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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.
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
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.
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
5.
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
6.
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
7.
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
8.
Clin Genet ; 96(5): 468-472, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31355908

RESUMO

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.


Assuntos
Calcinose/genética , Doenças Pulmonares Intersticiais/genética , Fenilalanina-tRNA Ligase/genética , Subunidades Proteicas/genética , Adolescente , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Calcinose/diagnóstico por imagem , Calcinose/patologia , Criança , Predisposição Genética para Doença , Humanos , Fígado/diagnóstico por imagem , Fígado/patologia , Pulmão/diagnóstico por imagem , Pulmão/patologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/patologia , Masculino , Mutação
9.
Adv Exp Med Biol ; 1211: 103-110, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31144241

RESUMO

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.


Assuntos
Antibacterianos/uso terapêutico , Infecções Comunitárias Adquiridas/tratamento farmacológico , Fidelidade a Diretrizes/estatística & dados numéricos , Pneumonia/tratamento farmacológico , Amoxicilina/uso terapêutico , Combinação Amoxicilina e Clavulanato de Potássio/uso terapêutico , Cefuroxima/uso terapêutico , Criança , Hospitais , Humanos , Pediatras , Guias de Prática Clínica como Assunto , Estudos Retrospectivos
10.
J Infect Chemother ; 22(5): 292-7, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-26919911

RESUMO

BACKGROUND: An increasing incidence of parapneumonic effusion and pleural empyema (PPE/PE) in children has been found in several studies published in the last decades. The aim of the study was to evaluate the incidence, etiology, clinical features, treatment strategies and outcomes of PPE/PE in children treated in a referral pulmonary center in central Poland. MATERIAL AND METHODS: We performed a retrospective analysis of clinical, radiological and laboratory data of all children aged between 1 month and 18 years with PPE/PE due to community acquired pneumonia (CAP) between January 2002 and December 2013. RESULTS: One thousand nine hundred and thirty three children with CAP were hospitalized between 2002 and 2013. Parapneumonic effusion or PE was diagnosed in 323 children (16.7%). The proportion of children with CAP related PPE/PE increased from 5.4% in 2002 to 18.8% in 2013. Streptococcus pneumoniae was the most common causative microorganism, responsible for 66.7% cases of known etiology. All children were treated with antibiotics and in 22.6%, and 74.3% of the patients therapeutic thoracentesis, pleural drainage with or without intrapleural fibrinolysis was performed, respectively. Approximately 3% of patients required surgical intervention. CONCLUSIONS: A significant increase in the incidence of PPE/PE in children with CAP treated in our institution in the last twelve years was found. S. pneumoniae was the most common causative microorganism. Antibiotic therapy with chest drain insertion ± intrapleural fibrinolysis is an effective treatment of PPE/PE and surgical intervention is seldom necessary. With proper management, the overall prognosis in children with CAP related PPE/PE is good.


Assuntos
Infecções Comunitárias Adquiridas , Empiema Pleural , Derrame Pleural , Pneumonia Bacteriana , Adolescente , Criança , Pré-Escolar , Infecções Comunitárias Adquiridas/complicações , Infecções Comunitárias Adquiridas/epidemiologia , Drenagem , Empiema Pleural/epidemiologia , Empiema Pleural/etiologia , Feminino , Humanos , Lactente , Tempo de Internação , Masculino , Derrame Pleural/epidemiologia , Derrame Pleural/etiologia , Pneumonia Bacteriana/complicações , Pneumonia Bacteriana/epidemiologia , Estudos Retrospectivos
11.
Pneumonol Alergol Pol ; 84(1): 22-8, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26676969

RESUMO

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.


