Treatment of the congenital thoracic deformity pectus excavatum.

Pectus excavatum is the most common chest wall deformity in the Czech Republic. This chest deformity is typically characterized by a wall depression with sternal rotation. If the excavation of the chest wall does not cause any physical or psychological problems, the patient does not need any specific treatment. However, if the deformity is painful, affects the function of the lungs, heart or results in psychological problems, we can propose an appropriate treatment for the specific age category of the patient. Up to 10 years, we choose a procedure that includes targeted exercises and rehabilitation; in the age group of 10-15 years, we can add to the exercises the vacuum bell therapy according to the patient's wishes and compliance; and in the age category of 16 years and above, the patient can be offered a surgical solution. The Nuss operation (so-called MIRPE - minimally invasive repair of pectus excavatum) is the gold standard in surgical treatment; during this surgery, a patient-shaped bar is inserted retrosternally into the patient's chest under thoracoscopic control and is left for 3 years. The aim of this article is to describe the most common modern methods used in the treatment of patients with pectus excavatum, supplemented by a historical overview.

CT findings predicting lung resection in children with complicated community-acquired pneumonia.

PURPOSE: To investigate computed tomography (CT) features which predict lung resection in children with complicated community-acquired pneumonia. METHODS: A retrospective study of CT findings of patients with complicated pneumonia treated between January 2010 and December 2019. Fisher's exact test and ROC curves were used for statistical analysis. RESULTS: The study cohort consisted of 84 patients who underwent chest CT for complicated pneumonia. Lung resection was performed in 36 patients, 3 patients were treated by lung decortication, 45 patients were cured conservatively. Seven CT features were found statistically significant among the patients who underwent lung resection. 80.5% of patients from the resection group had two or more of these features on the initial CT scan, 64% had three or more. According to ROC analysis, simultaneous occurrence of multiple cavities equal to or greater than 3 cm and lung abscess predicted a pulmonary resection. CONCLUSION: The combination of CT features which clearly predict lung resection are the simultaneous occurrence of multiple cavities ≥ 3 cm and lung abscess. The most common triple combination of CT signs in the resected group of patients were multiple cavities ≥ 3 cm, consolidation of lung tissue and pleural effusion < 3 cm.

Pediatric bronchoscopy: recent advances and clinical challenges.

Introduction: During the last 40 years equipment has been improved with smaller instruments and sufficient size working channels. This has ensured that bronchoscopy offers therapeutic and interventional options.Areas covered: We provide a review of recent advances and clinical challenges in pediatric bronchoscopy. This includes single-use bronchoscopes, endobronchial ultrasound, and cryoprobe. Bronchoscopy in persistent preschool wheezing and asthma is included. The indications for interventional bronchoscopy have amplified and included balloon dilatation, endoscopic intubation, the use of airway stents, whole lung lavage, closing of fistulas and air leak, as well as an update on removal of foreign bodies. Others include the use of laser and microdebrider in airway surgery. Experience with bronchoscope during the COVID-19 pandemic has been included in this review. PubMed was searched for articles on pediatric bronchoscopy, including rigid bronchoscopy as well as interventional bronchoscopy with a focus on reviewing literature in the past 5 years.Expert opinion: As the proficiency of pediatric interventional pulmonologists continues to grow more interventions are being performed. There is a scarcity of published evidence in this field. Courses for pediatric interventional bronchoscopy need to be developed. The COVID-19 experience resulted in safer bronchoscopy practice for all involved.

