Untitled 1 - Amernorrhea: Mllerian agenesis is a congenital...

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Amernorrhea: Müllerian agenesis is a congenital malformation in women characterised by a failure of the Müllerian ducts to develop, resulting in a missing uterus and variable malformations of the vagina . It is the second most common cause of primary amenorrhea . The condition is also called MRKH or Mayer-Rokitansky-Küster-Hauser Syndrome , named after August Franz Joseph Karl Mayer , Carl Freiherr von Rokitansky , Hermann Küster , and G.A.Hauser . Contents [ hide ] 1 Signs and sympto ms 2 Prevale nce 3 Treatm ent 4 See also 5 Referen ces 6 Externa l links [ edit ] Signs and symptoms A woman with this condition is hormonally normal; that is, she will enter puberty with development of secondary sexual characteristics including thelarche (breasts) and adrenarche (pubic hair). Her chromosome constellation will be 46,XX. Ovulation usually occurs. Typically, the vagina is shortened and intercourse may in some cases be difficult and painful. Medical examination supported by gynecologic ultrasonography demonstrates a complete or partial absence of the cervix , uterus , and vagina . If there is no uterus, women with MRKH cannot carry a pregnancy. However, it is possible for these women to have genetic offspring by in vitro fertilisation (IVF) and surrogacy . Uterine transplantation is currently not a treatment that can be offered as the technology is still in its infancy. Women with MRKH typically discover the condition when, during puberty years, the menstrual cycle does not start. Some women find out earlier through surgeries for other conditions, such as a hernia.
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Other, less common symptoms include kidney problems, hearing loss, and bone malformations. Renal anomalies occur in 25-35% of females with Müllerian agenesis. In 1988, a 15-year-old girl living in the small southern African nation of Lesotho came to local doctors with all the symptoms of a woman in labor. The doctors were puzzled, however, because she did not have a vagina, only a shallow skin dimple. Doctors traced her pregnancy to a knife wound to her abdomen 278 days earlier, after she had practiced fellatio on her boyfriend. The sperm had leaked from her stomach to her abdominal cavity and fertilized one of her eggs. This case was reported by Dr. Richard Paulson, head of the University of Southern California Fertility Program in Los Angeles, February 3, 2010. [ edit ] Prevalence The estimated prevalence is 1 in 5000 women. Queen Amalia of Greece was found postmortem to have had the syndrome. Her barrenness contributed to the overthrow of her husband, King Otto of Greece . [ edit ] Treatment Although there are treatments to increase the comfort in sexual intercourse, there are none to let the woman herself become pregnant. There are plans by UK and Swedish doctors for a uterine transplant that would allow these women to carry their own child, but no transplant has yet been successful in producing a child. Since the women do have ovaries, women with this condition can
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This note was uploaded on 12/10/2011 for the course ACCT Accountanc taught by Professor Jenkins during the Spring '11 term at Sacread Heart University.

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Untitled 1 - Amernorrhea: Mllerian agenesis is a congenital...

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