الفهرس 
Congenital Adrenal HyperplasiaOnly 14 pages are availabe for public view
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Abstract
Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive disorders characterized by impaired cortisol synthesis. The cortisol synthetic block leads to corticotropin stimulation of the adrenal cortex, with accumulation of cortisol precursors that are diverted to sex hormone biosynthesis. A cardinal feature of classic or severe virilizing CAH in newborn females is genital ambiguity If the disorder is not recognized and treated both girls and boys undergo rapid postnatal growth and sexual precocity or in the case of severe enzyme deficiency, neonatal salt loss and death. About 75% of classic CAH cases suffer aldosterone deficiency with salt wasting, failure to thrive and potentially fatal hypovolemia and shock.
Cognition in patients with CAH has not been as well studied as other aspects of psychological function. The evidence to date does not confirm that patients with CAH are more likely to have diagnosable learning disabilities when compared with their unaffected relatives but this issue has not been well studied with the appropriate psychoeducational assessments.
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The aim of the work was to assess cognitive functions in patients with congenital adrenal hyperplasia.
This study for one year duration conducted on Thirty boys and girls with congenital adrenal hyperplasia aged between eight and sixteen years following up at the Paediatric Endocrinology Clinic of Ain-Shams University Hospitals.
Patients were subjected to history taking and examination, Anthropometric evaluation, Tanner staging, Hormonal assay for androgen profile and Assessment of ccognitive functions using; Wechsler Intelligence Scale for Children (WISC), Wisconsin Card Sorting Test (WCST) the computerized version and Benton Visual Retention Test.
Results of this study show that
No significant difference between male: female ratios and socioeconomic standard in both patients and controls.
No significant difference was found between patients and controls in age and anthropometric data.
Patients had significantly lower mean scores on all domains of the WISC test including verbal performance as well total IQ than controls.
There is high significance decrease in the neurocognitive tests, Benton tests and Wisconsin Card Sorting Test in patients compared to controls.
There was no difference between scores of salt wasting and simple virilizing on in all cognitive tests.
There was no significant difference between patient subgroups according to hydrocortisone daily doses in Wechsler Intelligence Scale for Children (WISC) test, Benton test and Wisconsin Card Sorting Test.
No significant difference between patients of high and middle socioeconomic standard in Benton test.
There is high significant difference between patients of high and middle socioeconomic standard in Verbal Intelligence Quotient and Total Intelligence Quotient. Patients of high socioeconomic standard are better in both tests than patients of middle socioeconomic standard.
There is high significant difference between patients of high and middle socioeconomic standard in Wisconsin Card Sorting Test. Patients of high socioeconomic standard are better in this test than patients of middle socioeconomic standard.
No significant correlations were found between Total IQ and clinical or laboratory data.
A significant positive correlation was found between Difference Error Score as a part of Benton test and total hydrocortisone dose and age.
A significant positive correlation was found between Categories Completed as a part of Wisconsin Card Sorting Test and total hydrocortisone dose and age.