European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia

The diagnosis of primary ciliary dyskinesia is often confirmed with standard, albeit complex and expensive, tests. In many cases, however, the diagnosis remains difficult despite the array of sophisticated diagnostic tests. There is no "gold standard" reference test. Hence, a Task Force supported by the European Respiratory Society has developed this guideline to provide evidence-based recommendations on diagnostic testing, especially in light of new developments in such tests, and the need for robust diagnoses of patients who might enter randomised controlled trials of treatments. The guideline is based on pre-defined questions relevant for clinical care, a systematic review of the literature, and assessment of the evidence using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach. It focuses on clinical presentation, nasal nitric oxide, analysis of ciliary beat frequency and pattern by high-speed video-microscopy analysis, transmission electron microscopy, genotyping and immunofluorescence. It then used a modified Delphi survey to develop an algorithm for the use of diagnostic tests to definitively confirm and exclude the diagnosis of primary ciliary dyskinesia; and to provide advice when the diagnosis was not conclusive. Finally, this guideline proposes a set of quality criteria for future research on the validity of diagnostic methods for primary ciliary dyskinesia

Selective increase in blood dendritic cell antigen-3-positive dendritic cells in bronchoalveolar lavage fluid in allergic patients.

Dendritic cells (DCs) are specific antigen-presenting cells that play critical roles in the initiation and polarization of immune responses. DCs residing in the lungs might be detected in the bronchoalveolar lavage fluid (BALF). We analysed DC compartment in the peripheral blood and BALF of patients with allergy and in controls. Plasmacytoid and four distinct subsets of myeloid DCs [characterized by the expression of blood dendritic cell antigen (BDCA)-1+ and -3+ and CD16 positivity or negativity] were detected in both tested compartments. We further evaluated the expression of C-type lectins [mannose receptor (MR), dendritic cell-specific intercellular adhesion molecule-3-grabbing non-integrin (DC-SIGN) and dendritic and epithelial cells (DEC)-205] relevant to the pathogenesis of asthma. Interestingly, we found a selective increase in the frequency of myeloid DC-expressing BDCA-3 and MR particularly in BALF from allergic patients. Specific and highly statistically significant increase in BDCA-3+ and/or MR+ DCs brings a novel characteristic to BAL analysis in allergic patients.

Assessment of problematic severe asthma in children.

Assessment of problematic severe asthma in children should be performed in a step-wise manner to ensure an optimal approach. A four-step assessment scheme is proposed. First, a full diagnostic work-up is performed to exclude other diseases which mimic asthma. Secondly, a multi-disciplinary assessment is performed to identify issues that may need attention, including comorbidities. Thirdly, the pattern of inflammation is assessed, and finally steroid responsiveness is documented. Based upon these four steps an optimal individualised treatment plan is developed. In this article the many gaps in our current knowledge in all these steps are highlighted, and recommendations for current clinical practice and future research are made. The lack of good data and the heterogeneity of problematic severe asthma still limit our ability to optimise the management on an individual basis in this small, but challenging group of patients.

Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children.

Primary ciliary dyskinesia (PCD) is associated with abnormal ciliary structure and function, which results in retention of mucus and bacteria in the respiratory tract, leading to chronic oto-sino-pulmonary disease, situs abnormalities and abnormal sperm motility. The diagnosis of PCD requires the presence of the characteristic clinical phenotype and either specific ultrastructural ciliary defects identified by transmission electron microscopy or evidence of abnormal ciliary function. Although the management of children affected with PCD remains uncertain and evidence is limited, it remains important to follow-up these patients with an adequate and shared care system in order to prevent future lung damage. This European Respiratory Society consensus statement on the management of children with PCD formulates recommendations regarding diagnostic and therapeutic approaches in order to permit a more accurate approach in these patients. Large well-designed randomised controlled trials, with clear description of patients, are required in order to improve these recommendations on diagnostic and treatment approaches in this disease.

Bronchoalveolar lavage fluid cellular characteristics, functional parameters and cytokine and chemokine levels in interstitial lung diseases.

