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1.
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
2.
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
3.
Eur J Pediatr ; 181(11): 3931-3936, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36083314

RESUMO

After the SARS-CoV-2 pandemic, we noticed a marked increase in high-flow nasal cannula use for bronchiolitis. This study aims to report the percentage of children treated with high-flow nasal cannula (HFNC) in various seasons. The secondary outcomes were admissions for bronchiolitis, virological results, hospital burden, and NICU/PICU need. We conducted a retrospective study in four Italian hospitals, examining the medical records of all infants (< 12 months) hospitalized for bronchiolitis in the last four winter seasons (1 September-31 March 2018-2022). In the 2021-2022 winter season, 66% of admitted children received HFNC versus 23%, 38%, and 35% in the previous 3 years. A total of 876 patients were hospitalized in the study periods. In 2021-2022, 300 infants were hospitalized for bronchiolitis, 22 in 2020-2021, 259 in 2019-2020, and 295 in 2018-2019. The percentage of patients needing intensive care varied from 28.7% to 18%, 22%, and 15% in each of the four considered periods (p < 0.05). Seventy-seven percent of children received oxygen in the 2021-2022 winter; vs 50%, 63%, and 55% (p < 0.01) in the previous 3 years. NIV/CPAP was used in 23%, 9%, 16%, and 12%, respectively. In 2021-2020, 2% of patients were intubated; 0 in 2020-2021, 3% in 2019-2020, and 1% in 2018-2019. CONCLUSION: This study shows a marked increase in respiratory support and intensive care admissions this last winter. While these severity indexes were all driven by medical choices, more reliable indexes such as intubation rate and length of stay did not change. Therefore, we suggest that there is a more aggressive treatment attitude rather than a more severe disease. WHAT IS KNOWN: • COVID-19 pandemic deeply impacted bronchiolitis epidemiology, reducing hospitalizations to onetenth. In the 2021-2022 winter, bronchiolitis resurged to pre-pandemic numbers in Europe. WHAT IS NEW: • Bronchiolitis hospitalization rose much faster in the 2021-2022 winter period, peaking at a higher level. Respiratory supports and high-flow nasal cannula increased significantly compared to the pre-pandemic era.


Assuntos
Bronquiolite , COVID-19 , Médicos , Atitude do Pessoal de Saúde , Bronquiolite/epidemiologia , Bronquiolite/terapia , COVID-19/epidemiologia , COVID-19/terapia , Cânula , Criança , Humanos , Lactente , Oxigênio , Oxigenoterapia , Pandemias , Estudos Retrospectivos , SARS-CoV-2
4.
Eur Respir J ; 54(2)2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31151956

RESUMO

Rare variants in the T-box transcription factor 4 gene (TBX4) have recently been recognised as an emerging cause of paediatric pulmonary hypertension (PH). Their pathophysiology and contribution to persistent pulmonary hypertension in neonates (PPHN) are unknown. We sought to define the spectrum of clinical manifestations and histopathology associated with TBX4 variants in neonates and children with PH.We assessed clinical data and lung tissue in 19 children with PH, including PPHN, carrying TBX4 rare variants identified by next-generation sequencing and copy number variation arrays.Variants included six 17q23 deletions encompassing the entire TBX4 locus and neighbouring genes, and 12 likely damaging mutations. 10 infants presented with neonatal hypoxic respiratory failure and PPHN, and were subsequently discharged home. PH was diagnosed later in infancy or childhood. Three children died and two required lung transplantation. Associated anomalies included patent ductus arteriosus, septal defects, foot anomalies and developmental disability, the latter with a higher prevalence in deletion carriers. Histology in seven infants showed abnormal distal lung development and pulmonary hypertensive remodelling.TBX4 mutations and 17q23 deletions underlie a new form of developmental lung disease manifesting with severe, often biphasic PH at birth and/or later in infancy and childhood, often associated with skeletal anomalies, cardiac defects, neurodevelopmental disability and other anomalies.


