ESPE Abstracts

Introduction: A 5 month old boy is admitted to his local hospital with increased work of breathing secondary to a viral upper respiratory tract infection. The admitting team found pubic hair on examination, with otherwise normal infant genitalia, prompting a screen for precocious puberty. A low random cortisol was found at 15nmol/L. Following this, he had an inadequate response to a Synacthen test (peak Cortisol 190 nmol/L) demonstrating adrenal insufficiency. Awaiting results of further investigations, the infant developed bronchiolitis requiring local admission and then transfer to a tertiary unit for escalation of treatment.

Background: The infant was born at term and required a neonatal admission for respiratory distress. There were no antenatal concerns or family history of note. He had no dysmorphic features. However, there was difficulty passing a nasogastric tube and on ENT review he was diagnosed with pryifrom aperture stenosis. He was commenced on intranasal Betamethasone, 1 drop to each nostril BD from day 5 of life, which were continued on discharge.

Management: On admission to the tertiary hospital, in view of his failed Synacthen test and due to clinical deterioration, he was commenced on stress Hydrocortisone. On examination the infant was noted to have a markedly cushingoid appearance with hirsutism and pubic hair tanner stage 2. His testicular volume was pre pubertal with normal penile appearance for infancy. Regarding his investigation results; ACTH was low at <2ng/L. Remaining pituitary hormones, adrenal androgens and urine steroid profile were all normal. An MRI brain showed a normal pituitary gland and an ultrasound abdomen showed no adrenal abnormality. Adrenal Suppression secondary to long-term topical steroid treatment for nasal stenosis was diagnosed and Betamethasone were stopped. He was commenced on a gradual weaning Hydrocortisone regime over six weeks. A repeat Synacthen test post weaning steroids showed a satisfactory response and therefore resolved adrenal suppression.

Conclusion: This case supports the findings that all exogenous steroids put patients at risk of adrenal suppression and reinforces the recommendation that if glucocorticoids have been used for 3 weeks or longer, then an assessment of the integrity of the HPA axis should be performed. These parents had not been made aware of the potential side effects of glucocorticoids and had not attributed the growth of pubic hair to his nasal treatment signifying the importance of patient/parent education and awareness of adrenal insufficiency risk with glucocorticoids.