ESPE Abstracts

Introduction: Congenital adrenal hyperplasia (CAH) is a disorder with a wide spectrum of severity. Impaired cognition has been reported in patients with CAH, although the findings have been conflicting. It has been hypothesized that the major causes of the deficits are prenatal hormonal imbalances and/or excessive glucocorticoid treatment.

Objective: The objective of this study was to investigate cognitive function in children with CAH and to assess their anthropomentric measurements who were diagnosed early in life and were under good hormonal control.

Subjects and Methods: This study was conducted on children with CAH at a tertiary center for pediatric endocrinology at Alexandria University Children’s Hospital in Egypt. Anthropometric measurements were done for them. Cognitive function testing was done by using the Stanford-Binet Intelligence Scale, Forth Edition. Vineland Adaptive Behavior Scale (VABS) was used in children that refused to interact in the Stanford-Binet Intelligence Scale.

Results: Forty-six children and adolescents (their mean age 7.33 ± 3.89 years) were included, 14 males and 32 females. None of their mothers received Dexamethasone during their gestation. 41/46 were diagnosed during the first 3 months of their life and 5 during the first year of life. All children were on Prednisone therapy (3.72 ± 0.59 mg/m²/day) (equivalent to hydrocortisone dose = 14.82 ± 2.38 mg/m²/day) and fludrocortisone. Their doses were tailored to keep testosterone level (<9 ng/ml) and 17-OHP levels are < 600 ng/dl in the morning before medication. 34/46 had normal weight (>5 – <85 th percentile), 8/46 were overweight (85 - <95 th percentile) and 4/46 were obese ≥ 95 th percentile. Their mean height SDS (HtSDS) was –1.87 ± 1.43 SD and mean BMI was 20.3 ± 6.8 %. Mild mental retardation occurred in 13.7 %, slow learning occurred in 25.5% and low average IQ occurred in 21.6 % of them. Moderate retardation was found only in one girl. There was no significant difference in IQ score between boys and girls (t=0.481, P = 0.634).

Conclusions: Impaired cognitive function was observed in patients with CAH despite reasonable control of their androgens and 17- OHPlevels. These results may reflect prenatal adrenal androgen excess, and the potential psychosocial consequences of the disorder.