Assuntos
Deleção de Genes , Hipertensão Pulmonar/genética , Proteínas com Domínio T/genética , Adolescente , Adulto , Criança , Pré-Escolar , Variações do Número de Cópias de DNA , Feminino , Variação Genética , Heterozigoto , Humanos , Lactente , Recém-Nascido , Pulmão/crescimento & desenvolvimento , Transplante de Pulmão , Masculino , Mutação , Fenótipo , Resistência Vascular , Adulto Jovem
5.
Radiol Med ; 123(8): 577-585, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29569218

RESUMO

Diffuse lung disease in infancy includes a wide range of very rare and peculiar pulmonary conditions usually not seen in older children, in whom diffuse lung disease has much greater overlap with adult disorders. The acronym chILD (childhood Interstitial Lung Disease) commonly defines these disorders, although air spaces, airways, alveolar epithelium, vasculature, pleura, and pleural spaces can also be involved, besides the pulmonary interstitium. chILD can be caused by diffuse developmental disorders, alveolar growth abnormalities, surfactant dysfunction disorders, and other specific conditions of poorly understood etiology. Chest CT imaging studies play a pivotal role in the evaluation of chILD. In some conditions CT findings can be specific, and thus make it possible avoiding further testing. In other disorders, findings are nonspecific, although they may suggest a diagnostic pattern and guide further testing. Nevertheless, chILD disorders often remain unrecognized on imaging studies, as they are very rare. The aim of this article is to review the CT patterns of lung involvement in a series of infants with chILD.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico por imagem , Pulmão/anormalidades , Pulmão/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Algoritmos , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Recém-Nascido , Doenças Pulmonares Intersticiais/genética , Doenças Pulmonares Intersticiais/patologia , Masculino , Interpretação de Imagem Radiográfica Assistida por Computador , Tomografia Computadorizada por Raios X/instrumentação
6.
Arch Dis Child Educ Pract Ed ; 103(6): 300-301, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-28866618

RESUMO

A 4-year-old boy was admitted to our department with fever, cough and dyspnoea, unresponsive to salbutamol and antibiotic therapy. He had previously contracted bronchiolitis at 20 days of life, followed by intermittent episodes of wheeze that never required hospitalisation and responded to short inhaled corticosteroid cycles. He had an atopic family history. On examination, he had dyspnoea, persistent cough with bronchospasm but normal oxygen saturations. Bloods showed elevated eosinophils (2004 µL), a slightly elevated C-reactive protein (1.5 mg/dL) and total IgE (326 kU/L), and specific IgE was raised for various inhalant allergens (box). A chest X-ray was performed (figure 1).BoxPositive inhalant allergens Anthoxanthum odoratumCynodon dactylonDactylis glomerataDermatophagoides farinaeDermatophagoides pteronissimusHolcus lanatusPoa pratensisPhleum pratense edpract;103/6/300/F1F1F1Figure 1Chest X-ray of the patient. QUESTIONS: 1. What does the chest X-ray in figure 1 show? interstitial pneumoniapneumothoraxlung atelectasis with mild mediastinal shiftdiffuse air trappingenlargement of right hilar lymph nodes 2. Given the clinical picture and the chest X-ray, what would your differential diagnosis include from the following? plastic bronchitis (PB)mycoplasma infectiontuberculosisforeign body aspirationlung perforation 3. Are any of these conditions not associated with a specific type of cast/PB? Fontan procedurehaemophilialymphatic abnormalitiesasthma and other allergic disorderssickle cell disease.


Assuntos
Broncopatias/diagnóstico , Dispneia/etiologia , Broncopatias/cirurgia , Broncoscopia , Proteína C-Reativa/análise , Pré-Escolar , Humanos , Imunoglobulina E/sangue , Masculino , Atelectasia Pulmonar/diagnóstico por imagem , Tomografia Computadorizada por Raios X
7.
Minerva Pediatr ; 70(6): 589-599, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30334623

RESUMO

Bronchiolitis is the most frequent airway infection in the first 2 years of life, and the respiratory syncytial virus (RSV) is the most frequently responsible virus. In selected high-risk groups, RSV may cause severe respiratory disease leading to hospitalization, need for mechanical ventilation, and even death. These high-risk groups include children with congenital heart disease, infants with neuromuscular impairment, cystic fibrosis, Down Syndrome, immunodeficiency syndromes and others specific conditions. In these high-risk populations defined in literature as "special population", a 3- to 10-fold increase in the rate of RSV hospitalization has been observed, justifying RSV specific prophylaxis with palivizumab, a monoclonal antibody that binds a viral glycoprotein epitope and blocks the link between RSV and target cell. Evidence of safety and efficacy of RSV prophylaxis in these populations is lacking. Given the low incidence of these conditions, randomized clinical trials are not feasible. The purpose of this paper is to give an update from the literature of various conditions at higher risk to develop severe RSV infection, and to offer an overview of the efficacy of palivizumab in preventing RSV infection in these specific populations.


