼出气一氧化氮检测的临床应用

呼出气一氧化氮检测可反映气道嗜酸性粒细胞性炎症,近年来被广泛用于辅助支气管哮喘的诊断、监测支气管哮喘患者气道炎症以及监测患者对治疗效果的反应.呼出气一氧化氮检测作为一种无创性检查,能够得到成人及5岁以上儿童的高度配合,但在5岁以下儿童中的应用存在很多问题,目前国内尚缺乏婴幼儿及新生儿呼出气一氧化氮检测的正常值及相关大样本研究.该文主要从儿童呼出气一氧化氮检测的原理及方法、正常参考值、影响因素、临床应用等方面进行综述.     

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??The skeletal features of children are longitudinal growth and faster conversion of bone. Bone metabolism markers can sensitively reflect early bone metabolism status??which is useful in assessing growth situation??understanding the pathophysiology of metabolic bone disease and monitoring treatment. The different stages of age??race??sex??puberty??hormone regulation??nutritional status??diurnal changes and metabolic bone diseases can all affect the determination of bone metabolism markers in children and the establishment of the normal reference values. In this review??we will review the advances in bone formation and bone resorption markers and their applications in pediatric clinical practice.     

瑞芬太尼在重症患儿中的临床应用

镇痛和镇静治疗是重症患儿综合治疗的重要组成部分,随着镇痛镇静理念的发展,新的镇痛、镇静药物也不断推出,瑞芬太尼由于具有半衰期短、不产生蓄积、对患儿重要器官影响较小、可控性较好、镇痛效果较好及起效快等特点,现已较多的应用于重症患儿。本文探讨瑞芬太尼在重症患儿中的应用优势。     

血液透析在儿童危重症的临床应用

儿童血液透析(hemodialysis,HD)最早开始于20世纪50年代,临床主要应用于急性肾功能衰竭(ARF)的治疗,随着其治疗机制和相关技术的深入研究,又扩展到对多脏器衰竭、严重创伤、感染、中毒等危重病的救治[1].尽管已经建立了一些终末期肾病(ESRD)诊治指南,如美国肾脏病基金会提出透析预后及生存质量指南、急性透析患者生存质量指南以及欧洲儿童透析工作组(EPDWG)提出儿童透析实践指南,但有关儿童HD治疗时机、指征、透析剂量和HD效果的评价等尚未形成统一认识,这就导致在临床实践上存在差异.本文主要就儿童HD技术和临床应用进行介绍.     

小儿肝穿刺活体组织检查511例临床应用

目的探讨和推动小儿肝穿刺活体组织检查的开展和临床应用。方法对我院小儿肝病科１９８３年～１９９６年５１１例小儿肝活检情况进行回顾性研究。年龄最小１个月，最大１４岁，男∶女为２．６８∶１。均采用一秒钟肝穿法，按１９９５年５月中华传染病与寄生虫学术会议制订的病毒性肝炎病理诊断标准分型。肝穿刺指征如下：（１）原因不明的各种小儿肝病，包括原因不明肝脾肿大。（２）各型慢性病毒性肝炎，病程迁延反复，血清病毒标志持续阳性者。（３）准备实施抗病毒治疗（如干扰素）者。（４）部分重症肝炎。结果本组研究肝活检成功率较高，穿刺率逐年上升。病因中以乙、丙型病毒性肝炎为主，非甲～戊型约占６．５％，非病毒性肝病约占５．１％。结论小儿肝活检术是安全、简便、可行的，只要掌握指征，婴幼儿和年长儿同样成功与安全。通过小儿肝活检术的开展，可将小儿肝病的预防、诊断、治疗工作提高到一个新水平。     

Clinical characteristics of human bocavirus infections compared with other respiratory viruses in Spanish children

BACKGROUND: Human bocavirus (HBoV) can be found in a substantial proportion of children with respiratory tract diseases. The relative importance of HBoV in viral respiratory tract illnesses is not yet well known. OBJECTIVE: In this study, we looked for HBoV in pediatric patients to determine the incidence of HBoV as single infection and compared it with other commonly found respiratory viruses to describe the clinical differences associated with HBoV infections in children. PATIENTS AND METHODS: A prospective study was conducted on children less than 14 years old, admitted with respiratory infection from September 2005 to August 2007 to the Pediatrics Department of the Severo Ochoa Hospital, Madrid, Spain. We studied the frequency of HBoV and 15 other respiratory viruses in nasopharyngeal aspirates and compared the clinical course of the infections caused by HBoV with those caused by other common respiratory viruses. RESULTS: Positive results were confirmed in 435 (61.2%) of the 710 children studied. A single virus was detected in 308 patients. HBoV was found in 99 (13.9%) samples, but it was recovered as a single virus in only 35. Most of patients with HBoV infection (75%) were aged < or =26 months. The most common clinical diagnosis was recurrent wheezing (53%), followed by bronchiolitis (32%). Clinical differences were observed between HBoV and respiratory syncytial virus (RSV) infections (children were older and bronchiolitis less frequent), adenovirus (fever less frequent in HBoV group), and rhinovirus-associated infections (less hypoxia in HBoV group). CONCLUSIONS: HBoV was the fourth most frequent single virus after RSV, rhinovirus, and adenovirus in children hospitalized because of respiratory infection. It was associated with recurrent wheezing and bronchiolitis showing a different clinical course from other virus in terms of diagnosis, fever, and age.