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Cholestatic hepatitis as a result of severe cortisol deficiency in early infancy: report of two cases and review of literature.

Turk J Pediatr 2006 Oct-Dec;48(4):376-9

Division of Pediatric Endocrinology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Cholestatic hepatitis is identified as one of the features of hypopituitarism in the newborn, but the exact etiology of cholestasis in these cases has not been well established yet. We report here two infants, one with isolated glucocorticoid deficiency and the other with multiple pituitary hormone deficiency, indicating primary and central adrenal insufficiency, respectively, who presented with recurrent hypoglycemic seizures and cholestatic hepatitis. Severe cortisol deficiency in these cases was suggested to be the cause of cholestatic hepatitis. Review of the literature and our cases showed that the cortisol deficiency in both primary and central adrenal insufficiency occurring only during neonatal and early infancy period cause cholestatic hepatitis. The severity and the age of onset of cortisol deficiency are suggested to be the important predictors of cholestatic hepatitis in childhood.

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April 2007
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