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https://www.readbyqxmd.com/read/29751094/the-interest-of-women-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-and-laparoscopic-vecchietti-neovagina-in-uterus-transplantation
#1
Roman Chmel, Marta Novackova, Zlatko Pastor, Jiri Fronek
STUDY OBJECTIVE: The goal of this study was to assess a group of Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) women with surgically created neovaginas in the interest of uterus transplantation (UTx) and to recruit the first group of applicants for a uterus transplant trial. DESIGN: and Setting: This is an original prospective study using semistructured interviews. PARTICIPANTS: A study group of 50 MRKH syndrome women with Vecchietti neovaginas was recruited by letter of invitation...
May 8, 2018: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/29721502/diagnosis-and-management-of-ovarian-tumor-in-mayer-rokitansky-k%C3%A3-ster-hauser-mrkh-syndrome
#2
REVIEW
Yali Miao, Jirui Wen, Liwei Huang, Jiang Wu, Zhiwei Zhao
In the most recent publications on Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, the uterine remnants and ovaries in patients may develop uterine remnant leiomyoma, adenomyosis, or ovarian tumor, and this can lead to problems in differential diagnosis. Here we summarize the diagnosis methods and available interventions for ovarian tumor in MRKH syndrome, with emphasis on the relevant clinical findings and illustrative relevant case. According to the clinical findings and illustrative relevant case, with the help of imaging techniques, ovarian tumors can be detected in the pelvis in patients with MRKH syndrome and evaluated in terms of size...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29692510/management-of-female-congenital-short-patulous-urethra-with-urethral-tapering-and-pubovaginal-sling-a-report-of-two-cases
#3
Sumit Gahlawat, Hemant Goel, Nikhil Khattar
Congenital short patulous urethra is a rare entity and may be associated with developmental anomalies of mullerian ducts or urogenital sinus. We report the management of two cases of congenital short patulous urethra with stress urinary incontinence (SUI). Both the patients presented with SUI; one was diagnosed with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome and the other had uterus didelphys with longitudinally septated vagina. Both patients were successfully managed by excisional tapering of the urethra and pubovaginal sling placement...
April 2018: Indian Journal of Urology: IJU: Journal of the Urological Society of India
https://www.readbyqxmd.com/read/29582123/increased-incidence-of-abnormally-located-ovary-in-patients-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-a-retrospective-analysis-with-magnetic-resonance-imaging
#4
Yue Wang, Jingjing Lu, Lan Zhu, Rong Chen, Bo Jiang, Bo Hou, Feng Feng, Zhengyu Jin
PURPOSE: To explore the incidence of abnormally located ovary in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome as compared to a control group with magnetic resonance imaging. METHODS: Pelvic MR images of 83 MRKH patients and 60 age-matched normal females were retrospectively reviewed by two experienced gynecological radiologists in consensus. Characteristics including location of ovary, ovarian volume, and follicle counts were assessed. The incidence of abnormally located ovary was compared between MRKH patients and controls...
March 26, 2018: Abdominal Radiology
https://www.readbyqxmd.com/read/29581694/mayer-rockitansky-kuster-hauser-syndrome-presenting-as-irreducible-inguinal-hernia
#5
Radha Verma, Rajul Shah, Swarup Anand, Chirag Vaja, Kiran Gaikwad
Inguinal hernia is the most common type of hernia experienced in the surgical OPD due to the muscular anatomy of the region being weak along with the natural weaknesses like deep ring with indirect hernia being more common than direct. Inguinal hernias may have varying unusual sac contents, a case presentation of rudimentary uterus and ovaries as contents of inguinal hernia in an adult female with Mayer-Rokitansky-Kuster-Hauser (MRKH) type II syndrome. This syndrome may be attributed to abnormal development of blastema of cervicothoracic somites and pronephriducts...
