Adrenal enlargement was followed by serial ultrasonography in an infant 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 soon after replacement therapy, this infant with lipoid CAH showed persistent adrenal cortex enlargement due to massive accumulation of lipids and cholesterol resulting in a damaged glandular cyto-architecture.
Conclusion ultrasonographically persistent enlargement of the adrenals after replacement therapy is suggestive of the lipoid form of CAH.
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Received: 3 June 1997 / Accepted in revised form: 19 December 1997
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Takaya, J., Ishihara, R., Kino, M. et al. A patient with congenital lipoid adrenal hyperplasia evaluated by serial abdominal ultrasonography. Eur J Pediatr 157, 544–546 (1998). https://doi.org/10.1007/s004310050874
- Key words Congenital lipoid adrenal hyperplasia