Assuntos
Bronquiolite/prevenção & controle , Palivizumab/administração & dosagem , Infecções por Vírus Respiratório Sincicial/prevenção & controle , Antivirais/administração & dosagem , Bronquiolite/epidemiologia , Bronquiolite/virologia , Hospitalização/estatística & dados numéricos , Humanos , Infecções por Vírus Respiratório Sincicial/epidemiologia , Vírus Sincicial Respiratório Humano/isolamento & purificação , Fatores de Risco
8.
Eur J Pediatr ; 176(4): 493-499, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28168335

RESUMO

Nocturnal pulse oximetry has a high positive predictive value for polysomnographically diagnosed obstructive sleep apnoea (OSA) in children. When significant adenotonsillar hypertrophy is diagnosed, adenotonsillectomy (T&A) represents a common treatment for OSA in children. We investigated the role of pulse oximetry in predicting those patients, referred for suspected OSA, who subsequently needed T&A. At-home nocturnal pulse oximetry was performed on 380 children (65.7% males), median age 4.1(IRQ 3.0-5.6) years, referred for suspected OSA, and data were retrospectively analysed. For each recording McGill Oximetry Score (MOS) was categorized. Mean pulse rate (PR) z-score and pulse rate variability (PRV)-corrected (PRSD/meanPR) were significantly higher in children with abnormal MOS. Both parameters were significantly higher in subjects who underwent T&A compared with those not surgically treated. Both DI4 and PRV corrected showed a negative correlation with the elapsed time between pulse oximetry recordings and T&A. The logistic regression model showed a strong effect of an abnormal MOS as a predicting factor for T&A (adjusted odds ratio 19.7). CONCLUSIONS: In our study, children with OSA who subsequently needed T&A showed higher PRV compared to those without surgical indication. Children with abnormal MOS were nearly 20 times more likely to undergo T&A. What is Known: • Nocturnal pulse oximetry has a high positive predictive value for polysomnographically diagnosed obstructive sleep apnoea in children. • When significant adenotonsillar hypertrophy is diagnosed, adenotonsillectomy represents a common treatment for OSA in children. What is New: • An abnormal pulse oximetry highly predict the indication for adenotonsillectomy. • We suggest the use of at-home pulse oximetry as method to predict prescription of adenotonsillectomy, and this may be useful in contexts where polysomnography is not readily available.


Assuntos
Frequência Cardíaca/fisiologia , Oximetria/métodos , Valor Preditivo dos Testes , Apneia Obstrutiva do Sono/cirurgia , Tonsilectomia/estatística & dados numéricos , Adolescente , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Modelos Logísticos , Masculino , Polissonografia , Estudos Retrospectivos , Apneia Obstrutiva do Sono/diagnóstico , Inquéritos e Questionários
10.
J Allergy Clin Immunol ; 138(2): 413-420.e6, 2016 08.
Artigo em Inglês | MEDLINE | ID: mdl-27061250

