Fibrosing alveolitis in infancy and childhood.

Hewitt, C J; Hull, D.; Keeling, Jean W.

doi:10.1136/adc.52.1.22

Cited by 52 publications

(28 citation statements)

References 38 publications

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“…IPF with UIP phenotype is a lethal disease of unknown etiology in older adults characterized pathologically temporal heterogeneity and fibroblastic foci (18). Before the strict pathologic definition was established, there were many cases of IPF, UIP, and cryptogenic fibrosing alveolitis reported in the older pediatric literature (19)(20)(21)(22). However, to our knowledge, there (24); however, this is not consistent with IPF with UIP phenotypes because of the known surfactant dysfunction mutation.…”

Section: Original Researchcontrasting

confidence: 45%

Diffuse Lung Disease in Biopsied Children 2 to 18 Years of Age. Application of the chILD Classification Scheme

et al. 2015

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Rationale: Children's Interstitial and Diffuse Lung Disease (chILD) is a heterogeneous group of disorders that is challenging to categorize. In previous study, a classification scheme was successfully applied to children 0 to 2 years of age who underwent lung biopsies for chILD. This classification scheme has not been evaluated in children 2 to 18 years of age.Objectives: This multicenter interdisciplinary study sought to describe the spectrum of biopsy-proven chILD in North America and to apply a previously reported classification scheme in children 2 to 18 years of age. Mortality and risk factors for mortality were also assessed.Methods: Patients 2 to 18 years of age who underwent lung biopsies for diffuse lung disease from 12 North American institutions were included. Demographic and clinical data were collected and described. The lung biopsies were reviewed by pediatric lung pathologists with expertise in diffuse lung disease and were classified by the chILD classification scheme. Logistic regression was used to determine risk factors for mortality. Measurements and Main Results:A total of 191 cases were included in the final analysis. Number of biopsies varied by center (5-49 biopsies; mean, 15.8) and by age (2-18 yr; mean, 10.6 yr). The most common classification category in this cohort was Disorders of the Immunocompromised Host (40.8%), and the least common was Disorders of Infancy (4.7%). Immunocompromised patients suffered the highest mortality (52.8%). Additional associations with mortality included mechanical ventilation, worse clinical status at time of biopsy, tachypnea, hemoptysis, and crackles. Pulmonary hypertension was found to be a risk factor for mortality but only in the immunocompetent patients. Conclusions:In patients 2 to 18 years of age who underwent lung biopsies for diffuse lung disease, there were far fewer diagnoses prevalent in infancy and more overlap with adult diagnoses. Immunocompromised patients with diffuse lung disease who underwent lung biopsies had less than 50% survival at time of last follow-up.

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Section: Original Researchcontrasting

confidence: 45%

Diffuse Lung Disease in Biopsied Children 2 to 18 Years of Age. Application of the chILD Classification Scheme

et al. 2015

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“…Some authors, e.g. Hewitt et al [6], reject the classification of graded interstitial pneumonitis and propose a unique lung disease that shows either more desquamative or more fibrosing transitions in histology [10]. This disease is named fibrosing alveolitis [6].…”

Section: Discussionmentioning

confidence: 97%

Desquamative interstitial pneumonitis and alveolar lipoproteinosis: Diagnostic difficulties and therapy problems with an infant

et al. 1986

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A desquamative, interstitial pneumonitis was diagnosed histologically in a 9-month-old boy who first became ill at the age of 5 weeks. The desquamative interstitial pneumonitis was associated with an acquired cytomegalovirus (CMV) infection. Despite treatment with corticoids, acyclovir and artificial ventilation, the patient died of pulmonary insufficiency at the age of 15 months. The autopsy revealed an alveolar lipoproteinosis.

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“…18 In a large children's hospital in London only 10 patients were reported over 13 years. 19 Five of the 10 children reported by Hewitt et al 19 presented at birth and seven in the first 6 weeks of life. All of them had cough and dyspnea.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic Pulmonary Fibrosis in Two Saudi Children

Adam

1988

Ann Saudi Med

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Fibrosing alveolitis in infancy and childhood.

Cited by 52 publications

References 38 publications

Diffuse Lung Disease in Biopsied Children 2 to 18 Years of Age. Application of the chILD Classification Scheme

Diffuse Lung Disease in Biopsied Children 2 to 18 Years of Age. Application of the chILD Classification Scheme

Desquamative interstitial pneumonitis and alveolar lipoproteinosis: Diagnostic difficulties and therapy problems with an infant

Idiopathic Pulmonary Fibrosis in Two Saudi Children

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