European Journal of Pediatrics

, Volume 177, Issue 11, pp 1633–1640 | Cite as

Cognitive function in children with classic congenital adrenal hyperplasia

  • Sherifa Ahmed Hamed
  • Kotb Abbass Metwalley
  • Hekma Saad Farghaly
Original Article


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.

What is Known:

Congenital adrenal hyperplasia (CAH) is a group of inherited impairment of cortisol biosynthesis.

Studies of cognitive function in patients with congenital adrenal hyperplasia (CAH) are few and controversial.

What is New:

Children with CAH may have lower intelligent quotient (IQ) and cognitive deficits.

Early hyponatremic episodes, overtreatment with glucocorticoids, and high androgen levels may be possible causative factors for the cognitive deficits.


Congenital adrenal hyperplasia Cognition Glucocorticoids Memory Testosterone 



Congenital adrenal hyperplasia


Intelligence quotient


Stanford Binet Subsets Test version 4


Salt wasting


Wechsler Intelligence Scale for Children 3rd edition


Authors’ contributions

SH participated in the design of the protocol of the study, coordination of the research, performance of the clinical part, analyses of the data, and writing the draft of the paper. KM participated in the design of the protocol of the study and coordination of the research, performance of the clinical part, analyses of the data, and writing the draft of the paper. HF participated in performance of the clinical part, writing of the draft, and analyses of the results. All the authors read and approved the manuscript.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflicts of interest.

Ethical approval

The study protocol was approved by the Ethics Committee of the Faculty of Medicine, Assiut Children University Hospital, Assiut, Egypt.

Informed consent

Written informed consents were obtained from the parents of all participants.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Neurology and Psychiatry, Faculty of MedicineAssiut UniversityAssiutEgypt
  2. 2.Pediatric Endocrinology Unit, Department of Pediatrics, Faculty of MedicineAssiut UniversityAssiutEgypt

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