Background: There is controversy regarding cognitive affection in patients with congenital adrenal hyperplasia (CAH).
Objective and hypotheses: Assess cognitive functions in children with CAH, and their relation to hydrocortisone (HC) therapy and testosterone level.
Method: Thirty children with CAH due to 21 hydroxylase deficiency were compared with 20 age and sex matched healthy controls. Hydrocortisone daily dose and cumulative doses were calculated, socioeconomic standard was assessed, and free testosterone was measured. Cognitive function assessment was performed using Wechsler intelligence scale revised for children and adults (WISC) which reflects the intellectual performance through verbal, performance, and full scale IQ, Benton visual retention test to assess visual perception, memory and visual-constructive abilities, and Wisconsin card sorting test (WCST); a tool for recognizing frontal cortical dysfunction.
Results: The mean age (S.D.) of patients was 10.22 (3.17) years (11 males (36.7%), 19 females (63.3%)). Mean (S.D.) HC dose was 15.78 (4.36) mg/m2 per day. Mean (S.D.) cumulative HC dose 44 689. 9 (26 892.0) mg. No significant difference in age, gender, socioeconomic standard, and anthropometric data existed between patients and controls. Patients had significantly lower scores in all domains of WISC test, performed significantly worse in Benton Visual Retention test, as well as in the Wisconsin Card Sorting Test. There was no significant difference in cognitive performance when patients were subdivided according to daily HC dose (<10, 1015, >15 mg/m2 per day), or according to salt wasting state. A positive correlation existed between cumulative HC dose and worse results of Benton test. No correlation existed between free testosterone and any of the three tests.
Conclusion: Patients with CAH are at risk of cognitive impairment. Hydrocortisone therapy may be implicated. This study highlights the need to assess cognitive functions in CAH.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology