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1.
J Infect Chemother ; 2024 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-38631479

RESUMO

BACKGROUND: An increased incidence of group A Streptococcus (GAS) infections has been observed in pediatric population post-COVID-19 pandemic. While the majority of reports refer to scarlet fever or invasive GAS disease, detailed data on pulmonary manifestations such as complicated community-acquired pneumonia (CAP) are scarce. The aim of this study was to assess the contribution of GAS to complicated CAP in children during the 2022/2023 infectious season. METHODS: We retrospectively analyzed the etiology and clinical presentation of complicated CAP patients hospitalized in our tertiary care center in Warsaw, Poland, between August 2022 and May 2023. RESULTS: Among 91 patients with complicated CAP, GAS was the dominant cause constituting 24.2% (22/91; 95% CI 15.8-34.3%) of the study group. 68.2% of GAS pneumonia patients presented symptoms of scarlet fever, and 27.3% had preceding or concurrent viral infection. GAS complicated CAP was associated with longer hospitalization, higher incidence of chest tube insertion, but shorter duration of chest tube drainage than complicated CAP of other etiology. Children with GAS complicated CAP had higher procalcitonin concentration (28.1 vs. 1.5 ng/dL; p<0.0001) and a lower platelets level (254.5 vs. 422 × 103/µL; p = 0.0031) than those with non-GAS infection. CONCLUSIONS: GAS is currently the predominant pathogen of complicated CAP in children. Clinicians should be aware of the current epidemiological situation and a more severe course of GAS pneumonia in this age group, and should monitor patients presenting with symptoms of scarlet fever and preceding viral infection closely.

2.
ERJ Open Res ; 9(2)2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37101741

RESUMO

Background: Paediatric diffuse alveolar haemorrhage (DAH) is a rare heterogeneous condition with limited knowledge on clinical presentation, treatment and outcome. Methods: A retrospective, descriptive multicentre follow-up study initiated from the European network for translational research in children's and adult interstitial lung disease (Cost Action CA16125) and chILD-EU CRC (the European Research Collaboration for Children's Interstitial Lung Disease). Inclusion criteria were DAH of any cause diagnosed before the age of 18 years. Results: Data of 124 patients from 26 centres (15 counties) were submitted, of whom 117 patients fulfilled the inclusion criteria. Diagnoses were idiopathic pulmonary haemosiderosis (n=35), DAH associated with autoimmune features (n=20), systemic and collagen disorders (n=18), immuno-allergic conditions (n=10), other childhood interstitial lung diseases (chILD) (n=5), autoinflammatory diseases (n=3), DAH secondary to other conditions (n=21) and nonspecified DAH (n=5). Median (IQR) age at onset was 5 (2.0-12.9) years. Most frequent clinical presentations were anaemia (87%), haemoptysis (42%), dyspnoea (35%) and cough (32%). Respiratory symptoms were absent in 23%. The most frequent medical treatment was systemic corticosteroids (93%), hydroxychloroquine (35%) and azathioprine (27%). Overall mortality was 13%. Long-term data demonstrated persistent abnormal radiology and a limited improvement in lung function. Conclusions: Paediatric DAH is highly heterogeneous regarding underlying causes and clinical presentation. The high mortality rate and number of patients with ongoing treatment years after onset of disease underline that DAH is a severe and often chronic condition. This large international study paves the way for further prospective clinical trials that will in the long term allow evidence-based treatment and follow-up recommendations to be determined.

3.
Thorax ; 78(6): 587-595, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36808083

RESUMO

BACKGROUND: The 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. METHOD: Over 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. RESULTS: At 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, the ABCA3 sequence variants were missense variants, small insertions or deletions with in-silico tools predicting some residual ABCA3 transporter function. CONCLUSION: The natural history of ABCA3-related interstitial lung disease progresses during childhood and adolescence. Disease-modifying treatments are desirable to delay such disease course.