Assuntos
Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/fisiopatologia , Volume Expiratório Forçado , Pulmão/fisiopatologia , Capacidade Vital , Adolescente , Criança , Doenças do Tecido Conjuntivo/diagnóstico , Progressão da Doença , Feminino , Humanos , Medidas de Volume Pulmonar , Prevalência , Testes de Função Respiratória , Espirometria
12.
Adv Exp Med Biol ; 858: 31-8, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25468008

RESUMO

Interpretation of spirometry strongly depends on the applied predicted values. New Global Lung Initiative (GLI) reference values have recently been published but their influence on spirometry interpretation in children has not been widely evaluated. The aim of the study was to compare the interpretation of spirometry using GLI-2012 vs. Polish-1998 reference values. Spirometry results of 315 Caucasian children aged 4-18 were analyzed. Airway obstruction was defined as FEV1/FVCLLN. The findings were that FEV1 and FVC expressed as GLI-2012 or Polish-1998 z-scores differed significantly (p<0.05). The mean FEV1 z-score was -0.68±1.25 vs. -0.13±1.70 and the mean FVC was -0.34±1.08 vs. 0.30±1.15 for GLI-2012 and Polish-1998, respectively. There was no difference for FEV1/FVC z-scores. Obstructive and restrictive ventilatory patterns were diagnosed in 20.3% and 7.6% children using GLI-2012 values compared with 17.5% and 3.8% when using Polish-1998 reference values, respectively. In conclusion, the use of GLI-2012 reference values in the population of Polish children increases the number of detected lung function abnormalities compared with Polish-1998 reference values.


Assuntos
Volume Expiratório Forçado/fisiologia , Pulmão/fisiopatologia , Doença Pulmonar Obstrutiva Crônica/diagnóstico , Capacidade Vital/fisiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Pulmão/fisiologia , Masculino , Polônia , Guias de Prática Clínica como Assunto , Estudos Prospectivos , Doença Pulmonar Obstrutiva Crônica/etnologia , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Valores de Referência , Projetos de Pesquisa , Espirometria , População Branca
13.
Adv Exp Med Biol ; 857: 9-17, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25468010

RESUMO

Necrotizing pneumonia (NP) is an emerging complication of community acquired pneumonia (CAP) in children. This study aimed at the evaluation of etiology, clinical features, treatment, and prognosis of NP. The institutional database of children with CAP treated between April 2008 and July 2013 was searched to identify children with NP. Then, data on the NP characteristics were retrospectively reviewed and analyzed. We found that NP constituted 32/882 (3.7%) of all CAPs. The median age of children with NP was 4 (range 1-10) years. The causative pathogens were identified in 12/32 children (37.5%) with Streptococcus pneumoniae being the most common (6/32). All but one patient developed complications: parapneumonic effusion (PPE), pleural empyema or bronchopleural fistula (BPF), which required prompt local treatment. The median duration of hospital stay and antibiotic treatment was 26 (IQR 21-30) and 28 (IQR 22.5-32.5) days, respectively. Despite severe course of the disease no deaths occurred. A follow-up visit after 6 months revealed that none of the patients presented with any signs and symptoms which could be related to earlier pneumonia. Chest radiographs showed complete or almost complete resolution of pulmonary and pleural lesions in all patients. We conclude that necrotizing pneumonia is a relatively rare but severe form of CAP that is almost always complicated by PPE/empyema and/or BPF. It can be successfully treated with antibiotics and pleural drainage without major surgical intervention.


Assuntos
Doenças Transmissíveis , Empiema Pleural , Derrame Pleural , Pneumonia Pneumocócica , Streptococcus pneumoniae , Adolescente , Criança , Pré-Escolar , Doenças Transmissíveis/complicações , Doenças Transmissíveis/diagnóstico por imagem , Doenças Transmissíveis/epidemiologia , Doenças Transmissíveis/terapia , Empiema Pleural/diagnóstico por imagem , Empiema Pleural/epidemiologia , Empiema Pleural/etiologia , Empiema Pleural/terapia , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Derrame Pleural/diagnóstico por imagem , Derrame Pleural/epidemiologia , Derrame Pleural/etiologia , Derrame Pleural/terapia , Pneumonia Pneumocócica/complicações , Pneumonia Pneumocócica/diagnóstico por imagem , Pneumonia Pneumocócica/epidemiologia , Pneumonia Pneumocócica/terapia , Polônia/epidemiologia , Radiografia
14.
Pneumonol Alergol Pol ; 82(4): 358-63, 2014.
Artigo em Polonês | MEDLINE | ID: mdl-24964239

RESUMO

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.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico , Pulmão/patologia , Células Neuroendócrinas/patologia , Humanos , Hiperplasia/etiologia , Recém-Nascido , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/patologia , Masculino
15.
Pneumonol Alergol Pol ; 82(4): 377-84, 2014.
Artigo em Polonês | MEDLINE | ID: mdl-24964242

RESUMO

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.


