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https://www.readbyqxmd.com/read/28932808/two-sisters-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-and-serous-adenocarcinoma-of-the-ovary
#1
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
#2
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
#3
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: Turk J Obstet Gynecol
https://www.readbyqxmd.com/read/28893003/spectrum-of-mri-appearance-of-mayer-rokitansky-kuster-hauser-mrkh-syndrome-in-primary-amenorrhea-patients
#4
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
#5
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
https://www.readbyqxmd.com/read/28821413/uterine-transplantation-surgical-innovation-in-the-treatment-of-uterine-factor-infertility
#6
REVIEW
R Flyckt, A Davis, R Farrell, S Zimberg, A Tzakis, T Falcone
Uterine factor infertility (UFI) is a condition that affects thousands of women and is estimated to have a prevalence as high as one in five hundred reproductive-aged women. A wide range of circumstances can lead to UFI and include women with congenital absence of a uterus (Mayer Rokitansky Kuster Hauser or MRKH syndrome), women who have undergone iatrogenic removal of the uterus, or women who have uteri that are in situ but have been damaged by infection or surgical instrumentation. There have been 17 published reports of human uterine transplantation in the world...
August 15, 2017: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
https://www.readbyqxmd.com/read/28674963/evaluation-of-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-with-magnetic-resonance-imaging-three-patterns-of-uterine-remnants-and-related-anatomical-features-and-clinical-settings
#7
Yue Wang, Jingjing Lu, Lan Zhu, Zhijing Sun, Bo Jiang, Feng Feng, Zhengyu Jin
OBJECTIVE: To characterize the anatomical features and clinical settings of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and correlate them with patterns of uterine involvement. METHODS: Pelvic magnetic resonance images and medical records of 92 MRKH patients were retrospectively reviewed. Patients were subgrouped by uterine morphology: uterine agenesis, unilateral rudimentary uterus and bilateral rudimentary uteri. Uterine volume, presence of endometrium, location of ovary, endometriosis and pelvic pain were compared among groups...
July 3, 2017: European Radiology
https://www.readbyqxmd.com/read/28624115/evaluation-of-amnion-in-creation-of-neovagina-in-women-with-mayer-rokitansky-kuster-hauser-syndrome
#8
Richa Vatsa, Juhi Bharti, Kallol Kumar Roy, Sunesh Kumar, Jai Bhagwan Sharma, Neeta Singh, Seema Singhal, Jyoti Meena
OBJECTIVE: To assess the outcome of amnion vaginoplasty in cases of vaginal agenesis due to Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome managed at the authors' institution. DESIGN: Retrospective study. SETTING: Tertiary care hospital. PATIENT(S): Fifty women with MRKH who underwent neovaginoplasty. INTERVENTION(S): Modified McIndoe's vaginoplasty was done in all the patients, using human amnion graft...
August 2017: Fertility and Sterility
https://www.readbyqxmd.com/read/28606777/the-clinical-outcomes-of-vaginoplasty-using-tissue-engineered-biomaterial-mesh-in-patients-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#9
Xiaoli Zhang, Zhongyu Liu, Yizhuo Yang, Yuanqing Yao, Ye Tao
OBJECTIVE: The aim of the study is to evaluate the clinical outcomes of vaginoplasty using tissue-engineered biomaterial mesh in the patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. PATIENTS AND METHODS: Patients with MRKH syndrome underwent the vaginoplasty using tissue-engineered biomaterial mesh between January 2006 and August 2014 in PLA general hospital. We analyzed the anatomic results and collected the standardized FSFI (Female Sexual Function Index) questionnaire from each patient...
June 10, 2017: International Journal of Surgery
https://www.readbyqxmd.com/read/28600106/genetic-analysis-of-mayer-rokitansky-kuster-hauser-syndrome-in-a-large-cohort-of-families
#10
MULTICENTER STUDY
Lacey S Williams, Durkadin Demir Eksi, Yiping Shen, Amy C Lossie, Lynn P Chorich, Megan E Sullivan, John A Phillips, Munire Erman, Hyung-Goo Kim, Ozgul M Alper, Lawrence C Layman
OBJECTIVE: To study the genetic cause of Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH). Although a few candidate genes and genomic domains for have been reported for MRKH, the genetic underpinnings remain largely unknown. Some of the top candidate genes are WNT4, HNF1B, and LHX1. The goals of this study were to: 1) determine the prevalence of WNT4, HNF1B, and LHX1 point mutations, as well as new copy number variants (CNVs) in people with MRKH; and 2) identify and characterize MRKH cohorts...
