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SARS-CoV-2 has raised substantial concern for patients with chronic lung diseases. The aim of this study was to evaluate the clinical characteristics and outcomes of COVID-19 in children with persistent tachypnea of infancy (PTI).Data on the history of COVID-19, including diagnosis and clinical course of the infection, were collected during the regular follow-up visits of children previously diagnosed with PTI. The diagnosis of COVID-19 was based on laboratory criteria recommended by the European Centre for Disease Prevention and Control.Between January 1, 2021, and February 28, 2022, 62 patients with PTI (median age 3.7 years; 42 boys, 20 girls) were evaluated. COVID-19 was diagnosed in 38 patients (61.3%). Sixteen patients (42.1%) were asymptomatic, and 22 (57.9%) were symptomatic. The most common symptoms were rhinorrhea or nasal congestion, cough, and sore throat. Sixteen patients (42.1%) were classified as having a mild course of COVID-19, 3 children (7.9%) as moderate, and 3 children (7.9%) as severe. Follow-up performed after a median of 6 months (range 3-12 months) revealed that, after COVID-19, nearly all PTI patients returned to their prior status (32/38, 84.2%); 4 children (10.5%) suffered from exacerbating symptoms of PTI for 1 month. Two children (5.3%) became asymptomatic after COVID-19.The course of SARS-CoV-2 infection in children with PTI is usually mild, and the risk of hospitalization is low. The course of PTI did not change after COVID-19 in most children, and only a few patients experienced an exacerbation. This article is protected by copyright. All rights reserved.

BackgroundThe majority of patients with childhood interstitial lung disease (chILD) caused by pathogenic variants in ATP binding cassette subfamily A member 3 (ABCA3) develop severe respiratory insufficiency within their first year of life and succumb to disease if not lung transplanted. This register-based cohort study reviews patients with ABCA3 lung disease who survived beyond the age of 1 year.MethodOver a 21-year period, patients diagnosed as chILD due to ABCA3 deficiency were identified from the Kids Lung Register database. 44 patients survived beyond the first year of life and their long-term clinical course, oxygen supplementation and pulmonary function were reviewed. Chest CT and histopathology were scored blindly.ResultsAt the end of the observation period, median age was 6.3 years (IQR: 2.8–11.7) and 36/44 (82%) were still alive without transplantation. Patients who had never received supplemental oxygen therapy survived longer than those persistently required oxygen supplementation (9.7 (95% CI 6.7 to 27.7) vs 3.0 years (95% CI 1.5 to 5.0), p=0.0126). Interstitial lung disease was clearly progressive over time based on lung function (forced vital capacity % predicted absolute loss −1.1% /year) and on chest CT (increasing cystic lesions in those with repetitive imaging). Lung histology pattern were variable (chronic pneumonitis of infancy, non-specific interstitial pneumonia, and desquamative interstitial pneumonia). In 37/44 subjects, theABCA3sequence variants were missense variants, small insertions or deletions with in-silico tools predicting some residual ABCA3 transporter function.ConclusionThe natural history of ABCA3-related interstitial lung disease progresses during childhood and adolescence. Disease-modifying treatments are desirable to delay such disease course.

The goal of this study was to assess the pulmonary sequelae of COVID-19 pneumonia in children.Children (0-18 years old) diagnosed with COVID-19 pneumonia hospitalized between March 2020 and March 2021 were included in this observational study. All children underwent follow-up visit 3 months post-discharge, and if any abnormalities were stated, a second visit after the next 3 months was scheduled. Clinical assessment included medical history, physical examination, lung ultrasound (LUS) using standardized protocol, and pulmonary function tests (PFTs). PFTs results were compared with healthy children.41 patients with COVID-19 pneumonia (severe disease n=3, mechanical ventilation n=0) were included in the study. Persistent symptoms were reported by seven (17.1%) children, most common were decreased exercise tolerance (57.1%), dyspnea (42.9%), and cough (42.9%). The most prevalent abnormalities in LUS were coalescent B - lines (37%) and small subpleural consolidations (29%). The extent of LUS abnormalities was significantly greater at the first than at the second follow-up visit (p=0.03). There were no significant differences in PFTs results neither between the study group and healthy children, nor between the two follow-up visits in the study group.Our study shows that children might experience long-term sequelae following COVID-19 pneumonia. In the majority of cases these are mild and resolve over time. This article is protected by copyright. All rights reserved.

BACKGROUND Persistent tachypnea of infancy (PTI) is the most common interstitial lung disease in young children. As no standardized therapeutic guidelines exist, different pharmaceuticals are used to treat PTI; inhaled corticosteroids (ICS) and bronchodilators being mostly used. This observation assessed the effectiveness of bronchodilators and ICS in children with PTI enrolled in the children's interstitial lung diseases (chILD)-EU Register. METHODS Symptomatic children with PTI were observed according to a predetermined stepwise protocol including bronchodilators as the first choice treatment (6 weeks). In patients with incomplete response, additionally, ICS was given (12 weeks). Signs, symptoms, and pulmonary function were evaluated at three time points: at baseline, 6 (±1) weeks after initiation of bronchodilators, and 12 (±1) weeks after bronchodilators/ICS. RESULTS Thirty-one children (median age: 44 months, interquartile range [IQR]: 15-67) were included. The therapy was associated with a significant reduction of tachypnea (53.3% of patients, p = 0.02), exercise intolerance (52.2% of patients, p