Assuntos
Transportadores de Cassetes de Ligação de ATP , Doenças Pulmonares Intersticiais , Criança , Adolescente , Lactente , Humanos , Estudos de Coortes , Transportadores de Cassetes de Ligação de ATP/genética , Transportadores de Cassetes de Ligação de ATP/metabolismo , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/genética , Doenças Pulmonares Intersticiais/terapia , Pulmão/metabolismo , Tomografia Computadorizada por Raios X , Mutação
4.
Pediatr Pulmonol ; 58(3): 934-940, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36537617

RESUMO

BACKGROUND: Severe acute respiratory syndrome coronavirus 2 (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 coronavirus disease 2019 (COVID-19) in children with persistent tachypnea of infancy (PTI). METHODS: 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. RESULTS: 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, three children (7.9%) as moderate, and three 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. CONCLUSION: 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.


Assuntos
COVID-19 , Pneumopatias , Masculino , Criança , Feminino , Humanos , Lactente , Pré-Escolar , COVID-19/epidemiologia , SARS-CoV-2 , Taquipneia/epidemiologia , Tosse/etiologia
5.
Pediatr Pulmonol ; 58(4): 1042-1050, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36562653

RESUMO

OBJECTIVES: The goal of this study was to assess the pulmonary sequelae of COVID-19 pneumonia in children. STUDY DESIGN: 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 visits 3 months postdischarge, 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 a standardized protocol, and pulmonary function tests (PFTs). PFTs results were compared with healthy children. RESULTS: Forty-one 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, the most common was 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. CONCLUSIONS: 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.


Assuntos
COVID-19 , Humanos , Criança , Recém-Nascido , Lactente , Pré-Escolar , Adolescente , COVID-19/complicações , Assistência ao Convalescente , SARS-CoV-2 , Alta do Paciente , Pulmão/diagnóstico por imagem , Ultrassonografia/métodos
6.
Thorax ; 78(5): 476-483, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36572533

RESUMO

BACKGROUND: Monitoring 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. METHODS: This 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. RESULTS: Baseline data of 774 children were used to calculate the following MIDs: respiratory rate 1.3 (z-score), O2 saturation 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. CONCLUSIONS: For the first time, we provide estimates of MIDs for vital signs and HrQoL scores in a large cohort of chILD using different methods.


Assuntos
Doenças Pulmonares Intersticiais , Qualidade de Vida , Humanos , Criança , Qualidade de Vida/psicologia , Doenças Pulmonares Intersticiais/diagnóstico , Pulmão , Nível de Saúde , Inquéritos e Questionários
7.
Pediatr Pulmonol ; 58(1): 81-87, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36177553

RESUMO

BACKGROUND: 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. METHODS: 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 [DLCO ]) were assessed. RESULTS: Thirty-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 DLCO  were found in 73.3% and 90.9% of PTI patients, respectively. CONCLUSIONS: This study demonstrated that lung function is affected in most children with PTI. PFTs showed that peripheral airways are the major zone of functional impairment.


Assuntos
Pulmão , Taquipneia , Masculino , Feminino , Humanos , Lactente , Criança , Pré-Escolar , Capacidade Vital , Volume Expiratório Forçado , Testes de Função Respiratória , Espirometria
8.
Pediatr Pulmonol ; 58(1): 171-177, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36196557

RESUMO

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.


