Understanding klinefelter syndrome - lawley basecamp for

Understanding klinefelter syndrome
Hormone Solutions

Table of Contents
What is Klinefelter Syndrome? 1
How can I detect it? 1
Babies and Toddlers 1
School-age Boys 2
Puberty 3
When should I tell my son he has Klinefelter syndrome? 3
Adult Men 4
Klinefelter Facts 4
What are the variants? 4
Statistics 5
What causes Klinefelter syndrome? 5
Who discovered the syndrome? 5
What are my treatment options? 6
Specialists 8
Role of Testosterone in Humans 9
Riding the Lifecycle 10
Lowering Testosterone 10
Other Benefits of Testosterone 11
Homeopathic Treatments 12
Potential Risks of Testosterone Treatment 12
Contraindications 15
Precautions 16
What can we expect when we visit the doctor? 16
Which testosterone route is best for me? 17
About Lawley Pharmaceuticals 20
Our Mission Statement 20
Completed Clinical Studies 21
Recommended Reading for Patients 22
References for Medical Professionals 23
Glossary 24
Internet Education Reference Sites 26

What is Klinefelter Syndrome?
Klinefelter syndrome is the most common cause of male infertility. It is a
sex chromosome variation that occurs in 1 in 500 males. Most men do not
demonstrate symptoms. Normally, males have one X chromosome in their
cells. Males affected with Klinefelter syndrome have at least one extra X
chromosome in most of their cells. The extra chromosome impacts their:
• Bone strength • Language development
• Breast size • Learning
• Energy level • Muscle mass
• Hair growth • Social development
• Height
• Hip girth
• Thought processes
Not all men with KLINEFELTER SYNDROME are 100% infertile.
ICSI can produce a child – see page 9.
Klinefelter syndrome is also known as XXY syndrome.
How can I detect it?
Babies and Toddlers
A geneticist can diagnose Klinefelter syndrome before your son is born
through a fetal cytogenetic analysis.
Your pediatrician may suspect Klinefelter syndrome if your infant son has:
• Developmental delay
• Undescended testicles (cryptorchidism)
• Pea-sized testicles
• Hypospadias (urine dribbles out of an opening on the
underside of his penis)
However, 75% of boys with Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
1

School-age What is Klinefelter Boys Syndrome?
Your Klinefelter pediatrician syndrome may is investigate the most common your school-age cause of male infertility. It is a
son sex chromosome for Klinefelter variation syndrome that if he occurs has this in 1 in 500 males. Most men do not
characteristic demonstrate symptoms. appearance: Normally, males have one X chromosome in their
• cells. Tall Males statureaffected
with Klinefelter syndrome have at least one extra X
• chromosome Knock-knees in (genu most of valgum) their cells. The extra chromosome impacts their:
• High • Bone arches strength (pes cavus) • Language development
• Protruding • Breast size lips • Learning
• Projecting • Energy level jaw (prognathism) • Muscle mass
• Very • Hair widely-spaced growth eyes • Social (hypertelorism) development
• If • he Height is Caucasian or Black, • Thought epicanthal processes skin folds
on • Hip his upper girth eyelids at the inner corner,
giving him an oriental look
• Not Poor all coordination men with KLINEFELTER SYNDROME are 100% infertile.
• Fused bones in ICSI his can forearms produce that make a child it – see page 9.
diffi cult for him to rotate his arms
Klinefelter (radioulnar syndrome synostosis) is also known as XXY syndrome.
• Quiet, tractable personality with occasional tantrums
and aggression
How can I detect it?
You Babies may and ask your Toddlers pediatrician to investigate your son for Klinefelter
syndrome A geneticist if he can has: diagnose Klinefelter syndrome before your son is born
• through Attention a fetal Defi cytogenetic cit Disorder analysis. (ADD)
• Your Learning pediatrician disability may (especially suspect Klinefelter dyslexia, syndrome reading diffi if culty, your infant and data son has:
retrieval problems)
• Developmental delay
• Mental retardation (IQ drops 15 points for each additional X
• Undescended testicles (cryptorchidism)
chromosome)
• Pea-sized testicles
• Poor expressive and receptive language skills
• Hypospadias (urine dribbles out of an opening on the
• Poor short term memory
underside of his penis)
• Osteoporosis (bone thinning)
•
However,
Lack of
75%
sporting
of boys
ability
with Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
2
1

Puberty What Pubertyis
Klinefelter Syndrome?
Your Klinefelter Your doctor syndrome may suspect is the Klinefelter most common syndrome
cause of male infertility. It is a
when sex when chromosome your boy reaches variation puberty that occurs if he has:
in 1 in 500 males. Most men do not
• demonstrate • High-pitched symptoms. voice
Normally, males have one X chromosome in their
• cells. • Sparse Males beard affected and body with Klinefelter hair
syndrome have at least one extra X
• chromosome • Gynecomastia in most (enlarged of their breasts)
cells. The extra chromosome impacts their:
• Feminine • Bone strength fat distribution
• Language development
• Taurodontism • Breast size (enlarged • Learning molar teeth)
• Energy level • Muscle mass
• Breast cancer or germ cell tumors from
• Hair growth • Social development
elevated estradiol
• Height • Thought processes
If your doctor
• Hip doctor suspects
girthsuspects
your son has the androgen
deficiency defi ciency that accompanies Klinefelter syndrome,
he will likely be sent to a lab for these blood tests:
Not all men with KLINEFELTER SYNDROME are 100% infertile.
• FSH (follicle stimulating hormone)
ICSI can produce hormone) a child – see page 9.
• LH (luteinizing hormone)
•
Klinefelter • Estradiol
syndrome is also known as XXY syndrome.
• Testosterone
You How You may can ask your I detect doctor to it? perform genetic screening for
Klinefelter Babies Klinefelter and syndrome Toddlers if your son exhibits:
• A • geneticist Inability to can deal diagnose with stress
Klinefelter syndrome before your son is born
• through • Psychological a fetal cytogenetic problems, like analysis. anxiety, neurosis, depression,
Your
or psychosis
pediatrician
psychosis
may suspect Klinefelter syndrome if your infant son has:
When • When Developmental should delay I tell my son he has
Klinefelter • Klinefelter Undescended syndrome?
testicles (cryptorchidism)
• Pea-sized testicles
Experts recommend explaining to your affected child that he has
• Hypospadias
recommend
(urine
explaining
dribbles out
to your
of an
affected
opening
child
on the
that he has
Klinefelter syndrome when he reaches his mid-to-late teens. He should be
underside
syndrome
of his penis)
when he reaches his mid-to-late teens. He should be
old enough by then to understand its implications. Give him this booklet.
Knowing
However, Knowing that 75%
there of boys
is hope with
may Klinefelter
help him syndrome
to deal develop
with his symptoms.
normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
13
3

Adult What Men
is Klinefelter Syndrome?
Most Klinefelter men with syndrome Klinefelter is the syndrome most common do not cause exhibit
of male infertility. It is a
symptoms. sex chromosome You may variation first fi rst notice that occurs a problem in 1 when in when 500 males. Most men do not
you demonstrate experience:
symptoms. Normally, males have one X chromosome in their
• cells. Lack Males of libido
affected with Klinefelter syndrome have at least one extra X
• chromosome Sexual Dysfunction in most of (SD)
their cells. The extra chromosome impacts their:
• Infertility
• Bone strength • Language development
• Varicose • Breast veins size that tend • Learning to ulcerate or clot
(thrombosis)
• Energy level • Muscle mass
• Mitral • Hair valve growth prolapse
• Social development
• Poor • Height self-esteem
• Thought processes
• Psychological • Hip girth
• Psychological distress
Klinefelter Facts
Not all men with KLINEFELTER SYNDROME are 100% infertile.
• Girls do not develop ICSI can Klinefelter produce syndrome
a child – see page 9.
• It does not occur more in one race than
Klinefelter any other
syndrome is also known as XXY syndrome.
• • Klinefelter syndrome does not decrease the longevity of boys
• How Most can males I with detect the syndrome it? are undiagnosed until adulthood,
when they have reproductive problems
Babies when they and have Toddlers reproductive problems
• Older mothers tend to produce more Klinefelter syndrome babies
A geneticist Older mothers can diagnose tend to produce Klinefelter more syndrome Klinefelter before syndrome your son babies is born
through a fetal cytogenetic analysis.
What are the variants?
• Your
80% pediatrician
– 90% of affected may suspect
boys Klinefelter
have 47,XXY syndrome
(one additional if your infant
X
son has:
• chromosome). Developmental Most delayboys
boys with the 47,XXY karyotype have
• normal Undescended intelligence.
testicles (cryptorchidism)
• 10% Pea-sized have testicles mosaicism (46,XY/47,XXY). Men with Klinefelter
• syndrome Hypospadias mosaicism (urine dribbles are often out fertile of an and opening can father on the a child
through underside modern of his penis) technology.
• However, Very rare 75% and of more boys serious with Klinefelter variants are syndrome 48,XXYY; develop 48,XXXY; normal 49,XXXYY;
height
and 49,XXXXY; weight until 47,X,i(Xq)Y they reach and age 47,X,del(X)Y). 4 or 5. In 25% Boys of with boys, these there rare is a variants
telltale
condition have mental called retardation clinodactyly, that where increases the little with fi more nger curves X chromosomes.
toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
4
1

