J. Takaya et al., A PATIENT WITH CONGENITAL LIPOID ADRENAL-HYPERPLASIA EVALUATED BY SERIAL ABDOMINAL ULTRASONOGRAPHY, European journal of pediatrics, 157(7), 1998, pp. 544-546
Adrenal enlargement was followed by serial ultrasonography in an infan
t with congenital lipoid adrenal hyperplasia (lipoid CAH) from day 12
until 2 years and 4 months of age, when they were no longer detectable
. Contrary to other types of CAH in which the configuration changes so
on after replacement therapy, this infant with lipoid CAH showed persi
stent adrenal cortex enlargement due to massive accumulation of lipids
and cholesterol resulting in a damaged glandular cyto-architecture. C
onclusion ultrasonographically persistent enlargement of the adrenals
after replacement therapy is suggestive of the lipoid form of CAH.