February 2018: Indian Journal of Surgery
https://www.readbyqxmd.com/read/29534376/-development-and-validation-of-the-chinese-version-of-modified-body-imgae-scale-in-chinese-population
#6
X X Gao, L Zhu, S J Yu, T Xu
Objective: To develop the Chinese version of modified body image scale (MBIS) questionnaires, and to validate them in Chinese population. Methods: The original English MBIS questionnaire was translated into Chinese, following the WHO cross-cultural adaptation of health-related quality of life measures. The reliability and validity of the Chinese version of MBIS questionnaires were evaluated in Chinese population, MRKH syndrome patients. Results: Totally 50 patients with MRKH syndrome completed the MBIS and short-form 12-item health survey (SF-12) questionnaires...
February 25, 2018: Zhonghua Fu Chan Ke za Zhi
https://www.readbyqxmd.com/read/29527097/clinical-and-genetic-aspects-of-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#7
REVIEW
Susanne Ledig, Peter Wieacker
The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome [MIM 277000] is characterised by the absence of a uterus and vagina in otherwise phenotypically normal women with karyotype 46,XX. Clinically, the MRKH can be subdivided into two subtypes: an isolated or type I form can be delineated from a type II form, which is characterised by extragenital malformations. The so-called Müllerian hypoplasia, renal agenesis, cervicothoracic somite dysplasia (MURCS) association can be seen as the most severe phenotypic outcome...
2018: Medizinische Genetik: Mitteilungsblatt des Berufsverbandes Medizinische Genetik E.V
https://www.readbyqxmd.com/read/29434669/copy-number-variation-and-regions-of-homozygosity-analysis-in-patients-with-m%C3%A3-llerian-aplasia
#8
Durkadin Demir Eksi, Yiping Shen, Munire Erman, Lynn P Chorich, Megan E Sullivan, Meric Bilekdemir, Elanur Yılmaz, Guven Luleci, Hyung-Goo Kim, Ozgul M Alper, Lawrence C Layman
Background: Little is known about the genetic contribution to Müllerian aplasia, better known to patients as Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. Mutations in two genes ( WNT4 and HNF1B ) account for a small number of patients, but heterozygous copy number variants (CNVs) have been described. However, the significance of these CNVs in the pathogenesis of MRKH is unknown, but suggests possible autosomal dominant inheritance. We are not aware of CNV studies in consanguineous patients, which could pinpoint genes important in autosomal recessive MRKH...
2018: Molecular Cytogenetics
https://www.readbyqxmd.com/read/29415121/mayer-rokitansky-kuster-hauser-syndrome-a-unique-case-presentation
#9
Bao T Nguyen, Katherine L Dengler, Rhiana D Saunders
Background: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a congenital condition characterized by aplasia of the vagina with or without concurrent uterine and/or cervical aplasia. Type II (MURCS) is a rare form involving MUllerian agenesis, Renal agenesis, and Cervicothoracic Somite anomalies. Case: A 17-yr-old virginal female presented for evaluation of primary amenorrhea and pelvic pain. Her medical history was significant for a bicuspid aortic valve and right radial dysplasia...
February 5, 2018: Military Medicine
https://www.readbyqxmd.com/read/29366966/laparoscopic-assisted-uterus-retrieval-from-live-organ-donors-for-uterine-transplant-our-experience-of-two-patients
#10
Shailesh Puntambekar, Milind Telang, Pankaj Kulkarni, Seema Puntambekar, Sanjeev Jadhav, Mangesh Panse, Ravindra Sathe, Nikhil Agarkhedkar, Neeta Warty, Sandesh Kade, Manoj Manchekar, Hirav Parekh, Kajal Parikh, Riddhi Desai, Mehul Mehta, Mihir Chitale, Bhushan Kinholkar, Joy Shankar Jana, Avinash Pare, Abhay Sadre, Swapnil Karnik, Abhay Mane, Giriraj Gandhi, Shailendra Kanade, Uday Phadke
STUDY OBJECTIVE: To report the first ever laparoscopic-assisted live donor uterus retrieval in 2 patients for uterus transplant. DESIGN: Case study (Canadian Task Force classification III). SETTING: Galaxy CARE Laparoscopy Institute, Pune, India. PATIENTS: Two patients with absolute uterine factor infertility with their mothers as donors. INTERVENTIONS: In vitro fertilization and uterine transplant...