Assuntos
Refluxo Gastroesofágico , Doenças Pulmonares Intersticiais , Criança , Humanos , Lactente , Monitoramento do pH Esofágico/efeitos adversos , Estudos Prospectivos , Estudos Transversais , Refluxo Gastroesofágico/complicações , Refluxo Gastroesofágico/epidemiologia , Refluxo Gastroesofágico/diagnóstico , Impedância Elétrica , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/complicações , Concentração de Íons de Hidrogênio
9.
Diagnostics (Basel) ; 12(4)2022 Mar 29.
Artigo em Inglês | MEDLINE | ID: mdl-35453887

RESUMO

OBJECTIVES: IP-10 has been proposed as a new diagnostic biomarker for Mycobacterium tuberculosis infection (MTBI). However, data on IP-10 concentration in bronchoalveolar lavage fluid (BALF) for pediatric tuberculosis are lacking. AIM: To determine IP-10 levels in unstimulated BALF and plasma in children with and without MTBI. METHODS: IP-10 concentrations in BALF and plasma were measured in children hospitalized with suspected tuberculosis or other respiratory disease and scheduled for bronchoscopy. Thirty-five children were enrolled: 13 with suspected tuberculosis and 22 controls. The association between IP-10 and age was examined. RESULTS: The IP-10 expression was increased in BALF compared to plasma (p = 0.008). We noticed higher BALF IP-10 levels in children with asthma, interstitial lung disease, and lung anomaly than in children with MTBI and other respiratory tract infections, but the differences were statistically insignificant. There was a moderate correlation between plasma and BALF IP-10 concentrations (rs = 0.46, p = 0.018). No correlation between IP-10 level and age was detected. CONCLUSIONS: IP-10 is detectable in unstimulated BALF in children with respiratory diseases, reaches higher concentrations in unstimulated BALF vs plasma, and does not correlate with age. However, it could not discriminate MTBI from other respiratory diseases.

10.
Thorax ; 77(8): 781-789, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35149583

RESUMO

BACKGROUND: No data on healthcare utilisation and associated costs for the many rare entities of children's interstitial lung diseases (chILD) exist. This paper portrays healthcare utilisation structures among individuals with chILD, provides a pan-European estimate of a 3-month interval per-capita costs and delineates crucial cost drivers. METHODS: Based on longitudinal healthcare resource utilisation pattern of 445 children included in the Kids Lung Register diagnosed with chILD across 10 European countries, we delineated direct medical and non-medical costs of care per 3-month interval. Country-specific utilisation patterns were assessed with a children-tailored modification of the validated FIMA questionnaire and valued by German unit costs. Costs of care and their drivers were subsequently identified via gamma-distributed generalised linear regression models. RESULTS: During the 3 months prior to inclusion into the registry (baseline), the rate of hospital admissions and inpatient days was high. Unadjusted direct medical per capita costs (€19 818) exceeded indirect (€1 907) and direct non-medical costs (€1 125) by far. Country-specific total costs ranged from €8 713 in Italy to €28 788 in Poland. Highest expenses were caused by the disease categories 'diffuse parenchymal lung disease (DPLD)-diffuse developmental disorders' (€45 536) and 'DPLD-unclear in the non-neonate' (€47 011). During a follow-up time of up to 5 years, direct medical costs dropped, whereas indirect costs and non-medical costs remained stable. CONCLUSIONS: This is the first prospective, longitudinal study analysing healthcare resource utilisation and costs for chILD across different European countries. Our results indicate that chILD is associated with high utilisation of healthcare services, placing a substantial economic burden on health systems.


Assuntos
Custos de Cuidados de Saúde , Doenças Pulmonares Intersticiais , Criança , Europa (Continente) , Humanos , Estudos Longitudinais , Doenças Pulmonares Intersticiais/terapia , Aceitação pelo Paciente de Cuidados de Saúde , Estudos Prospectivos
11.
Thorax ; 77(8): 799-804, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35149584

