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https://www.readbyqxmd.com/read/29234256/congenital-malformations-of-the-reproductive-tract-in-a-patient-with-poland-syndrome-is-there-a-connection
#1
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
#2
Michael W Slater, Xochitl Vinaja, Islam Aly, Marios Loukas, Mark Terrell, Justine Schober
INTRODUCTION: 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...
November 27, 2017: 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
#3
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...
November 24, 2017: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/29133152/laparoscopic-assisted-uterus-retrieval-from-live-organ-donors-for-uterine-transplant
#4
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
OBJECTIVE: The authors present the first ever laparoscopic assisted uterus retrieval in live donor for uterus transplant. DESIGN: A step-by-step surgical demonstration SETTING: Galaxy CARE Laparoscopy Institute, Pune, India PATIENT(S): Two patients, ages 21 and 26 with Mayer Rokitansky Kuster Hauser (MRKH) syndrome and Ashermann's syndrome respectively, with their mothers as donors. INTERVENTION(S): A Twelve member team was formed. After review of available literature on uterine transplant a protocol was formulated and submitted to the IRB...
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
#5
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...
September 28, 2017: 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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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...
December 2017: European Radiology
https://www.readbyqxmd.com/read/28624115/evaluation-of-amnion-in-creation-of-neovagina-in-women-with-mayer-rokitansky-kuster-hauser-syndrome
#13
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
#14
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
#15
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
#16
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
#17
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...
November 2017: International Urogynecology Journal
https://www.readbyqxmd.com/read/28397650/-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#18
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
#19
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
#20
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
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