How are Childhood Hypogonadism symptoms treated?
The main cause of  hypogoadism is Klinefelter, Turner and Kallmann syndromes. These are all genetic conditions that have no cure. It’s up to hormone replacement therapy to control symptoms and improve the quality of life of sufferers.
Affected children do not need a special diet, or to restrict their activities. Hospital care is not required. Treatment is on an out-patient basis.
Boys receive testosterone replacement therapy (TRT) beginning at 11 or 12 years old. Testosterone reduces the gonadotropin level to high normal and gradually virilizes the affected boy, giving him male secondary sex characteristics, like a beard, body hair, and a male-pattern fat distribution.
If childhood hypogonadism is treated correctly with hormone replacement therapy, then affected men and women can go on to lead a normal life.
Childhood hypogonadism symptoms and treatment options
Boys with Klinefelter syndrome do not need a special diet, or to restrict their activities. Hospital care is not required. Treatment is on an out-patient basis.
Testosterone is the treatment of choice for Klinefelter syndrome. Testosterone can reduce the gonadotropin level to high normal. Gradually, testosterone will virilize the boy, giving him male secondary sex characteristics, like a beard, body hair, and a male-pattern fat distribution.
When your son is 11 or 12 years old, the doctor will commence testosterone supplementation. Testosterone treatment options include injections, gels and scrotal cream. Here is a list of Klinefelter treatment options, also used for Hypogonadism.
For all other causes of hypogonadism, the doctor must treat the underlying condition, for example, diabetes, drug abuse, or sickle cell anemia. Treatment is tailored specifically to the condition, and often requires specialists. For example:
- A dieter with anorexia nervosa needs multifaceted treatment at an eating disorders clinic with an endocrinologist, psychologist, and nurse specialist.
- If a tumor is causing your hypogonadism, you will need surgery and/or chemotherapy to remove it.
The cost of Assisted Reproductive Technology (ART) presently ranges from $8,000 – $15,000 per cycle, depending on the complexity of the method the doctor uses. ART is usually successful in three cycles. Ask your doctor to define ‘success’ before you make your down-payment, because some clinics define success as any conception, and others define it as taking home a baby.
How can I decrease the symptoms of Hypogonadism?
Males may inadvertently lower their testosterone level by consuming foods containing too much protein and too few carbohydrates. Fad diets with too many carbohydrate and too little fat deplete testosterone. Your son can marginally increase his testosterone level with exercise. To produce enough testosterone, his body requires the:
- Minerals boron and zinc
- Vitamins A, B6, and C
- Branched Chain Amino-acids (BCAA) valine, isoleucine, and leucine
Girls with Turner syndrome have very fragile bones, and should supplement their diet with Vitamin D, calcium, and Vitamin E.
Both genders must eat a well-balanced diet and perform exercise at least three times a week for 20 minutes.
People of both genders who have sexual dysfunction may benefit from psychotherapy, sex therapy, and fertility treatments.
Who is most likely to have hypogonadism?
Your child of either gender is more likely to have hypogonadism if he or she has:
- Type I Diabetes
- Sickle cell anemia, which mostly affects people of African descent
- Exposure to heroin, methadone, marijuana, spironolactone, or cyproterone
- Cancer chemotherapy
- Radiation exposure to the head or pelvis
Type II Diabetic women and girls with juvenile rheumatoid arthritis are more likely to have Turner’s syndrome. Boys who contracted mumps after puberty have a high possibility of being sterile.
Childhood hypogonadism can be linked to certain genetic disorders. The table below highlights particular genes that when affected can result in a hypogonadal disorder.
If there is a family history of genetic disorders you should consult a geneticist.
| KAL | Kallmann syndrome |
| DAX1 | Adrenal hypoplasia congenital |
| GNRHR | GnRH deficiency |
| PC1 | Prohormone processing defect |
| PROP1 and POU1F1 | Pituitary deficiency |
| HESX1 | Septo-optic dysplasia |
What is a normal appearance? How do do dr’s compare normal children with those who have Hypogonadism symptoms?
Boys
The doctor either compares your son’s testes to a model called an orchidometer or measures their length and width. Normal testes before puberty measure 2 centimeters long and their volume is 1 to 3 cm3. When the boy reaches puberty, his testicles should expand to 25 cm3 in volume. The boy’s scrotum should be completely closed (fused). His urethra should open on the end of his penis, not on the bottom of his penis. Your family doctor will suspect hypogonadism if your son is taller than 75% of his male classmates, with disproportionately long legs, small, soft testes, and female-like breast development. This is called a eunuchoid body habitus. If your son’s testes are not palpable (able to be felt) and the doctor is not sure if there is any testicular tissue in the scrotum, then your son will be given an hCG (human chorionic gonadotrophin) injection and his testosterone response is later measured by a blood test. Your son can have prosthetic testes surgically implanted to give him a normal appearance if the testes are absent.
