支气管肺泡灌洗在小儿呼吸系统疾病中的临床应用

支气管肺泡灌洗(bronchoalveolar lavage,BAL)是通过对支气管肺泡灌洗液(bronchoalveolar lavage fluid,BALF)成分进行分析,探讨肺部疾病局部免疫病理过程和发病机制,也可以通过注入药物直接起治疗作用.BAL在成人呼吸科已广泛开展,目前BAL在儿科主要用于呼吸道感染病原学诊断、喘息性疾病和慢性咳嗽病因/发病机制研究、新生儿疾病预后判断及难治性肺炎的治疗等.     

支气管肺泡灌洗在儿科中的应用进展

支气管肺泡灌洗 (BAL)是一种非常有价值的诊疗手段 ,儿科领域已广泛应用 ,而且安全有效 ,可用于研究气道与肺部疾病的发病机制、在肺泡灌洗液中寻找疾病的标志、用药物直接进行灌洗治疗疾病、评价药物作用等。但BAL在技术方法上仍不完善 ,缺乏标准化的方法 ,需要更多的探索。该文就近年来国外儿科领域在BAL的方法学、安全性、组分分析及临床应用中的一些新进展作一综述。     

支气管肺泡灌洗在儿科中的应用进展

支气管肺泡灌洗(BAL)是一种非常有价值的诊疗手段,儿科领域已广泛应用,而且安全有效,可用于研究气道与肺部疾病的发病机制、在肺泡灌洗液中寻找疾病的标志、用药物直接进行灌洗治疗疾病、评价药物作用等.但BAL在技术方法上仍不完善,缺乏标准化的方法,需要更多的探索.该文就近年来国外儿科领域在BAL的方法学、安全性、组分分析及临床应用中的一些新进展作一综述.     

支气管肺发育不良早产儿出生早期支气管肺泡灌洗液中相关因子检测的临床意义

支气管肺发育不良(bronchopulmonary dysplasia,BPD)是早产儿,尤其是极低、超低出生体重儿的较严重并发症之一,目前临床上尚缺乏早期检测BPD的手段.近年来,随着新生儿支气管肺泡灌洗技术(bronchoalveolar lavage,BAL)的开展,人们发现BPD早产儿在出生早期,其支气管肺泡灌洗液(bronchoalveolar lavage fluid,BALF)中的某些炎症因子、生长因子、蛋白和酶的水平即有不同程度的改变,可为临床早期预测BPD提供监控指标.     

支气管肺泡灌洗在儿童肺部感染病原学诊断中的应用

支气管肺泡灌洗(bronchoalveolar lavage,BAL)是指通过纤维支气管镜(简称纤支镜)或在气管插管下对目的肺段进行灌洗并回收小气道与肺泡衬液的一种检查方法.     

小儿支气管镜检及灌洗液细胞成分及其细胞因子水平的研究

支气管镜检查及支气管肺泡灌洗术(bronchoalveolarlavage,BAL)是支气管镜或导管通过获取肺泡内液体进行肺部疾病研究的一种方法。BAL作为一项新兴呼吸科技术,在国内外成人肺科已有许多成果,在儿科也有一些发展,但将其用于儿科应考虑其伤害性及安全性。因此使推广应用受到一定的限制。实践证明,在复合麻醉下,在保证通气和充分供氧情况下,适度灌洗,仍是安全的、通过支气管肺泡灌洗液(BALF)对其中的细胞因子进行检测,分析与疾病的关系,未见文献报道,现就其综述如下。     

气道阻塞幼儿支气管镜检查和支气管肺泡灌洗58例临床观察

目的 探讨支气管镜检和支气管肺泡灌洗在幼儿气道阻塞性疾病中的应用价值.方法 58例发生气道阻塞常规治疗欠佳的难治性肺部疾病患儿适时给予支气管镜检及支气管肺泡灌洗,对其临床资料进行回顾性分析.结果 58例患儿经交气管镜检明确诊断,经吸除分泌物、取出异物、清除肉芽、灌洗后及对症处理后,症状改善明显,疗效显著.结论 对保守治疗疗效不佳的难治性肺部疾病,支气管镜检和支气管肺泡灌洗起着重要的诊断和治疗作用.     