RESUMO

BACKGROUND: There is no agreed upon definition of systemic corticosteroid response in asthmatic children. Moreover, pediatric severe therapy-resistant asthma (STRA) is heterogeneous, and thus response to steroids is unlikely to be uniform in all patients. OBJECTIVE: We sought to evaluate the utility of a multidomain approach incorporating symptoms, lung function, and inflammation to determine steroid responsiveness in pediatric patients with STRA. METHODS: Eighty-two children (median age, 12 years) with STRA received a clinically indicated dose of intramuscular steroid. Changes in 4 separate domains were assessed 4 weeks after intramuscular triamcinolone acetonide: normalization of (1) symptoms (Asthma Control Test score, >19/25 or 50% increase), (2) spirometric results (FEV1 ≥80% of predicted value or ≥15% increase), (3) fraction of exhaled nitric oxide levels (<24 ppb), and (4) sputum eosinophil counts (<2.5%). Fifty-four of 82 children had complete data in all 4 domains. RESULTS: Twenty-three (43%) of 54 children had a symptom response, 29 (54%) of 54 had a lung function response, 28 (52%) of 54 had a fraction of exhaled nitric oxide response, and 29 (54%) of 54 had a sputum eosinophil response. Although a similar proportion of children responded to systemic corticosteroids in each domain, there were no reliable predictors of a response pattern. Seven (13%) of 54 were complete responders (response in all domains), 8 (15%) of 54 were nonresponders (no response in any domain), and 39 (72%) of 54 were partial responders (response in ≥1 domain). CONCLUSIONS: A multidomain evaluation of systemic steroid responsiveness using pragmatic clinical assessments confirms childhood STRA is heterogeneous and that a complete response in symptoms and inflammatory and physiologic parameters is rare. Individual response patterns to systemic steroids might be useful in guiding the choice of add-on therapies in each child as a step toward achieving personalized medicine.


Assuntos
Corticosteroides/uso terapêutico , Antiasmáticos/uso terapêutico , Asma/diagnóstico , Asma/tratamento farmacológico , Adolescente , Corticosteroides/administração & dosagem , Antiasmáticos/administração & dosagem , Biomarcadores , Criança , Eosinófilos/patologia , Feminino , Volume Expiratório Forçado , Humanos , Contagem de Leucócitos , Masculino , Óxido Nítrico/metabolismo , Índice de Gravidade de Doença , Espirometria , Escarro/citologia , Resultado do Tratamento , Fluxo de Trabalho
11.
Int J Mol Sci ; 18(3)2017 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-28335406

RESUMO

Community-acquired pneumonia (CAP) is still the most important cause of death in countries with scarce resources. All children (33 months ± 35 DS) discharged from the Pediatric Unit of Itigi Hospital, Tanzania, with a diagnosis of CAP from August 2014 to April 2015 were enrolled. Clinical data were gathered. Dried blood spot (DBS) samples for quantitative real-time polymerase chain reaction (PCR) for bacterial detection were collected in all 100 children included. Twenty-four percent of patients were identified with severe CAP and 11% died. Surprisingly, 54% of patients were admitted with a wrong diagnosis, which increased complications, the need for antibiotics and chest X-rays, and the length of hospitalization. Comorbidity, found in 32% of children, significantly increased severity, complications, deaths, need for chest X-rays, and oxygen therapy. Malnourished children (29%) required more antibiotics. Microbiologically, Streptococcus pneumonia (S. p.), Haemophilus influenza type b (Hib) and Staphylococcus aureus (S. a.) were the bacteria more frequently isolated. Seventy-five percent of patients had mono-infection. Etiology was not correlated with severity, complications, deaths, oxygen demand, or duration of hospitalization. Our study highlights that difficult diagnoses and comorbidities negatively affect clinical evolution. S. p. and Hib still play a large role; thus, implementation of current vaccine strategies is needed. DBS is a simple and efficient diagnostic method for bacterial identification in countries with scarce resources.


Assuntos
Infecções Comunitárias Adquiridas/epidemiologia , Hospitais de Distrito , Pneumonia Bacteriana/epidemiologia , Criança , Pré-Escolar , Infecções Comunitárias Adquiridas/diagnóstico , Infecções Comunitárias Adquiridas/microbiologia , Infecções Comunitárias Adquiridas/mortalidade , Comorbidade , Feminino , Humanos , Lactente , Recém-Nascido , Tempo de Internação , Masculino , Fenótipo , Pneumonia Bacteriana/diagnóstico , Pneumonia Bacteriana/microbiologia , Pneumonia Bacteriana/mortalidade , Prognóstico , Fatores de Risco , Índice de Gravidade de Doença , Tanzânia/epidemiologia
13.
J Allergy Clin Immunol ; 136(2): 312-22.e7, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25746970