Childhood interstitial lung disease (chILD) is a rare group of respiratory tract disorders. One of the factors suggested to be associated with its etiopathogenesis is microaspiration related to gastroesophageal reflux disease (GERD). The aim of the present study was to determine the frequency and characteristics of GERD in children with chILD, with a particular focus on proximal GER episodes.This was a prospective cross-sectional study. Consecutive patients with chILD underwent 24-h multichannel intraluminal pH-impedance monitoring. Different types of gastroesophageal reflux episodes (GER) were recorded and compared with regard to the GERD diagnosis.Sixty-two children (median age of 1.22 years) were included. GERD was diagnosed in 20 (32.3%) of them. The GERD (+) and GERD (-) subgroups differed mainly in their esophageal exposure to acid content (2.9 vs. 1.0%, p = 0.02) and bolus exposure (3.0 vs. 1.4%, p 0.0001), as well as total number of GER (72.5 vs. 42.0 p = 0.0004), acid GER (35.5 vs. 15.0 p = 0.004), and acid proximal GER (21.0 vs. 12.0 p = 0.02). There were no differences in the number of proximal GER comparing GERD (+) and GERD (-) subgroups.The frequency of GERD seems to be relatively high in the population of children with chILD used in this study. However, it has not demonstrated an association between proximal GER and GERD diagnosis in chILD, which casts uncertainty over the microaspiration theory proposed to link the two diseases. The latter conclusion, however, needs to be confirmed using more accurate aspiration assessment methods.

Data on the prevalence and type of lung function impairment in preschool and school-aged children previously diagnosed with persistent tachypnea of infancy (PTI) are scarce. Therefore, this study aims to assess pulmonary function in this age group.Children diagnosed with PTI over 3 years old were admitted for follow-up visits and healthy controls were enrolled. The study group included children who were able to complete pulmonary function tests (PFTs). Medical history, physical examination, and pulmonary function (spirometry, body plethysmography, impulse oscillometry, nitrogen multiple breath washout test, diffusing capacity for carbon monoxide [DLThirty-seven children (26 boys, 11 girls; median age: 5.6 years) diagnosed with PTI and 37 healthy controls were recruited. Forced expiratory volume in 1 s and forced vital capacity were significantly lower (-1.12 vs. 0.48, p = 0.002 and -0.83 vs. 0.31, p = 0.009, respectively); respiratory resistance at 5 Hz (0.06 vs. -0.62, p = 0.003), resonant frequency (1.86 vs. 1.36, p = 0.04), residual volume (RV) (2.34 vs. -1.2, p 0.0001), RV%TLC (total lung capacity) (2.63 vs. -0.72, p 0.0001), and specific airway resistance (5.4 vs. 2.59, p = 0.04) were significantly higher in PTI patients as compared with controls (data were presented as median z-score). Air trapping was found in 60.0%, and abnormally high lung clearance index and DLThis study demonstrated that lung function is affected in most children with PTI. PFTs showed that peripheral airways are the major zone of functional impairment.

BackgroundMonitoring disease progression in childhood interstitial lung diseases (chILD) is essential. No information for the minimal important difference (MID), which is defined as the smallest change in a parameter that is perceived as important prompting a clinician to change the treatment, is available. We calculated MIDs for vital signs (respiratory rate, peripheral oxygen saturation in room air, Fan severity score) and health-related quality of life (HrQoL) scores.MethodsThis study used data from the Kids Lung Register, which is a web-based management platform that collects data of rare paediatric lung disorders with a focus on chILD. Data of vital signs and HrQoL scores (Health Status Questionnaire, chILD-specific questionnaire and PedsQL V.4.0) were collected. MIDs were calculated according to distribution-based (one-third SD) and anchor-based methods (using forced expiratory volume in 1 s and forced vital capacity) as anchors.ResultsBaseline data of 774 children were used to calculate the following MIDs: respiratory rate 1.3 (z-score), O2saturation in room air 3.0%, Fan severity score 0.2–0.4, Health Status Questionnaire 0.4–0.8, chILD-specific questionnaire 4.4%–8.2%, physical health summary score 7.8%–8.9%, psychosocial health summary score 3.4%–6.9% and total score 5.1%–7.4%. Results of the responsiveness analysis generally agreed with the MIDs calculated.ConclusionsFor the first time, we provide estimates of MIDs for vital signs and HrQoL scores in a large cohort of chILD using different methods.

Purpose Proton-pump inhibitors (PPIs) are frequently used to treat gastroesophageal reflux disease (GERD) in children, but recent evidence suggests a potential association between PPI treatment and some types of infections. The aim of this study was to assess the effectiveness of Lactobacillus rhamnosus GG (LGG) for the prevention of gastrointestinal and respiratory tract infections in children with GERD treated with PPI (omeprazol). Methods Children younger than 5 years with GERD were assigned by a computer-generated list to receive LGG (109 colony-forming units) or placebo, twice daily, concomitantly with PPI treatment for 4-6 weeks; they were followed up for 12 weeks after therapy. The primary outcome measures were the percentage of children with a minimum of one episode of respiratory tract infection and the percentage of children with a minimum of one episode of gastrointestinal infection during the study. Results Of 61 randomized children, 59 patients (LGG n=30; placebo n=29, mean age 11.3 months) were analyzed. There was no significant difference found between the LGG and placebo groups, either for the proportion of children with at least one respiratory tract infection (22/30 vs. 25/29, respectively; relative risk [RR] 0.85, 95% confidence interval [CI] 0.66-1.10) or for the proportion of children with at least one gastrointestinal infection (9/30 vs. 9/29, respectively; RR 0.97, 95% CI 0.45-2.09). Conclusion LGG was not effective in the prevention of infectious complications in children with GERD receiving PPI. Caution is needed in interpreting these results, as the study was terminated early due to slow subject recruitment.

