Cognitive functions in children with congenital adrenal hyperplasia.

Nermine Hussein Amr,Abdelgawad Khalifa,Alaa Youssef Baioumi,Mohamed Nagy Serour,Nermine Mahmoud Shaker

doi:10.20945/2359-3997000000125

Nermine Hussein Amr, Abdelgawad Khalifa + Show 3 more

Open Access

https://doi.org/10.20945/2359-3997000000125

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Abstract

There is controversy regarding cognitive function in patients with congenital adrenal hyperplasia (CAH). This study is aimed at the assessment of cognitive functions in children with CAH, and their relation to hydrocortisone (HC) therapy and testosterone levels. Thirty children with CAH due to 21 hydroxylase deficiency were compared with twenty age- and sex-matched healthy controls. HC daily and cumulative doses were calculated, the socioeconomic standard was assessed, and free testosterone was measured. Cognitive function assessment was performed using the Wechsler Intelligence Scale - Revised for Children and Adults (WISC), the Benton Visual Retention Test, and the Wisconsin Card Sorting Test (WCST). The mean age (SD) of patients was 10.22 (3.17) years [11 males (36.7%), 19 females (63.3%)]. Mean (SD) HC dose was 15.78 (4.36) mg/m 2 /day. Mean (SD) cumulative HC dose 44,689. 9 (26,892.02) mg. Patients had significantly lower scores in all domains of the WISC test, performed significantly worse in some components of the 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, 10 - 15, > 15 mg/m 2 /day). A positive correlation existed between cumulative HC dose and worse results of the Benton test. No correlation existed between free testosterone and any of the three tests. Patients with CAH are at risk of some cognitive impairment. Hydrocortisone therapy may be implicated. This study highlights the need to assess cognitive functions in CAH.

Highlights

Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive diseases, the most common enzyme defect of which is 21-hydroxylase deficiency
Memory performance defects were demonstrated in some patients with congenital adrenal hyperplasia (CAH) [5], some showed defects in mental rotation [6], executive functions [7], spatial abilities [8], while others showed no differences in cognitive functions between patients and controls [9]
The present study shows that young patients with CAH have intellectual disabilities as reflected by lower total, verbal, and performance IQ scores

Summary

Introduction

Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive diseases, the most common enzyme defect of which is 21-hydroxylase deficiency. Memory performance defects were demonstrated in some patients with CAH [5], some showed defects in mental rotation [6], executive functions [7], spatial abilities [8], while others showed no differences in cognitive functions between patients and controls [9]. The aim of this study is to assess the cognitive functions in children with CAH, and their relation to hydrocortisone (HC) therapy and testosterone level.

Results

Conclusion