The clinical and morphological features are presented of eight infants
who developed a chronic respiratory illness in the first year of life
characterised by tachypnoea and inspiratory crackles on auscultation.
The median age at the onset of symptoms was 1 month (range 1 day to 7
months). Five of the infants required supplemental oxygen. Chest radi
ographs showed only non-specific perihilar alveolar shadowing and gene
ralised hyperinflation. Investigations into the cause of the illness i
n these infants, including studies for infection, cardiac disease, and
gastrooesophageal reflux, were negative, and an open lung biopsy samp
le was subsequently taken from each infant. Histological examination s
howed mild lymphocytic bronchiolar infiltration only in six of the eig
ht infants. The other two biopsy samples were normal. Seven of the inf
ants were given a trial of steroids by mouth (mean duration of treatme
nt four months) without any apparent benefit. These infants have now b
een followed up for a median duration of 21 months (range 1-9 years).
All have shown continuous improvement, with either the end of oxygen t
reatment (three patients) or the use of low flow oxygen only at night
(two patients). All have normal development and all but one have norma
l growth. Chest examination has become normal in four subjects. In the
remainder the inspiratory crackles have persisted, although they have
decreased markedly in all patients. It is proposed that these infants
have a previously unreported respiratory entity and that the clinical
and lung biopsy sample findings reported here predict a favourable me
dium term prognosis.