Download د.هالة عبدالغني الراوي 2017 المرحلة الخامسة Hirsutism Definition:growth

‫هالة عبدالغني الراوي‬.‫د‬
2017
‫المرحلة الخامسة‬
Hirsutism
Definition:growth of terminal hair on the body of a women in the same pattern
sequence as that which develops in the post-pubertal male.
Incidence:
Approximately 9%
Pathogenesis:
The androgen receptors interact with dihydrotestosterone(the active metabolite
of testosterone).This interaction results in an increase in the size of the hair
follicle & the type of hair produced by the follicle.
Causes:
1- Idiopathic 2-polycystic ovary 3-androgen secreting tumor e.g. that of ovary
4-congenital adrenal hyperplasia 5-cushing syndrome
Clinical features:
In order to maintain consistency of diagnosis and to monitor therapeutic
intervention, it is important to have a standard grading system.
The most common is the Ferriman & Gallwey grading system, which scores 11
areas of the body on a scale of 1 to 4 according to the degree of terminal hair
growth. The scores then added together.
•
• Diagnosis:
• Onset of hirsutism
1) Gradual onset of hirsutism is associated with acne, oily skin, weight gain,
and irregular menstrual cycles. This suggests an underlying endocrine
condition, such as polycystic ovary syndrome (PCOS).
2) Abrupt onset or rapidly worsening hirsutism with signs of virilization should
prompt concern for an androgen-producing tumor.
3) Presence or absence of virilization
4) Drug ingestion. Drugs are usually associated with hypertrichosis, but
androgenic drugs (e.g., steroids and phenytoin) may cause hirsutism.
5) Family history. A family history of hirsutism may indicate an inherited
disorder (i.e., familial hypertrichosis).
6) Ethnic background. The pattern of hair growth is genetically predetermined
and is associated with differences in 5α-reductase activity at hair follicles.
7) Local trauma. Changes in skin and hair growth may occur.
8) Regularity of menstrual cycles
9) Patients with regular menstrual cycles and hirsutism often have idiopathic,
ethnic, or familial hirsutism.
10)
Some anovulatory hirsute patients (as many as 40%) appear to have
regular menstrual cycles; thus, testing is necessary to determine whether
ovulation is occurring.
11)
History of infertility
2.Physical examination
The most sensitive marker for increased androgen production is
1.hirsutism. Followed in order by:
2. acne
3.oiliness of the skin,
4. increased libido,
5. clitoromegaly,
5.masculinization.
Abdominal,
Pelvic ex.
For adrenal, ovarian tumors .
Skin ex. for acanthosis nigricans
1.Serum testosterone is a marker of ovarian and adrenal activity.
 Total testosterone levels greater than 200 ng/dL suggest an
androgen-producing tumor. However, 10% to 20% of
patients with androgen-producing tumors may have low
testosterone levels. Imaging is warranted.
 Pelvic ultrasound is best to provide an image of the ovaries.
 Computed tomography or magnetic resonance imaging
views the adrenal glands.
 Elevated total testosterone levels but less than 200 ng/dL
associated with anovulation and hirsutism suggest PCOS.
 Testosterone maybe in the normal range when measured in
patients with PCOS.
 Serum DHEAS is almost exclusively produced by the adrenal
glands and reflects adrenal androgen activity.
 Levels greater than 700 ng/dL suggest an adrenal tumor.
 Moderately elevated DHEAS levels may occur with
anovulation, PCOS, or adrenal hyperplasia.
Normal DHEAS levels indicate that adrenal disease is less probable
and that ovarian androgen production is more likely
Treatment:
An explanation of the condition with a discussion about the expectation of
successful treatment and the length of time that this may require is extremely
important at the first consultation.
Physical methods of hair removal
-Bleaching: Hydrogen peroxide, remove the hair color but occasionally may lead
to discoloration of the skin.
-shaving: this does not affect the rate of hair growth.
-Electrolyis: effective means of permanent hair removal, similarly laser may be
used that generate thermal energy to destroy hair follicles.