Assuntos
Sequestro Broncopulmonar/complicações , Malformação Adenomatoide Cística Congênita do Pulmão/complicações , Sequestro Broncopulmonar/diagnóstico , Sequestro Broncopulmonar/terapia , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico , Malformação Adenomatoide Cística Congênita do Pulmão/terapia , Feminino , Humanos , Recém-Nascido , Gravidez , Diagnóstico Pré-Natal
16.
Pediatr Pulmonol ; 2024 Jun 27.
Artigo em Inglês | MEDLINE | ID: mdl-38934775

RESUMO

BACKGROUND: Neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease of unknown origin associated with hyperplasia of pulmonary neuroendocrine cells (PNECs). Diagnosis is based on the characteristic clinical picture and typical radiological imaging, and, in some cases, on lung biopsies. To date, no biochemical indicators of the disease have been identified. AIM: We aimed to determine biomarkers that could be useful in the management of children diagnosed with NEHI. METHODS: Patients with NEHI and healthy children were enrolled. Concentrations of serum biomarkers secreted by PNECs (calcitonin gene-related peptide and gastrin-releasing peptide) and biomarkers of the destruction of alveolar capillary membrane (surfactant proteins A and D [SP-A and SP-D]; glycoprotein Krebs von den Lungen-6 [KL-6]; metalloproteinases 7 and 9 [MMP-7 and MMP-9]; tissue inhibitor of metalloprotease 1) were measured. RESULTS: Fifty-two children with NEHI and 23 healthy children were included in the study. The median age of children with NEHI was 3.9 years. There were no differences in serum levels of biomarkers secreted by PNECs between groups. KL-6 levels were significantly higher in children with NEHI than in healthy ones (median 119.6 vs. 92.1 U/mL, p = 0.003); however, concentrations of KL-6 were low in both groups. No significant differences existed between groups for the remaining biomarkers associated with the destruction of the alveolar-capillary membrane. CONCLUSIONS: Measurement of serum biomarkers released by PNECs and those associated with the destruction of the alveolar-capillary membrane does not appear to be useful in the management of children with NEHI.

17.
Pediatr Pulmonol ; 2024 Jun 05.
Artigo em Inglês | MEDLINE | ID: mdl-38838063

RESUMO

INTRODUCTION: Childhood interstitial lung disease (chILD) is a heterogeneous group of mostly chronic respiratory disorders. Assessment of health-related quality of life (HrQoL) in chILD has become increasingly important in clinical care and research. The aim of this study was to assess differences between patient-reported (self) and caregiver-reported (proxy) HrQoL scores. METHODS: This study used data obtained from the chILD-EU Register. After inclusion (baseline), the patient's health status was followed up at predefined study visits. At each study visit, caregivers and patients were handed validated, age-specific HrQoL questionnaires. HrQoL data entered at baseline were used to compare self- and proxy-reported HrQoL scores. For the longitudinal analysis, we compared HrQoL scores between the baseline and the next follow-up visit. RESULTS: No differences between patient- and caregiver-reported HrQoL scores were found for school functioning, chILD-specific questionnaire score, and physical health summary score. Self-reported HrQoL scores were higher for the subscales emotional functioning (77.4 vs. 70.7; p < .001), social functioning (81.9 vs. 76.2; p < .001), as well as psycho-social summary score (76.5 vs. 71.8; p < .001) and total score (74.7 vs. 70.8; <.001). The longitudinal analysis showed that a significant change in a patient-reported HrQoL score resulted in a significant change in a caregiver-reported HrQoL score after a mean time of 11.0 months (SD 9.4). CONCLUSIONS: We found a good agreement between children- and caregiver-related HrQoL scores. In chILD, caregivers are able to sense changes in children's HrQoL scores over time and may be used as a proxy for children unable to complete HrQoL questionnaires.

18.
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
19.
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
20.
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
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