Lung ultrasound (LUS) has been increasingly used in diagnosing and monitoring of various pulmonary diseases in children. The aim of the current study was to evaluate its usefulness in children with persistent tachypnea of infancy (PTI). This was a controlled, prospective, cross-sectional study that included children with PTI and healthy subjects. In patients with PTI, LUS was performed at baseline and then after 6 and 12 months of follow-up. Baseline results of LUS were compared to (a) baseline high-resolution computed tomography (HRCT) images, (b) LUS examinations in control group, and (c) follow-up LUS examinations. Twenty children with PTI were enrolled. B-lines were found in all children with PTI and in 11 (55%) control subjects (P < .001). The total number of B-lines, the maximal number of B lines in any intercostal space, the distance between B-lines, and pleural thickness were significantly increased in children with PTI compared to controls. An irregularity of the pleural line was found in all patients with PTI and in none of the healthy children. There were no significant changes in LUS findings in patients with PTI during the study period. The comparison of HRCT indices and LUS findings revealed significant correlations between the mean lung attenuation, skewness, kurtosis and fraction of interstitial pulmonary involvement, and the number of B-lines as well as the pleural line thickness. LUS seems to be a promising diagnostic tool in children with PTI. Its inclusion in the diagnostic work-up may enable to reduce the number of costly, hazardous, and ionizing radiation-based imaging procedures.

In about 3% of children, viral infections of the airways that develop in early childhood lead to narrowing of the laryngeal lumen in the subglottic region resulting in symptoms such as hoarseness, abarking cough, stridor, and dyspnea. These infections may eventually cause respiratory failure. The disease is often called acute subglottic laryngitis (ASL). Terms such as pseudocroup, croup syndrome, acute obstructive laryngitis and spasmodic croup are used interchangeably when referencing this disease. Although the differential diagnosis should include other rare diseases such as epiglottitis, diphtheria, fibrinous laryngitis and bacterial tracheobronchitis, the diagnosis of ASL should always be made on the basis of clinical criteria.

Periostin is considered to be a marker of eosinophilic inflammation in patients with asthma. However, there are no literature data on exhaled breath condensate (EBC) periostin level in pediatric patients with asthma. The aim of this study was to analyze EBC periostin concentration in children with mild asthma and to evaluate the potential usefulness of EBC periostin level as a biomarker for the disease.EBC and serum periostin concentrations were measured by enzyme-linked immunosorbent assay in 23 children with asthma and 23 healthy controls.EBC periostin concentration was 250- to 780-fold lower than that found in serum. No significant differences between serum nor EBC periostin concentration in asthmatics and the control group were showed. The comparison between children with Th2 and non-Th2 type of asthma did not show significant differences in periostin concentration, both in serum and EBC. Serum periostin concentration inversely correlated with BMI and age not only in asthma patients but also in controls.In children with mild asthma, periostin may be measured not only in serum but also in EBC. The low periostin level in patients with mild asthma and lack of difference between asthmatic subjects and controls indicate that EBC periostin may not be useful as an asthma biomarker in this group.

Pathogenic variants in genes encoding aminoacyl-tRNA synthetases cause numerous disorders characterized by involvement of neurons, muscles, lungs and liver. Recently, biallelic FARSB defects have been shown to cause severe growth restriction with combined brain, liver and lung involvement (Rajab interstitial lung disease [ILD] with brain calcifications). Herein, for the first time, we present a patient with similar condition associated with biallelic mutations in FARSA (NM_004461.3: c.766T>C:p.Phe256Leu and c.1230C>A:p.Asn410Lys). Both detected FARSA variants are ultrarare and predicted to be damaging by in silico programs. Furthermore, they are both located in the active site of phenylalanyl-tRNA synthetase (PheRS) with Asn410Lys directly affecting a residue forming the wall of the phenylalanine-binding pocket. Clinical features shared between our patient and the FARSB syndrome include ILD with cholesterol pneumonitis, growth delay, hypotonia, brain calcifications with cysts and liver dysfunction. Our findings indicate that a disease similar to a syndrome associated with FARSB defects can also be caused by biallelic FARSA mutations. These findings are consistent with molecular structure of PheRS which is a tetramer including both FARSA and FARSB proteins.

Background Persistent tachypnea of infancy (PTI) is a rare pediatric lung disease of unknown origin. The diagnosis can be made by clinical presentation and chest high resolution computed tomography after exclusion of other causes. Clinical courses beyond infancy have rarely been assessed. Methods Patients included in the Kids Lung Register diagnosed with PTI as infants and now older than 5 years were identified. Initial presentation, extrapulmonary comorbidities, spirometry and clinical outcome were analyzed. Results Thirty-five children older than 5 years with PTI diagnosed as infants were analyzed. At the age of 5 years, 74% of the patients were reported as asymptomatic and did not develope new symptoms during the observational period at school-age (mean, 3.9 years; range, 0.3-6.3). At the age of about 10 years, none of the symptomatic children had abnormal oxygen saturation during sleep or exercise anymore. Lung function tests and breathing frequency were within normal values throughout the entire observational period. Conclusions PTI is a pulmonary disease that can lead to respiratory insufficiency in infancy. As at school age most of the previously chronically affected children became asymptomatic and did not develop new symptoms. We conclude that the overall clinical course is favorable.

Introduction: chILD comprises various rare respiratory conditions and partially overlaps with adult ILD. Lung fibrosis is the most common disease entity in adults; however, there are no data on the prevalence, clinical, radiological and histopathological presentation of pulmonary fibrosis in chILD. Aim: To assess the prevalence of fibrosis in lung biopsies from the international management platform, the European chILD registry (NCT02852928), and to correlate paediatric lung fibrosis with radiological findings. Methods: Features of fibrosis in biopsies were defined by the presence of any of the following: description of interstitial fibrosis, fibroblastic foci or honeycombing. A histological diagnosis of chronic pneumonitis of infancy (CPI) was also included. Fibrosis by chest computed tomography (CT) scans was defined by the presence of linear or reticular opacities, honeycombing, traction bronchiectasis or architectural distortion. Results: Lung biopsies were available for 149/825 chILD cases (18%); 46 biopsies (31%) had features of fibrosis and 11 were identified by CPI pattern alone. Mean age at biopsy was 6.4 years (range 0–26). Thirty cases were related to alveolar surfactant region (ABCA3: n=11; SFTPC: n=3), five to systemic disease processes (e.g. connective tissue disease: n=2) and three to immunodeficiency. Individual cases accounted for the other categories. Only 21/46 cases (46%) with biopsy-proven features of fibrosis had fibrosis features by CT. Conclusions: Features of fibrosis were already present in 31% of cases with a diagnostic lung biopsy. Concordance between biopsy- and CT-diagnosed fibrosis was lower than expected and requires further evaluation.