RESUMO

BACKGROUND: The mechanism underlying severe asthma with fungal sensitization (SAFS) is unknown. IL-33 is important in fungus-induced asthma exacerbations, but its role in fungal sensitization is unexplored. OBJECTIVE: We sought to determine whether fungal sensitization in children with severe therapy-resistant asthma is mediated by IL-33. METHODS: Eighty-two children (median age, 11.7 years; 63% male) with severe therapy-resistant asthma were included. SAFS (n = 38) was defined as specific IgE or skin prick test response positivity to Aspergillus fumigatus, Alternaria alternata, or Cladosporium herbarum. Clinical features and airway immunopathology were assessed. Chronic exposure to house dust mite and A alternata were compared in a neonatal mouse model. RESULTS: Children with SAFS had earlier symptom onset (0.5 vs 1.5 years, P = .006), higher total IgE levels (637 vs 177 IU/mL, P = .002), and nonfungal inhalant allergen-specific IgE. Significantly more children with SAFS were prescribed maintenance oral steroids (42% vs 14%, P = .02). SAFS was associated with higher airway IL-33 levels. In neonatal mice A alternata exposure induced higher serum IgE levels, pulmonary IL-33 levels, and IL-13(+) innate lymphoid cell (ILC) and TH2 cell numbers but similar airway hyperresponsiveness (AHR) compared with those after house dust mite exposure. Lung IL-33 levels, IL-13(+) ILC numbers, TH2 cell numbers, IL-13 levels, and AHR remained increased with inhaled budesonide during A alternata exposure, but all features were significantly reduced in ST2(-/-) mice lacking a functional receptor for IL-33. CONCLUSION: Pediatric SAFS was associated with more oral steroid therapy and higher IL-33 levels. A alternata exposure resulted in increased IL-33-mediated ILC2 numbers, TH2 cell numbers, and steroid-resistant AHR. IL-33 might be a novel therapeutic target for SAFS.


Assuntos
Antiasmáticos/uso terapêutico , Asma/tratamento farmacológico , Asma/imunologia , Budesonida/uso terapêutico , Interleucinas/imunologia , Micoses/tratamento farmacológico , Micoses/imunologia , Adolescente , Alternaria/imunologia , Animais , Animais Recém-Nascidos , Anticorpos Anti-Idiotípicos/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Aspergillus fumigatus/imunologia , Asma/complicações , Asma/patologia , Criança , Cladosporium/imunologia , Modelos Animais de Doenças , Feminino , Humanos , Imunoglobulina E/genética , Imunoglobulina E/imunologia , Proteína 1 Semelhante a Receptor de Interleucina-1 , Interleucina-13/genética , Interleucina-13/imunologia , Interleucina-33 , Interleucinas/genética , Masculino , Camundongos , Micoses/complicações , Micoses/patologia , Omalizumab , Pyroglyphidae/química , Pyroglyphidae/imunologia , Receptores de Interleucina/deficiência , Receptores de Interleucina/genética , Receptores de Interleucina/imunologia , Índice de Gravidade de Doença , Testes Cutâneos , Células Th2/imunologia , Células Th2/patologia
14.
J Allergy Clin Immunol ; 131(4): 1024-32, 1032.e1-16, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-23069488

RESUMO

BACKGROUND: Increased airway smooth muscle (ASM) is a feature of established asthma in schoolchildren, but nothing is known about ASM in preschool wheezers. OBJECTIVE: We sought to determine endobronchial biopsy specimen ASM area fraction in preschool wheezers and its association with asthma at school age. METHODS: ASM area, reticular basement membrane thickness, and mucosal eosinophil and ASM mast cell values were quantified in endobronchial biopsy specimens previously obtained from preschool children undergoing clinically indicated bronchoscopy: severe recurrent wheezers (n=47; median age, 26 months) and nonwheezing control subjects (n=21; median age, 15 months). Children were followed up, and asthma status was established at age 6 to 11 years. Preschool airway pathology was examined in relation to asthma at school age. RESULTS: Forty-two (62%) of 68 children had 1 or more evaluable biopsy specimens for ASM. At school age, 51 of 68 children were followed up, and 15 (40%) of 37 preschool wheezers had asthma. Children who had asthma and an evaluable biopsy specimen had increased preschool ASM area fraction (n=8; median age, 8.2 years [range, 6-10.4 years]; median ASM, 0.12 [range, 0.08-0.16]) compared with that seen in children without asthma (n=24; median age, 7.3 years [range, 5.9-11 years]; median ASM, 0.07 [range, 0.02-0.23]; P=.007). However, preschool reticular basement membrane thickness and mucosal eosinophil or ASM mast cell values were not different between those who did or did not have asthma at school age. CONCLUSION: Increased preschool ASM is associated with those children who have asthma at school age. Thus a focus on early changes in ASM might be important in understanding the subsequent development of childhood asthma.