January 31, 2018: Journal of Minimally Invasive Gynecology
https://www.readbyqxmd.com/read/29234256/congenital-malformations-of-the-reproductive-tract-in-a-patient-with-poland-syndrome-is-there-a-connection
#11
Tian Meng, Ming Bai, Ru Zhao
Background: Poland syndrome and Müllerian duct agenesis (Mayer-Rokitansky-Küster-Hauser(MRKH)-like syndrome) are two rare congenital anomalies with unclear etiology. Case Report: An 18-year-old female presented with hypoplasia of the left pectoralis muscles and subcutaneous tissue, hypoplasia of the left breast and nipple, and dextrocardia, typical in those with a severe form of Poland syndrome. She also showed aplasia of the vagina and uterus, bilateral ovarian agenesis, and hyperandrogenism, presenting as MRKH-like syndrome...
October 2017: Breast Care
https://www.readbyqxmd.com/read/29178543/neovaginal-construction-with-pelvic-peritoneum-reviewing-an-old-approach-for-a-new-application
#12
Michael W Slater, Xochitl Vinaja, Islam Aly, Marios Loukas, Mark Terrell, Justine Schober
Transgender reassignment surgeries have become a major topic of interest within the last decade. Although there are numerous surgical techniques for male-to-female reassignment, a physician must assess each patient's anatomy and past medical history to determine the most suitable technique. Additionally, patient preference is important. The neovaginal constructive technique most commonly used in male-to-female reassignment surgery is penile skin inversion, but various other techniques and tissues can also be used including pelvic peritoneum, buccal mucosa, and bowel...
March 2018: Clinical Anatomy
https://www.readbyqxmd.com/read/29177592/hyperandrogenemia-and-ovarian-reserve-in-patients-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-type-1-and-2-potential-influences-on-ovarian-stimulation
#13
Melanie Henes, Ludmila Jurow, Andreas Peter, Birgitt Schoenfisch, Florin Andrei Taran, Markus Huebner, Harald Seeger, Sara Yvonne Brucker, Kristin Katharina Rall
PURPOSE: This study aimed to analyze the hormone profiles, to detect the rate of hyperandrogenemia and to investigate the potential effect of Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) on ovarian reserve, as reflected by the serum Anti-Mullerian hormone (AMH) levels. Clinical implications were analyzed by including our own experiences with three patients after ovarian stimulation in preparation for uterus transplantation. METHODS: Serum samples of 100 patients with MRKHS (50 patients with MRKHS type 1 and 50 with type 2) were analyzed and compared to 50 individually age-matched healthy controls...
February 2018: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/29133152/laparoscopic-assisted-uterus-retrieval-from-live-organ-donors-for-uterine-transplant
#14
Shailesh Puntambekar, Milind Telang, Pankaj Kulkarni, Sanjeev Jadhav, Ravindra Sathe, Neeta Warty, Seema Puntambekar, Sandesh Kade, Mangesh Panse, Nikhil Agarkhedkar, Giriraj Gandhi, Manoj Manchekar, Hirav Parekh, Kajal Parikh, Riddhi Desai, Mehul Mehta, Mihir Chitale, Sambit Nanda
STUDY OBJECTIVE: The authors present the first ever laparoscopic-assisted uterus retrieval in a live donor for uterus transplant. DESIGN: A step-by-step surgical demonstration. SETTING: Galaxy CARE Laparoscopy Institute, Pune, India. PATIENTS: Two patients, ages 21 and 26 years, with Mayer-Rokitansky-Küster-Hauser syndrome and Asherman syndrome, respectively, with their mothers as donors. INTERVENTIONS: A 12-member team was formed...
November 10, 2017: Journal of Minimally Invasive Gynecology
https://www.readbyqxmd.com/read/28960241/intensive-vaginal-dilation-using-adjuvant-treatments-in-women-with-mayer-rokitansky-kuster-hauser-syndrome-retrospective-cohort-study
#15
Ashradha Ketheeswaran, Jennifer Morrisey, Jason Abbott, Michael Bennett, Jan Dudley, Rebecca Deans
AIMS: To evaluate the effect of adjuvants during intensive vaginal dilator therapy for functional and anatomical neovagina creation in women with Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH). METHODS: This retrospective cohort study included 75 women with MRKH undergoing intensive vaginal dilator treatment between 2000 and 2014. One specialist nurse performed non-surgical vaginal dilation aided by adjuvants, during inpatient admissions for several dilation sessions per day...
