Mayer Rokitansky Küster Hauser Syndrome; condition that affects the reproductive system скачать в хорошем качестве

Mayer Rokitansky Küster Hauser Syndrome; condition that affects the reproductive system

🧬 Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome: A Rare Congenital Condition MRKH syndrome is a congenital disorder affecting females, characterized by the underdevelopment or absence of the uterus and upper vagina, despite normal external genitalia and functioning ovaries. It’s typically discovered during adolescence when menstruation fails to begin (primary amenorrhea). 🧠 What Happens in MRKH Syndrome? The Müllerian ducts, which normally form the uterus, cervix, and upper vagina, fail to develop properly during fetal life. Ovaries and external genitalia are usually normal, so puberty progresses with breast development and pubic hair. Most individuals have a 46,XX karyotype and normal female hormone levels. 📊 Types of MRKH Syndrome Type Description Type I (Isolated) Only the uterus and upper vagina are affected. Type II (Associated) Includes malformations of kidneys, spine, ears, or heart. Also known as MURCS association. ⚠️ Symptoms No menstrual periods by age 15–16 (primary amenorrhea) Pain or difficulty during intercourse due to a shortened vaginal canal Possible kidney anomalies, scoliosis, or hearing loss (in Type II) 🧪 Diagnosis Physical exam: Short or absent vaginal canal Imaging: Ultrasound or MRI to assess internal reproductive organs and kidneys Genetic testing: May be done to explore associated anomalies 🛠️ Treatment Options Goal Approach Create functional vagina Vaginal dilation or surgical vaginoplasty Fertility options IVF with gestational surrogacy or experimental uterine transplant Emotional support Counseling and support groups for coping and identity 🌱 Living with MRKH Individuals can lead full lives, including sexual relationships and parenthood through assisted reproductive technologies. Emotional and psychological support is crucial—many find strength in community and advocacy groups. #Syndrome