Pulmonary vascular disorders masquerading as interstitial lung disease.
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.
Department of Pediatrics, The Children's Hospital, University of Colorado Health Sciences Center, Denver, USA.
H M
HM
Sondheimer
M C
MC
Lung
S M
SM
Brugman
D N
DN
Ikle
L L
LL
Fan
C W
CW
White
D000293:Adolescent; D002318:Cardiovascular Diseases / Q000175:diagnosis* / Q000503:physiopathology; D002648:Child; D002908:Chronic Disease; D003937:Diagnosis, Differential; D004452:Echocardiography; D004562:Electrocardiography; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000503:physiopathology; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D016015:Logistic Models; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000503:physiopathology; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis / Q000503:physiopathology; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000175:diagnosis / Q000503:physiopathology