What Statistics is Klinefelter Syndrome?
Klinefelter • Most males syndrome who are is infertile the most and common have small cause testicles of male (grape infertility. size) It is a
sex due chromosome to a chromosomal variation disorder that occurs have in Klinefelter 1 in 500 males. syndrome Most with men a do not
demonstrate 47,XXY karyotype symptoms. (extra Normally, X and Y males chromosomes) have one or X a chromosome variant. in their
cells. • In Australia, Males affected 1 in 650 with males Klinefelter is born syndrome with Klinefelter have at syndrome. least one In extra the X
chromosome U.S.A., 1 in in 500-1,000 most of their males cells. has The an extra sex chromosome chromosome. impacts Klinefelter their:
syndrome • Bone strength is signifi cantly • Language under-diagnosed development in the general population.
If • your Breast healthcare size practitioner • Learning is unfamiliar with the fi ner details
of • Klinefelter Energy level syndrome, • Muscle there mass is a list of references at the end of
this • Hair booklet. growth • Social development
• Height • Thought processes
What causes Klinefelter syndrome?
• Hip girth
Klinefelter syndrome occurs after the mother’s egg is fertilized.
Chromosome
Not all men
pairs
with
are
KLINEFELTER
supposed to separate,
SYNDROME
so that
are
two
100%
daughter
infertile.
cells receive one
ICSI
chromosome
can produce
each.
a
In
child
Klinefelter
– see page
syndrome,
9.
the pair
does not disjoin (separate). Both of the chromosomes in the pair go
to one daughter cell. The other daughter cell receives none.
Klinefelter
This problem
syndrome
happens
is
more
also known
frequently
as XXY
in older
syndrome.
mothers, and is
called meiotic nondisjunction.
How can I detect it?
As a result of this genetic problem, the Klinefelter syndrome baby
Babies develops and an abnormal Toddlers pituitary gland, testicles, and hypothalamus
A portion geneticist of the can brain. diagnose The boy’s Klinefelter testicles syndrome degenerate, before so your they son cannot is born
through produce a enough fetal cytogenetic sperm in later analysis. life. Healthy tissue is replaced by clear,
Your
glassy
pediatrician
collagen fi bers,
may suspect
called hyaline.
Klinefelter
The
syndrome
boy has scar
if your
tissue
infant
(fi brosis)
son has:
in
his seminiferous tubules, where his sperm form. Feminine breasts develop
•
in
Developmental
late puberty for
delay
50% of Klinefelter syndrome adolescents. Urine tests
• show Undescended elevated gonadotropin testicles (cryptorchidism)
levels, which mean the male is sterile.
• Psychosocial Pea-sized testicles problems and low self-esteem result from the feminizing
• effects Hypospadias of the hormone (urine dribbles estradiol. out of an opening on the
underside of his penis)
However, Who discovered 75% of boys with the Klinefelter syndrome? syndrome develop normal height
and Dr. Harry weight Klinefelter until they discovered reach age the 4 or syndrome 5. In 25% while of boys, working there with is a telltale nine
condition male patients called at clinodactyly, Massachusetts where General the little Hospital fi nger in curves Boston toward in 1942. the ring
fi The nger actual because genetics the middle were worked bone is out wedge-shaped later, from 1956 instead – 1959 of rectangular. by
Drs. Joe Hin Tjio, Albert Lavan, and Patricia Jacobs.
15
5

6 6
What What are is are Klinefelter my treatment Syndrome? options?
Boys Klinefelter Boys with Klinefelter syndrome Klinefelter syndrome is syndrome the most do common do not not need cause a a special of male diet, infertility. or or to to It is a
restrict sex restrict chromosome their activities. variation Hospital that care occurs care is is not in not 1 required. in required. 500 males. Treatment Most men is is do not
on demonstrate on an an out-patient symptoms. basis.
Normally, males have one X chromosome in their
Testosterone cells. Testosterone Males affected is is the the treatment with Klinefelter of of choice syndrome for for Klinefelter have at syndrome. least syndrome. one extra X
Testosterone chromosome Testosterone can in can most reduce of their the the gonadotropin cells. gonadotropin The extra level chromosome to to high normal.
impacts their:
Gradually, • Bone testosterone strength testosterone • will Language will virilize the development
the boy, giving him male secondary sex sex
characteristics, • Breast size like like a a beard, • Learning body hair, and a a male-pattern fat fat distribution.
When • Energy your son level son is is 11 11 or or • 12 Muscle 12 years old, mass old, the the doctor will will commence
testosterone • Hair growth supplementation. • Social Testosterone development
Testosterone treatment options include
injections, • Height gels gels and and scrotal • Thought cream.
processes
• Hip girth
Common testosterone injections options include:
Not Drug all name men with KLINEFELTER SYNDROME Trade Nameare
100% infertile.
ICSI can produce a child – see page 9.
Testosterone enanthate Delatestryl ® Primoteston ® Drug name Trade Name
Testosterone enanthate Delatestryl Depot
® Primoteston ® Depot
Klinefelter syndrome is also known as XXY syndrome.
How can I detect it?
Babies and Toddlers
A geneticist can diagnose Klinefelter syndrome before your son is born
through a fetal cytogenetic analysis.
Testosterone injection treatment
(using short-acting injections)
Your pediatrician may suspect Klinefelter
usually begins
syndrome
with
if
a
your
50mg
infant
dose
son has:
• Developmental delay on a monthly basis.
• Undescended testicles (cryptorchidism)
• Pea-sized testicles
Transdermal gels (1% testosterone gel
• Hypospadias (urine dribbles out AndroGel of an opening on the
underside of his penis)
However, 75% of boys with Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
® , Testogel ® or Testim ® ) can
be applied daily to the arms, chest,
back and shoulders.
Testosterone cream (Andromen ® 2% and Andromen ® Testosterone esters Sustanon
Forte 5%) can be
applied either transdermally, but preferably scrotally once daily.
®
Testosterone cyprionate Depo-Testosterone
Testosterone undeconate
Reandron
(slow release)
® , Nebido ®
Testosterone esters Sustanon
Not available in the USA
®
Testosterone cyprionate Depo-Testosterone
Testosterone undeconate
Reandron
(slow release)
® , Nebido ®
Not available in the USA
Testosterone injection treatment
(using short-acting injections)
usually begins with a 50mg dose
on a monthly basis.
Transdermal gels (1% testosterone gel
AndroGel ® , Testogel ® or Testim ® ) can
be applied daily to the arms, chest,
back and shoulders.
Testosterone cream (AndroForte ® 2 and AndroForte ® 5) can be
applied either transdermally, but preferably scrotally once daily.
1

What is Klinefelter Syndrome?
Klinefelter syndrome is the most common cause of male infertility. It is a
sex chromosome variation that occurs in 1 in 500 males. Most men do not
demonstrate symptoms. Normally, males have one X chromosome in their
cells. Males affected with Klinefelter syndrome have at least one extra X
chromosome in most of their cells. The extra chromosome impacts their:
• Bone strength • Language development
• Breast size • Learning
• Energy level • Muscle mass
• Hair growth • Social development
The
•
doctor
Height
will closely monitor
• Thought
your
processes
son’s growth and the development
of male
• Hip
secondary
girth
sex characteristics. Your son will need to have blood
drawn to check the gonadotropin hormone levels.
In Not years all past, men adult with males KLINEFELTER with Klinefelter SYNDROME syndrome visited are 100% the doctor infertile.
every two or three ICSI weeks can to produce receive a an child intramuscular – see page injection 9. of 200 – 250
milligrams of testosterone enanthate, esters or cyprionate or at 6-monthly
intervals for 600 –1000 milligrams testosterone implants.
Klinefelter syndrome is also known as XXY syndrome.
More recently testosterone gels (AndroGel
How can I detect it?
Babies and Toddlers
A geneticist can diagnose Klinefelter syndrome before your son is born
through a fetal cytogenetic analysis.
Your pediatrician may suspect Klinefelter syndrome if your infant son has:
• Developmental delay
• Undescended testicles (cryptorchidism)
• Pea-sized testicles
• Hypospadias (urine dribbles out of an opening on the
underside of his penis)
However, 75% of boys with Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
® , Testogel ® and Testim ® )
have allowed patients to self administer their testosterone requirements.
Testosterone gels are applied to the body - abdomen, chest, shoulders
and arms.
AndroForte ® 2 and AndroForte ® lethargy, loss of motivation, decreased muscle mass and strength,
depression, irritability and mood changes.
Applied topically to the skin, Andromen
5 scrotal testosterone creams are
the most recent and user friendly advance in testosterone administration.
Scrotal skin is significantly more receptive to testosterone absorption due
to its high blood flow, thin skin and low fat content. Testosterone cream
applied to the scrotum achieves significantly higher testosterone blood
levels than the equivalent amount of testosterone applied to other areas
of the body. This represents signifi cant cost savings to patients. Because
testosterone gels are alcohol based they cannot be applied scrotally due
to the sensitivity of scrotal skin.
Unlike intramuscular injections and implants, the cream is painless.
The daily dose you receive with cream is even. Injections produce
uneven testosterone blood levels because they wear off and have to be
replenished every 7 – 22 days.
® Forte and Andromen ®
Testosterone Creams for men are the world’s only clinically trialed
and tested pharmaceutical grade testosterone creams using natural
bio-identical testosterone. Andromen ® Forte and Andromen ® Testosterone
Creams are listed with the Australian government (AUST L 95334 / L 70886).
Andromen ® Prescribing Information and Consumer Medicine Information
can be downloaded from http://www.hormonesolutions.com.au
(or by clicking on the link).
The doctor will closely monitor your son’s growth and the development
of What male secondary can we sex expect characteristics. when Your we son visit will need the to doctor? have blood
drawn to check the gonadotropin hormone levels.
All visits begin with the same three steps, and then vary according to your
In reason years for past, attending adult males the doctor: with Klinefelter syndrome visited the doctor
every two or three weeks to receive an intramuscular injection of 200 – 250
1. Your drug dose is calculated based on your size, so your nurse records
milligrams of testosterone enanthate, esters or cyprionate or at 6-monthly
your height and weight. Your nurse records baseline readings of your
intervals for 600 –1000 milligrams testosterone implants.
vital signs (temperature, pulse and respiration rates, and blood pressure)
More for comparison recently testosterone to your results gels (AndroGel once your treatment is underway, so that
complications such as hypertension can be detected early if they occur.
2. Your doctor takes a history. Expect many personal questions about
your health and sex life, or that of your child. Your doctor will enquire
about genetic disease in your family. Your doctor will ask you to list
previous illnesses, drugs (prescribed and street drugs for recreational
use), and stressors.
3. Your doctor next performs a physical examination. Your doctor will feel
(palpate) your groin and lymph nodes for testicular cancer and perform
a digital rectal exam (DRE) to check for prostate cancer. If the doctor
feels a swelling in your testicles, he or she will shine a light through your
scrotum to determine if it is possibly a benign (non-cancerous) cyst.
® , Testogel ® and Testim ® )
have allowed patients to self administer their testosterone requirements.
Testosterone gels are applied to the body - abdomen, chest, shoulders
and arms.
AndroForte ® 2 and AndroForte ® 5 scrotal testosterone creams are
the most recent and user friendly advance in testosterone administration.
Scrotal skin is signifi cantly more receptive to testosterone absorption due
to its high blood fl ow, thin skin and low fat content. Testosterone cream
applied to the scrotum achieves signifi cantly higher testosterone blood
levels than the equivalent amount of testosterone applied to other areas
of the body. This represents signifi cant cost savings to patients. Because
testosterone gels are alcohol based they cannot be applied scrotally due
to the sensitivity of scrotal skin.
Unlike intramuscular injections and implants, the cream is painless.
The daily dose you receive with cream is even. Injections produce
uneven testosterone blood levels because they wear off and have to be
replenished every 7 – 22 days.
117
77

Specialists What is Klinefelter Syndrome?
You Klinefelter and your syndrome child can is also the most benefi common t from consulting cause of male with infertility. these specialists: It is a
sex Geneticist: chromosome A geneticist variation can that diagnose occurs in Klinefelter 1 in 500 males. syndrome Most before men do not
demonstrate your son is symptoms. born through Normally, a fetal cytogenetic males have analysis. one X chromosome A genetic in their
cells. counselor Males affected may help with you Klinefelter explain Klinefelter syndrome syndrome have at least thoroughly one extra to X
chromosome your affected in son. most of their cells. The extra chromosome impacts their:
• Bone strength • Language development
Endocrinologist: A hormone specialist can regularly monitor
• Breast size • Learning
the effectiveness of the testosterone replacement therapy. The
• Energy level • Muscle mass
endocrinologist will order blood tests for testosterone, FSH, LH, and
• Hair growth • Social development
estradiol. The endocrinologist may order an echocardiogram for mitral
• Height • Thought processes
valve prolapse, x-rays, and a bone density test for osteoporosis.
• Hip girth
Physiotherapist (PT): Your affected son may have weak, fl accid muscles
and Not slow all men refl exes with (hypotonia). KLINEFELTER Klinefelter SYNDROME syndrome are could 100% make infertile. him
clumsy, unbalanced, ICSI can uncoordinated, produce a child and with – see poor page posture. 9. Ask your
family physician to refer you to a physiotherapist familiar with
Klinefelter
Klinefelter
syndrome
syndrome.
is also known as XXY syndrome.
Speech therapist: Your son may require help to understand complex
How language can before I detect he starts it? school. Ask your family physician for a
referral to a speech therapist.
Babies and Toddlers
A geneticist Occupational can diagnose Therapist Klinefelter (OT): Your syndrome son may have before motor your dyspraxia, son is born
through a nervous a fetal system cytogenetic disorder analysis. where he has diffi culty planning and
Your executing pediatrician complex may movements suspect Klinefelter and tasks. syndrome Obsolete if your terms infant for dyspraxia son has:
are clumsy child syndrome, congenital maladroitness, and sensory
• Developmental delay
integration disorder. Dyspraxia often co-occurs with learning disabilities,
•
dyslexia,
Undescended
and attention
testicles
defi
(cryptorchidism)
cit disorder. An Occupational Therapist (OT)
• can Pea-sized train your testicles son to appear less clumsy. The OT can fi t your child with
• small, Hypospadias inconspicuous, (urine dribbles and inexpensive out of an opening assistive on devices, the like pen grips.
underside of his penis)
Psychologist: Enlarged breasts place psychological stress on
However, 75% of boys with Klinefelter syndrome develop normal height
affected men, so seek help from a psychologist familiar with Klinefelter
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
syndrome. Visit The Australian Psychological Society at www.psychology.
condition called clinodactyly, where the little fi nger curves toward the ring
org.au to fi nd a local psychologist suitable for your needs. The American
fi nger because the middle bone is wedge-shaped instead of rectangular.
Psychological Association at www.apa.org offers a similar service.
8 1

What is Klinefelter Syndrome?
Special Ed: Get a thorough psychoeducational examination through
your son’s school. The written evaluation you will receive from
Klinefelter syndrome is the most common cause of male infertility. It is a
the Special Education Department lists your son’s strengths and
sex chromosome variation that occurs in 1 in 500 males. Most men do not
weaknesses, and recommends an appropriate classroom placement.
demonstrate symptoms. Normally, males have one X chromosome in their
A psychoeducational exam will list additional resources available in
cells. Males affected with Klinefelter syndrome have at least one extra X
your area, so you can tailor your son’s education.
chromosome in most of their cells. The extra chromosome impacts their:
Surgeon: • Bone strength You may want • Language to consult development a surgeon about mastectomy
(breast • Breast removal) size or breast • Learning reduction. Around 10% of XXY males have
breast • Energy enlargement level great • Muscle enough mass to require surgery. Gynecomastia
increases • Hair growth the chance • of Social breast development
cancer.
Fertility • Height Experts: Not • all Thought men with processes Klinefelter syndrome are infertile.
Some • Hip have girtholigospermia
(low sperm production). If you wish to father
a child and have a low sperm count, a fertility expert may be able to
extract Not all sperm men with directly KLINEFELTER from your testicles SYNDROME during a are biopsy, 100% choose infertile. one
that is viable, and ICSI inject can produce it into a woman’s a child – egg. see This page process 9. is called
ICSI (intracytoplasmic sperm injection). The resulting child will not have
Klinefelter a risk of developing syndrome is Klinefelter also known syndrome as XXY syndrome. above that of the general
population. If the specialist fi nds more than one viable sperm, you
How may choose can I to detect have them it? frozen for future pregnancies. To date, more
than 60 children have been born to Klinefelter men around the world
Babies through and ICSI. Toddlers
A geneticist can diagnose Klinefelter syndrome before your son is born
through a fetal cytogenetic analysis.
Role of Testosterone in Humans
Your pediatrician may suspect Klinefelter syndrome if your infant son has:
Natural testosterone is a steroid hormone, normally produced by the
Leydig
• Developmental
cells in the
delay
testes of humans and animals. Females produce
far • Undescended less testosterone testicles in their (cryptorchidism)
ovaries than males do in their testicles.
The • Pea-sized small amount testicles of testosterone present in females does not have a
masculinizing • Hypospadias effect (urine on dribbles them. Testosterone out of an opening increases on the libido and affects
mood underside in both of sexes. his penis)
Testosterone However, 75% is of classifi boys ed with as Klinefelter an androgen syndrome (masculinizing develop substance). normal height
Androgens and weight control until they masculine reach age secondary 4 or 5. In sex 25% characteristics, of boys, there like is a male telltale
hair condition growth called patterns clinodactyly, (beard, armpits, where the chest little and fi nger groin), curves deep toward voice, the and ring
male fi nger fat because distribution. the middle Testosterone bone is is wedge-shaped crucial for the instead development of rectangular. and
maintenance of the male sex organs (testes and penis).
9
1

10
What is Klinefelter Syndrome?
Testosterone is also an anabolic, meaning it encourages bulky, strong
muscle growth. Testosterone has systemic anabolic effects. It infl uences
Klinefelter syndrome is the most common cause of male infertility. It is a
fluid balance by making the male retain electrolytes (sodium, potassium,
sex chromosome variation that occurs in 1 in 500 males. Most men do not
and chloride), water, and nitrogen. Testosterone infl uences bone growth
demonstrate symptoms. Normally, males have one X chromosome in their
by encouraging the retention of calcium and phosphate. Testosterone
cells. Males affected with Klinefelter syndrome have at least one extra X
makes the skin more vascular and less fatty.
chromosome in most of their cells. The extra chromosome impacts their:
Riding the Lifecycle
• Bone strength • Language development
Testosterone • Breast size production • Learning increases when a boy enters puberty.
Testosterone • Energy level production • Muscle decreases mass when a man turns 50. A good
testosterone • Hair growth target range • Social for an development
adult Klinefelter man to maintain is
300 • – Height 1,000 nanograms • per Thought deciliter processes (ng/dl) of blood serum
(or 10.5 • Hip – girth 35 nmol/L). Ideally the level should be greater than 500 ng/dl.
Applying 1 gram (50mg testosterone) of AndroForte
Not all men with KLINEFELTER SYNDROME are 100% infertile.
ICSI can produce a child – see page 9.
Klinefelter syndrome is also known as XXY syndrome.
How can I detect it?
Babies and Toddlers
A geneticist can diagnose Klinefelter syndrome before your son is born
through a fetal cytogenetic analysis.
Your pediatrician may suspect Klinefelter syndrome if your infant son has:
• Developmental delay
• Undescended testicles (cryptorchidism)
• Pea-sized testicles
• Hypospadias (urine dribbles out of an opening on the
underside of his penis)
However, 75% of boys with Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
® 5 natural
testosterone cream every night to your scrotum will help maintain this
target range. Allow the cream to absorb into the skin before dressing.
Wash your hands well with soapy water after use.
Lowering Testosterone
You may inadvertently lower your testosterone level by consuming foods
containing too much protein and too few carbohydrates. If you go on a
fad diet with too many carbs and too little fat, it can deplete testosterone.
Other factors, separate to Klinefelter Syndrome that may lower
testosterone levels include:
• Acute critical illness, burns, major trauma or surgery
• Drug use (e.g., opiates, glucocorticoids, anabolic steroids,
some anticonvulsants)
• Chronic disease and its treatment
• Alcohol abuse
• Smoking
• Ageing
Most of the above cause an increase in Sex Hormone Binding Globulin
(SHBG). SHBG is a transporter protein found in the blood. It acts as a
1

What carrier to is move Klinefelter hormones around Syndrome? the body.
Klinefelter Up to 99% syndrome of testosterone is the produced most common is bound cause to of SHBG. male Once infertility. bound It is to a
sex SHBG chromosome the testosterone variation is inactive. that occurs in 1 in 500 males. Most men do not
demonstrate Testosterone symptoms. to which SHBG Normally, does not males attach have is one the X biologically chromosome in their
cells. available Males testosterone affected with that Klinefelter is free to act syndrome on cells have throughout at least the one body extra X
chromosome (free testosterone). in most of their cells. The extra chromosome impacts their:
You • can Bone marginally strength increase • Language your testosterone development level with exercise.
To produce • Breast enough size testosterone, • Learning your body requires the:
• Minerals • Energy boron level and zinc • Muscle mass
• Vitamins
• Hair growth
A, B6, and C
• Social development
• Height • Thought processes
• Branched Chain Amino-acids (BCAA) valine, isoleucine, and leucine
• Hip girth
Other Benefi ts of Testosterone
Not all men with KLINEFELTER SYNDROME are 100% infertile.
Testosterone supplementation helps boys and men with Klinefelter
ICSI can produce a child – see page 9.
syndrome immensely, but it also benefi ts people with other conditions:
• Testosterone is the primary hormone responsible for sexual function,
Klinefelter syndrome is also known as XXY syndrome.
sexual motivation, sexual arousal and fantasy in men of all ages
• Testosterone is responsible for maintaining muscle mass
How can I detect it?
and muscle strength
Babies • AIDS patients and Toddlers often use testosterone to curtail unwanted
A geneticist weight loss can and diagnose muscle wasting Klinefelter syndrome before your son is born
through • Testosterone a fetal cytogenetic plays a pivotal analysis. role in bone metabolism
Your
Testosterone
pediatrician
slows
may
bone
suspect
loss
Klinefelter
and builds
syndrome
replacement
if your
bone
infant son has:
• Cancer patients who have had their testicles removed or suffered
• Developmental delay
permanent and irreversible testicular damage due to chemo or
• Undescended
radiotherapy may
testicles
use testosterone
(cryptorchidism)
to compensate for the loss
•
Pea-sized
Testosterone
testicles
exerts an strong infl uence on mood, energy levels
• Hypospadias and concentration
(urine dribbles out of an opening on the
underside of his penis)
However, 75% of boys with Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
111
11

What is Klinefelter Syndrome?
• Androgen Deficient Ageing Males (ADAM) or late-onset hypogonadal
males apply natural testosterone cream to combat their:
Klinefelter syndrome is the most common cause of male infertility. It is a
– Changes in mood (fatigue, depression, anger)
sex chromosome variation that occurs in 1 in 500 males. Most men do not
demonstrate – Decreased symptoms. body hair Normally, (feminization) males have one X chromosome in their
cells. – Decreased Males affected bone with mineral Klinefelter density syndrome and possible have resulting at least one osteoporosis extra X
chromosome – Decreased in lean most body of their mass cells. and The muscle extra strength chromosome impacts their:
– • Decreased Bone strength libido and • Language erectile quality development
– • Increased Breast size abdominal • Learning fat
– • Rudimentary Energy level breast • development Muscle mass (man-boobs or gynecomastia)
– • Low Hair sperm growth in the semen • Social development
Women • Height take reduced-dose • Thought testosterone processes to treat poor libido. This is
a common • Hip girth “off-label” practice among doctors in the USA, where no
testosterone product is officially approved for use by women. The
situation Not all in men Australia with is KLINEFELTER distinctly different. SYNDROME Lawley Pharmaceuticals
are 100% infertile.
produces a 1% testosterone ICSI can produce cream (AndroFeme a child – see page 9.
Klinefelter syndrome is also known as XXY syndrome.
® ), tailored especially for
women. AndroFeme ® is a popular testosterone treatment option for use
in women because it involves no surgery, no pain, is applied by the woman
in the privacy of her own home, and the dose is accurately controlled.
How can I detect it?
Homeopathic Treatments
Babies and Toddlers
Homeopathy is a complementary therapy. Homeopaths claim that like
cures
A geneticist
like. Essentially,
can diagnose
homeopaths
Klinefelter
believe
syndrome
that if
before
a substance
your son
causes
is born
a
disease,
through a
then
fetal
you
cytogenetic
can cure it
analysis.
by taking a very minute, diluted amount of
the Your same pediatrician substance. may If suspect you or your Klinefelter son have syndrome Klinefelter if your syndrome, infant son you has:
should • Developmental know that homeopathic delay treatments contain NO testosterone
nor • Undescended have they been testicles demonstrated (cryptorchidism) to cause any change in testosterone
levels.
• Pea-sized
Testosterone
testicles
is the worldwide gold standard for treating Klinefelter
syndrome and androgen deficiency.
• Hypospadias (urine dribbles out of an opening on the
underside of his penis)
Potential Risks of Testosterone Treatment
(Short However, 75% and of Long boys with Term) Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
Testosterone condition called should clinodactyly, not be used where in men the little with fi breast nger curves cancer toward or known the or ring
suspected fi nger because prostate the middle cancer.
bone is wedge-shaped instead of rectangular.
12 1

Patients with heart disease, liver disease or kidney disease are not
recommended to use testosterone supplements.
Before initiating TRT your doctor should check for prostate abnormalities
by means of a digital rectal examination (insertion of the fi nger through
the anus and feeling the hardness of the prostate gland) and a blood test
for Prostate Specifi c Antigen (PSA).
These tests will ensure complications of the prostate should not arise due
to testosterone usage.
Side effects can occur if testosterone is used in excess quantities.
These may include:
• Too frequent or persistent erections of the penis (priapism)
• Nausea and vomiting
• Swelling of the ankles
• Acne
• Headache
• Gynecomastia (breast development)
• Increased appetite
These effects are usually associated with excessive levels of serum
testosterone due to incorrect dose. Due to their mode of administration,
testosterone gels and creams generally keep testosterone blood levels
within the normal therapeutic range for men and therefore side effects are
unlikely to occur.
Prostate Disease
A. Benign prostatic hyperplasia (enlarged prostate): The use of
testosterone will increase the size of the prostate mainly during the fi rst
six months of treatment. Men with testosterone defi ciency often have
reduced prostate size and most increases in prostate size result in a return
to “normal” prostate volume.
A number of medical studies have failed to show any deterioration in
obstructive symptoms attributable to benign prostatic hyperplasia during
treatment and urinary retention has not been reported at rates higher than
in control subjects.
13

14
B. Prostate cancer: The most important theoretical danger of testosterone
treatment is to increase the risk of developing prostate cancer. Whilst
lowering of testosterone levels is a standard treatment for metastatic
prostate cancer, there is no available evidence to suggest that replacement
of low testosterone levels into the normal range, leads to any increase
in the occurrence of the disease. Numerous medical papers have shown
that there was no signifi cant increase in the occurrence of prostate cancer
and a variable increase in the levels of prostate specifi c antigen (PSA). The
PSA is often below normal in hypogonadal men and is generally restored
to normal with testosterone supplementation. The authors of one paper
concluded that “there is no compelling evidence that testosterone has
a causative role in prostate cancer... (nor) increases the risk”. During the
monitoring of testosterone replacement therapy, regular digital rectal
examination and measurement of PSA are recommended.
Adverse Changes in Serum Lipids
Synthetic testosterone derivatives are associated with adverse changes
in serum lipids. However, the use of pure testosterone (e.g. testosterone
implants, patches, creams and gels) is not associated with any changes to
cholesterol or serum lipid concentrations.
There is no known interaction between testosterone and lipid
lowering medications.
Coronary Heart Disease
A major theoretical concern regarding testosterone administration is the
possibility that it could increase the risk of cardio-vascular disease. Such a
concept is based on the higher incidence of cardio-vascular events in men
than in women. However, this may be much more readily explicable by
protective effects of estrogen in women. There is little data to support a
causal relationship between high testosterone levels and heart disease
and in fact, a signifi cant body of evidence suggests that the opposite
may be true and that men with low testosterone levels may be at higher
cardio-vascular risk. There are reports that testosterone replacement
can improve symptoms of chronic stable angina and there are direct
observations showing vasodilation following intra-coronary injections of

testosterone. There are no reports of increasing incidence of
cardio-vascular disease including myocardial infarction, stroke
or angina in reports of testosterone replacement therapy.
Polycythemia (an abnormal increase in red blood cells)
A well know side effect of chronic testosterone administration, particularly
using the intra muscular route (injections), where high serum testosterone
levels are present for some days following each injection, is the
occurrence of polycythemia, with a rise in haematocrit (the percent of
whole blood that is composed of red blood cells). It is noteworthy that
men with hypogonadism tend to have anaemia and reduced hematocrit
concentrations and testosterone replacement leads to normalisation.
There is a direct dose relationship between the testosterone dose and the
incidence of polycythemia. This effect, while not life threatening or severe
requires the need for regular monitoring (yearly) by a medical professional
of this parameter during testosterone replacement therapy.
Long term risks with testosterone replacement therapy are minimal,
particularly in regard to the major concerns addressed above.
Side effects from excessive testosterone dosing are noted, but
such adverse reactions are extremely unlikely with testosterone cream
or gel topical administration.
Contraindications
If you are a woman, you cannot take testosterone during pregnancy
to avoid giving birth to a son with Klinefelter syndrome. The genetic
problem already occurred when the cells divided and is irreversible.
Do not take testosterone while you are breastfeeding, as it will adversely
affect your child through the milk. The benefi ts do not outweigh the risks.
If you are a man, you cannot take testosterone if you have any of these
pre-existing conditions:
• Known hypersensitivity or allergy to testosterone
• Existing cancer of the breast or prostate gland
• Severe heart, kidney, or liver disease
• Excessive calcium in the blood (hypercalcemia)
15

16
Precautions
Before you embark on a course of testosterone replacement therapy,
or decide to place your son on TRT, take these simple precautions:
1. Ask your doctor to rule out these similar conditions before
diagnosing Klinefelter syndrome:
• Kallmann syndrome
• 46,XX karyotype
• Infertility
2. Get a blood test for testosterone. The normal range is 300 – 1,200 ng/dl
or 10 – 35 nmol/L. It will be highest in the morning.
3. Your doctor needs to undertake a physical examination of your
prostate gland and conduct a blood test to measure the PSA
(prostate specifi c antigen). This is to ensure that you do not have
prostate cancer. Testosterone should not be used if there is prostate
cancer or irregularities. If you have chronic liver or kidney disease
testosterone should only be used with strict medical supervision.
4. Get genetic testing to see if the cause is myotonic dystrophy.
In adult males, during therapy, get a regular (yearly) PSA test for early
detection of prostate cancer and rectal examination of the prostate. You
need regular hemoglobin, hematocrit, liver function, and cholesterol tests
and frequent monitoring by your doctor for the risks discussed above.
What can we expect when we visit the doctor?
Your doctor will ask about your health and sex history, or that of your child.
Your doctor will enquire about genetic disease in your family. Your doctor
will ask you to list previous illnesses, drugs (prescribed and street drugs
for recreational use), and stressors. Your doctor will perform a digital rectal
exam to check for prostate cancer.
Explain to your child that he needs blood tests, x-rays, and a cardiogram.
The x-rays and cardiogram do not hurt. The blood test is a minor prick.
Accompany him during testing to make him feel secure. Maintain a calm
demeanor. Bring a treat as positive reinforcement for your child’s good
behavior. Praise your child for his cooperation.

What is Klinefelter Syndrome?
Klinefelter syndrome is the most common cause of male infertility. It is a
sex chromosome variation that occurs in 1 in 500 males. Most men do not
demonstrate symptoms. Normally, males have one X chromosome in their
cells. Males affected with Klinefelter syndrome have at least one extra X
chromosome in most of their cells. The extra chromosome impacts their:
Which How to testosterone determine which route testosterone is best for me? treatment?
• Bone strength • Language development
• Breast size • Learning
If one Googles “natural testosterone cream” or “testosterone gel” there
• Energy level • Muscle mass
are dozens of products claiming to be the “best” and “authentic” natural
• Hair growth • Social development
testosterone creams or gel. Just how does a man contemplating using
• Height • Thought processes
a natural testosterone cream determine which product is most suited
• Hip girth
to his requirements? The following is an outline of basic manufacturing
processes to help you decide:
Not all men with KLINEFELTER SYNDROME are 100% infertile.
The three quality standards of natural testosterone cream are:
ICSI can produce a child – see page 9.
1. Pharmaceutical Grade: The manufacturer operates to international
standards of Good Manufacturing Practice (GMP). GMP means all
Klinefelter syndrome is also known as XXY syndrome.
production processes are standardized and controlled from the time
the raw material is procured through to the expiry date printing on the
How can I detect it?
fi nished product. The Australian government, like the US and European
Babies regulators, and Toddlers enforce rigid government controls on the manufacturing
A geneticist facility and can its equipment, diagnose Klinefelter processes, syndrome and packaging. before your Andromen son is born
through a fetal cytogenetic analysis.
Your pediatrician may suspect Klinefelter syndrome if your infant son has:
• Developmental delay
• Undescended testicles (cryptorchidism)
• Pea-sized testicles
• Hypospadias (urine dribbles out of an opening on the
underside of his penis)
However, 75% of boys with Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
® and
Andromen ® If one Googles “natural testosterone cream” or “testosterone gel” there
are dozens of products claiming to be the “best” and “authentic” natural
testosterone creams or gel. Just how does a man determine which
product is most suited to his requirements? The following is an outline
of basic manufacturing processes to help you decide. The three quality
standards of natural testosterone cream are:
1. Pharmaceutical Grade: The manufacturer operates to international
standards of Good Manufacturing Practice (GMP). GMP means all
production processes are standardized and controlled from the time
the raw material is procured through to the expiry date printing on
the finished product. The Australian government, like the U.S. and
European regulators, enforces rigid government controls on the
manufacturing facility and its equipment, processes, and packaging.
AndroForte
Forte natural testosterone creams are guaranteed stable,
effective, and potent. The fi nal product has detailed documentation
and is backed by clinical trials that substantiate its therapeutic claims.
2. Cosmetic Grade: This is the quality sold over-the-counter in drug,
department and grocery stores. Cosmetic grade products are 70% pure.
Often, brand-names have exactly the same ingredients as generics,
just with a different label. Cosmetic grade products are allowed a high
bacterial content, so their shelf-life is very limited (usually 3 – 6 months).
Cosmetic manufacturers are not required to register their products with
the government because cosmetic products do not require clinical trials
to prove their worth.
® 2 and AndroForte ® 5 natural testosterone creams
are guaranteed stable, effective, and potent. The final product
has detailed documentation and is backed by clinical trials that
substantiate its therapeutic claims.
2. Cosmetic Grade: This is the quality sold over-the-counter in drug,
department and grocery stores. Cosmetic grade products are 70% pure.
Often, brand-names have exactly the same ingredients as generics,
just with a different label. Cosmetic grade products are allowed a high
bacterial content, so their shelf-life is very limited (usually 3 – 6 months).
Cosmetic manufacturers are not required to register their products with
the government because cosmetic products do not require clinical trials
to prove their worth.
17 117

18
What is Klinefelter Syndrome?
3. Compounded Product: Natural health products from pharmacists,
herbalists, homeopaths, naturopaths, and practitioners of traditional
Klinefelter syndrome is the most common cause of male infertility. It is a
Indian and Chinese medicines are compounded. This means the
sex chromosome variation that occurs in 1 in 500 males. Most men do not
product is tailored to the patient’s individual needs in the delivery
demonstrate symptoms. Normally, males have one X chromosome in their
system most desired. Pharmacists compound drugs that are not
cells. Males affected with Klinefelter syndrome have at least one extra X
commercially available, or in a different strength than that readily
chromosome in most of their cells. The extra chromosome impacts their:
available. A compounded product may be needed to make a drug
palatable. • Bone strength A compounded • Language product development may be needed if the patient reacts
to • Breast dyes, preservatives, size • Learning and allergens found in commercial products.
Compounded • Energy level products • Muscle do not mass undergo any form of production
control, • Hair growth concentration, • Social impurity, development stability or efficacy testing. Safe shelflife
• Height is usually extremely • Thought short, if processes at all known. Compounded items are
time-consuming • Hip girth to make, so generally they are more expensive.
The only pharmaceutical grade natural testosterone creams for men
Not all men with KLINEFELTER SYNDROME are 100% infertile.
available worldwide are AndroForte
ICSI can produce a child – see page 9.
® 2 and AndroForte ® 5 cream from
Lawley Pharmaceuticals, Australia.
AndroForte
Klinefelter syndrome is also known as XXY syndrome.
How can I detect it?
Babies and Toddlers
A geneticist can diagnose Klinefelter syndrome before your son is born
through a fetal cytogenetic analysis.
Your pediatrician may suspect Klinefelter syndrome if your infant son has:
• Developmental delay
• Undescended testicles (cryptorchidism)
• Pea-sized testicles
• Hypospadias (urine dribbles out of an opening on the
underside of his penis)
® 2 and AndroForte ® 5 testosterone creams are specifically
targeted for use in men with declined or lowered serum testosterone levels
due to genetic disorders, surgical or chemical interventions or ageing.
Low testosterone in men is associated with declined libido, diminished
sexual function, fatigue, lethargy, loss of motivation, decreased muscle
mass and strength, depression, irritability and mood changes. Applied
topically to the skin, AndroForte ® 5 and AndroForte ® 2 Testosterone Creams
for men are the world’s only clinically trialed and tested pharmaceutical
grade testosterone creams using natural bio-identical testosterone.
AndroForte ® 5 and AndroForte ® 2 Testosterone Creams are listed with the
Australian government (AUST L 166238 / L 166239).
However, 75% of boys with Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
1