Assuntos
Asma/diagnóstico , Asma/patologia , Brônquios/patologia , Músculo Liso/patologia , Sons Respiratórios/fisiopatologia , Asma/imunologia , Biópsia , Brônquios/imunologia , Broncoscopia , Criança , Pré-Escolar , Diagnóstico Precoce , Eosinófilos/imunologia , Eosinófilos/patologia , Feminino , Seguimentos , Humanos , Masculino , Mastócitos/imunologia , Mastócitos/patologia , Músculo Liso/imunologia , Testes de Função Respiratória , Mucosa Respiratória/imunologia , Mucosa Respiratória/patologia , Sons Respiratórios/imunologia
15.
J Allergy Clin Immunol ; 132(3): 676-685.e13, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-23759184

RESUMO

BACKGROUND: TH2 cytokines are not responsible for the ongoing symptoms and pathology in children with severe therapy-resistant asthma (STRA). IL-33 induces airway hyperresponsiveness, but its role in airway remodeling and steroid resistance is unknown. OBJECTIVE: We sought to investigate the relationship between IL-33 and airway remodeling in pediatric patients with STRA. METHODS: IL-33 levels were quantified in neonatal mice given inhaled house dust mite (HDM), and the effect of blocking IL-13 on remodeling and IL-33 levels was assessed. HDM-induced allergic airways disease (AAD) in neonatal ST2(-/-) mice lacking the IL-33 receptor was assessed, together with collagen production after IL-33 administration. The effect of steroid therapy on IL-33 levels in patients with neonatal AAD was explored. IL-33 expression was quantified in endobronchial biopsy (EB) specimens from children with STRA and related to remodeling, and collagen production by airway fibroblasts from pediatric patients stimulated with IL-33 and budesonide was quantified. RESULTS: Blocking IL-13 after AAD was established in neonatal mice and did not reduce remodeling or IL-33 levels; airway hyperresponsiveness was only partially reduced. IL-33 promoted collagen synthesis both from asthmatic fibroblasts from pediatric patients and after intranasal administration in mice. Increased cellular expression of IL-33, but not IL-13, was associated with increased reticular basement membrane thickness in EB specimens from children with STRA, whereas remodeling was absent in HDM-exposed ST2(-/-) mice. IL-33 levels were maintained, whereas IL-13 levels were abrogated by steroid treatment in neonatal HDM-exposed mice and in EB specimens from children with STRA. CONCLUSION: IL-33 is a relatively steroid-resistant mediator that promotes airway remodeling in patients with STRA and is an important therapeutic target.


Assuntos
Remodelação das Vias Aéreas , Asma/patologia , Hiper-Reatividade Brônquica/imunologia , Interleucinas/imunologia , Adolescente , Remodelação das Vias Aéreas/fisiologia , Animais , Animais Recém-Nascidos , Anti-Inflamatórios/uso terapêutico , Asma/tratamento farmacológico , Asma/imunologia , Hiper-Reatividade Brônquica/patologia , Budesonida/uso terapêutico , Criança , Colágeno/imunologia , Resistência a Medicamentos , Feminino , Glucocorticoides/uso terapêutico , Humanos , Interleucina-13/imunologia , Interleucina-33 , Interleucinas/administração & dosagem , Masculino , Camundongos , Camundongos Endogâmicos BALB C , Camundongos Knockout , Pyroglyphidae/imunologia
16.
Front Pediatr ; 12: 1345265, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38873586

RESUMO

Background: Primary ciliary dyskinesia (PCD) is considered a rare cause of chronic rhinosinusitis with nasal polyposis (CRSwNP), which is reported in 6% of children with PCD. The forms of PCD associated with the variants of the GAS8 gene identified so far seem to be linked to recurrent respiratory infections (sinusitis, otitis, and bronchiectasis) without situs inversus. Case presentation: We report a case of an 11-year-old girl with recurrent otitis media, productive cough, and chronic rhinosinusitis with nasal polyposis with homozygosity for a novel nonsense mutation in the GAS8. Conclusion: Children with CRSwNP should be treated in a multidisciplinary manner (ENT, pulmonologist, allergist, pathologist, pediatrician, and geneticist) because nasal polyposis often hides etiologies that must be recognized.

