The topic of Matsumi Ladies Clinic Mita English blog in June is about the nose.

In the final part, I create a slightly scientific article about a disease of the nose, which is accompanied with a gynecological disorder from a medical perspective.

 

The name of this disease is Kallmann syndrome, the symptom of which is impairment of starting or fully completing puberty.

Kallmann syndrome is a genetic disorder, which is categorized in the group of hypogonadotropic hypogonadism.

The additional unique symptom of Kallmann syndrome is lack or reduced sense of smell.

Impaired puberty causes poorly defined secondary sexual characteristics, infertility and a resulting increased risk of developing osteoporosis due to low levels of the sex hormones, estrogen.

 

The underlying mechanism of this disease is a failure in the correct production or activity of gonadotropin-releasing hormone by the hypothalamus in the brain.

This causes low levels of estrogen along with progesterone in female.

Specialized fertility treatments and hormone replacement therapy (HRT) is required for the patients.

 

An estimated incidence of Kallmann syndrome is reported to be 1 in about 50,000 people.

It is normally difficult to distinguish a case of Kallmann syndrome from a straightforward constitutional delay of puberty.

However, if puberty has not started and one or more of the non-reproductive features mentioned below are present, then a referral to gynecologists might be recommended.

The features are markedly reduced sense of smell, color-blindness and midline cranio-facial defects such as cleft palate and cleft lip.