July 2017: Fertility and Sterility
https://www.readbyqxmd.com/read/28434104/mullerian-dysgenesis-a-critical-review-of-the-literature
#11
REVIEW
Souzana Choussein, Dimitrios Nasioudis, Dimitrios Schizas, Konstantinos P Economopoulos
PURPOSE: To present an update of the genetic, clinical, diagnostic, and therapeutic aspects of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. METHODS: Studies were considered eligible if they have evaluated patients with MRKH syndrome. Eligible articles were identified by a search of MEDLINE bibliographical database from 1950 to August 2016. A purely descriptive approach was adopted concerning all outcomes examined by the individual studies. RESULTS: MRKH syndrome is defined as congenital aplasia of the upper vagina and impairment of uterine development in normal 46XX females...
June 2017: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/28405713/vaginal-vault-prolapse-in-a-patient-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-a-video-case-presentation
#12
Carolyn Botros, Shilpa Iyer, Alexis M Tran, Roger P Goldberg
INTRODUCTION AND HYPOTHESIS: Several reports have described vaginal prolapse in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome patients after creation of a neovagina. To our knowledge, no reports of primary vaginal prolapse of a blind pouch without previous intervention, or surgery for this condition, have been described. METHODS: In this case report, we describe a 19-year-old woman with MRKH and complete prolapse of her shortened vaginal pouch. Surgical correction utilizing permanent suture-based sacrospinous ligament fixation was performed...
April 12, 2017: International Urogynecology Journal
https://www.readbyqxmd.com/read/28397650/-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#13
Morten Herlin, Michael Bjørn Petersen
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital anomaly characterized by uterovaginal agenesis in females with normal secondary sex characteristics and normal karyotype (46,XX). The prevalence of MRKH syndrome is one in 5,000 live female births as recently confirmed by a nationwide population-based study in Denmark. This review kaleidoscopically summarizes the current knowledge of the history, genetics, diagnostics, treatment of vaginal agenesis, psychosexual aspects, and fertility options in MRKH syndrome...
March 27, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/28369817/giant-leiomyoma-with-extensive-myxoid-degeneration-in-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#14
Richa Sharma, Kiran Guleria, Amita Suneja, Sruthi Bhaskaran, Nadeem Tanveer
No abstract text is available yet for this article.
July 2017: International Journal of Gynaecology and Obstetrics
https://www.readbyqxmd.com/read/28278272/additional-regulatory-activities-of-mrkh-for-the-transcriptional-expression-of-the-klebsiella-pneumoniae-mrk-genes-antagonist-of-h-ns-and-repressor
#15
Miguel A Ares, José L Fernández-Vázquez, Sabino Pacheco, Verónica I Martínez-Santos, Ma Dolores Jarillo-Quijada, Javier Torres, María D Alcántar-Curiel, Jorge A González-Y-Merchand, Miguel A De la Cruz
Klebsiella pneumoniae is a common opportunistic pathogen causing nosocomial infections. One of the main virulence determinants of K. pneumoniae is the type 3 pilus (T3P). T3P helps the bacterial interaction to both abiotic and biotic surfaces and it is crucial for the biofilm formation. T3P is genetically organized in three transcriptional units: the mrkABCDF polycistronic operon, the mrkHI bicistronic operon and the mrkJ gene. MrkH is a regulatory protein encoded in the mrkHI operon, which positively regulates the mrkA pilin gene and its own expression...
2017: PloS One
https://www.readbyqxmd.com/read/28263220/ectopic-pelvic-fibroid-in-a-woman-with-uterine-agenesis-and-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#16
Thelina Amaratunga, Iain Kirkpatrick, Yi Yan, Fern Karlicki
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare disorder characterized by aplasia or hypoplasia of the uterus and vagina due to arrest in the development of the müllerian ducts. Women with this syndrome have the normal 46 XX karyotype, normal female secondary sex characteristics, and primary amenorrhea. Only a few cases have been described in the literature where a fibroid develops from a rudimentary, nonfunctioning uterus in patients with MRKH syndrome. In even rarer instances, a fibroid can develop in patients with a congenitally absent uterus...
