11
Long-Term Management of Patients With Congenital Adrenal
Hyperplasia
Transition to Adult Care
➤ In adolescent patients with congenital adrenal hyperplasia, ES suggests
that the transition to adult care begins several years prior to dismissal
from pediatric endocrinology. (2|⊕
)
Technical remark: We advise the use of joint clinics comprised of pediatric, reproductive,
and adult endocrinologists and urologist during this transition.
➤ In adolescent females with congenital adrenal hyperplasia, ES suggests a
gynecological history and examination to ensure functional female anatomy
without vaginal stenosis or abnormalities in menstruation. (2|⊕⊕
)
Genetic Counseling
➤ In children with congenital adrenal hyperplasia, adolescents transitioning
to adult care, adults with nonclassic congenital adrenal hyperplasia upon
diagnosis, and partners of patients with congenital adrenal hyperplasia
who are planning a pregnancy, ES recommends that medical professionals
familiar with congenital adrenal hyperplasia provide genetic counseling.
(1|⊕⊕
)
Fertility Counseling
➤ In individuals with congenital adrenal hyperplasia and impaired fertility,
ES suggests referral to a reproductive endocrinologist and/or fertility
specialist. (2|⊕⊕
)
Management of Congenital Adrenal Hyperplasia and Nonclassic
Congenital Adrenal Hyperplasia During Pregnancy
➤ In women with nonclassic congenital adrenal hyperplasia who are infertile
or have a history of prior miscarriage, ES recommends treatment with a
glucocorticoid that does not traverse the placenta. (1|⊕⊕
)
➤ In women with congenital adrenal hyperplasia who are pregnant, ES
advises management by an endocrinologist familiar with congenital
adrenal hyperplasia. (UGPS)
➤ In women with congenital adrenal hyperplasia who become pregnant
ES recommends continued prepregnancy doses of hydrocortisone/
prednisolone and fludrocortisone therapy, with dosage adjustments if
symptoms and signs of glucocorticoid insufficiency occur. (1|⊕⊕
)
Technical remark: Clinicians should evaluate the need for an increase in glucocorticoid
during the second or third trimester and administer stress doses of glucocorticoids during
labor and delivery.
