Infants and Young Children with Children's Interstitial Lung Disease

Deterding, Robin R.

doi:10.1089/ped.2010.0011

Cited by 73 publications

(76 citation statements)

References 47 publications

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“…In the last decade, NEHI was described as an ILD that exclusively affected infants, with characteristic clinical findings (tachypnea, retractions, hypoxemia, and crackles) and tomographic findings (GGO in the central regions of the lungs, especially in the middle lobe and lingula, and air trapping), and good prognosis (9,10,13) ; similar results were found in our case series, only differing in the need for oxygen therapy, which was lower than in previous reports.…”

Section: Discussionsupporting

confidence: 87%

Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case series

et al. 2013

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Objective: Neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease characterized by tachypnea, retractions, crackles, and hypoxia. The aim of this study was to report and discuss the clinical, imaging, and histopathological findings in a series of NEHI cases at a tertiary pediatric hospital, with an emphasis on diagnostic criteria and clinical outcomes. Methods: Between 2003 and 2011, 12 full-term infants were diagnosed with NEHI, based on clinical and tomographic findings. Those infants were followed for 1-91 months. Four infants were biopsied, and the histopathological specimens were stained with bombesin antibody. Results: In this case series, symptoms appeared at birth in 6 infants and by 3 months of age in the remaining 6. In all of the cases, NEHI was associated with acute respiratory infection. The most common initial chest HRCT findings were ground-glass opacities that were in the middle lobe/lingula in 12 patients and in other medullary areas in 10. Air trapping was the second most common finding, being observed in 7 patients. Follow-up HRCT scans (performed in 10 patients) revealed normal results in 1 patient and improvement in 9. The biopsy findings were nonspecific, and the staining was positive for bombesin in all samples. Confirmation of NEHI was primarily based on clinical and tomographic findings. Symptoms improved during the follow-up period (mean, 41 months). A clinical cure was achieved in 4 patients. Conclusions: In this sample of patients, the diagnosis of NEHI was made on the basis of the clinical and tomographic findings, independent of the lung biopsy results. Most of the patients showed clinical improvement and persistent tomographic changes during the follow-up period, regardless of the initial severity of the disease or type of treatment.Keywords: Lung diseases, interstitial/diagnosis; Lung diseases, interstitial/treatment; Tomography, X-ray computed. ResumoObjetivo: A hiperplasia de células neuroendócrinas do lactente (HCNEL) é uma forma de doença pulmonar intersticial da infância caracterizada por taquipneia, retrações, estertores e hipóxia. O objetivo deste estudo foi descrever e discutir os achados clínicos, histopatológicos e de imagem em uma série de casos de HCNEL em um hospital pediátrico terciário, enfatizando critérios de diagnóstico e desfechos clínicos. Métodos: Entre 2003 e 2011, 12 lactentes nascidos a termo foram diagnosticados com HCNEL, com base em critérios clínico-tomográficos e acompanhados por 1-91 meses. Quatro lactentes foram submetidos a biopsia pulmonar, e as amostras histopatológicas foram coradas com anticorpo para bombesina. Resultados: Nesta série de casos, os sintomas surgiram ao nascimento em 6 lactentes e em até 3 meses de idade nos outros 6. Em todos os casos, HCNEL estava associada com infecção respiratória aguda. Os achados iniciais em TCAR de tórax foram opacidades em vidro fosco em lobo médio e língula, em 12 pacientes, e em outras regiões medulares, em 10. O aprisionamento aéreo foi o segundo achad...

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Section: Discussionsupporting

confidence: 87%

Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case series

et al. 2013

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“…As recent attention has been given to the infant with chILD, with the application of a novel classifi cation scheme to diffuse lung disease in young children described by Deutsch et al 2 ; an Amercian Thoracic Society guidelines for the classifi cation, evaluation, and management of chILD in infants currently near completion; and the review by Deterding in this issue 3 focuses on ILD presenting in children older than 2 years of age. A classifi cation of the disorders seen in this population is presented in Table 1 .…”

Section: Introductionmentioning

confidence: 99%

Interstitial Lung Disease in Children Older Than 2 Years

Vece

Fan

2010

Pediatric Allergy, Immunology, and Pulmonology

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The spectrum of childhood interstitial lung diseases (chILD) encompasses a group of heterogeneous, rare disorders in children characterized by diffuse pulmonary infi ltrates and disordered gas exchange. Whereas the disorders that present in early life are unique to children, those that present in older children are also seen in adults. This review will concentrate on chILD presenting in children older than 2 years of age with a focus on the idiopathic interstitial pneumonias, connective tissue diseases, alveolar hemorrhage, and hypersensitivity pneumonitis. A systematic approach to diagnosis that includes a careful history and physical, computed tomography of the chest, bronchoalveolar lavage, and lung biopsy can be very helpful in establishing the correct diagnosis. Treatment approaches are described, including general supportive measures, indications for a trial of systemic corticosteroids, or other immunomodulating therapies, and when lung transplantation reserved for those with end-stage lung disease should be considered.

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“…Compared with adult disease, 16 chILD occurs far less frequently, with a prevalence of idiopathic ILD among immunocompetent children aged 0 to 16 years being estimated at 3.6 cases per million, 17 although this is likely to be an underestimate. 18 Our understanding of chILD remains incomplete. However, important advances in disease mechanisms, diagnostic approach, and management have been recently made.…”

Section: Doi: 101542/peds2015-2725mentioning

confidence: 99%

Interstitial Lung Disease in Children Younger Than 2 Years

Spagnolo

Bush

2016

Pediatrics

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Childhood interstitial lung disease (chILD) represents a highly heterogeneous group of rare disorders associated with substantial morbidity and mortality. Although our understanding of chILD remains limited, important advances have recently been made, the most important being probably the appreciation that disorders that present in early life are distinct from those occurring in older children and adults, albeit with some overlap. chILD manifests with diffuse pulmonary infiltrates and nonspecific respiratory signs and symptoms, making exclusion of common conditions presenting in a similar fashion an essential preliminary step. Subsequently, a systematic approach to diagnosis includes a careful history and physical examination, computed tomography of the chest, and some or all of bronchoscopy with bronchoalveolar lavage, genetic testing, and if diagnostic uncertainty persists, lung biopsy. This review focuses on chILD presenting in infants younger than 2 years of age and discusses recent advances in the classification, diagnostic approach, and management of chILD in this age range. We describe novel genetic entities, along with initiatives that aim at collecting clinical data and biologic samples from carefully characterized patients in a prospective and standardized fashion. Early referral to expert centers and timely diagnosis may have important implications for patient management and prognosis, but effective therapies are often lacking. Following massive efforts, international collaborations among the key stakeholders are finally starting to be in place. These have allowed the setting up and conducting of the first randomized controlled trial of therapeutic interventions in patients with chILD.

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Infants and Young Children with Children's Interstitial Lung Disease

Cited by 73 publications

References 47 publications

Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case series

Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case series

Interstitial Lung Disease in Children Older Than 2 Years

Interstitial Lung Disease in Children Younger Than 2 Years

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