-Weight loss. (PCOS)
Pharmacological methods:
1. The oral contraceptive pills: suppress ovarian androgen activity & increase sex
hormone binding globulin, thus decreasing free testosterone ex.Dianette
(combined OCP with the antiandrogen cyproterone acetate)
2. Cyproterone acetate (andrucure): decrease ovarian androgen should be given
with O.C.P.to prevent pregnancy because it is teratogenic.
3. Spironolactone: is an oral aldosterone antagonist with antiandrogenic
properties acts by blocking androgen receptors.
4. Flutamide: antiandrogen that appears to work only at the androgen receptor
.
5. Finasteride: 5α-reductase inhibitor.
Virilism:
Is excessive androgen secretion that causes
1. musculinization of female: hirsuitism, horsness of voice, enlargement or
hypertrophy of clitoris,temporal balding ,increase libido.
2. defeminization:atrpphy of the breast & vagina
Hyperprolactinemia
Is the elevation of prolactine hormone which is secreted
from anterior pituitary gland
Etiology of Hyperprolactinemia :
A-physiological causes :
1- idiopathic 2- pregnancy 3- postpartum 4- sleep
5- stress 6-major surgery 7-intercourse 8-eating
B-Hypothalamic condition.
1-craniopharangioma 2-tuberculosis 3- cystic glyoma 3-radiation 4-sarcoidosis
which damage the pituitary stalk and prevent dopamine-mediated inhibition of
PRL secretion
C-Pituitary condition .
1-acromegaly 2-addisons disease 3-cushing syndrome 4-hypothyrodism 5pituitary adenoma(Prolactin-secreting adenomas) 6-metastatic tumor .
D-metabolic dysfunction
1-hepatic cirrhosis 2-renal failure .
E-Drugs: Prolactin is primarily regulated by tonic dopamine inhibition of secretion.
Prolactin secretion is increased by serotonin, norepinephrine, opioids, estrogen,
Methyl dopa , cimitidine, metochlopromide and TRH. Therefore, medications that
block dopamine receptor action (phenothiazines) or deplete catecholamine levels
(monoamine oxidase inhibitors [MAOIs]) may increase PRL levels
F-Chest wall stimulation :(sukling,Herpes zoster infection,breast examination)
G-Primary hypothyroidism is also associated with mild elevations in serum PRL
levels. Low circulating thyroid hormone levels produce a reflex increase in
hypothalamic TRH levels due to loss of feedback inhibition. As a rule, thyroid
function tests should be measured when confirming a diagnosis of
hyperprolactinemia, as a patient may require thyroid replacement rather than
further evaluation for pituitary adenoma.
Diagnosis of Hyperprolactinemia
Most affected women present with microadenomas and signs of PRL excess such
as galactorrhea and amenorrhea
Review the history, physical examination, excludes the physiological causes, drugs
1-Measurement of serum prolactin levels by radioimmunoassay
PRL levels are drawn in the morning. Prior to testing, breast examination is
avoided to prevent false-positive results. If a mildly elevated PRL level is found,
sampling should be repeated because PRL levels vary throughout the day.
Moreover, many factors including the stress of venipuncture may produce false
elevations.
PRL levels rise nearly 10-fold during pregnancy and make detection of a
prolactinoma difficult at this time.
2-T.S.H.for assessment of thyroid function
3-X ray of the sella tursica to see any destruction by macro adenoma of the
pituitary gland
4-Magnetic resonance imaging is advisable for all patients with confirmed
hyperprolactinemia. Some experts advocate limiting imaging to women with a
PRL level exceeding 100 ng/mL, as lower levels are most likely due to small
microadenomas. Although this is undoubtedly a safe approach in most women,
mildly elevated PRL levels also may be due to pituitary stalk compression by a
non-prolactin-secreting macroadenoma or a craniopharyngioma, which are
diagnoses with potentially severe consequences.
