Disorders of sex hormones

The table below summarises the findings in patients who have disorders of sex hormones:

Disorder LH Testosterone
Primary hypogonadism (Klinefelter's syndrome) High Low
Hypogonadotrophic hypogonadism (Kallman's syndrome) Low Low
Androgen insensitivity syndrome High Normal/high
Testosterone-secreting tumour Low High

Klinefelter's syndrome

Klinefelter's syndrome is associated with karyotype 47, XXY

Features

Diagnosis is by chromosomal analysis

Kallman's syndrome

Kallman's syndrome is a recognised cause of delayed puberty secondary to hypogonadotrophic hypogonadism. It is usually inherited as an X-linked recessive trait. Kallman's syndrome is thought to be caused by failure of GnRH-secreting neurons to migrate to the hypothalamus.

The clue given in many questions is lack of smell (anosmia) in a boy with delayed puberty

Features