Idiopathic pulmonary fibrosis (IPF), hypersensitivity pneumonitis (HP) and sarcoidosis belong to interstitial lung diseases (ILD) where an imbalance of regulatory, profibrotic and antifibrotic cytokines is hypothesized. The relationship of bronchoalveolar lavage (BAL) fluid (BALF) cytokines, BALF cell profile and ILD course is supposed. The aim of our study was to correlate BALF cytokine and chemokine levels with BALF cellular characteristics and lung function parameters in different ILD. Twenty-two sarcoidosis, seven IPF and 11 HP patients underwent lung function tests and BAL. The BALF differential cell counts and superficial cell markers were characterized, and MCP-1, MIP-1alpha, MIP-1beta, RANTES, epithelial neutrophil-activating protein (ENA)-78, FGF, G-CSF, GM-CSF, IFN-gamma, interleukin (IL)-1alpha, IL-1RA, IL-1beta, -2beta, -4beta, -5beta, -6beta, -8beta, -10beta, -17beta, tumour necrosis factor (TNF)-alpha, thromobopoietin (Tpo) and vascular endothelial growth factor (VEGF) values measured. The BALF VEGF values were highest in sarcoidosis (P = 0.0526). IL-1RA values were higher in IPF and HP compared with sarcoidosis (P = 0.0334). IL-8/ENA-78 ratio positively correlated with BALF neutrophil counts in IPF (r = 0.89, P = 0.04). Vital capacity and TL(CO) values positively correlated with VEGF and negatively with IL-8 BALF levels in all ILDs but the correlations were most significant in sarcoidosis group. We suppose that VEGF plays a role in ILDs' early phases and has rather angiogenic than profibrotic effect. On the contrary, IL-8 is probably upregulated in advanced ILDs with prominent fibrosis and marked lung functions decline. We state that BALF VEGF, IL-8 and ENA-78 levels and IL-8/ENA-78 ratio could become useful markers of ILDs' phase, activity and prognosis. They might also be helpful in treatment modality choice.

Prevalence of tidal expiratory flow limitation in preschool children with and without respiratory symptoms: application of the negative expiratory pressure (NEP) method.

Negative expiratory pressure (NEP) applied at the mouth during tidal expiration provides a non-invasive method for detecting expiratory flow limitation. Forty-two children were studied, i.e. 25 children with different respiratory symptoms (R) and 17 without any respiratory symptoms (NR). Children were examined without any sedation. A preset NEP of -5 cm H(2)O was applied; its duration did not exceed duration of tidal expiration. A significance of FL was judged by determining of a flow-limited range (in % of tidal volume). FL was found in 48 % children of R group. No patient of the NR group elicited FL (P<0.001 R vs. NR). The frequency of upper airway collapses was higher in R group (12 children) than in NR group (5 children). In conclusion, a high frequency of tidal FL in the R group was found, while it was not present in NR group. A relatively high frequency of expiratory upper airway collapses was found in both groups, but it did not differ significantly. NEP method represents a reasonable approach for tidal flow limitation testing in non-sedated preschool children.

Definition, assessment and treatment of wheezing disorders in preschool children: an evidence-based approach.

There is poor agreement on definitions of different phenotypes of preschool wheezing disorders. The present Task Force proposes to use the terms episodic (viral) wheeze to describe children who wheeze intermittently and are well between episodes, and multiple-trigger wheeze for children who wheeze both during and outside discrete episodes. Investigations are only needed when in doubt about the diagnosis. Based on the limited evidence available, inhaled short-acting beta(2)-agonists by metered-dose inhaler/spacer combination are recommended for symptomatic relief. Educating parents regarding causative factors and treatment is useful. Exposure to tobacco smoke should be avoided; allergen avoidance may be considered when sensitisation has been established. Maintenance treatment with inhaled corticosteroids is recommended for multiple-trigger wheeze; benefits are often small. Montelukast is recommended for the treatment of episodic (viral) wheeze and can be started when symptoms of a viral cold develop. Given the large overlap in phenotypes, and the fact that patients can move from one phenotype to another, inhaled corticosteroids and montelukast may be considered on a trial basis in almost any preschool child with recurrent wheeze, but should be discontinued if there is no clear clinical benefit. Large well-designed randomised controlled trials with clear descriptions of patients are needed to improve the present recommendations on the treatment of these common syndromes.

