Pediatric Kallmann Syndrome
Kallmann syndrome is a condition that causes delayed or absent puberty and an impaired sense of smell.
Kallmann syndrome is one form of hypogonadotropic hypogonadism, a condition in which the body does not produce enough hormones needed for sexual development. Normally, hormones made in the hypothalamus of the brain direct the body to develop secondary sex characteristics during puberty. When a child has Kallmann syndrome, they experience delayed or absent puberty and an impaired sense of smell.
- In boys, secondary sex characteristics means they may not develop facial hair and their voice may not deepen. They may also have undescended testes and/or micropenis.
- In girls, secondary sex characteristics means they may not experience breast development or the start of menstruation.
Boys and girls with Kallmann syndrome will not experience the typical growth spurt seen at puberty (between the ages of 10 and 14 for girls and between the ages of 12 and 16 for boys).
In addition to affecting sexual development, Kallmann syndrome affects the sense of smell. A child with Kallmann syndrome may have diminished sense of smell or may not be able to smell at all. Many times, a child does not realize that their sense of smell is impaired.
Kallmann syndrome happens when the hypothalamus in the brain is unable to release the gonadotropin releasing hormone. At the same time, the pituitary gland is unable to release the luteinizing hormone (LH) and follicle stimulating hormone (FSH) hormones, which activate the ovaries and testes during puberty.
The symptoms of Kallmann syndrome may include:
- Absent or diminished sense of smell
- Cleft lip and palate
- Dental problems
- Hand movements that mirror each other
- Hearing problems
- Lack of menstruation (in girls of menstruating age)
- Lack of secondary sex characteristics
- Micropenis (in boys)
- Poor balance
- Renal agenesis (only one kidney developed)
- Shorter than average stature for age
- Undescended testes (in boys)