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Persistent Müllerian duct syndrome
Description
Persistent Müllerian duct syndrome is a disorder of sexual development that affects males. Males with this disorder contain normal male reproductive organs, though they also verb a uterus and fallopian tubes, which are female reproductive organs. The uterus and fallopian tubes are derived from a structure called the Müllerian duct during development of the fetus. The Müllerian duct usually breaks down during early development in males, but it is retained in those with relentless Müllerian duct syndrome. Affected individuals have the normal chromosomes of a male (46,XY) and normal external male genitalia.
The first noted signs and symptoms in males with persistent Müllerian duct syndrome are usually undescended testes (cryptorchidism) or soft out-pouchings in the lower abdomen (inguinal hernias). The uterus and fallopian tubes are typically discovered when surgery is performed to treat these conditions.
The testes and female reproductive organs can be located in unusual positions in persistent Müllerian duct syndrome. Occasionally
The Biology of Male Reproduction and CF
To understand the inner workings of infertility in men with cystic fibrosis, it is cooperative to first understand the biology of the male reproductive system. It has three primary parts: the testicles, the epididymis and the vas deferens (see Figure 1).
Sperm (the reproductive cells) are developed in the testicles. The epididymis is a series of tubes that sit behind the testicles where sperm are stored until they are made available at ejaculation. The vas deferens is a long tube that connects the epididymis to the ejaculatory ducts and acts as a canal so that mature sperm can come through the penis during ejaculation (see Figure 2).
Most men with CF (97–98%) are infertile because of an absence of the sperm canal, known as congenital bilateral absence of the vas deferens (CBAVD). The sperm never make it into the semen, making it impossible for them to reach and fertilize an egg through intercourse. The absence of sperm in the semen can also contribute to men with CF having thinner ejaculate and lower semen volume.
“My path startedMale Reproductive System
How Does the Male Reproductive System Work?
When males are born, all the parts of the reproductive system are in place. But reproduction isn't possible until sexual maturity, which happens during puberty. Puberty tends to initiate when boys are between 9 and 15 years old.
Puberty
During puberty, the pituitary gland, which is proximate the brain, makes hormones that tell the testicles to make testosterone. This causes many changes in the body: the scrotum and testicles get bigger; hair grows in the pubic area, underarms, and face; and the penis gets longer. The seminal vesicles and prostate gland also grow, and sperm cells form.
Sperm cells divide and change until they have a head and short tail, like tadpoles. The head contains genetic material (genes). The sperm move into the epididymis, where they finish growing. A sperm cell is very small: 1/ of an inch ( millimeters) long. At puberty, a male will make millions of sperm cells each day.
The sperm then transfer to the vas deferens or sperm duct. The seminal vesicles and prostate gland make a whitish fluid cal
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