Diagnosis and treatment of asthma in childhood: a PRACTALL consensus report.

Asthma is the leading chronic disease among children in most industrialized countries. However, the evidence base on specific aspects of pediatric asthma, including therapeutic strategies, is limited and no recent international guidelines have focused exclusively on pediatric asthma. As a result, the European Academy of Allergy and Clinical Immunology and the American Academy of Allergy, Asthma and Immunology nominated expert teams to find a consensus to serve as a guideline for clinical practice in Europe as well as in North America. This consensus report recommends strategies that include pharmacological treatment, allergen and trigger avoidance and asthma education. The report is part of the PRACTALL initiative, which is endorsed by both academies.

Markers of eosinophilic inflammation and tissue re-modelling in children before clinically diagnosed bronchial asthma.

Chronic inflammatory changes in the bronchial mucosa have been well documented in patients with established asthma. Much less is known of the changes, which occur in the airways of children early in the evolution of their disease with most of the information based on indirect markers of inflammation only. We evaluated markers of inflammation and tissue re-modelling in bronchial biopsies from children with early respiratory symptoms before a clear clinical diagnosis of bronchial asthma could be made. We examined bronchial biopsies performed in 27 children between the ages of 1.2 and 11.7 yr who were bronchoscoped for a clinical indication because of recurrent or chronic respiratory symptoms. The patients were re-evaluated 22-80 months after the original bronchoscopy to determine whether or not they had subsequently developed bronchial asthma. There were more eosinophils in the bronchial mucosa (129.4 vs. 19.1 cells/mm2 of lamina propria, p <0.001) and the thickness of the subepithelial lamina reticularis was greater (4.65 vs. 3.72 microm, p=0.044) in children with bronchial asthma diagnosed at follow-up, compared with the children who did not progress to asthma. Eosinophilic inflammation and airway re-modelling occur early in the natural history of bronchial asthma and are present even before asthma would be diagnosed based on clinical symptoms. Recognition of these changes and their significance for clinical disease should emphasize the need for timely detection and diagnosis of asthma in children to facilitate the early introduction of anti-asthma therapy.

Flexible endoscopy of paediatric airways.

Paediatric fibreoptic bronchoscopy is used for ever wider indications, and increasingly used in many contexts, including paediatric and neonatal intensive care. The report of this Task Force contains an overview on the current applications of paediatric bronchoscopy. The report discusses the facilities and equipment needed for the procedure, including the newly developed bronchoscopes which are allowing intervention even in very small children. The indications of both flexible and rigid bronchoscopes in the context of newer and smaller flexible endoscopic equipment are also considered. The care of the instruments, including disinfection and sterilisation, is fully documented. Patient management is described, including the relative merits of conscious sedation and general anaesthesia, as well as special settings for the procedure, including the needs in intensive care. Special procedures, increasingly performed bronchoscopically are described. These include bronchoalveolar lavage, endobronchial and transbronchial biopsy, laser therapy, bronchography, and endoscopic intubation and drug therapy. Finally, neonatal bronchoscopy is discussed, and the ethics of bronchoscopic procedures, including bronchoscopic research in children. Advances in instrumentation, and also improved anaesthetic techniques, allow fibreoptic bronchoscopy to be safely performed in even very small, sick infants, provided proper precautions are taken.

Lung transplantation for cystic fibrosis: immune system and autoimmunity.