Introduction: The “Maps of Health Needs” project has been carried out in Poland since 2016 and its purpose is to implement quality-promoting and organisational solutions in the Polish healthcare system. This paper is the analysis of hospitalisations for chronic respiratory diseases recorded in Polish National Health Fund databases in 2014. Material and methods: The study included 122,000 hospitalisations of adults and 22,000 hospitalisations of children. Epidemio-logical parameters (incidence and prevalence) and major hospitalisation parameters were determined through statistical analysis. Results: The highest registered incidence was observed in asthma patients (548 per 100,000 inhabitants) followed by COPD patients (233 per 100,000 inhabitants). Asthma patients were also characterised by the highest prevalence, with lower values being observed in COPD patients. In the group of adults, patients aged 65 years or older and 80 years or older accounted for 44% and 14% of hospitalised adults respectively. The analysis also revealed that 66% of hospitalisations of adults included patients with asthma, COPD and respiratory failure. The development of respiratory failure prolongs hospitalisation and increases both in-hospital and post-discharge mortality. In children, 90% of the identified hospitalisations were for asthma, chronic inflammatory lung diseases and cystic fibrosis. Conclusions: The results of the study demonstrate that pulmonary obstructive diseases are associated with a considerable burden. Therefore, corrective actions within the Polish healthcare system are required to decrease the number of hospitalisations for these diseases.

We performed a prospective, observational, cohort study of children newly diagnosed with children’s interstitial lung disease (ChILD), with structured follow-up at 4, 8, 12 weeks and 6 and 12 months. 127 children, median age 0.9 (IQR 0.3–7.9) years had dyspnoea (68%, 69/102), tachypnoea (75%, 77/103) and low oxygen saturation (SpO2) median 92% (IQR 88–96). Death (n=20, 16%) was the most common in those 2

Od drugiej połowy XX wieku obserwuje się w Polsce systematyczny spadek liczby zachorowań na gruźlicę. Mimo to aktualne wskaźniki epidemiologiczne uzasadniają kontynuację powszechnych szczepień BCG w naszym kraju w najbliższych latach. Poza ochroną przed ciężkimi, krwiopochodnymi postaciami gruźlicy, szczepienie to zmniejsza ryzyko zakażenia prątkiem gruźlicy. Przeciwwskazania do szczepienia BCG to między innymi pierwotne i nabyte niedobory odporności, w tym zaburzenia immunologiczne związane ze stosowanym leczeniem. Do najczęstszych niepożądanych odczynów po szczepieniu BCG należą zmiany w miejscu szczepienia i zmiany odczynowe w lokalnych węzłach chłonnych, zazwyczaj niewymagające leczenia. Metody zapobiegania gruźlicy, szczególnie zalecane w krajach o niskiej zapadalności na tę chorobę, to: diagnostyka osób stykających się z chorym na gruźlicę płuc oraz aktywne wykrywanie i leczenie utajonego zakażenia prątkiem gruźlicy. Utajone zakażenie prątkiem gruźlicy rozpoznaje się na podstawie dodatnich wyników odczynu tuberkulinowego (OT) lub testu wydzielania interferonu gamma, po wykluczeniu gruźlicy aktywnej. Stan ten wymaga leczenia profilaktycznego.

Since the second half of the 20th century the incidence of tuberculosis has been declining in Poland. Despite this, current epidemiological data still support the need for the continued mass BCG vaccination in Poland in the near future. Apart from the protection against severe hematogenous forms of tuberculosis, vaccination lowers the risk of infection with Mycobacterium tuberculosis. Primary and acquired immunodeficiency, including immunity disorders associated with an ongoing treatment, are contraindications to BCG vaccination. The most common adverse effects following BCG vaccination are reactions at the site of injection and in regional lymph nodes, which usually does not require treatment. Methods of tuberculosis prevention, particularly recommended in low-incidence countries, include: diagnostic investigations of patients who had contacts with pulmonary tuberculosis as well as an active detection and treatment of latent Mycobacterium tuberculosis infection. Latent tuberculosis infection can be identified on the basis of positive results of the tuberculin skin test or interferon-gamma release assays after the active disease has been ruled out. This condition does require prophylactic treatment.

Aim The aim of the study was to describe the epidemiology and clinical characteristic of children hospitalized with pneumonia complicated by lung abscess, as well as to evaluate the long-term sequelae of the disease. Methods A retrospective review of medical records of all patients treated for pulmonary abscess in two tertiary centers was undertaken. Pulmonary function tests and lung ultrasound were performed at a follow-up. Results During the study period, 5151 children with pneumonia were admitted, and 49 (0.95%) cases were complicated with lung abscess. In 38 (77.5%) patients, lung abscess was treated solely with antibiotics, and in nine cases (16.3%) surgically. In 21 (51.21%) children complete radiological regression was documented. The mean time for radiological abnormalities regression was 84.14 ± 51.57 days, regardless of the treatment mode. Fifteen patients were followed up at 61.6 ± 28.3 months after discharge. Lung ultrasound revealed minor residual abnormalities: pleural thickening, subpleural consolidations and line B artefacts in 11 (73.3%) children. Pulmonary function tests results were abnormal in eight (53.3%) patients, the most frequent abnormality being hyperinflation. We did not find a restrictive disorder in any of the children. There were no deaths in our study. Conclusions Lung abscess is a rare but severe complication of pneumonia in children. Most children recover uneventfully with no significant long-term pulmonary sequelae.