Pituitary Adenomas
Pituitary adenomas are the most common cause of acquired pituitary dysfunction
and comprise approximately 10 percent of all intracranial tumors. Clinically,
symptoms of galactorrhea, menstrual disturbances, or infertility may lead to its
diagnosis. Most tumors are benign, with only an estimated 0.1 percent of
adenomas developing into frank carcinoma with metastasis Nevertheless,
pituitary adenomas may cause striking abnormalities in both endocrine and
nervous system function
Tumors are now classified by their hormonal expression pattern as determined by
immunohistochemistry. Adenomas are further grouped by size into
microadenomas (<10 mm in diameter) and macroadenomas (>10 mm in
diameter).
Most adenomas secrete PRL, however, adenomas may secrete any of the pituitary
hormones either as a single hormone (monohormonal adenoma) or in
combinations (multihormonal adenoma)
Pituitary Adenoma Symptoms
Endocrinopathy
Pituitary adenomas may cause symptoms via excess hormone secretion and lead
to clinical conditions such as hyperprolactinemia, acromegaly, or Cushing disease.
Alternatively, adenomas may result in hormone deficiency due to damage of
other pituitary cell types or of the pituitary stalk by an expanding adenoma or
following treatment of the primary lesion.
The anterior pituitary gland borders both the optic chiasm and cavernous sinus.
Disruption of the optic chiasm by suprasellar growth of the pituitary mass may
present as bitemporal hemianopia in which patients lose of the outer portion of
the right and left visual fields
Reproductive Effects
Any pituitary mass or infiltrate can present as an abnormality in reproductive
function that may include delayed puberty, anovulation, oligomenorrhea, and
infertility. Hyperprolactinemia results in a reflex increase in central dopamine.
Stimulation of the dopaminergic receptors on the GnRH neurons alters GnRH
pulsatility, thereby disrupting folliculogenesis.
Treatment of Hyperprolactinemia
Treatment of hyperprolactinemia should begin with treatment of the underlying
cause. For example, medications should be changed, if possible, or
hypothyroidism should be treated. The management of pituitary adenomas
includes dopamine agonists, such as bromocriptine. Bromocriptine therapy can be
reasonable instituted in a woman with a mildly elevated prolactin level and a
normal pituitary on imaging. These patients can be presumed to have a small
microadenoma.
Specifically, for those with prolactin-secreting adenomas, most tumors grow
slowly, and asymptomatic patients with a microprolactinoma may be managed
conservatively with serial MR imaging and serum PRL levels every 1 to 2 years, as
the risk of progression to a macroadenoma is less than 10 percent .These women
should be followed for even mild changes in menstrual cyclicity, as they are at risk
for developing hypoestrogenism and resultant risk for osteopenia or osteoporosis
When tumors of any size are associated with symptoms of amenorrhea or
galactorrhea, therapy should be considered .Neurosurgical evaluation is
mandatory when visual field defects or severe headaches are present. In general,
first-line treatment is medical for both micro- and macroadenomas. Specifically,
women should receive a dopamine agonist such as the nonspecific dopamine
receptor agonist, bromocriptine (Parlodel), or the dopamine receptor type 2
agonist, cabergoline (Dostinex).
These dopamine agonists decrease PRL secretion and shrink tumor size. However,
bromocriptine treatment is associated with a number of common side effects
including headache, postural hypotension, blurred vision, drowsiness, and leg
cramps. Cabergoline treatment is generally better tolerated than bromocriptine.
Cabergoline also has a longer half-life than bromocriptine, allowing once- or
twice-weekly dosing compared with the multiple daily doses that may be required
for bromocriptine. Typical initial cabergoline dosages are 0.25 mg orally twice
weekly. Cabergoline has been found to be more effective than bromocriptine in
normalizing PRL levels but, cabergoline is expensive. Most patients can tolerate
bromocriptine if started at a low dose—½ tablet or 0.125 mg—each night to
minimize associated nausea and dizziness. This dose can be slowly increased to
three times daily as tolerated. Reliable measurement of posttreatment serum
prolactin levels can be obtained 1 month following a steady medication dose.
Surgery: Neurosurgery is required for refractory tumors or those causing acutely
worsening symptoms. Radiation therapy may be used for patients with surgically
nonresectable or persistent tumors.
Gene therapy has been proposed as a treatment for pituitary tumors