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.
Ital J Pediatr ; 50(1): 25, 2024 Feb 13.
Artigo em Inglês | MEDLINE | ID: mdl-38350986

RESUMO

BACKGROUND: The coronavirus 2019 (COVID-19) related containment measures led to the disruption of all virus distribution. Bronchiolitis-related hospitalizations shrank during 2020-2021, rebounding to pre-pandemic numbers the following year. This study aims to describe the trend in bronchiolitis-related hospitalization this year, focusing on severity and viral epidemiology. METHODS: We conducted a retrospective investigation collecting clinical records data from all infants hospitalized for bronchiolitis during winter (1st September-31th March) from September 2018 to March 2023 in six Italian hospitals. No trial registration was necessary according to authorization no.9/2014 of the Italian law. RESULTS: Nine hundred fifty-three infants were hospitalized for bronchiolitis this last winter, 563 in 2021-2022, 34 in 2020-2021, 395 in 2019-2020 and 483 in 2018-2019. The mean length of stay was significantly longer this year compared to all previous years (mean 7.2 ± 6 days in 2022-2023), compared to 5.7 ± 4 in 2021-2022, 5.3 ± 4 in 2020-2021, 6.4 ± 5 in 2019-2020 and 5.5 ± 4 in 2018-2019 (p < 0.001), respectively. More patients required mechanical ventilation this winter 38 (4%), compared to 6 (1%) in 2021-2022, 0 in 2020-2021, 11 (2%) in 2019-2020 and 6 (1%) in 2018-2019 (p < 0.05), respectively. High-flow nasal cannula and non-invasive respiratory supports were statistically more common last winter (p = 0.001 or less). RSV prevalence and distribution did not differ this winter, but coinfections were more prevalent 307 (42%), 138 (31%) in 2021-2022, 1 (33%) in 2020-2021, 68 (23%) in 2019-2020, 61 (28%) in 2018-2019 (p = 0.001). CONCLUSIONS: This study shows a growth of nearly 70% in hospitalisations for bronchiolitis, and an increase in invasive respiratory support and coinfections, suggesting a more severe disease course this winter compared to the last five years.


Assuntos
Bronquiolite , Coinfecção , Infecções por Vírus Respiratório Sincicial , Lactente , Humanos , Pandemias , Estudos Retrospectivos , Coinfecção/epidemiologia , Bronquiolite/epidemiologia , Bronquiolite/terapia , Hospitalização , Infecções por Vírus Respiratório Sincicial/epidemiologia , Infecções por Vírus Respiratório Sincicial/terapia
20.
Pediatr Pulmonol ; 58(6): 1640-1650, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36929867

RESUMO

Asthma is a common disease in childhood with a minority of affected children suffering from severe asthma. Patients with severe asthma require high dose inhaled glucocorticoids plus a second controller and/or systemic corticosteroids to be well-controlled or remain uncontrolled despite such treatment. Although only a small subset of children and adolescents falls in this category, the management of affected patients represents a major concern for pediatricians. Guidelines and recommendations have been designed to guide the management of this group of patients. Though the terms "recommendations" and "guidelines" are often used interchangeably, it should be noted that the first one should be used more narrowly to identify specific actions and the second one to broadly refer to the umbrella under which multiple recommendations for a specific condition are provided. Moreover, the availability of several and sometimes-conflicting documents on severe asthma management both in adult and pediatric age could generate confusion among health care professionals. The manuscript analyses seven papers addressing severe asthma, comparing any key aspects and differences. Finally, we tried to create a more practical document for physicians to simplify the interpretation of the several available documents on severe asthma management focusing the pediatric age.


Assuntos
Antiasmáticos , Asma , Médicos , Adulto , Criança , Humanos , Adolescente , Asma/tratamento farmacológico , Glucocorticoides/uso terapêutico , Corticosteroides/uso terapêutico , Antiasmáticos/uso terapêutico
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