September 2017: Ultrasound Quarterly
https://www.readbyqxmd.com/read/28246310/hyperandrogenemia-and-high-prolactin-in-congenital-utero-vaginal-aplasia-patients
#17
Patricia G Oppelt, Andreas Mueller, Liana Stephan, Ralf Dittrich, Johannes Lermann, Christian Büttner, Arif B Ekici, Gabi Conzelmann, Harald Seeger, Dorit Schöller, Katharina Rall, Matthias Wilhelm Beckmann, Pamela Lynn Strissel, Sara Brucker, Reiner Strick
Patients with the Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) have a congenital utero-vaginal-cervical aplasia, but normal or hypoplastic adnexa and develop with normal female phenotype. Although reports mostly demonstrated regular steroid hormone levels in small MRKH cohorts, single MRKH patients with hyperandrogenemia and clinical presentation, like hirsutism and acne has also been shown. Genetically a correlation of WNT4 mutations with singular MRKH patients and hyperandrogenemia was noted. This present study analyzed the hormone status of 215 MRKH patients by determining the levels of luteinizing hormone (LH), follicle stimulating hormone (FSH), estradiol, 17-OH progesterone, testosterone, dehydroepiandrosterone sulfate (DHEAS), sex hormone-binding globulin (SHBG) and prolactin in order to determine the incidence of hyperandrogenemia and hyperprolactinemia in MRKH patients...
February 28, 2017: Reproduction: the Official Journal of the Society for the Study of Fertility
https://www.readbyqxmd.com/read/28216916/a-rare-case-of-46-xx-gonadal-dysgenesis-and-mayer-rokitansky-kuster-hauser-syndrome
#18
Sriharibabu Manne, C H Veeraabhinav, Mounica Jetti, Yalamanchali Himabindu, Kiranmai Donthu, Mutyalarayudu Badireddy
46,XX gonadal dysgenesis is a rare genetically heterogeneous disorder characterized by underdeveloped ovaries with consequent, impuberism, primary amenorrhea, and hypergonadotropic hypogonadism. Mullerian agenesis or Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper part (2/3) of the vagina in a woman with normal development of secondary sexual characteristics and a normal 46,XX karyotype. The phenotypic manifestations of MRKH syndrome may sometimes overlap with various other syndromes and require accurate delineation...
October 2016: Journal of Human Reproductive Sciences
https://www.readbyqxmd.com/read/28216131/assessing-the-experience-of-vaginal-dilator-use-and-potential-barriers-to-ongoing-use-among-a-focus-group-of-women-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#19
Oluyemisi A Adeyemi-Fowode, Jennifer E Dietrich
STUDY OBJECTIVE: To assess the experience of dilator use for neovagina creation in women diagnosed with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and identify barriers affecting patient compliance with dilator use. DESIGN AND SETTING: This was a qualitative study using a structured questionnaire to investigate perception and identify barriers regarding vaginal dilator use. A questionnaire was administered after participants viewed a 2-minute patient educational video to additionally examine the usefulness of a patient education video tool as an adjunct to counseling in clinic on vaginal dilator use...
August 2017: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/28210483/intra-peritoneal-leiomyoma-of-the-round-ligament-in-a-patient-with-mayer-rokitansky-k%C3%A3-ster-hauser-mrkh-syndrome
#20
G Salem Wehbe, R Bitar, T Zreik, M Samaha, C Walter, Z Sleiman
BACKGROUND: The occurrence of an extra-uterine leiomyoma, arising from the intra-peritoneal portion of the round ligament in a lady with Müllerian agenesis diagnosed at the age of forty is extremely rare. We report a case of this rare combination in a Middle Eastern woman. CASE: A 40 years old lady, primarily amenorrheic, presented to our clinic for an infertility consultation. The work- up showed features suggestive of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with a leiomyoma arising from the intra-peritoneal part of the round ligament...
December 2016: Facts, Views & Vision in ObGyn
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