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.
Sondheimer
H M
HM
Department of Pediatrics, The Children's Hospital, University of Colorado Health Sciences Center, Denver, USA.
Lung
M C
MC
Brugman
S M
SM
Ikle
D N
DN
Fan
L L
LL
White
C W
CW
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