气道阻塞幼儿支气管镜检查和支气管肺泡灌洗58例临床观察

目的 探讨支气管镜检和支气管肺泡灌洗在幼儿气道阻塞性疾病中的应用价值.方法 58例发生气道阻塞常规治疗欠佳的难治性肺部疾病患儿适时给予支气管镜检及支气管肺泡灌洗,对其临床资料进行回顾性分析.结果 58例患儿经交气管镜检明确诊断,经吸除分泌物、取出异物、清除肉芽、灌洗后及对症处理后,症状改善明显,疗效显著.结论 对保守治疗疗效不佳的难治性肺部疾病,支气管镜检和支气管肺泡灌洗起着重要的诊断和治疗作用.     

气道阻塞幼儿支气管镜检查和支气管肺泡灌洗58例临床观察

目的 探讨支气管镜检和支气管肺泡灌洗在幼儿气道阻塞性疾病中的应用价值.方法 58例发生气道阻塞常规治疗欠佳的难治性肺部疾病患儿适时给予支气管镜检及支气管肺泡灌洗,对其临床资料进行回顾性分析.结果 58例患儿经交气管镜检明确诊断,经吸除分泌物、取出异物、清除肉芽、灌洗后及对症处理后,症状改善明显,疗效显著.结论 对保守治疗疗效不佳的难治性肺部疾病,支气管镜检和支气管肺泡灌洗起着重要的诊断和治疗作用.     

支气管肺泡灌洗在儿科的应用

支气管肺泡灌洗是指通过获取肺泡内液体进行肺部疾病研究的一种方法，近年在国内外日益受到重视。支气管肺泡灌洗在成人肺科应用广泛，但在儿科应用尚未推广。本文对儿科进行支气管肺泡灌洗的方法、临床应用、安全性等作一综述。     

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.     

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.     

Whole-body mineral measurements in Swedish adolescents at 17 years compared to 15 years of age

Aim: To provide reference data for bone mineral variables in 15- and 17-y-old adolescents and to analyse the relationships between these variables and measures of bone and body size, gender, puberty, growth, various lifestyle and environmental factors and socioeconomic background.

Methods: In the same 321 randomly selected adolescents (147 boys and 174 girls) living in two different regions of Sweden, the total bone mineral content (TBMC), bone area (BA) and total bone mineral density (TBMD) were assessed by dual-energy X-ray absorptiometry at ages 15 and 17 y. The effects of bone and body size, gender, growth, sexual maturity, physical activity, region of domicile, social conditions, food habits, smoking and alcohol intake on TBMC and TBMD were examined in multivariate analyses.

Results: In the 15-y-old adolescents, BA, height, gender, physical activity, maturity and weight explained 91% and 48%, of the variance in TBMC and TBMD, respectively. In similar analyses in the 17-y-olds, the corresponding figures were 92% and 62%, respectively, when BA, height, growth, physical activity, gender and region emerged as significant in the model. In all these analyses, BA explained most of the variance in TBMC and TBMD. No significant reduction of variance was found when different measures of social conditions, smoking, food habits, alcohol or dietary intakes of energy, calcium or vitamin D were included in the models. The reason why region of domicile had a significant impact on TBMC in the 17-y-olds is not known. The fact that the normal fluoride concentration in drinking water (1.1 mg/L) is 10 times higher in the region where TBMC was higher than in the other region is an interesting observation.

Conclusion: Almost 90% of the variance in TBMC and 50% of that in TBMD was explained by measures of bone and body size and only a few percent by gender, physical activity, Tanner stage, growth and region of domicile.     

