21-hydroxylase deficiency natural history, complications and prognosis
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Congenital adrenal hyperplasia due to 21-hydroxylase deficiency Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]
Overview
The prognosis of 21-hydroxylase deficient congenital adrenal hyperplasia is usually good, if not treated appropriately, it usually leads to adrenal crisis which is fatal.
Natural History
While a boy (or girl) with simple virilizing congenital adrenal hyperplasia is taller than peers at that point, he will have far fewer years remaining to grow, and may go from being a very tall 7-year-old to a 62-inch 13-year-old who has completed growth.
Complications
Complication associated with 21-hydroxylase deficient congenital adrenal hyperplasia include:
- Adrenal crisis
- Infertility
- Precocious puberty
- Virilization
- Dehydration
Prognosis
Even after diagnosis and initiation of treatment, a small percentage of children and adults with infancy or childhood onset congenital adrenal hyperplasia die of adrenal crisis. Deaths from this are entirely avoidable if the child and his family understand that the daily glucocorticoids cannot be allowed to be interrupted by an illness. When a person is well, missing a dose, or even several doses, may produce little in the way of immediate symptoms. However, glucocorticoid needs are increased during illness and stress, and missed doses during an illness such as the "flu" (or viral gastroenteritis) can lead within hours to reduced blood pressure, shock, and death.