Objectives To assess whether children ≤5 years of age, produce sufficient amounts of interferon gamma (IFN-ɣ) in response to phytohaemagglutinin (mitogen), and Mycobacterium tuberculosis antigens (TB antigens) in the QuantiFERON-TB Gold in-Tube test (QFT-GIT), (Cellestis Ltd., Australia). Working hypothesis Is TB-antigen-induced IFN-ɣ response in children ≤5 years sufficient to consider QFT-GIT a possible tool for TB diagnostics? Study design, patient-subject selection, and methods: We recruited children 0-17 years old suspected of TB infection to this cross-sectional study, in whom QFT-GIT and TST were performed. We analyzed the median IFN-ɣ levels in mitogen and TB antigen tubes in children ≤5 years and >5 years, and the correlation between IFN-ɣ level in both tubes and age. Results A total of 153 children were enrolled, age median was 7.8 (IQR:8), 45 (29.4%) aged ≤5 years (median 3.4, IQR:1.7), 108 > 5 years (median 10.55, IQR:5.93). In the mitogen tubes, the median IFN-ɣ level was higher in children >5 years (median 17.87, IQR:2.1 vs 16.77, IQR:7.6), but surprisingly in the TB antigen tubes it was higher in the younger group (median 0.12, IQR:0.21vs 0.06, IQR:0.09, P = 0.04). We proved a positive correlation between IFN-ɣ level and age in mitogen tubes (r = 0.18, P = 0.03) and a negative correlation in TB antigen tubes (r = -0.17, P = 0.04). In latent tuberculosis infection patients, the latter correlation was found to be even stronger (r = -0.39, P = 0.01). Conclusions The youngest children release sufficient amount of IFN-ɣ in response to TB antigens thus QFT-GIT might be a useful tool for TB diagnostics in this age group.

Experts of four scientific societies and the independent experts described the position paper concerning the role of inhalation chambers in inhalation therapy in children. This document is directed to all paediatricians and family doctors. We have reviewed the most important original and review papers, together with an analysis of the holding chambers market in the country. Recommendations containing indications and principles of the selection as well as methods of inhalation from these devices in children at different age were pointed out.

Community-acquired pneumonia in children is still an important health problem, even in high-income countries. In adults, a combination of clinical signs, symptoms and laboratory tests (e.g. CURB-65) is used to estimate the severity and prognosis. In children, the assessment of severity is based mainly on clinical symptoms and there is no objective, universal score system for this purpose. Therefore, authors undertook a study which aimed at the evaluation of whether N-terminal pro-brain natriuretic peptide (NT-proNB), as a marker of myocardial stress, can be used to differentiate children with severe and non-severe pneumonia. Material and methods: The study included children with a diagnosis of pneumonia, divided into two groups severe/non-severe pneumonia according to clinical symptoms. All patient underwent clinical evaluation, chest x-ray and laboratory tests: blood morphology, c-reactive protein (CRP) and NT-proBNP. Results: Forty-three patients were included in the study (mean age 89.5 ± 61.7 months, 44.2% males). There was 13/30 severe/non-severe pneumonia diagnosis respectively. The median duration of hospitalization was 8.5 days (2 - 35 d.) and it was significantly longer in the group with severe pneumonia (20 vs.8 d.). NT-proBNP was elevated in 29 patients (67%), including all 13 patients with severe pneumonia and 16 with non-severe. The NT-proBNP concentration, CRP and leucocyte count was significantly higher in the group with severe pneumonia (p= 0.00067, p = 0.0394 and p = 0.0229 respectively). The length of hospitalization was positively correlated with NT pro-BNP concentration (r = 0.354 p Conclusion: NT-proBNP seems to be a promising marker for the childhood pneumonia severity stratification.

This is a retrospective study whose main objective was to analyze the influence of the Polish Guidelines for the Management of Respiratory Tract Infections of 2010 (PGMRTI) on in-hospital treatment of children with community-acquired pneumonia (CAP). Files from four Warsaw hospitals were reviewed to identify children with uncomplicated CAP, treated before (2008-2009) (pre-PGMRTI) and after (2011-2012) (post-PGMRTI) publication of the guidelines. Predefined data on the management were compared. A cohort of 2,359 children (1,081 pre-PGMRTI and 1,278 post-PGMRTI) was included. We found that co-amoxiclav was the most common first-line therapy in children3 months of age (34.6% and 40.4% pre- and post-PGMRTI, respectively), followed by cefuroxime (31.8% and 20.9% pre- and post-PGMRTI, respectively; p 0.0001) and macrolides (17.4% and 24.5% pre- and post-PGMRTI, respectively; p 0.0001). Amoxicillin was rarely used (5.4% and 4.9%, pre- and post-PGMRTI, respectively). The study revealed an overuse of inhaled bronchodilators, corticosteroids, and mucoactive drugs. Blood diagnostic tests were applied to a significant percentage of patients: blood cultures (41.2% and 44.5% pre-and post-PGMRTI, respectively) and serology for atypical pathogens (27.9% and 44.9% pre-and post-PGMRTI, respectively; p 0.0001). The number of follow-up chest X-rays increased (30.5% and 53.8% pre- and post-PGMRTI, respectively; p 0.0001). In conclusion, the study demonstrates an unsatisfactory influence of the guidelines on in-hospital management of CAP in children. Despite an explicit recommendation for the use of amoxicillin, it was still underused. Other methods of education and guideline dissemination are needed to optimize the prescribing of antibiotics.