RESUMO

INTRODUCTION: Acute exacerbations (AEs) increase morbidity and mortality of patients with chronic pulmonary diseases. Little is known about the characteristics and impact of AEs on children's interstitial lung disease (chILD). METHODS: The Kids Lung Register collected data on AEs, the clinical course and quality of life (patient-reported outcomes - PRO) of rare paediatric lung diseases. Characteristics of AEs were obtained. RESULTS: Data of 2822 AEs and 2887 register visits of 719 patients with chILD were recorded. AEs were characterised by increased levels of dyspnoea (74.1%), increased respiratory rate (58.6%) and increased oxygen demand (57.4%). Mostly, infections (94.4%) were suspected causing an AE. AEs between two register visits revealed a decline in predicted FEV1 (median -1.6%, IQR -8.0 to 3.9; p=0.001), predicted FVC (median -1.8%, IQR -7.5 to 3.9; p=0.004), chILD-specific questionnaire (median -1.3%, IQR -3.6 to 4.5; p=0.034) and the physical health summary score (median -3.1%, IQR -15.6 to 4.3; p=0.005) compared with no AEs in between visits. During the median observational period of 2.5 years (IQR 1.2-4.6), 81 patients died. For 49 of these patients (60.5%), mortality was associated with an AE. CONCLUSION: This is the first comprehensive study analysing the characteristics and impact on the clinical course of AEs in chILD. AEs have a significant and deleterious effect on the clinical course and health-related quality of life in chILD.


Assuntos
Doenças Pulmonares Intersticiais , Qualidade de Vida , Criança , Humanos , Pulmão , Inquéritos e Questionários
12.
Pediatr Pulmonol ; 56(12): 3952-3959, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34520130

RESUMO

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 < 0.001), chest retractions (43.8% of patients, p = 0.04), and crackles (29.2% of patients, p = 0.02). Also, a significant improvement in forced expiratory volume in 1 s (FEV1 ) (median z score: -2.21 vs. -0.47, p = 0.03), residual volume (RV) (median z score 5.28 vs. 1.07, p = 0.007), RV% total lung capacity (TLC) (median z score: 6.05 vs. 1.48, p = 0.01), sRaw (median z score: 6.6 vs. 4.64, p = 0.01), R5 (median z score: 1.27 vs. 0.31, p = 0.009), and R5-R20 (median: 0.58 vs. 0.26 kPa/(l/s), p = 0.002) was demonstrated. CONCLUSIONS: Inhaled bronchodilators and ICS may exert a positive effect on the severity of symptoms and pulmonary function test (PFT) in symptomatic children with PTI. However, a randomized control trial should be conducted to confirm their effectiveness.


Assuntos
Corticosteroides , Broncodilatadores , Administração por Inalação , Corticosteroides/uso terapêutico , Broncodilatadores/uso terapêutico , Criança , Pré-Escolar , Volume Expiratório Forçado , Humanos , Pulmão , Taquipneia
13.
J Asthma ; 58(1): 60-68, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-31496317

RESUMO

OBJECTIVE: 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. METHODS: EBC and serum periostin concentrations were measured by enzyme-linked immunosorbent assay in 23 children with asthma and 23 healthy controls. RESULTS: 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. CONCLUSIONS: 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.


Assuntos
Asma/diagnóstico , Moléculas de Adesão Celular/análise , Adolescente , Biomarcadores/análise , Testes Respiratórios , Criança , Estudos Transversais , Feminino , Humanos , Masculino , Estudos Prospectivos , Índice de Gravidade de Doença
14.
Front Immunol ; 11: 1950, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32973798

RESUMO

Primary immunodeficiencies (PIDs) are rare disorders of the immune system encompassing inborn errors of immunity. Primary antibody deficiencies constitute the largest group of PID with common variable immunodeficiency (CVID) being the most common symptomatic form. Combined immunodeficiencies (CID) accompanied by antibody deficiency can mimic CVID and these patients need the verification of the final diagnosis. Respiratory involvement, especially interstitial lung disease (ILD), poses a relevant cause of morbidity and mortality among patients with PID and in some cases is the first manifestation of immunodeficiency. In this study we present a retrospective analysis of a group of children with primary immunodeficiency and ILD - the clinical, radiological, histological characteristics, treatment strategies and outcomes. Eleven children with PID-related ILD were described. The majority of them presented CVID, in three patients CID was recognized. All patients underwent detailed pulmonary diagnostics. In eight of them histological analysis of lung biopsy was performed. We noted that in two out of 11 patients acute onset of ILD with respiratory failure was the first manifestation of the disease and preceded PID diagnosis. The most common histopathological diagnosis was GLILD. Among the analyzed patients three did not require any immunosuppressive therapy. All eight treated children received corticosteroids as initial treatment, but in some of them second-line therapy was introduced. The relevant side effects in some patients were observed. The study demonstrated that the response to corticosteroids is usually prompt. However, the resolution of pulmonary changes may be incomplete and second-line treatment may be necessary.


