Treatment
8
Prenatal Treatment of Congenital Adrenal Hyperplasia
➤ ES advises that clinicians continue to regard prenatal therapy as
experimental. Thus, ES does not recommend specific treatment protocols.
(UGPS)
➤ In pregnant women at risk for carrying a fetus affected with congenital
adrenal hyperplasia and who are considering prenatal treatment, ES
recommends obtaining prenatal therapy only through protocols approved
by Institutional Review Boards at centers capable of collecting outcomes
from a sufficiently large number of patients, so that risks and benefits can
be defined more precisely. (1|⊕⊕⊕
)
➤ ES advises that research protocols for prenatal therapy include genetic
screening for Y-chromosomal DNA in maternal blood to exclude male
fetuses from potential treatment groups. (UGPS)
Treatment of Classic Congenital Adrenal Hyperplasia
➤ In growing individuals with classic congenital adrenal hyperplasia,
ES recommends maintenance therapy with hydrocortisone. (1|⊕⊕⊕
)
➤ In growing individuals with congenital adrenal hyperplasia, ES
recommends against the use of oral hydrocortisone suspension and
against the chronic use of long-acting potent glucocorticoids. (1|⊕⊕⊕
)
➤ In the newborn and in early infancy, ES recommends adding
fludrocortisone and sodium chloride supplements to the treatment
regimen. (1|⊕⊕⊕
)
➤ In adults with classic congenital adrenal hyperplasia, ES recommends
using daily hydrocortisone and/or long-acting glucocorticoids plus
mineralocorticoids, as clinically indicated. (1|⊕⊕⊕
)
➤ In all individuals with classic congenital adrenal hyperplasia, ES
recommends monitoring for signs of glucocorticoid excess, as well as
for signs of inadequate androgen normalization, to optimize the adrenal
steroid treatment profile. (1|⊕⊕⊕
)
➤ In all individuals with classic congenital adrenal hyperplasia, ES
recommends monitoring for signs of mineralocorticoid deficiency or
excess. (1|⊕⊕⊕
)
Stress Dosing
➤ In all patients with congenital adrenal hyperplasia who require
glucocorticoid treatment, for situations such as febrile illness (>38.5°C),
gastroenteritis with dehydration, major surgery accompanied by
general anesthesia, and major trauma, ES recommends increasing the
glucocorticoid dosage. (1|⊕⊕⊕
)