This is a retrospective study whose main objective was to analyze the influence of the Polish Guidelines for the Management of Respiratory Tract Infections of 2010 (PGMRTI) on in-hospital treatment of children with community-acquired pneumonia (CAP). Files from four Warsaw hospitals were reviewed to identify children with uncomplicated CAP, treated before (2008–2009) (pre-PGMRTI) and after (2011–2012) (post-PGMRTI) publication of the guidelines. Predefined data on the management were compared. A cohort of 2,359 children (1,081 pre-PGMRTI and 1,278 post-PGMRTI) was included. We found that co-amoxiclav was the most common first-line therapy in children >3 months of age (34.6% and 40.4% pre- and post-PGMRTI, respectively), followed by cefuroxime (31.8% and 20.9% pre- and post-PGMRTI, respectively; p < 0.0001) and macrolides (17.4% and 24.5% pre- and post-PGMRTI, respectively; p < 0.0001). Amoxicillin was rarely used (5.4% and 4.9%, pre- and post-PGMRTI, respectively). The study revealed an overuse of inhaled bronchodilators, corticosteroids, and mucoactive drugs. Blood diagnostic tests were applied to a significant percentage of patients: blood cultures (41.2% and 44.5% pre-and post-PGMRTI, respectively) and serology for atypical pathogens (27.9% and 44.9% pre-and post-PGMRTI, respectively; p < 0.0001). The number of follow-up chest X-rays increased (30.5% and 53.8% pre- and post-PGMRTI, respectively; p < 0.0001). In conclusion, the study demonstrates an unsatisfactory influence of the guidelines on in-hospital management of CAP in children. Despite an explicit recommendation for the use of amoxicillin, it was still underused. Other methods of education and guideline dissemination are needed to optimize the prescribing of antibiotics.

Background An increasing incidence of parapneumonic effusion and pleural empyema (PPE/PE) has been reported in recent studies. As only few data on etiology of PPE/PE in Central Europe have been reported, we undertook a study on the etiology of PPE/PE in children, using both standard culture and molecular techniques. Methods This prospective study was conducted between June 2011 and December 2013. Consecutive children with PPE/PE complicating community acquired pneumonia, who required diagnostic/therapeutic thoracentesis were included. Blood and pleural fluid samples for microbiological cultures were collected. Molecular methods were applied to identify Streptococcus pneumonia, Haemophilus influenzae, Staphylococcus aureus, Streptococcus pyogenes, Mycoplasma pneumoniae, Chlamydophila pneumoniae, and respiratory viruses in pleural fluid. Results The study group included 64 children, median age 4 (1–15). Seven of 64 (10.9%) blood cultures and 11 of 64 (17.2%) pleural fluid cultures revealed bacterial growth. The most common bacteria detected was S. pneumoniae (13 blood and pleural fluid samples from 11/64 (17.2%) children). DNA sequences of typical bacteria were found in 29/64 (45.3%) pleural fluid samples. S. pneumoniae was identified in 90% of these samples. The most common serotypes were: serotype 6B in 9/26 (36.6%), 19A in 6/26 (23%), serotype 3 in 3/26 (11.5%), 6A and 23F (both in 2/26 i.e. 7.7%) patients. Molecular methods identified atypical bacteria in 8/58 (13.8%) and respiratory viruses in 12/58 (20.7%) pleural fluid samples. Conclusions S. pneumoniae, in particular serotype 6B and 19A, is the most common etiologic agent of PPE/PE in Polish children. The use of PCR significantly improves pathogen identification in pleural fluid., Highlights • Pleural empyema is challenging complication of childhood community acquired pneumonia. • S. pneumoniae is the most common pathogen responsible for pleural empyema in children. • Serotypes 6B and 19A were most commonly identified as causative pathogens. • The use of PCR improves the efficacy of pathogen identification in pleural fluid. • Molecular methods can help to detect atypical bacteria and viruses in pleural fluid.

Introduction: Connective tissue diseases (CTDs) of childhood are rare inflammatory disorders, involving various organs and tissues including respiratory system. Pulmonary involvement in patients with CTDs is uncommon but may cause functional impairment. Data on prevalence and type of lung function abnormalities in children with CTDs are scarce. Thus, the aim of this study was to asses pulmonary functional status in children with newly diagnosed CTD and follow the results after two years of the disease course. Material and methods: There were 98 children (mean age: 13 ± 3; 76 girls), treated in Department of Pediatric Rheumatology, Institute of Rheumatology, Warsaw and 80 aged-matched, healthy controls (mean age 12.7 ± 2.4; 50 girls) included into the study. Study procedures included medical history, physical examination, chest radiograph and PFT (spirometry and whole body-plethysmography). Then, the assessment of PFT was performed after 24 months. Results: FEV1, FEV1/FVC and MEF50 were significantly lower in CTD as compared to control group, there was no difference in FVC and TLC. The proportion of patients with abnormal lung function was significantly higher in the study group, 41 (42%) vs. 9 (11%). 24-months observation didn’t reveal progression in lung function impairment. Conclusions: Lung function impairment is relatively common in children with CTDs. Although restrictive ventilatory pattern is considered typical feature of lung involvement in CTDs, airflow limitation could also be an initial abnormality.

A boy aged 8, with chronic diarrhoea, was admitted to clinic for diagnosis of Crohn's disease. The diagnosis was confirmed and total enteral nutrition initiated. Due to intolerance of oral intake, it was decided to insert gastric tube for nutrition. Two attempts of insertion were unsuccessful due to stubborn vomiting and cough – only minor amount of polymeric diet was administered. During next 24 hours the general state of patient worsened sharply. Dyspnoea and tachypnoea were observed, dull percussion sound and decreased breath sound were heard. The imaging of chest revealed presence of a fluid in the right pleural cavity. Pleural drain was inserted and yellow-white fluid (biochemical features of chylous and complicated parapneumonic effusion) was obtained. Chylothorax and necrotizing pneumonia were diagnosed. Pleural drainage was kept for next 12 days, along with antibiotics, fat free diet, albumins and intensive symptomatic treatment. The improvement of patient's general state and resolution of pulmonary changes were achieved. A rupture of thoracic duct leading to chylothorax in children due to stubborn vomiting has been already discussed in literature – however, such a case has never been described in association with Crohn's disease.