Girls
Your family doctor will look at the extent of your daughter’s hair growth, breast development, and if her vagina has matured with regard to length and secretions. Your family doctor will suspect hypogonadism if your daughter is short, with widely spaced nipples, many moles (pigmented nevi), a webbed neck (pterygium colli), short fourth metacarpal bones in her hands, and a high arch on her palate.
Your doctor needs to determine if your child has real hypogonadism or just a constitutional delay in growth and maturation. The doctor should routinely screen for hypogonadotropic hypogonadism if your child develops: Central Nervous System (CNS) disorders; tumors of the head, eyes, throat or gonads; malnutrition; any chronic systemic disorder; or loss of menstrual periods from over-exercising.
What can I expect during my doctor’s visit to discuss Hypogonadism symptoms?
A nurse usually records the vital signs (height, weight, blood pressure and temperature). Your doctor records the history. Expect many personal questions. Your endocrinologist will ask about your child’s birth, stressors during your pregnancy, and will want to know if there was any abnormality noted about your child’s genitals, like undescended testicles or hypospadias. You will be asked about your child’s general health, sex history, and genetic diseases in your family. Bring any medications your child is currently taking, in their original bottles. You must list previous illnesses, drugs (prescribed and street drugs for recreational use), and stressors. Your doctor then performs a physical examination:
- Eyes for droopy or pale lids, abnormal movements, dryness,and visual loss or disturbance
- Nailbeds for pallor, indicating anemia
- Ears for hearing loss and unusual ear placement
- Reflexes and abnormal movements (synkinesia)
- Hair in your child’s underarms (axilla) and on the genitalia, scored according to Tanner criteria
- Pelvic exam for cancer, benign tumors, lesions, discharge, and venereal disease. If your child is male, the doctor measures both penis and testicles, and manually examines the prostate gland via the rectum. If your child is female, she reclines on the examination couch while your doctor inserts a clean clamp (speculum) to hold her vagina open and shines a bright light on her perineum to see well. If she has not had a pap smear in the past year, the doctor scrapes her cervix with a wooden Popsicle stick, smears the sample on a slide, and sends it to the Pathology Lab for expert examination.
Depending on the findings of the physical examination, the doctor orders lab tests:
- Adult males return with a semen sample to find if you produce enough sperm.
- Sexually active females give a urine sample or a hCG blood test as a routine precaution to ensure they are not pregnant before beginning hormone therapy. This is a standard precaution because of irregular periods.
- Blood tests are drawn for T3; T4; TSH; FSH; LH; estrogen; estradiol; testosterone; CBC; ferritin; TIBC; karyotyping and antiovarian antibody will be added for girls.
If your doctor suspects congenital adrenal hyperplasia, then a cosyntropin (ACTH 1-24) stimulation test is performed by injecting your child with cosyntropin injection, followed by a blood test one hour later. If your doctor suspects hypogonadotropic hypogonadism, then a stimulation test with LHRH is performed by injecting the LHRH and taking blood for LH and FSH levels at 15 or 30-minute intervals.
Your doctor may order bone age and bone density from Radiology to distinguish hypogonadism from constitutional delay. Onset of puberty depends more on bone age than chronological age. The nurse may book an abdominal ultrasound for your daughter to measure her ovaries or to find undescended testicles in your son.
If the semen results state the male over 12 years of age has azoospermia (no sperm) or few sperm (oligospermia), then his testicles are biopsied. A fine needle aspiration (FNA) biopsy in the doctor’s office is the preferred method, and is just a pinprick. If an FNA is not possible, the male will be sent for open biopsy at a surgical centre. Antiseptic and anesthetic are applied to the scrotum. It is draped with a sterile cloth. A small incision is made through the skin, and a tiny piece of the underlying tissue is removed. The scrotum is stitched closed (sutured). If the testicles are not functioning, the doctor suggests removing them to prevent cancer (orchiectomy) and replacing them with prosthetics. Hormone replacement therapy will not cause testicles to grow where they did not exist before. Administering human chorionic gonadotropin (hCG) and measuring testosterone response may be helpful.
If your daughter’s karyotype contains a Y chromosome, then she is at high risk for developing cancer and the doctor may recommend removal of the ovaries (oophorectomy). Comprehensive specialist medical and psychological advice should be sought if this option is to pursued.
Understand more on Childhood Hypogonadism:
What is childhood Hypogonadism?
More on Hypogonadism in Children and adults
The information in this article has been taken with permission from the official Lawley booklet on Childhood Hypogonadism.