Faculty Bibliography

The medical management of children with congenital adrenal hyperplasia (CAH) can be challenging with regard to optimizing final height. Insufficient glucocorticoid suppression of adrenal hormone production will result in acceleration of bone maturation and premature epiphyseal fusion, while appropriate or excessive glucocorticoid therapy can be accompanied by suppression of the growth axis. The extent of the growth suppression appears to be affected by the type and dose of glucocorticoid. Some studies suggest that such growth suppression can be ameliorated through concomitant use of growth hormone (GH) therapy. Available data published over the last 10 years on height outcomes in CAH patients treated with glucocorticoids will be reviewed

Acanthosis nigricans in children is usually a benign condition most commonly associated with obesity. Generalized acanthosis nigricans is a very rare condition, especially in childhood. We report a 6-year-old boy with a 4-year history of generalized hyperpigmentation and velvety thickening of the skin. Despite an extensive examination, no evidence for an underlying neoplastic or endocrinologic disease was found.

We describe three siblings with congenital myopathy, bullous eruption of the skin, secretory diarrhea, apparent zinc deficiency, failure to thrive, deafness, and microcephaly. The parents are not consanguineous and there are no other affected relatives. This new syndrome, which follows an apparent autosomal recessive pattern, appears to be distinct from known syndromes of secretory diarrhea, myopathy, deafness, microcephaly, and zinc deficiency.