VARICELLA ZOSTER VIRUS INFECTIONS IN THE PEDIATRIC STEM CELL TRANSPLANT PATIENT

Varicella zoster virus (VZV), a member of the human herpesvirus family, causes the clinical syndromes of chickenpox during primary infection and shingles on later reactivation. In immunocompromised patients, including those undergoing hematopoietic stem cell transplantation, VZV can produce life-threatening infections. The most serious forms of VZV infection involve hematogenous dissemination of the virus to vital organs, such as the lung, brain, and liver. Advances in immunoprophylaxis, antiviral chemotherapy, and vaccine development have provided effective tools to limit the morbidity and mortality previously associated with VZV infection in hematopoietic stem cell transplant patients. In this review, we discuss virologic aspects of VZV, pathogenesis of VZV infection, methods of viral diagnosis, clinical manifestations of infection in both normal and immunocompromised patients, and available preventative and therapeutic measures.     

The potential role of rapamycin in pediatric transplantation as observed from adult studies

Although pediatric patient and renal graft survival rates have shown marked improvements during the past decade, the persistent toxicities of immunosuppressive drugs and chronic allograft attrition remain major obstacles in transplant therapy. Results in adult patients suggest that complete steroid withdrawal is possible in the majority of recipients under treatment with a cyclosporin A-rapamycin (CsA RAPA) regimen. Furthermore, preliminary studies suggest that a marked reduction in the dose of CsA may be possible under the umbrella of RAPA coverage. The gain in immunosuppressive efficacy afforded by RAPA has not only been obtained without an increased morbidity owing to infectious or neoplastic causes, but also with the potential for reducing the incidence and/or progression of chronic rejection.     

Génétique des schizophrénies : mise en perspective des schizophrénies à début précoce et autres pathologies du développement

Schizophrenia (SCZ) is a severe brain disorder characterized by hallucinations, delusions, flat and/or inappropriate affect and cognitive impairment. The lifetime risk is about 0.5% with heritability of 65–85%. The prevalence of early-onset schizophrenia (defined here as before 15 years of age) has not been well studied, but is likely to be 5–10% of all cases. The rarity of early-onset SCZ has made it difficult to study. We focus on genetic studies of adults with schizophrenia, highlighting results for early-onset schizophrenia where available. Prior to the past 5 years, studies failed to find replicable association or linkage between SCZ and specific genes when appropriate statistical corrections for multiple testing were used. Many false positive results were probably reported using the candidate gene approach. Recently, the development of single nucleotide polymorphism (SNP) “chips” has permitted large genome-wide association study (GWAS) analyses that suggest that across all age groups, a proportion of genetic risk can be attributed to a large number of common SNP, each with a very small effect on risk (odds ratios of 1.1 or less). The greatest known genetic effect is conferred by the 1.5–3 Mb 22q.11.2 deletions, which occurs in ∼ 1/4000–1/6000 births with SCZ developing in 20–30% of carriers. Large SNP and aCGH microarray studies have now identified associations between SCZ and other rare, large copy number variations (CNV, insertions and deletions) with high odds ratios (5–10), including deletions of 1q21, 2p16.3 (neurexin-1 gene), 3q29 and 15q13.3, and duplications of 16p11.2. Some of these CNV are also associated with autism or other developmental disorders as well as epilepsy or intellectual deficiency, suggesting some overlap in the mechanisms that contribute to risks of these disorders. Based on preliminary data from larger-scale analyses in progress, approximately 1–2% of cases carry a CNV that has been clearly associated with SCZ (ORs 4–12). Whole exome and genome sequencing studies of large adult samples will be the next steps to identify rarer SCZ-associated mutations, including point mutations and smaller as well as rarer CNV. Genetic findings are beginning to contribute to an understanding of biological mechanisms of SCZ risk and may lead to new approaches to treatment.     

