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Mullerian agenesis

Mullerian agenesis refers to a condition in a female where the mullerian ducts fail to develop and a uterus will not be present. Primary amenorrhea is a leading symptom. more...

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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 and adrenarche. Her chromosome constellation will be 46,XX. Ovulation usually occurs. Typically the vagina is shortened and intercourse will be difficult and painful. Medical examination supported by gynecologic ultrasonography demonstrates vaginal and uterine absence.

Prevalence

The estimated prevalence is 1:5000. A genetic cause is likely (see OMIM).

Treatment

It may be necessary to use vaginal dilators or surgery to develop a functioning vagina to allow for satisfactory sexual intercourse. Women with this condition can have children through IVF with embryo transfer to a gestational carrier.

Other

The condition is also called Mayer-Rokitansky-Küstner-Hauser (MRKH) Syndrome, named after August Franz Joseph Karl Mayer, Carl Freiherr von Rokitansky, Hermann Küster, and G.A.Hauser.

Read more at Wikipedia.org


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Don't overlook diagnosis of mullerian agenesis.(Gynecology) : An article from: OB GYN News $5.95

Don't overlook diagnosis of mullerian agenesis
PORTLAND, ORE. -- Some cases of apparent imperforate hymen are actually mullerian agenesis, and that possibility should be in the differential diagnosis, ...
ACOG opinion paper on vaginal agenesis - American College of Obstetricians and Gynecologists Committee on Adolescent Health Care - Brief Article
The Committee on Adolescent Health Care of the American College of Obstetricians and Gynecologists (ACOG) has issued an opinion paper on nonsurgical ...
Endometriosis is easily missed in adolescents - Typically Early-Stage Disease
BIG SKY, MONT. -- Endometriosis in adolescents has a very different appearance than in adults, Dr. Joseph S. Sanfilippo said at an ob.gyn. update sponsored ...
Diagnosing Pubertal Developmental Disorders - Rare But Often Overlooked
BALTIMORE -- If you don't particularly like evaluating the 17-year-old who has not reached menarche yet, refer her to an expert, Dr. Leo Plouffe Jr.

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