Assuntos
Doenças Pulmonares Intersticiais/etiologia , Doenças da Imunodeficiência Primária/complicações , Adolescente , Corticosteroides/uso terapêutico , Fatores Etários , Criança , Pré-Escolar , Feminino , Humanos , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Masculino , Polônia , Doenças da Imunodeficiência Primária/diagnóstico , Doenças da Imunodeficiência Primária/tratamento farmacológico , Estudos Retrospectivos , Resultado do Tratamento
15.
Adv Respir Med ; 88(4): 297-304, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32869262

RESUMO

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.


Assuntos
Hospitalização/estatística & dados numéricos , Doença Pulmonar Obstrutiva Crônica/epidemiologia , Doença Pulmonar Obstrutiva Crônica/terapia , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Feminino , Humanos , Pessoa de Meia-Idade , Programas Nacionais de Saúde/normas , Alta do Paciente/estatística & dados numéricos , Polônia , Prevalência , Pneumologia/normas , Fatores de Risco
16.
Pediatr Pulmonol ; 55(11): 3119-3125, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32761949

RESUMO

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.


Assuntos
Taquipneia/fisiopatologia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Testes de Função Respiratória , Taquipneia/epidemiologia
17.
Pediatr Gastroenterol Hepatol Nutr ; 23(3): 251-258, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32483546

RESUMO

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.

18.
Pediatr Pulmonol ; 55(4): 1028-1036, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31978279

RESUMO

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.


Assuntos
Taquipneia/diagnóstico , Criança , Estudos Transversais , Feminino , Humanos , Pulmão/diagnóstico por imagem , Pneumopatias , Masculino , Pleura , Estudos Prospectivos , Tomografia Computadorizada por Raios X , Ultrassonografia/métodos
19.
Thorax ; 75(2): 172-175, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31748256

RESUMO

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 <6 months of age with SpO2<94% and developmental/surfactant disorders. We report for the first time that ChILD survivors improved multiple clinical parameters within 8-12 weeks of diagnosis. These data can inform family discussions and support clinical trial measurements.


Assuntos
Corticosteroides/administração & dosagem , Azitromicina/administração & dosagem , Hidroxicloroquina/administração & dosagem , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Adolescente , Causas de Morte , Criança , Pré-Escolar , Estudos de Coortes , Quimioterapia Combinada , Europa (Continente) , Feminino , Seguimentos , Humanos , Lactente , Estimativa de Kaplan-Meier , Estudos Longitudinais , Doenças Pulmonares Intersticiais/epidemiologia , Masculino , Monitorização Fisiológica/métodos , Estudos Prospectivos , Sistema de Registros , Testes de Função Respiratória , Medição de Risco , Índice de Gravidade de Doença , Análise de Sobrevida , Fatores de Tempo
20.
Adv Respir Med ; 87(5): 308-316, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31680234

RESUMO

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.


Assuntos
Laringite/complicações , Laringite/diagnóstico , Infecções Respiratórias/complicações , Doença Aguda , Obstrução das Vias Respiratórias/etiologia , Infecções Bacterianas/complicações , Criança , Crupe/etiologia , Dispneia/etiologia , Humanos , Laringite/terapia , Infecções Respiratórias/diagnóstico
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