What is Klinefelter Syndrome?
AndroForte
Klinefelter syndrome is the most common cause of male infertility. It is a
sex chromosome variation that occurs in 1 in 500 males. Most men do not
demonstrate symptoms. Normally, males have one X chromosome in their
cells. Males affected with Klinefelter syndrome have at least one extra X
chromosome in most of their cells. The extra chromosome impacts their:
® Prescribing Information and Consumer Medicine Information
can be downloaded from http://www.androforte.com (or by clicking on the
links above)
AndroForte®
AndroForte®
AndroForte® 5
5
AndroForte® 5
5
Cream containing 5% w/v (50mg/mL) Testosterone B.P. (17-β- Hydroxyandrost-4-en-3-one)
PRODUCT INFORMATION
Normal range serum testosterone concentrations are reached during the first
day of dosing using a 50mg single dose applied to the scrotum. There is no
• Bone strength accumulation • of testosterone Language after steady-state level is reached. A steady-state development
level is achieved within 10 days of initiating therapy. A single daily application of
0.5-1.0ml of cream (25-50mg testosterone) of ANDROFORTE
• Breast size • Learning
• Energy level • Muscle mass
• Hair growth • Social development
• Height • Thought processes
• Hip girth
® 5 testosterone
cream to the scrotum results in a serum concentration profile within the normal
therapeutic range for healthy young males (10-35 nmol/L or 300-1100 ng/dL).
Indications
ANDROFORTE ® 5 testosterone cream is indicated for testosterone replacement
therapy for symptomatic testosterone deficient males, including confirmed
primary hypogonadism, secondary hypogonadism and late-onset hypogonadism.
Contraindications
Testosterone is contraindicated in men with known or suspected carcinoma of the
prostate, known or suspected carcinoma of the breast, known or suspected
androgen-dependent neoplasia, nephrotic syndrome or hypercalcaemia.
Known sensitivity to testosterone, ANDROFORTE ® 5 testosterone cream or
any of its components. ANDROFORTE ® 5 testosterone cream contains almond
oil. ANDROFORTE ® 5 testosterone cream has not been evaluated in women and is
contraindicated in pregnancy and while lactating. The product is not suitable for children.
Precautions
EXTREMELY IMPORTANT: Before initiating ANDROFORTE ® 5 testosterone
cream treatment, surveillance for prostate cancer by means of digital rectal
examination (DRE) and a blood test for Prostate-Specific Antigen (PSA) is
recommended. Caution is warranted if the PSA is normal-high because in
hypogonadal patients the PSA is generally inappropriately low. Furthermore a
post treatment increase in PSA more than the normal range should raise
suspicion about prostate disease. International guidelines recommend reviewing
PSA each 3 months for the first 12 months then each 12 months thereafter. If
there is a 1.5 increase of PSA during a 12 month period further evaluation is
required even if under age-related normal cut-off. Hemoglobin and hematocrit
should be checked periodically to detect polycythemia in patients receiving
androgen therapy. Liver function, PSA, total and HDL cholesterol should also be
monitored. Patients with pre-existing cardiac, hepatic or renal diseases need to be
monitored closely when undergoing androgen treatment. High level athletes need
to be aware of the rules governing androgen use if prescribed ANDROFORTE ® Composition Active: Testosterone.
Chemical Name: 17-β-Hydroxyandrost- 4 - en-3-one
Molecular Weight: 288.4.
CAS: 58-22-0
Description
ANDROFORTE
5
testosterone cream. Androgen supplementation in geriatric patients may increase
the risk for the development of prostatic hyperplasia.
® 5 testosterone cream is a transdermal drug delivery system
consisting of a white oil-in-water cream intended for scrotal administration.
ANDROFORTE ® 5 testosterone cream contains dl-α- tocopherol acetate (vitamin
E) and almond oil formulated to optimize systemic absorption of the active
ingredient. Also contains cetomacrogol 1000, cetostearyl alcohol, butylated
hydroxytoluene, anhydrous citric acid, triethanolamine, carbomer 940, B & J
Phenonip ® OH
CH3
CH3 H
H H
O
Testosterone
C19H28O2
and purified water.
Pharmacology
Testosterone is the primary androgenic hormone. Testosterone and its 5α-reduced
metabolite dihydrotestosterone (DHT) activate the intracellular androgen
receptor and modulate gene transcription.Testosterone is produced by the
interstitial (Leydig) cells of the testes and the adrenal glands in males and by
Prior to using ANDROFORTE the ovary and adrenal glands in females. Testosterone is responsible for the
normal growth and development of the male sex organs and for maintenance
of secondary sex characteristics. Secondary sex characteristics include growth
and maturation of the prostate, seminal vesicles, penis and scrotum; male hair
distribution, deepening of the voice, changes in fat distribution and muscle mass.
Pharmacokinetics
The majority of testosterone produced (98-99%) is biologically inactive due to
binding to sex hormone-binding globulin (SHBG) and albumin (55% and 45%
respectively to the bound portion). Testosterone also circulates unbound as a free
hormone (1-2%) and is considered biologically active. Testosterone is metabolized
primarily in the liver and also in peripheral tissue. Dihydrotestosterone (DHT)
and estradiol (E2) are products of testosterone metabolism. DHT is produced by
reduction through the action of the enzyme 5- alpha reductase, which is present
in genital tissue, skin and the prostate. DHT is further metabolized to 3-alpha
and 3-beta androstanediol. DHT binds with greater affinity to SHBG than does
testosterone. E2 is produced by aromatisation of testosterone. DHT:testosterone
and E2:testosterone ratios in normal adult males are 1:10 and 1: 200 respectively.
90% of testosterone is excreted in the urine as glucuronide and sulphate
® 5
ANDROFORTE ® 5 cream is only for use in men.
conjugates of testosterone and its metabolites.
Scrotal skin is significantly more receptive to the absorption of steroids than other
sites of application including the back, axilla and forearms¹. Daily application of
ANDROFORTE ® 5 to the scrotum results in a serum testosterone concentration
profile within the normal range observed in healthy young men. Maximum serum
testosterone concentration occurs 3-5 hours after application as shown in Figure 1.
Fig. 1 Mean (SD) plasma testosterone concentrations using 50mg testosterone
Paediatric Use
This product is not suitable for children. Care should be taken to ensure that
children do not come into contact with ANDROFORTE ® 5 application sites. In the
event of contact, wash with soap and water as soon as possible.
Adverse Reactions
Potential side-effects from excessive dosing may include:
• Nausea, vomiting, jaundice or swelling of the ankles
• Increased body hair
• Increased acne
• Signs of virilization
• Weight gain
• Persistent headaches
• Increased appetite
• Deepening of the voice
Not all men with KLINEFELTER • Electrolyte disturbances SYNDROME are 100% infertile.
• Too frequent or persistent erections of the penis (priapism)
• Gynecomastia
(2 units) scrotally once daily application of ANDROFORTE ICSI can produce a child – see page 9.
® It is recommended that patients do not swim or shower until at least
one hour after application of ANDROFORTE
5 in 8 hypogonadal
males.
® How to use ANDROFORTE
5 cream. Close skin
contact with the area of application within an hour of application by
a partner should be avoided. This may result in the partner absorbing
some testosterone via skin contact.
® 5
Your doctor or pharmacist will explain how to apply ANDROFORTE ® 5.
Follow all directions exactly as they are explained. If you are unclear
talk to your doctor. The dose of ANDROFORTE ® 5 will be determined
for you by your doctor. This dose is specific to your condition and
should not be varied unless directed to do so by your doctor. You will
need to return to your doctor at regular intervals so that the dose can
be checked via a blood test.
www.hormonesolutions.com www.hormonesolutions.com www.hormonesolutions.com www.hormonesolutions.com
Klinefelter syndrome is also known as XXY syndrome.
How can I detect it?
Cream containing 5% w/v (50mg/mL) Testosterone B.P (17-β- Hydroxyandrost-4-en-3-one)
CONSUMER MEDICINE INFORMATION
What is in this leaflet
This leaflet answers some of the common questions about
ANDROFORTE ® 5. It does not contain all the available information.
It does not take the place of talking to your doctor or pharmacist.
All medicines have risks and benefits. Your doctor has weighed the
possible risks of you using ANDROFORTE ® 5 against the benefits it can
have for you.
If you have any concerns about using ANDROFORTE ® 5 talk to your
doctor or pharmacist. Keep this leaflet with the medicine. You may
need to read it again.
What ANDROFORTE ® 5 is used for?
ANDROFORTE ® 5 contains the active ingredient testosterone. This
form of testosterone is identical to the testosterone produced by
the testes of men and the ovaries of women. Testosterone plays an
important role in physical and sexual development, sexual arousal,
sexual response, libido, maintenance of bone, energy levels and
well-being in men and women. When men suffer from a deficiency
of testosterone one or more of these areas may be affected and
ANDROFORTE ® 5 provides testosterone to address this deficiency. The
skin readily absorbs testosterone and ANDROFORTE ® 5 is a simple and
effective means of getting testosterone into the bloodstream.
ANDROFORTE ® 5 cream should not be used if you are allergic
to testosterone or any of the ingredients contained within
ANDROFORTE ® 5. These are listed at the end of this leaflet. This
product contains almond oil.
If you have used ANDROFORTE ® 5 or any other testosterone
preparation before and became unwell, tell your doctor or pharmacist
before using the cream. Other testosterone containing preparations
include Testogel ® , (AndroGel ® and Testim ® USA only), Reandron ® ,
Sustanon ® , Andriol ® , Primodian ® depot and Primoteston ® depot.
Do not use ANDROFORTE ® 5 if you have cancer especially prostatic
cancer, breast cancer, kidney disease or have high calcium levels in the
blood. If you have heart disease, high blood pressure or liver disease
make sure your doctor is aware of these conditions before using
ANDROFORTE ® 5 because it may not be appropriate for you. Check
with your doctor or pharmacist if you are unsure about whether you
have any of these conditions before starting ANDROFORTE ® 5. If you
are taking other medications check with your doctor or pharmacist
before starting ANDROFORTE ® 5. High level athletes need to be aware
of the rules governing androgen use if prescribed ANDROFORTE ® 5
cream. Do not use ANDROFORTE ® 5 after the expiry date which is
printed on the base (crimp) of the tube. If you use this medicine after
the expiry date has passed, it may not work as well.
Do not use ANDROFORTE ® 5 if the foil seal at the top of the tube is
damaged or broken.
ANDROFORTE ® 5 is not indicated for use in children or women.
www.hormonesolutions.com www.hormonesolutions.com www.hormonesolutions.com
Babies and Toddlers
A geneticist can diagnose Klinefelter syndrome before your son is born
through a fetal cytogenetic analysis.
Your pediatrician may suspect Klinefelter syndrome if your infant son has:
• Developmental delay
• Undescended testicles (cryptorchidism)
• Pea-sized testicles
• Hypospadias (urine dribbles out of an opening on the
underside of his penis)
Opening the tube
To open the tube remove the cap and peel off the foil seal. Dispose of
the foil seal and replace the cap firmly after using the cream.
Measuring the correct dose of ANDROFORTE ® 5
cream
A measuring applicator (syringe style) in a sealed sleeve is enclosed in
the ANDROFORTE ® 5 box. The dose of ANDROFORTE ® 5 is measured
in millilitres. The applicator is marked with 0.5ml graduations for dosing
accuracy. Your doctor will have determined which dose is appropriate
for you. To measure the correct dose of cream insert the tip of the
applicator into the open nozzle of ANDROFORTE ® 5 cream so that
the nozzle and the shoulder of the applicator are in contact. GENTLY
squeeze the base of the ANDROFORTE ® 5 tube until cream reaches the
open nozzle of the tube. At the same time slowly withdraw the plunger
of the applicator. The cream will flow into the barrel of the applicator.
Fill to the required dose. For example: a 1ml dose of ANDROFORTE ® 5
(50mg testosterone) needs the flat part of the plunger level with the
1ml mark. If there are any air bubbles in the measured dose fill slightly
past the required dose mark then depress the plunger so that the
excess cream flows back into the tube. Stop at the required dose mark.
Remove the applicator from the nozzle of the tube and replace the cap
firmly on the tube. Depress the plunger of the applicator containing
the dose of ANDROFORTE ® 5 directly onto the scrotal area. Massage
the cream into the scrotal area until absorbed. Rinse the applicator
in warm water after use and replace in box with ANDROFORTE ® 5
testosterone cream ready for the next day's application. Each 1ml of
ANDROFORTE ® 5 contains 50mg of testosterone.
Where to apply ANDROFORTE ® 5 cream
Always apply ANDROFORTE ® 5 cream to clean dry skin. The best
area to use ANDROFORTE ® 5 is the scrotum (the skin sack that
holds the testicles). This is the recommended site of application
because absorption
of testosterone is
greatest from this
part of the body. It is
recommended that
a dose of no greater
than 1ml (50mg
testosterone) once
daily be applied when
initiating scrotal application. Many men experience a warming sensation
of the scrotal skin for a few minutes after applying ANDROFORTE ® 5 – this
is normal and quickly subsides. No perfume, deodorant or moisturizing
creams or gels should be used on the area because this may interfere
with ANDROFORTE ® 5 from being absorbed. Never apply the cream to
broken or damaged skin. ANDROFORTE ® 5 cream should be applied at
approximately the same time each day.
However, 75% of boys with Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
0.5
1
1.5
2
ml
Scrotum - The skin sack
that holds the testicles
19 1

