Mayer-Rokitansky-Kuster-Hauser syndrome

(redirected from MRKH Syndrome)

May·er-Ro·ki·tan·sky-Küs·ter-Hau·ser syn·drome

(mī'yĕr rō-kĭ-ton'skē kēs'tĕr hoyz'er),
primary amenorrhea due to müllerian duct agenesis, resulting in absence of the vagina, or presence of a short vaginal pouch, and absence of the uterus with normal karyotype and ovaries.

Mayer-Rokitansky-Kuster-Hauser syndrome

A female-limited, autosomal dominant embryopathy (OMIM:277000) characterised by impaired müllerian duct differentiation, resulting in a rudimentary and cordlike uterus and vaginal agenesis with normal ovaries and normal femininising puberty, as well as primary amenorrhoea due to müllerian duct agenesis due to the defective uterus.

Frequency
1:5000 live female births.

Molecular pathology
LHX1, which encodes a homeobox transcription factor that plays a key role in the development of the renal and urogenital systems, is a candidate gene for Mayer-Rokitansky-Kuster-Hauser syndrome.

Hauser,

G.A., 20th century German gynecologist.
Mayer-Rokitansky-Küster-Hauser syndrome - see under Mayer, Paul
Rokitansky-Küster-Hauser syndrome - Synonym(s): Mayer-Rokitansky-Küster-Hauser syndrome

Küster,

Herman, early 20th century German gynecologist.
Mayer-Rokitansky-Küster-Hauser syndrome - see under Mayer, Paul
Rokitansky-Küster-Hauser syndrome - Synonym(s): Mayer-Rokitansky-Küster-Hauser syndrome

Mayer,

Paul, German histologist, 1848-1923.
Mayer hemalum stain - a progressive nuclear stain also used as a counterstain.
Mayer mucicarmine stain
Mayer mucihematein stain
Mayer-Rokitansky-Küster-Hauser syndrome - congenital absence of the vagina. Synonym(s): Rokitansky-Küster-Hauser syndrome

Rokitansky,

Karl Freiherr von, Austrian pathologist, 1804-1878.
Mayer-Rokitansky-Küster-Hauser syndrome - see under Mayer, Paul
Rokitansky disease - (1) Synonym(s): acute yellow atrophy of the liver - (2) Chiari syndrome
Rokitansky diverticulum
Rokitansky hernia - separation of the muscular fibers of the bowel allowing protrusion of a sac of the mucous membrane.
Rokitansky kidney
Rokitansky pelvis - Synonym(s): spondylolisthetic pelvis
Rokitansky tumor
Rokitansky-Aschoff sinuses - small outpocketings of the mucosa of the gallbladder which extend through the muscular layer.
Rokitansky-Küster-Hauser syndrome - Synonym(s): Mayer-Rokitansky-Küster-Hauser syndrome
References in periodicals archive ?
Generally, patients of the MRKH syndrome undergo a procedure called vaginoplasty that helps create a ( neo) vagina.
Type I MRKH syndrome is characterised by the absence of the proximal two-thirds of the vagina and the uterus, while type II affects additional parts of the body.
Congenital uterovaginal agenesis is most common in women with MRKH syndrome.
MRKH syndrome is one of the most common causes of primary amenorrhea (i.
For, the 45- year- old was the surrogate for her daughter Bhavika Kathwadia, who can't bear children as she is afflicted by Mayer- Rokitansky- Kuster- Hauser Syndrome, or MRKH Syndrome.