Cushing's disease in prepubertal children

Cushing's disease (CD) in prepubertal children, while rare, presents problems for diagnosis and management. Dias et al. have reported a retrospective case note review of 17 patients (aged 5.7-14.1 years) who presented at St Bartholomew's and the Royal London Hospitals between 1985 and 2008.

The study has found that the clinical features of Cushing's disease in prepubertal patients can be subtle, and noted that growth failure and excessive weight gain were seen in all patients. A change in facial appearance was also a commonly observed feature.

The test with highest sensitivity showed an excessive increase in serum cortisol in response to i.v. corticotrophin-releasing hormone (CRH), which contributed to diagnosis. Pituitary imaging was only effective in identifying the microadenoma in a small percentage, as in this age group they are usually too small to be identified. However, bilateral simultaneous inferior petrosal sinus sampling (BSIPSS) was highly effective and in the patients in whom it was used, appeared to contribute to positive surgical outcome and cure. R P Dias et al., European Journal of Endocrinology.

DOI: 10.1530/EJE-09-0509