USMLE Step 2 CK Lecture Notes 2019: Obstetrics/Gynecology (Kaplan Test Prep)

GYN TRIAD

Congenital  Adrenal Hyperplasia 21-OH   Deficiency

Congenital adrenal hyperplasia (21-hydroxylase deficiency): typically the
onset has been gradual in the second or early third decade of life and is
associated with menstrual irregularities and anovulation. Precocious puberty
with short stature is common. Family history may be positive. Late-onset CAH
is one of the most common autosomal recessive genetic disorders.

Gradual-onset   hirsutism

Normal  exam

↑   17-OH   progesterone

Examination.    Physical    examination will    show    evidence    of  hirsutism   without

virilization.   Pelvic  examination is  unremarkable.

Laboratory  tests.  Serum   17-OH   progesterone    level   is  markedly    elevated.

Management. Continuous  corticosteroid  replacement to  arrest  the signs   of

androgenicity   and restore ovulatory   cycles.