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early in the course of the disease the girls are taller and heavier than
their chronologic peers who have not experienced the growth spurt
however, although the patient is tall as a child, her eventual
adult height will be shorter than normal
without therapy, approximately 50% of females with
precocious puberty will not reach a height of 5 feet
Premature Thelarche
COMPREHENSIVE GYNECOLOGY
PRECOCIOUS PUBERTY
COMPREHENSIVE GYNECOLOGY
PRECOCIOUS PUBERTY
thecomas and luteomas are usually much smaller than
granulosa cell tumors and usually cannot be palpated
these tumors are rare during childhood, with only 5% of
granulosa cell tumors and 1% of thecomas occurring before
puberty
infrequently, follicular cysts of the ovary may emerge spontaneously
and secrete enough estrogen to be the cause, rather than the result, of
precocious puberty
the ability of many tumors, including teratomas, choriocarcinomas,
and dysgerminomas, to secrete estrogen, human chorionic
gonadotropin (HCG), -fetoprotein, and other markers has been
established
McCune-Albright Syndrome is a rare triad of cafau-lait spots,
fibrous dysplasia, and cysts of the skull and long bones
a.k.a. polyostotic fibrous dysplasia
these patients also have facial asymmetry
40% of girls with MAS have associated isosexual precocious puberty
adrenocortical neoplasms may produce either isosexual or
heterosexual precocious puberty
the relationship between congenital adrenal hyperplasia and puberty
depends on the time of initial diagnosis and therapy
if the disease is diagnosed in the neonatal period and treated,
normal puberty ensues
if the disease is untreated, the girl over time usually develops
heterosexual precocious puberty (signs of androgen excess)
from the adrenal androgens
if congenital adrenal hyperplasia is diagnosed late in
childhood, isosexual precocious puberty may follow initial
treatment of the adrenal disease
hypothyroidism most commonly is associated with delayed pubertal
development
in RARE cases, untreated hypothyroidism results in isosexual,
GnRH-dependent, or GnRH-independent precocious puberty
the hypothyroidism associated with precocious puberty is due to
primary thyroid insufficiency, usually Hashimoto's thyroiditis, and not
a deficiency in pituitary TSH
pathophysiology: diminished negative feedback of
thyroxine, resulting in an increased production of TSH,
which may be accompanied by an increase in production
of gonadotropins
Diagnosis:
begins with a meticulous history and physical examination
primary emphasis: rule out life-threatening neoplasms of the
ovary, adrenal gland, or CNS
secondary emphasis: delineate the speed of the maturation process,
for this is crucial in making decisions concerning therapy
the height of the girl and the exact stage of pubertal development,
including Tanner stage, should be recorded
a battery of tests, including imaging studies of the brain, serum
estradiol levels, FSH levels, and thyroid function tests, may be needed
to establish the diagnosis
with this acceleration of development, the sex steroids and adrenal
androgen (DHEA-S) are elevated regardless of the cause
acceleration of growth is one of the earliest clinical features of
precocious puberty
bone age should be determined by handwrist films and compared
with standards for a patient's age
usually these films are repeated at 6-month intervals to
evaluate the rate of skeletal maturation and correspondingly
the need for active treatment of the disease
advancement of bone age more than 95% of the norm for the
child's chronologic age is indicative of an estrogen effect
disease of the CNS are suggested during the history by symptoms such
as headaches, seizures, trauma to the head, and encephalitis
these conditions are confirmed or excluded by a series of
tests, including neurologic and ophthalmologic examinations,
EEGs, and brain imaging
serum levels of FSH, LH, prolactin, TSH, E2, testosterone, DHEA or
DHEA-S, HCG, androstenedione, 17-hydroxyprogesterone, T3, and T4
may be of value in establishing the differential diagnosis
sometimes a GnRH stimulation test is diagnostic in differentiating
incomplete from true precocious puberty, but this test does not
specifically identify children with CNS lesions
LH responses to gonadotropin stimulation after reaching a basal level
are similar in cases of true precocious puberty to the responses of a
mature adult. In contrast, a child with precocious puberty secondary
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PRECOCIOUS PUBERTY
to a feminizing ovarian neoplasm does not have a significant elevation
in LH response to exogenous gonadotropins. In summary, a
stimulation test with exogenous GnRH is fundamental in helping to
delineate the underlying pathophysiology
Management:
the treatment of precocious puberty depends on the cause, the extent
and progression of precocious signs, and whether the cause may be
removed operatively
the present drug of choice for GnRH-dependent precocious puberty is
one of the potent GnRH agonists
these drugs are typically given by monthly injections or,
rarely, intranasally
GnRH agonists are safe and effective treatments for children with the
disease second-ary to disturbances in the hypothalamicpituitary
ovarian axis
therapy should be initiated as soon as possible after the
diagnosis is established in order to achieve maximal adult
height.
the effect on adult height depends on the chronologic age at
which therapy is initiated
the therapy is MORE effective in 4- to 6-year-olds
continuous chronic administration of the drug is maintained
until the median age of puberty
medical therapy produces involution of secondary sexual
characteristics, with amenorrhea and regression of both breast
development and amount of pubic hair LH and FSH pulsations are
abolished. Most importantly the drug not only reverses the ovarian
cycle but definitely changes the growth pattern
growth velocity is usually decreased by approximately 50%
patients with McCuneAlbright syndrome may be treated with
aromatase inhibitors, which prevent the conversion to biologically
active estrogens
this treatment leads to diminished circulating estrogen levels,
diminished frequency of menses, and a decreased rate of
growth and skeletal maturation
both the child with precocious puberty and her family need
intensive counseling
COMPREHENSIVE GYNECOLOGY