20
About What is Lawley Klinefelter Pharmaceuticals
Syndrome?
Lawley Klinefelter Pharmaceuticals syndrome is the is a most privately common owned cause pharmaceutical of male infertility. company It is a
which sex chromosome focuses on the variation transdermal that occurs administration in 1 in 500 of males. the naturally Most men occurring do not
hormones demonstrate testosterone, symptoms. progesterone Normally, males and have estradiol. one X chromosome in their
Founded cells. Males in affected 1995 by pharmacist with Klinefelter Michael syndrome Buckley, have Lawley at least Pharmaceuticals
one extra X
has chromosome grown to become in most of a world their cells. leader The in extra research chromosome and development impacts their: of
transdermal • Bone strength hormone preparations.
• Language development
As the • Breast principal size of Lawley • Learning Pharmaceuticals Mr. Buckley has presided over
the • development, Energy level research, • Muscle clinical mass trial program, regulatory process,
development • Hair growth and marketing • Social of development
the company.
• Height • Thought processes
The
•
Lawley
Hip girth
Pharmaceuticals portfolio of products includes:
• AndroFeme 1% cream – Testosterone for women
• AndroForte
Not all men with KLINEFELTER SYNDROME are 100% infertile.
ICSI can produce a child – see page 9.
Klinefelter syndrome is also known as XXY syndrome.
How can I detect it?
Babies and Toddlers
A geneticist can diagnose Klinefelter syndrome before your son is born
through a fetal cytogenetic analysis.
Your pediatrician may suspect Klinefelter syndrome if your infant son has:
• Developmental delay
• Undescended testicles (cryptorchidism)
• Pea-sized testicles
• Hypospadias (urine dribbles out of an opening on the
underside of his penis)
However, 75% of boys with Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
® 2 and AndroForte ® 5 creams –
Testosterone for men
• Pro-Feme ® 3.2% and 10% creams – Progesterone for women
• Natragen ® 0.2% cream – Estradiol for women
Our Mission Statement
Lawley Pharmaceuticals provides optimal delivery systems for the
administration of naturally occurring hormones to counter endocrine
deficiency states.
Our philosophy centres on the principle to replace “like with like”, to use
a bio-identical hormone in preference to a synthetic hormone analogue
when a viable clinical option and to advance areas of clinical research that
has had little or no investigation using naturally occurring hormones.
Our goal is to establish, through evidence based medical research, bioidentical
hormones as cornerstone treatments for diseases such as breast
disease, infertility, male hypogonadism, female androgen deficiency, post
partum depression and endometriosis.
Lawley Pharmaceuticals has established strong links with centres
of medical excellence around the world and continues to push the
boundaries of medical research.
1

Completed What is Klinefelter Clinical Syndrome?
Studies
1. Klinefelter Effect of syndrome sequential is transdermal the most common progesterone cause of cream male on infertility. endometrium, It is a
sex bleeding chromosome pattern, variation and plasma that occurs progesterone in 1 in 500 and males. salivary Most progesterone men do not
demonstrate levels in postmenopausal symptoms. Normally, women. males Wren have BG et one al. X Climacteric chromosome 2000 in their
cells. 3:155–160. Males affected with Klinefelter syndrome have at least one extra X
2. chromosome Distribution in and most metabolism of their cells. of topically The extra applied chromosome progesterone impacts in their: a rat
model. • Bone Waddell strength B and • Language O’Leary PJ. development J Ster Biochem & Mol Biol. 80 (2002)
449–455. • Breast size • Learning
3. Plasma • Energy and level saliva concentrations • Muscle mass of progesterone in pre- and postmenopausal
• Hair growth women • after Social topical development application of progesterone cream.
O’Leary • Height PJ et al. Presented • Thought at the processes Annual Congress of the Australian
Menopause • Hip girth Society held in Perth, Australia in October 1997
4. Long-term pharmacokinetics and clinical efficacy of AndroForte 5
Not cream all for men androgen with KLINEFELTER replacement in SYNDROME hypogonadal are men. 100% Handelsman infertile. DJ
et al. ANZAC Research ICSI can produce Institute, Department a child – see of page Andrology, 9. Concord
Hospital, Sydney, 2004.
5.
Klinefelter
Transdermal
syndrome
testosterone
is also known
therapy
as
improves
XXY syndrome.
well-being, mood, and
sexual function in premenopausal women. Goldstat R et al. Menopause
How 2003; can 10 (5): I 390-398. detect it?
6. The pharmacokinetics pilot study of ANDROFEME
Babies and Toddlers
A geneticist can diagnose Klinefelter syndrome before your son is born
through a fetal cytogenetic analysis.
Your pediatrician may suspect Klinefelter syndrome if your infant son has:
• Developmental delay
• Undescended testicles (cryptorchidism)
• Pea-sized testicles
• Hypospadias (urine dribbles out of an opening on the
underside of his penis)
However, 75% of boys with Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
® 1% testosterone
cream following two week, once daily application in testosterone
deficient women. Eden JA et al. Presented at the 4th Annual Congress
of the Australasian Menopause Society held in Adelaide 5-7th
November 2000.
7. A double-blind, randomized, placebo-controlled trial of the effect
of testosterone cream on the sexual motivation of menopausal
hysterectomized women with hypoactive sexual desire disorder. El-Hage
et al Climacteric 2007; 10: 335–343.
8. Pharmacokinetics Of AndroForte 5 Cream: A Dose Finding Study.
Kelleher S et al. ANZAC Research Institute, Department of Andrology,
Concord Hospital, Sydney, 2002.
121

Recommended What is Klinefelter Reading Syndrome? for Patients
1. Klinefelter http://www.andrologyaustralia.org
syndrome is the most common cause of male infertility. It is a
sex chromosome variation that occurs in 1 in 500 males. Most men do not
2. http://www.nichd.nih.gov/health/topics/klinefelter_syndrome.cfm
demonstrate symptoms. Normally, males have one X chromosome in their
3. cells. Eunice Males Kennedy affected Shriver with Klinefelter National syndrome Institute of have Child at Health least one and extra Human X
chromosome Development, in most NIH, of DHHS. their cells. (1997). The extra chromosome impacts their:
Understanding
• Bone strength
Klinefelter
• Language
Syndrome:
development
A Guide for XXY Males
and
• Breast
Their
size
Families (97-3202).
• Learning
• Energy level • Muscle mass
Washington, • Hair growth DC: U.S. • Social Government development Printing Office.
• Height • Thought processes
4. http://www.genome.gov/19519068
• Hip girth
5. http://www.nlm.nih.gov/medlineplus/tutorials/lowtestosterone/htm/
Not index.htm all men with KLINEFELTER SYNDROME are 100% infertile.
6. http://www.medem.com/medlb/article_detaillb.cfm?article_
ICSI can produce a child – see page 9.
ID=ZZZO7PDVDLC&sub_cat=57
Klinefelter syndrome is also known as XXY syndrome.
7. http://clinicaltrials.gov/search/open/condition=%22Klinefelter+Syndro
me%22
How can I detect it?
8. Babies http://ghr.nlm.nih.gov/condition=klinefeltersyndrome
and Toddlers
9. A geneticist http://www.genetic.org/knowledge/support/action/C130/
can diagnose Klinefelter syndrome before your son is born
through a fetal cytogenetic analysis.
10. http://kidshealth.org/teen/sexual_health/changing_body/delayed_
Your pediatrician may suspect Klinefelter syndrome if your infant son has:
puberty.html
• Developmental delay
11. • Undescended http://www.hormonesolutions.com.au/?page=pages/frontpage
testicles (cryptorchidism)
12. • Pea-sized http://www.lawleypharm.com.au/
testicles
• Hypospadias (urine dribbles out of an opening on the
underside of his penis)
However, 75% of boys with Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
22 1

References for Medical Professionals
1. Bojesen A, Gravholt CH. Klinefelter syndrome in clinical practice.
Nat Clin Pract Urol. Apr 2007;4(4):192-204.
2. Swerdlow AJ, Higgins CD, Schoemaker MJ, et al. Mortality in patients
with Klinefelter syndrome in Britain: a cohort study. J Clin Endocrinol
Metab. Dec 2005;90(12):6516-22.
3. Schiff JD, Palermo GD, Veeck LL, et al. Success of testicular sperm
injection and intracytoplasmic sperm injection in men with Klinefelter
syndrome. J Clin Endocrinol Metab. Nov 2005;90(11):6263-7.
4. Denschlag D, Tempfer C, Kunze M, Wolff G, Keck C. Assisted
reproductive techniques in patients with Klinefelter syndrome:
a critical review. Fertil Steril. Oct 2004;82(4):775-9.
5. Kamischke A, Baumgardt A, Horst J, Nieschlag E. Clinical and
diagnostic features of patients with suspected Klinefelter syndrome.
J Androl. Jan-Feb 2003;24(1):41-8.
6. Advani SK, Chadha MD, Khan NM. Klinefelter syndrome with
unelevated serum gonadotropin levels (a case report).
J Postgrad Med 1991;37:171
7. Klinefelter HF Jr, Reifenstein EC Jr, Albright F. Syndrome characterized
by gynecomastia aspermatogenesis without a-Leydigism and increased
excretion of follicle-stimulating hormone. J Clin Endocr Metabl.
1942;2:615-624.
8. Anawalt BD, Bebb RA, Matsumoto AM, et al. Serum inhibin B levels
refl ect Sertoli cell function in normal men and men with testicular
dysfunction. J Clin Endocrinol Metab. Sep 1996;81(9):3341-5.
9. Bender BG, Harmon RJ, Linden MG, Robinson A. Psychosocial
adaptation of 39 adolescents with sex chromosome abnormalities.
Pediatrics. Aug 1995;96(2 Pt 1):302-8.
10. Bhasin S, Ma K, Sinha I, et al. The genetic basis of male infertility.
Endocrinol Metab Clin North Am. Dec 1998;27(4):783-805, viii.
23

