Endocrine Abstracts

Introduction: There is a known association between delayed puberty in autoimmune Addison’s disease in presence of polyglandular involvement, but constitutional delay still needs to be considered in these cases.

Case: We report a 14-year-old boy who presented with a 4 month history of dizziness, fainting episodes and weight loss at the age of 12 years. He was stabilised on a reasonable dose of hydrocortisone and fludrocortisone after a diagnosis of Addison’s was made.

He then presented with growth failure and pubertal delay after being stable on steroid replacement for the next seventeen months. Examination revealed a reduced testicular size for age and prepubertal tanner staging and reduced height velocity and static weight for the last 5 months with a bone age delay of ten months. Investigations at this stage, suggested that he was well controlled with no evidence of over or under replacement and with no acute hospital attendances or periods of being unwell.

A provisional diagnosis of constitutional delay of growth and puberty was made and he was started on a trial of 4weekly testosterone for next 7 months during which marked improvement of his height velocity and pubertal development was noted.

Conclusions: i) Growth and pubertal delay in well controlled Addison’s could present as a diagnostic dilemma. ii) Appropriate investigations for growth and pubertal delay with optimising replacement before starting hormonal treatment is probably best practice in this condition, as this case demonstrates.

iii) Although linear growth is normal in adequately treated Addisons disease, further studies will be needed to see whether these patients achieve their final predictive height based on their appropriate mid parental centiles.

References:

Grant et al. ADC 1985 60 925–928.

Mehta et al. BMJ Best practice, May’12.