Endocrine Abstracts

Context: Prior studies of children with endogenous Cushing syndrome (CS) have identified cognitive decline despite reversal of brain atrophy after remission as well as residual impairment of quality of life measures. Although parental observations support personality changes with CS, significant psychopathology has not been described. We investigated the feasibility of using paediatric Patient-reported outcomes (PROMIS) using a computer interface to assess patient perception of burden of illness and correlation with parent proxy report using Child Health Questionnaire (CHQ).

Setting: Subjects were enrolled in a clinical protocol at the National Institutes of Health Clinical Center in Bethesda, MD, USA.

Method: We report 9 children (6F, 12±3.5 years) diagnosed with Cushing disease (CD). Prior to surgical treatment, subjects completed paediatric PROMIS measures and parents completed CHQ using an online secure website.

Results: PROMIS measures scores (mean±S.D.): anxiety (54±9), depression (55±11) anger (50±9), and emotional support (52±10) (higher scores indicate more of the concept being measured); CHQ total Psychosocial score (mean±S.D.) (43±10) (lower values are associated with a greater deficit in functioning). A significant correlation was found between Psychosocial summary score of CHQ and paediatric PROMIS measures of anxiety (r=−70.5; P<0.03) and depression (r=−0.9; P<0.002). No correlation was found between urinary free cortisol level or midnight serum cortisol and PROMIS measures.

Conclusion: Prior to treatment for CS, a significant correlation was found between children’s endorsement of anxiety and depression symptoms and parental proxy report of psychosocial function. Our findings support that paediatric PROMIS measures represent feasible and potentially valuable instruments for future studies to assess behavioural symptoms in children diagnosed with CS prior to and after treatment.