INACTIVATION OF PULMONARY SURFACTANT AND THE TREATMENT OF ACUTE LUNG INJURIES

Inactivation of pulmonary surfactant may be important in acute lung injury and acute respiratory distress syndrome. Treatment of surfactant dysfunction by instilling exogenous surfactants may improve gas exchange and pulmonary mechanics. Surfactants used for treatment vary in their attributes and effects, so when various surfactants are considered for therapy, resistance to inactivation is an important consideration. Animal models of acute lung injury exist in which the relative merits of surfactants can be compared. We hypothesize that the surfactants most resistant to inactivation in vitro will be the ones that are most effective in treatment of animal models of acute lung injury. Surfactants with higher concentrations of surfactant proteins (specifically A, B, and C) are more resistant to inactivation. Nonionic polymers mimic surfactant proteins in preventing surfactant inactivation under some conditions. Adding nonionic polymers to surfactant containing minimal amounts of SP-B and SP-C markedly improves lung function of animals with lung injury. Making surfactants more "inactivation-proof" may improve surfactant therapy of acute lung injuries.     

Plasma Proinsulin and C-Peptide Concentrations in Children with Hyperinsulinaemic Hypoglycaemia

ABSTRACT. Plasma concentrations of proinsulin and C-peptide were measured in five children presenting with svere hypoglycaemia associated with elevated plasma levels of immunoreactive insulin (IRI) in order to determine whether the profile of circulating B-cell products related to the underlying pathophysiology of the pancreas. Results were compared with data from 13 normal infants. Four children, three neonates and a nine year old girl, were subjected to partial or total pancreatectomy. The neonates had nesidioblastosis, nesidioblastosis with a microadenoma, and a functional abnormality without histological derangement respectively; the older child had a localised adenoma. The remaining child, a neonate, had transient hypoglycaemia and elevated IRI levels associated with hyperlactataemia and hyperalaninae-mia. All the children had markedly elevated plasma proinsulin concentrations; the highest levels were seen in the child with an isolated adenoma and in the neonate with nesidioblastosis and a microadenoma. Both of these children also had substantially elevated plasma C-peptide concentrations. The remaining three neonates had plasma C-peptide levels, which although in the normal range for normoglycaemia were inappropriately elevated during hypoglycaemia. It is concluded that elevated proinsulin and C-peptide concentrations are seen in children with hypoglycaemia associated with increased plasma IRI levels and that the profile of the concentrations does not provide a reliable marker for the nature of the underlying pancreatic abnormality.     

Growth tracks in early childhood

Aim: Child growth is modulated by numerous factors and, particularly in infancy and early childhood, often tends to follow apparently irregular patterns, with many centiles crossed before the later growth channels are reached. The aim of this study was to visualize the diversity of individual growth. Design: The study investigated 333 girls and 329 boys without chronic illnesses from four paediatric practices in Kiel, Germany. The children were measured on natural     

Residual pulmonary hypertension in children after treatment with inhaled nitric oxide: a follow-up study regarding cardiopulmonary and neurological symptoms

Inhaled nitric oxide is a potent vasodilator in acute severe pulmonary hypertension and is increasingly used as rescue treatment in intensive care algorithms aiming at reducing severe hypoxaemia in neonates and children. Although the immediate effects may seem impressive, longterm outcome regarding residual pulmonary hypertension and other sequelae has been studied in only a very few patients. The aim of the present study was to evaluate residual pulmonary hypertension, cardiopulmonary or neurological symptoms in children after treatment with inhaled nitric oxide in severely hypoxaemic and/or pulmonary hypertensive mechanically ventilated children. The study was performed in four paediatric intensive care units in university hospitals in Sweden, Norway and Australia. Patients who had received inhaled nitric oxide as part of their intensive care treatment for severe hypoxaemia and/or pulmonary hypertension, and in whom 6 mo had elapsed since treatment, were included for evaluation. Thus 36 paediatric or neonatal patients were examined for circulatory, respiratory or neurological disorders with clinical examination, echocardiography, chest X-ray and a capillary blood sample. Four patients with congenital heart disease had residual pulmonary hypertension. Nine patients were receiving bronchodilators. Sixteen patients had minor (n = 15) or moderate (n = 1) changes on a chest X-ray. One patient had a possible delay in psychomotor development. Conclusions: In spite of the severity of their primary illness, we found that the overwhelming majority of the surviving children were asymptomatic and doing well. The few residual circulatory and respiratory symptoms could be related to the initial condition.