24
11. Meschede D, Louwen F, Nippert I. Low rates of pregnancy termination
for prenatally diagnosed Klinefelter syndrome and other sex
chromosome polysomies. Am J Med Genet. Dec 4 1998;80(4):330-4.
12. Robinson A, Bender BG, Linden MG. Prognosis of prenatally
diagnosed children with sex chromosome aneuploidy. Am J Med
Genet. Oct 1 1992;44(3):365-8.
Glossary
You may hear these terms discussed in reference to yourself or your son:
Azoospermia: The ejaculate contains no sperm at all.
Cryptorchidism: Undescended testicles. One or both testes stay in the
abdominal cavity as they are before birth, instead of entering the scrotum.
The doctor can try to manipulate the testes down into the scrotum, or
can move them surgically if manipulation fails. It is dangerous to leave
the testes in the abdomen, as it increases the risk of testicular cancer. The
testes need to be kept cool in the scrotum to produce sperm. The intense
core heat of the abdomen kills sperm.
Fibrosis: Scar tissue replaces healthy tissue as a result of degeneration,
injury, or infection.
Genotype: Genetic makeup, as opposed to appearance.
Gynecomastia: Enlargement of one or both male breasts, sometimes with
milk production. This is not pubertal hypertrophy, where a tender disc
of enlarged tissue forms under the boy’s nipple and disappears within a
year. Causes of gynecomastia include: Klinefelter syndrome; hormone
imbalance; weight gain; taking steroids or estrogen; cirrhosis of the liver;
tumor in the testicles, breast, or lung. Gynecomastia should always be
evaluated by a doctor.
Hyalinized: Healthy tissue is replaced by hyaline (clear or translucent
white, glassy collagen fi bers) due to degeneration.
Hypospadias: A birth defect where the boy’s urethra opens onto the
underside of the penis or below it, instead of the end of the glans.

Hypothalamus: The section of the brain that regulates body temperature,
chemical balance, the pituitary gland, and the autonomic nervous system.
The hypothalamus is part of the limbic system, so it regulates sexual
appetite, eating, sleep, and emotions. It infl uences heart and breathing
rates and blood pressure. The hypothalamus is located in the grey matter,
below the thalamus, in the center of the brain. The pituitary gland hangs
on a stalk below the hypothalamus.
Karyotype: Number, form, and size of chromosomes.
Oligospermia: The ejaculate contains fewer sperm than normal.
Phenotype: The physical characteristics of the boy comprised of his
genetic makeup and his environment.
Pituitary gland: Connected to the hypothalamus, the pituitary controls
growth hormone, prolactin for milk production, and follicle stimulating
hormone (FSH) to stimulate ovaries. The pituitary stimulates the adrenal
glands and the thyroid.
Seminiferous tubules: Two or three convoluted tubes in the testicles,
where sperm are made.
Gonadotropin levels: The pituitary gland secretes a group of hormones
called gonadotropins, which stimulate the testicles and ovaries. Boys
with Klinefelter syndrome possess at least one extra X chromosome(s),
which usually causes their pituitary glands to produce too much of the
gonadotropins FSH (follicular stimulating hormone) and LH (luteinizing
hormone). FSH and LH suppress the testicles’ normal function.
Drs. S.K. Advani, M.D. Chadha, and N.M. Khan, endocrinologists in
Bombay, reported examining two men in their twenties with 47XXY
Klinefelter syndrome who did not have elevated FSH and LH. These
doctors suggest all males with suspected Klinefelter syndrome should
have a karyotype performed before starting therapy with hCG. If the
karyotype test result shows 47 XXY and the serum gonadotropin levels are
not elevated, then the males should undergo detailed imaging studies of
their pituitary-hypothalamic complexes.
25

Internet What is Klinefelter Education Syndrome?
Reference Sites
ANDROFORTE Klinefelter syndrome www.androforte.com
is the most common cause of male infertility. It is a
ANDROFEME sex chromosome www.androfeme.com
variation that occurs in 1 in 500 males. Most men do not
PROFEME demonstrate www.profeme.com
symptoms. Normally, males have one X chromosome in their
NATRAGEN cells. Males affected www.natragen.com
with Klinefelter syndrome have at least one extra X
HORMONE chromosome SOLUTIONS in most of their www.hormonesolutions.com.au
cells. The extra chromosome impacts their:
HORMONESOLUTIONS • Bone strength • Language www.hormonesolutions.com
development
ANDROPAUSE • Breast size www.understandingandropause.com
• Learning
ANOVULATION • Energy level www.understandinganovulation.com
• Muscle mass
BREAST • Hair DISEASE growth www.understandingbenignbreastdisease.com
• Social development
BREAST • Height DISEASE www.understandingbreastdisease.com
• Thought processes
BREAST • Hip DISEASE girth www.understandingbreastdisorders.com
CASTRATION www.understandingcastration.com
DUB www.understandingdub.com
Not all men with KLINEFELTER SYNDROME are 100% infertile.
DYSFUNCTIONAL UTERINE BLEEDING
ICSI can produce a child – see page 9.
www.understandingdysfunctionaluterinebleeding.com
DYSMENORRHEA www.understandingdysmenorrhea.com
DYSPAREUNIA Klinefelter syndrome www.understandingdyspareunia.com
is also known as XXY syndrome.
EARLY MENOPAUSE www.understandingearlymenopause.com
ENDOMETRIAL How can I detect HYPERPLASIA it?
www.understandingendometrialhyperplasia.com
Babies and Toddlers
ENDOMETRIOSIS www.understandingendometriosis.com
A geneticist can diagnose Klinefelter syndrome before your son is born
ESTROGEN DOMINANCE www.understandingestrogendominance.com
through a fetal cytogenetic analysis.
FEMALE SEXUAL DYSFUNCTION
www.understandingfemalesexualdysfunction.com
Your pediatrician may suspect Klinefelter syndrome if your infant son has:
FIBROCYSTIC • Developmental BREAST delay DISEASE
www.understandingfibrocysticbreastdisease.com
• Undescended testicles (cryptorchidism)
FSD • Pea-sized www.understandingfsd.com
testicles
GYNECOMASTIA • Hypospadias (urine www.understandinggynecomastia.com
dribbles out of an opening on the
HEAVY underside PERIODS of his www.understandingheavyperiods.com
penis)
HORMONE MIGRAINE www.understandinghormonemigraine.com
However, 75% of boys with Klinefelter syndrome develop normal height
HOT FLASHES www.understandinghotflashes.com
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
HYPOGONADISM www.understandinghypogonadism.com
condition called clinodactyly, where the little fi nger curves toward the ring
HYSTERECTOMY www.understandinghysterectomy.com
fi nger because the middle bone is wedge-shaped instead of rectangular.
INFERTILITY www.understandinginfertility.biz
26 1

What is Klinefelter Syndrome?
IRREGULAR PERIODS www.understandingirregularperiods.com
KLINEFELTER SYNDROME www.understandingklinefeltersyndrome.com
Klinefelter syndrome is the most common cause of male infertility. It is a
LIBIDO www.understandinglibido.com
sex chromosome variation that occurs in 1 in 500 males. Most men do not
LOW LIBIDO www.understandinglowlibido.com
demonstrate symptoms. Normally, males have one X chromosome in their
LOW TESTOSTERONE www.understandinglowtestosterone.com
cells. Males affected with Klinefelter syndrome have at least one extra X
MENOPAUSE www.understandingmenopause.biz
chromosome in most of their cells. The extra chromosome impacts their:
MENOPAUSE www.understandingmenopause.info
MENORRHAGIA • Bone strength www.understandingmenorrhagia.com
• Language development
MISCARRIAGE • Breast size www.understandingmiscarriage.com
• Learning
MOOD • Energy CHANGES level www.understandingmoodchanges.com
• Muscle mass
NIGHT • Hair SWEATS growth www.understandingnightsweats.com
• Social development
OOPHORECTOMY • Height www.understandingoophorectomy.com
• Thought processes
OVARIAN • Hip girth CYSTS www.understandingovariancysts.com
PCOS www.understandingpcos.com
PERIMENOPAUSE Not all men with www.understandingperimenopause.com
KLINEFELTER SYNDROME are 100% infertile.
PMDD www.understandingpmdd.com
ICSI can produce a child – see page 9.
POLYCYSTIC OVARIAN SYNDROME
www.understandingpolycysticovariansyndrome.com
Klinefelter syndrome is also known as XXY syndrome.
POSTNATAL DEPRESSION www.understandingpostnataldepression.com
POSTPARTUM How can I DEPRESSION detect it?
www.understandingpostpartumdepression.com
PREGNANCY Babies and Toddlers www.understandingpregnancy.biz
PREMENSTRUAL A geneticist can diagnose SYNDROME Klinefelter syndrome before your son is born
www.understandingpremenstrualsyndrome.com
through a fetal cytogenetic analysis.
UTERINE Your pediatrician FIBROIDS may www.understandinguterinefibroids.com
suspect Klinefelter syndrome if your infant son has:
• Developmental delay
• Undescended testicles (cryptorchidism)
• Pea-sized testicles
• Hypospadias (urine dribbles out of an opening on the
underside of his penis)
However, 75% of boys with Klinefelter syndrome develop normal height
and weight until they reach age 4 or 5. In 25% of boys, there is a telltale
condition called clinodactyly, where the little fi nger curves toward the ring
fi nger because the middle bone is wedge-shaped instead of rectangular.
27
1

© Lawley Pharmaceuticals 2008
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free
free
fax:
fax:
1-800-961-7650
1-800-961-7650
Hormone Solutions