BACKGROUND: In the current study we focused on changes in the immune parameters of patients with CF after lung transplantation (Tx), with particular emphasis on the interaction of the immune system, infection, the autoimmune phenomenon observed in some CF patients, and immunosuppression. MATERIAL AND METHODS: Seven transplant patients with CF were investigated, 3 men and 4 women; the average age at Tx was 24.2 years (20.2-32.3). The parameters of both humoral immunity (immunoglobulins, complement, CRP, antinuclear and antineutrophil cytoplasmic antibodies) and cellular immunity (T and B lymphocytes, NK cells) were traced. RESULTS: We observed marked initial hyperimmunoglobulinemia, with a sharp drop in immunoglobulin levels within 1 month after Tx. Positivity for antineutrophil cytoplasmic antibodies (ANCA) was found in 3 patients before Tx. A strong ANCA positivity persisted 2 months after Tx despite deep introductory immunosuppression. In one patient ANCA positivity, after a transient negative result at months 2 and 12 after Tx, reappeared one year after Tx. The Burkholderia cepacia infections found in 2 patients proved to be lethal. CONCLUSIONS: In our series of CF lung transplant recipients, we found Burkholderia cepacia infection to be a risk factor. The robust appearance of autoantibodies and their persistent positivity for many months despite deep immunosuppression is a remarkable feature observed in some CF patients.

Serum ECP taken in the acute episode of bronchial obstruction can predict the development of bronchial asthma in young children.

For the early institution of anti-asthma treatment, reliable markers distinguishing the children with asthma from children with virus-associated wheeze are needed. Serum eosinophilic cationic protein (ECP) has been suggested as a marker correlating with the intensity of eosinophilic inflammation. We have studied 27 children (age 3 to 35 months) admitted with acute bronchial obstruction. Each child had been followed for 12 months after the first episode and then assigned to one of two groups (asthma or non-asthma) based on the clinical course. Serum ECP (s-ECP) was taken at the acute episode and again at least 6 months later, when the child was completely symptom-free. Serum ECP was analyzed using the Pharmacia CAP ECP FEIA immunofluorescence system. Mean s-ECP during the acute episode was 26.5 micrograms/L (5.5-69) in the asthma group (n = 14) and 9.7 (5.2-17 micrograms/L) in the non-asthmatics (n = 13), p < 0.01. There was no difference in the s-ECP analyzed during the symptom-free period. Elevated values of serum ECP taken during, but not outside, the acute episodes of bronchial obstruction may be helpful in predicting the development of bronchial asthma in young children with acute obstructive episodes.

Progression from allergic sensitization to asthma.

Asthma is one of the atopic diseases strongly associated with allergy. High aeroallergen exposure in the immediate postnatal period has been associated with higher risk of sensitization and chronic asthma. It is proposed that following in utero allergen sensitization, postnatal high dose allergen exposure localizes inflammation to the airways. In association with adjuvantizing effects of some virus infections, eosinophils and neutrophils are recruited which contribute to epithelial damage and the initiation of the remodelling process. Eventually, the latter processes lead to sufficient airway narrowing to manifest as the first symptoms of asthma. Thus, the immunopathology of asthma is fully established by the time of first symptoms and future strategies will need to identify those at risk of developing the disease before irreversible changes in the airways are established.

Issues in understanding childhood asthma.

Asthma and related allergic disorders in childhood have increased considerably in prevalence over the last few decades. During the same period of increasing morbidity from childhood asthma in the community, there have been dramatic advances in understanding of the basic immunopathologic features of the disease and consequently the development of a far more rational approach to its treatment. The immunopathologic condition of eosinophil-mediated airway inflammation is established very early in the evolution of asthma in childhood. It may even antedate the onset of symptoms. The present state of the art dictates that early intervention with potent therapies cannot be justified on the basis of symptoms alone and may in any case have no influence on the natural history of the condition. This means that current cautious therapeutic guidelines should continue to be followed. However, with the development of more accurate markers predicting ongoing disease, it will be possible to evaluate a whole range of early interventions in the future. Much evidence, though indirect, points to the possibility that the only true prophylaxis that will affect the natural history of asthma will need to be commenced before clinical features are manifest.