Introduction: Isolated pulmonary Langerhans cell histiocytosis (PLCH) is very rare disease in paediatric population. Objective: Herein, we present a case report of a progressive PLCH disease in a teenager who was qualified for second line treatment with cladribine Case Report: Ten year old boy was admitted to our department due to the chest pain and exercise intolerance. Cystic lesions found in the chest X-ray were confirmed in computed tomography. Specimen obtained during bronchoscopy contained cells which had positive immunoreactivity for CD1a. Based on histopathologic examination of the lung biopsy LCH was diagnosed. Other localizations of the LCH were excluded. Twelve weeks treatment with vinblastin and prednisone according to the recommended protocols for PLCH was conducted. Due to the stable appearance of cystic lesions in control HRCT treatment was stopped. After 4 years the boy was admitted due to the progression of the disease and exercise intolerance. The boy was constantly exposed to tobacco smoke. On physical examination he presented with diminished breath sounds in both lung fields. Pulmonary function tests showed an obstructive pattern. In the PET-CT study active infiltrations in both lung apices were found. He was qualified to the second line treatment with cladribine as a single agent. After four 5-day- courses at monthly intervals of cladribine PET study revealed remission of active infiltrates. The improvement in exercise tolerance was observed. Conclusions: Cladribine therapy may be effective in patient with progressive PLCH disease in children.

Introduction: Childhood Interstitial lung disease (chILD) is a group of rare lung disorders of different origin. The symptoms of chILD are non specific and involve not only respiratory system. Clinicians usually focus on daytime functioning. There is very limited data concerning sleep characteristic in children with interstitial lung disease. Aim of the study: The goal of this study was to assess impact of chILD on sleep quality, architecture and respiratory parameters as well as to evaluate whether children with chILD are susceptible to sleep apnea due to chronic lung disease. Methods: Patients with chILD diagnosed on the basis of clinical history, chest computed tomography and lung biopsy (if taken), who were admitted to our department between May 2017 and February 2018 for a check-up visit had a polysomnography performed. All of the patients were in stable state during the study, one child used supplemental oxygen during sleep. Results: Six patients were qualified for a sleep study. The age in this group ranged from 11 months to 11 years. Polysomnography revealed that five patients had obstructive sleep apnea (OSA). Apnea - hypopnea index varied between 0,9 and 16,5. Two patients had mean oxygen saturation below 92%, desaturation index varied from 4,6 to 16,9 per hour. There were no significant abnormalities in the sleep architecture in all patients. Conclusions: Interstitial lung disease can affect sleep. Patients with chILD are susceptible to obstructive sleep apnea, therefore screening for OSA should be considered in all of them. More studies are needed to evaluate accurately the influence of chronic lung disease on different parameters of sleep. Hence study on a bigger group of patients is planned.

Lung ultrasound (LUS) is as an easily accessible, radiation-free imaging technique that might be used as a diagnostic tool in community-acquired pneumonia (CAP). The aim of the study was to evaluate the usefulness and accuracy of LUS in the diagnosis and monitoring of childhood CAP. One hundred six consecutive children aged between 1 and 213 (median 52.5) months referred to the hospital with suspicion of CAP were enrolled. All patients underwent LUS on the day of admission, followed by chest radiograph (CXR). Lung ultrasound was also performed in 25 children between 5th-7th and 31 children between 10th-14th day after admission. Radiographic signs of pneumonia were demonstrated in 76 children, while lung ultrasound revealed pulmonary abnormalities consistent with pneumonia in 71 children. LUS gave false negative results in 5 patients with parahilar pulmonary infiltrates demonstrated by CXR. Almost perfect overall agreement between LUS and CXR was found in terms of pneumonia diagnosis (Cohen kappa coefficient of 0.89). The diagnostic performance of LUS in demonstration of lung involvement was as follows: sensitivity of 93.4%, specificity of 100%, positive predictive value of 100%, negative predictive value of 85.7% and accuracy of 95.3%. Our study showed that LUS is a sensitive and highly specific diagnostic method in children with CAP. Therefore, LUS may be considered as the first imaging test in children with suspicion of CAP. A diagnostic algorithm of CAP which includes LUS should be validated in prospective studies. Lung ultrasound can also be used to follow-up resolution of pneumonic lesions.

Background: In 2010 in Poland was published Recommendations for Management of Respiratory Tract Infections (RMRTI) which support medical practitioners in every aspect of management of community acquired pneumonia (CAP). Objective: To analyze the impact of RMRTI on antibiotic prescribing in children with CAP. Material and Methods: We assessed prehospital and hospital antibiotic use in CAP in 4 pediatric hospitals before (2008-2009) and after (2011-2012) RMRTI publication. Results: A group of 2359 children with CAP was enrolled. In outpatients the most common first-line antibiotics were: second generation cephalosporins (CEF), co-amoxiclav and macrolides. There were no differences between pre-recommendation group (PreRG) (n=1081) and post-recommendation group (PostRG) (n=1278). In hospitalized children significant change in first-line therapy was observed in PreRG and PostRG periods: CEF (57.1% vs 16.5% p Conclusion: The study showed that publishing recommendations is not a sufficient form of education. Despite recommendations amoxicillin is still rarely prescribed in CAP in children. Other forms of active teaching are needed to convince clinicians to adhere to the guidelines.

Introduction: Children’s interstitial lung disease (chILD) comprises a heterogeneous group of over 200 different entities of diffuse lung diseases with symptom onset in childhood. Only 1-3 of one million children are affected per year. No evidence based treatment guidelines exist. Aim: First analysis of the distribution of chILD disease entities using a classification system also including cases without lung biopsy (Orphanet J Rare Dis 2015 10:122), as well as respiratory medications prescribed. Methods: Patients with chILD reported to the European Management Platform for Childhood Interstitial Lung Diseases between December 2012 and November 2016 were included. Baseline and follow up visits including information on disease course and respiratory treatments used in the whole cohort and specific subcategories were analysed. Results: 366 peer-reviewed patients received a final diagnosis: the most common was chILD related to alveolar surfactant region (category A4, n=194), followed by chILD related to systemic disease (B1, n=60), specific conditions of undefined etiology (A3, n=59) and chILD of the immune intact host (B2, n=46). The most commonly used drugs at baseline were glucocorticosteroids in 175 cases (48%), especially in the subcategories A4 and B1. Azithromycin was used in 24% and hydroxychloroquine in 14%. Conclusion: The knowledge of most frequently diagnosed subcategories of chILD and most commonly used medication in these groups will inform controlled studies in the future, especially for glucocorticosteroids and azithromycin in the subcategories A4 and B1.

