Treatment
Of HypogonadismTreatment is very specific according to the underlying
condition. Here is a treatment summary for the conditions mentioned above:
| Hormone deficiencies
of testicles and ovaries (Treatment details) |
| Primary
hypogonadism | | Condition
| Details of treatment: |
| Klinefelter
syndrome | small amounts of testosterone replacement
prevent atrophy of left temporal lobe (Ref. 6); IVF methods can be used in 40%
of patients to father children with normal chromosomes (Ref.5) |
| Bilateral
anorchia | replacement therapy of testosterone starting
in puberty and slowly increasing over 2 years to adult replacement dosage (injection
or transdermally); laparoscopic exploration (Ref. 7) |
| Congenital adrenal hyperplasia
(CAH) | replacement of glucocorticoid (prednisone or
hydrocortisone orally); fludrocortisone given orally to replace aldosterone; in
female infants reduction clitoroplasty and creation of vaginal opening |
| Turner
syndrome | replacement with female hormones brings
on normal puberty; careful check for mosaics with 45,X and 46,XY who require removal
of gonadal remnants to prevent gonadal malignancies (Ref.8) |
| Secondary hypogonadism |
| Condition | Details
of treatment: | | Panhypopituitarism | treat
underlying pituitary or hypothalamic abnormality; adult males may be treated with
gonadotropins (FSH/LH) to induce spermatogenesis (Ref. 10) |
| Kallman
syndrome | hCG treatment can treat
cryptorchidism and render patients fertile; consider GnRH with portable sc. pump
to achieve normal male appearance and fertility |
Klinefelter
syndrome It is interesting that 40% of patients with Klinefelter
syndrome (according to Ref. 5) have sperm in the testicular tubules and 94% of
these samples checked out genetically normal! In the last view years there are
several groups throughout the word that reported in vitro fertilization (=IVF)
with Klinefelter syndrome sperm resulting in genetically normal babies. There
is still a serious fertility problem in this group of patients, but things are
not as bleak as they were thought of in the past. High-resolution MRI scan studies
with Klinefelter syndrome children in puberty showed that treatment with low dose
testosterone lead to more volume of the left temporal gray matter of the brain.
This correlated with improved verbal and language skill scores when compared to
normal controls and untreated Klinefelter patient controls.
Bilateral
anorchia Patients with this condition have to be carefully
studied with the hCG test to ensure that there is no other cause for the finding
of missing testicles, such as cryptorchidism. If there is even the slightest increase
of testosterone on this test from the prior baseline, the authors of Ref. 7 suggest
that an examination under a general anesthetic and possible laparoscopy should
be done. This study involved more than 900 boys. With early correction of cryptorchidism
malignancy and testicular torsion of the testicles will be prevented.
Congenital
adrenal hyperplasia (=CAH) This condition
can be present in males or females and the basic primary defect is the same, namely
an absence of glucocorticosteroid production in the outer layer of the adrenal
glands as well as an inability to produce aldosterone as well. The pediatrician
or endocrinologist needs to watch these children as over treatment with prednisone
or hydrocortisol leads to Cushing's disease, but undertreatment leads to ACTH
overproduction because of inadequate feedback on the pituitary gland.
This
ACTH overproduction leads to an overproduction of androgens (a group of adrenal
gland hormones with male attributes), which causes virilization and growth abnormalities.
The growth is initially exaggerated, but because of the closing of the growth
plates of the long bones under the influence of androgens later leads to permanent
short stature. For this reason compliance to the regimen of treatment must be
monitored with regular visits to the doctor's office for weight and height measurements.
Growth tables are used to monitor that the growth rate is as close to the normal
curve as possible. At the same time blood tests are taken from time to time to
ensure normal blood hormone levels. Females affected with CAH often need corrective
surgery early on as a baby to do a clitoroplasty and create a vaginal opening.
Later in life further corrective surgery might be necessary. Some psychosexual
counseling may be required. However, these patients will have normal, fulfilled
sexual lives and normal childbearing.
Turner
syndrome Turner syndrome, as mentioned before consists of
a wide spectrum of mosaic Turner syndrome, which is responsible for the wide variation
of symptoms. Hormone studies are used to aid in the decision of how much hormone
replacement would be necessary. The goal is to mimic normal hormone activity and
normal growth. A cardiologist needs to investigate the narrowing at the beginning
of the aorta (medically termed "coarctation of the aorta"), and help
to decide whether or not surgery is required (Ref. 11). In severe cases
of coarctation the newborn becomes blue and needs immediate intubation and oxygen.
Prostaglandin E-1 is given intravenously to reopen the "ductus arteriosus",
which is the artery that during the course of pregnancy bypassed the blood from
the lungs. With the first breath when the lungs expand this bypassing artery closes,
but the normal circulation depends then on a patent aorta. The first step of defense
for a Turner syndrome patient with this malformation of the aorta is to reopen
the ductus arteriosus as stated. At age 5 to 7 (Ref. 11) elective removal of the
stenotic piece of aorta can then be done by the cardiovascular surgeon. Another
area of concern is to spot those patients who are mosaics with a Y-bearing cell
line (for instance 45,x and 46,XY). It is this subpopulation who is at a high
risk for developing a malignancy of the nonfunctioning gonadal tissue, called
"gonadoblastoma". This tissue is removed prophylactically and then the
risk disappears. With hormone replacement and some corrective surgeries, including
some plastic surgery regarding the webbing of the neck, Turner syndrome patients
will be able to lead a normal life. However, infertility in most patients cannot
be avoided.
Panhypopituitarism This
condition needs to be treated according to the underlying pathology. If there
is an underlying tumor in the hypothalamus or in the pituitary gland, appropriate
neurosurgery or radiotherapy may have to be performed . At the same time hormone
replacement is given with either hCG injections or GnRH, if the pituitary function
is intact. It is interesting to note that endurance athletes, like marathon runners
etc., can also have reduced gonadotropin activity, which can lead to low testosterone
levels and infertility due to diminished sperm production (Ref. 9). Other acquired
cases have also been described (Ref. 10) where the pituitary gland no longer produces
enough gonadotropins of its own and replacement hCG by injection has to be given
to bring testosterone and sperm levels up to normal.
Kallman
syndrome This conditionneeds to be treated
with hCG to treat cryptorchidism and render the patients fertile. Alternatively,
as the pituitary FSH and LH cells are functional, GnRH can be given via a portable
pump subcutaneously and programmed in a way how to simulate sporadic release of
gonadotropin hormones from the pituitary gland. In this way normal testicular
hormone production, normal male appearance and normal fertility will occur. Here
is a link
to a Mayo Clinic site with more info on hypogonadism.
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