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Cognitive function in children with classic congenital adrenal hyperplasia

Research Authors
Sherifa Ahmed Hamed1 & Kotb Abbass Metwalley2 & Hekma Saad Farghaly2
Research Department
Research Journal
European Journal of Pediatrics
Research Publisher
NULL
Research Rank
1
Research Vol
Vol. 177
Research Website
NULL
Research Year
2018
Research_Pages
pp. 1633–1640
Research Abstract

Studies of cognitive function in patients with congenital adrenal hyperplasia (CAH) are few and controversial. This
study aimed to investigate general intelligence and specific cognitive functions in children with salt wasting (SW)
form of CAH and their relationship to demographic, clinical, and laboratory variables. This study included 36 children
with classic 21 hydroxylase deficiency SW type of CAH (males = 12; females = 24; mean age = 15.6 ± 2.3 years).
Intelligence quotient (IQ) and cognition were assessed using Wechsler Intelligence Scale for Children 3rd edition
(WISC-III) and Stanford Binet Subsets Test version 4 (SBST4). Compared to controls, patients had lower mean
full-scale (FS) IQ (P = 0.01) score, particularly performance IQ score (P = 0.001), and comprehension, pattern analysis,
quantitation, bead memory, and memory for sentences of SBST4 (P = 0.05, P = 0.014, P = 0.001, P = 0.002, and P =
0.05, respectively). Lower IQ was observed in poorly controlled compared with well-controlled patients on medical
treatment. Significant correlations were observed between FSIQ with age (r = − 0.810; P = 0.001), duration of treatment
(r = − 0.887; P = 0.01), dose of glucocorticoids (r = − 0.463; P = 0.01), 17-OHP (r = − 0.543; P = 0.01) and testosterone
(r = − 0.462; P = − 0.006) levels, and number of hyponatremic episodes (r = − 0.350; P = 0.05). In multivariate analysis,
the independent risks of low FSIQ were the dose of glucocorticoids (OR = 1.14; 95% CI = 1.08–1.23, P = 0.0001), 17-
OHP levels (OR = 2.25; 95% CI = 1.19–2.85, P = 0.01), and number of hyponatremic episodes (OR = 4.34; 95% CI =
2.05–5.15, P = 0.01).
Conclusion: Patients with SW form of CAH may have lower IQ and cognitive deficits which may be related to the dose of
glucocorticoids, androgen excess, and number of hyponatremic episodes.