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.

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.

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.

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.

[Pharmacokinetics and chronotherapy of delayed action theophylline (Euphyllin CR)]. / Farmakokinetika a chronoterapie protrahovane pusobícím teofylinem (Euphyllin CR).

Monitoring of theophyllinaemia in the course of 24 h during treatment with theophylline anhydrate capsules (Euphyllin CR) was used in 20 children (9-15 years) and in 20 adults (18-61 years) with bronchial asthma. The doses used in children were 5.96 mg/kg at 7 a.m. and 10.2 mg/kg at 7 p.m., while in adults the morning and evening doses were similar (4.35 and 4.89 mg/kg). After the first dose in the morning the serum theophylline concentration (STC) did not differ in children and adults (10.4 and 10.5 mg/l). In adults the half-time of absorption was twice as high as in children (0.63 and 0.3 h), as well as the time when the peak theophylline concentration was reached (2.7 and 1.39 h). In children a double dose of the drug at night ensured a STC of 11.2 mg/l and a maximum theophyllinaemia (C max = 13.5 mg/l between 8 p.m. and 6 a.m. (measured by the EMIT method). In adults the values during the night were significantly lower than in day-time (STC: ANOVA test P less than 0.05, C max: T-test P less than 0.01). The percentage fluctuation in individual subjects was small (36-49) interindividual, fluctuations in children and adults were considerable (105 to 207). From the chronoprofile of the pharmacokinetics of Euphyllin CR ensues that at the age of 9-15 years the most important indicators are similar to those in adults and that both groups should be given at night a double dose, as compared with that administered in the morning.

[Therapeutic approach to the treatment of acute asthma in childhood]. / Terapeutické postupy pri lécbe akutního astmatu v detském veku.

Acute deterioration of bronchial asthma with development of dyspnoea which involves the risk of failure of vital functions call for rapid and specialized therapeutic action which will prevent further deterioration of the condition and an as rapid as possible return of pulmonary functions to the range of normal values. In the treatment of acute asthma in the field as well as in in-patient departments different procedures are applied frequently based on tradition of the given department. In the submitted paper the author recapitulates recent views on the role of different procedures and the most frequently used drugs. The basis of modern therapy of acute asthma is above all inhalation treatment, high and frequent doses of beta 2-adrenergic preparations in particular during the initial stages of treatment and anti-inflammatory treatment with steroids. Theophyllines are considered drugs of second choice, the administration of infusions is also of limited importance. To ensure early treatment of acute asthma satisfactory and skilled cooperation of the patient and his closest surroundings is essential. Early therapeutic response to a deteriorating condition can substantially reduce the risk of development of serious conditions and reduce the need of hospital treatment.

[Use of the interruption method in the measurement of respiratory tract resistance in children]. / Vyuzití prerusovací metody pri merení odporu dýchacích cest u detí.

In a group of 50 children with a negative case- history as regards airways and lacking acute respiratory disease, the authors assessed the resistance of the airways on a whole-body plethysmograph and by using the interruptor method on a Bronchoscreen (Jaeger) apparatus. The authors found a satisfactory correlation of the two methods (r = 0.7). When relating the resulting values of resistance to height, greater differences were found between the two methods in younger children; with increasing height the differences disappear. For the relationship of the resistance assessed by the interruptor method and height in our group a Rint regression equation was calculated [kPa/(l/s)] = -0.00765 X height (cm) + 1,565.

[Bronchomotor tests in childhood]. / Bronchomotorické testy v detském veku.

The authors present a review of bronchoconstriction and bronchodilation tests which can be used in the diagnosis of respiratory diseases in children. Basic procedures and principles of evaluation are given.

[Study of bronchial reactivity by means of cold air inhalation]. / Vysetrování bronchiální reaktivity inhalací studeného vzduchu.

In 24 children with bronchial asthma and 16 children of a control group provocation by three-minute inhalation of cold air was applied under eucapnic condition on a RHES apparatus (E. Jaeger, GFR). After hyperventilation changes in the resistance of the airways were investigated by the occlusion method and repeatedly parameters of ventilation were examined by the flow-volume loop method. In the group of asthmatic children a significantly increased resistance was recorded after provocation with an onset already during the first minute after completed hyperventilation, and it persisted to the 9th minute. In the investigated parameters of the flow-volume loop (FEV 1, MEF 25/75) the maximum drop was recorded between the 3rd and 5th minute after provocation. In the control group no significant changes developed after provocation. Provocation by eucapnic hyperventilation of cold air is in particular in paediatric pneumological and allergological diagnosis a perspective method which does not cause discomfort to the patient and is well tolerated.

[Initial experience with surgical treatment of tracheal stenosis in small children using extracorporeal circulation]. / Prvé zkusenosti s chirurgickým resením stenózy trachey u malých detí s pouzitím mimotelního obehu.

Severe tracheal stenosis represents a life threatening malformation which necessitates early surgery. Two patients aged one and two years, respectively, were followed-up for signs of congenital stridor. Following respiratory infection both children became critically ill with severe dyspnoea necessitating intubation and artificial ventilation. In the first patient, echocardiography, tracheobronchoscopy and other investigations revealed a pulmonary artery sling with tracheal compression and hypoplasia of the whole trachea. In the second child, presence of a short local tracheal stenosis was found, the cause of which could not be clarified. In the patient with the pulmonary sling, resection and reimplantation of the anomalous left pulmonary artery was performed first. In both children, however, surgical reconstruction of the lower airways was necessary. Surgery was performed from a midline sternotomy approach in extracorporeal circulation. The hypoplastic trachea with circular rings in the first child was enlarged with a pericardial patch. In the second child, the local tracheal stenosis was resected and a direct anastomosis of the trachea was performed. In both patients, transient formation of granulations was observed. Both children, however, survived and their clinical condition remains good 18 and 9 months, respectively, after surgery. Tracheobronchoscopic controls show very good result. Our experience confirms the possibility of successful surgical reconstruction of lower airways in young children using extracorporeal circulation. Good interdisciplinary cooperation between the surgeon and other specialists is an important prerequisite of good surgical results.