Abstract
During the acquisition of a series of 92 children with interstitial lung disease (ILD) over a 14 year period, a significant minority (8/92 or 9%) were initially diagnosed as having ILD, but were subsequently found to have a variety of arterial, venous, and/or capillary disorders that explained their initial pulmonary findings. This subgroup of patients has had a very high morbidity and mortality, with only three of eight patients currently surviving. The presentation, evaluation, and natural history of these eight children were reviewed. We developed a strategy of cardiac and pulmonary evaluation for children presenting with clinical and radiographic features of ILD that helped us to identify rapidly those with pulmonary vascular disorders.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Adolescent
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Cardiovascular Diseases / diagnosis*
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Cardiovascular Diseases / physiopathology
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Child
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Chronic Disease
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Diagnosis, Differential
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Echocardiography
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Electrocardiography
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Humans
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Hypertension, Pulmonary / diagnosis*
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Hypertension, Pulmonary / physiopathology
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Incidence
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Infant
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Infant, Newborn
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Logistic Models
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Lung Diseases, Interstitial / diagnosis*
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Lung Diseases, Interstitial / physiopathology
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Pulmonary Veno-Occlusive Disease / diagnosis
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Pulmonary Veno-Occlusive Disease / physiopathology
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Retrospective Studies
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Risk Factors
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Survival Rate
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Telangiectasia, Hereditary Hemorrhagic / diagnosis
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Telangiectasia, Hereditary Hemorrhagic / physiopathology