February 2018: Australian & New Zealand Journal of Obstetrics & Gynaecology
https://www.readbyqxmd.com/read/28932808/two-sisters-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-and-serous-adenocarcinoma-of-the-ovary
#16
Sarah P Huepenbecker, Laura Divine, Christina S Chu, David G Mutch
BACKGROUND: Mayer-Rokitansky-Küster-Hauser syndrome is a rare entity with proposed genetic underpinnings. Ovarian carcinoma has well-described genetic associations and syndromes, although much of the etiology of the disease remains unknown. CASES: Two sisters present in the 1970s with primary amenorrhea, 46, XX karyotypes, and absent uteri consistent with MRKH syndrome. In the 2010s, both sisters again present for care. Case 1 presents one sister with stage IIIC serous ovarian adenocarcinoma and negative BRCA panel...
November 2017: Gynecologic Oncology Reports
https://www.readbyqxmd.com/read/28922305/mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-with-bilateral-ovarian-sertoli-cell-tumors-review-of-the-literature-and-report-of-a-rare-case
#17
Alexander L Juusela, Ilana Naghi, Suresh Thani
BACKGROUND: Patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome are infertile secondary to hypoplasia or complete agenesis of the uterus, yet they remain at risk of primary neoplasms of the ovaries because embryologically the uterus and ovaries develop via separate mechanisms. CASE: A 72-year-old nulliparous woman with a history of primary amenorrhea underwent an exploratory laparotomy for a suspected uterine fibroid. In addition to the pelvic mass, the patient was found to have findings consistent with MRKH syndrome...
September 15, 2017: Female Pelvic Medicine & Reconstructive Surgery
https://www.readbyqxmd.com/read/28913139/mayer-rokitansky-kuster-hauser-syndrome-associated-with-rectovestibular-fistula
#18
Charu Tiwari, Hemanshi Shah, Mukta Waghmare, Kiran Khedkar
A female neonate with two openings in the introitus and an absent anal opening at the anal site presents a diagnostic challenge. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome associated with rectovestibular fistula, though rare, should be kept in mind as a differential diagnosis of this presentation. We present such a case in a one-year-old female child with MRKH syndrome and rectovestibular fistula.
March 2017: Turkish Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28893003/spectrum-of-mri-appearance-of-mayer-rokitansky-kuster-hauser-mrkh-syndrome-in-primary-amenorrhea-patients
#19
Deb Kumar Boruah, Shantiranjan Sanyal, Bidyut Bikash Gogoi, Kangkana Mahanta, Arjun Prakash, Antony Augustine, Sashidhar Achar, Hiranya Baishya
INTRODUCTION: Mayer-Rokitansky-Kuster-Hauser (MRKH) synd-rome is a malformation in female genital tract due to interrupted embryonic development of para-mesonephric ducts leading to uterine and proximal vagina aplasia or hypoplasia. AIM: To analyse the MRI findings in females suspected of MRKH syndrome in a primary amenorrhea workup. MATERIALS AND METHODS: A cross-sectional study comprising of 11 patients of MRKH syndrome presented in a tertiary care centre from March 2016 to February 2017 evaluated in 1...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28851338/a-modified-neo-vagina-procedure-in-a-low-resource-urogynecological-unit-a-case-report-of-a-21%C3%A2-year-old-with-mayer-rokitansky-k%C3%A3-ster-hauser-mrkh-syndrome-operated-at-mbarara-referral-hospital-southwestern-uganda
#20
Musa Kayondo, Joseph Njagi, Peter Kivuniike Mukasa, Tom Margolis
BACKGROUND: Although vaginal agenesis as may occur in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare condition, it is associated with not only anatomical problems but also serious psychological and social problems like painful sexual intercourse, primary amenorrhea and infertility. Surgery, which is aimed at reconstruction of a vagina of adequate length and width to serve the function, is the main method of treatment. Many methods for vaginal reconstruction have been described but each has its complications and limitations...
August 29, 2017: BMC Urology
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