How to treat Cranfield syndrome?

summary

Congenital seminiferous tubule hypoplasia syndrome, also known as Klinefelter syndrome (Klinefelter syndrome), is a common genetic disease of sex chromosome aberration. The features of the disease are similar to that of the patients without testis, male breast development, small testis, azoospermia and increased gonadotropin in urine. The sex chromosome of the patients with this disease is 47, XXY, which is one more X chromosome than that of normal men, so this disease is also called 47, XXY syndrome. Now let's talk about how to treat Cranfield syndrome?

How to treat Cranfield syndrome?

1. Patients with congenital seminiferous tubule hypoplasia syndrome have no abnormality in childhood, but often appear abnormality in adolescence or adulthood. The patient has a higher body shape, slender lower limbs, delicate skin, sharp voice, no beard, less body hair, etc. About half of the patients had bilateral breast hypertrophy. The external genitalia is often normal male like, but the penis is shorter than normal male, the testicles on both sides are significantly smaller, mostly less than 3 cm, hard texture, poor sexual function, semen without sperm, patients often seek treatment because of infertility or low sexual function. Intelligence development is normal or slightly low.

2. Generally, it is difficult to make a diagnosis before the development period. Infertility or sexual dysfunction are the main reasons for patients to see a doctor. The typical symptoms are high body size, small bilateral testicles and hypertrophy of bilateral breasts. X-Body positive, karyotype 47, XXY can be diagnosed.

3. Some cases decreased. Due to the increase of sex hormone binding globulin (SHBG), the total plasma testosterone can be in the normal range, which can not reflect the androgen level, and the bioactive free testosterone is decreased.

matters needing attention

Long term supplement of male hormone to improve the secondary sexual characteristics, but the effect is not ideal. Testosterone propionate (testosterone propionate) or methyltestosterone (methyltestosterone) tablets are usually taken sublingually. More convenient is to give long-acting testosterone, such as testosterone heptanoate or testosterone cyclopentyl propionate. HCG may also be given at the same time. The medicine only has certain help to masculinity, but it can't change the female breast, so for the breast hypertrophy, the breast and fat tissue can be removed.