Background: Neuroendocrine cell hyperplasia of infancy (NEHI) is an idiopathic interstitial lung disease (ILD) of childhood. HRCT is a gold standard in diagnosis of NEHI. As HRCT has some limitations, lung ultrasound (LUS) arouses great interest as an alternative imagine method in some pulmonary diseases in children. Objective: The study was aimed to evaluate the usefulness of LUS in diagnosis and monitoring of NEHI. Methods: This was a prospective study that included children with NEHI diagnosed between 2014 and 2015. In patients with NEHI LUS and HRCT was performed at baseline. Follow-up LUS was repeated after 6 and 12 months from the initial diagnosis. Control group underwent LUS at baseline. Results: Thirteen children with NEHI (11 boys, median age 13 months (8-47 IQR)) were included. The main USG findings were B-lines, pleural line irregularities and pleural line thickening. B-lines were found in 13 children (100%) from the study group and in 6 (46.2%) from the control group (p=0.002). The number of B-lines in all assessed lung areas was significantly higher in children with NEHI. The study groups differed significantly in terms of pleural line irregularities and pleural line thickening. ILD indices found in a quantitative CT analysis correlated significantly with the number of B-lines in every assessed area. We did not found significant changes in LUS abnormalities between baseline and follow-up assessment. Conclusions: Although LUS is sensitive in diagnosing lung involvement in NEHI, its diagnostic specificity is low and does not enable a reliable diagnosis of NEHI. It seems that LUS might be useful in following patients with NEHI, but longer observation is needed.

Neuroendocrine cell hyperplasia of infancy is a rare form of children’s interstitial lung disease recognised usually in infancy and in children younger than two years old. The typical clinical scenario, such as chest retractions, tachypnoea, hypoxaemia, crackles, characteristic changes in high-resolution computed tomography and histological examination of the lung parenchyma, is the cornerstone for diagnosis. In the article, the authors describe clinical manifestation of neuroendocrine cell hyperplasia and a present case of an infant with this rare interstitial lung disease.

This paper presents a case report of an infant, with a prenatally diagnosed congenital lung malformation, which proved to be pulmonary sequestration and congenital cystic adenomatoid malformation. The authors discuss current knowledge on diagnosis, clinical course and suggestions of ante- and postnatal management of patients with pulmonary sequestration or/and congenital cystic lung malformation.

Summary. Background: Although inflammatory bowel diseases (IBD) affect mainly the gastrointestinal tract, the extra-intestinal manifestations are not uncommon. Different diagnostic methods have been applied to assess pulmonary involvement in patients with IBD, but majority of these methods show significant limitations in children. The aim of our study was to evaluate the usefulness of exhaled breath condensate (EBC) measurements of pro-inflammatory cytokines in children with IBD. Material and Methods: Twenty-two children with Crohn’s disease (CD) (mean age 13.8 � 3.3 years), 25 with ulcerative colitis (UC) (mean age 14.1 � 3.3 years) and 37 healthy volunteers (mean age 13.9 � 3.6 years) were studied. IBD activity was assessed using appropriate scoring systems. None of the patients had signs or symptoms of pulmonary disease. Exhaled breath condensate was collected and EBC concentration of interleukin 6 (IL-6), tumor necrosis factor-a (TNF-a), interleukin-1b (IL-1b), and interleukin 8 (IL-8) was measured. Results: The concentrations of all the assessed cytokines were significantly higher in the study group as compared to controls. A negative correlation between IL-1b and CD activity index was found. There were no significant relationships between TNF-a, IL-6, or IL-8 level and CD activity index as well as between IL-1b, TNF-a, IL-6, IL-8 and UC activity index. No significant correlation between the concentration of IL-1b, TNF-a, IL-6, IL-8, and IBD duration or treatment duration was found. Conclusions: Elevated concentration of pro-inflammatory cytokines in EBC in children with IBD maysuggestthepresenceofasymptomaticinflammationinthelowerairways.PediatrPulmonol.

Rak płuca jest najczęstszym nowotworem zło- śliwym w większości uprzemysłowionych krajów świata [...]

Background Interstitial lung disease (ILD) is an umbrella term for more than 200 rare entities, with a high mortality in childhood and scarce information about healthcare and disease management. Objectives This study seeks to fill the knowledge gap about healthcare utilization of childhood ILD patients. Methods For the analysis we used data from the observational study chILD-EU (Children9s Interstitial Lung Disease in Europe) providing incident and prevalent cases from 4 countries and a substantial amount of medical information. In a baseline questionnaire parents answered questions about healthcare utilization of the last 3 months (in- and outpatient treatment, visiting healthcare professionals, use of medical aid and participation in rehabilitation programs). Results Study population included 129 children (56.6% male) with a median age of 4.5 years (IQR 1.1-10.9) and median disease duration of 1.8 years (IQR 0.6-4.7). 51 different disease entities were presented; median disease severity was 3, based on FAN 5 point scale physicians9 estimation. 49.6% of the children needed oxygen and 7.8% non-invasive ventilation. Within the last three months, 57.4% were hospitalized (if so median 18 days, IQR 6-56), 67.4% of the children had at least one visit at a physician or outpatient clinic (if so median 3 visits IQR 2-6), 38.0% had at least one visit at healthcare professionals (if so median 10 visits IQR 3-24), and 4.7% participated in rehabilitation programs (range 1-92 days). Conclusion Many childhood ILDs are associated with substantial use of healthcare services. Identification of predictive factors and their contribution are crucial for health services planning. Fund: grant FP7-305653-chILD-EU.