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https://www.readbyqxmd.com/read/28434104/mullerian-dysgenesis-a-critical-review-of-the-literature
#1
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...
April 22, 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
#2
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
#3
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
#4
Richa Sharma, Kiran Guleria, Amita Suneja, Sruthi Bhaskaran, Nadeem Tanveer
Fibroids and fibroid degeneration are common among women with a normal uterus. However, the present report describes a rare case of fibroids arising from a rudimentary horn and degenerations in a patient with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. The patient provided consent for publication. A woman with MRKH syndrome aged 45 years presented at the gynecology outpatient department at University College of Medical Sciences and Guru Teg Bahadur Hospital, Delhi, India, with complaints of an abdominopelvic mass and pain for the previous 2 years...
March 31, 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
#5
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
#6
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...
March 3, 2017: Ultrasound Quarterly
https://www.readbyqxmd.com/read/28246310/hyperandrogenemia-and-high-prolactin-in-congenital-utero-vaginal-aplasia-patients
#7
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
#8
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
#9
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...
February 16, 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
#10
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
https://www.readbyqxmd.com/read/28003020/vertebral-defect-anal-atresia-cardiac-defect-tracheoesophageal-fistula-esophageal-atresia-renal-defect-and-limb-defect-association-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-in-co-occurrence-two-case-reports-and-a-review-of-the-literature
#11
Thomas Bjørsum-Meyer, Morten Herlin, Niels Qvist, Michael B Petersen
BACKGROUND: The vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome are rare conditions. We aimed to present two cases with the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser co-occurrence from our local surgical center and through a systematic literature search detect published cases...
December 21, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27865679/percutaneous-closure-of-isolated-ostium-secundum-type-atrial-septal-defect-in-a-patient-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#12
Murat Akcay, Okan Gulel, Korhan Soylu, Murat Meric, Muzaffer Elmali
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital anomaly characterized by complete or partial aplasia of the uterus and the upper part of the vagina. It is reported to be associated with cardiovascular disorders including atrial septal defect, anomalous pulmonary venous return, aortopulmonary window, pulmonary valve stenosis, mitral valve prolapse, tetralogy of Fallot, truncus arteriosus, and patent ductus arteriosus. Herein, for the first time in the medical literature, we present percutaneous closure of an isolated ostium secundum atrial septal defect in this syndrome...
December 2016: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/27843659/rare-case-of-leiomyoma-and-adenomyosis-in-mayer-rokitansky-kuster-hauser-syndrome
#13
P S Hoo, A R Norhaslinda, J N Shah Reza
We report a case of adenomyosis which developed from a hypoplastic uterus and leiomyoma in a patient with MRKH syndrome. A 45-year-old Malay female with primary amenorrhoea and primary infertility presented with abdominal mass and abdominal pain. She is phenotypically female, has well developed secondary sexual characteristics, and has normal female external genitalia with shallow vagina dimple. Transabdominal ultrasonography showed a homogenous adnexal mass of 10 × 8 cm, uterus sized 5 × 4 cm, and normal kidneys...
2016: Case Reports in Obstetrics and Gynecology
https://www.readbyqxmd.com/read/27716927/genetics-of-mayer-rokitansky-k%C3%A3-ster-hauser-mrkh-syndrome
#14
REVIEW
L Fontana, B Gentilin, L Fedele, C Gervasini, M Miozzo
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, also referred to as Müllerian agenesis, is the second most common cause of primary amenorrhea. It is characterized by congenital absence of the uterus, cervix, and the upper part of the vagina in otherwise phenotypically normal 46,XX females. MRKH syndrome has an incidence of about 1 in 4,500-5,000 newborn females and it is generally divided into two subtypes: MRKH type 1, in which only the upper vagina, cervix and the uterus are affected, and MRKH type 2, which is associated with additional malformations generally affecting the renal and skeletal systems, and also includes MURCS (MÜllerian Renal Cervical Somite) characterized by cervico-thoracic defects...
February 2017: Clinical Genetics
https://www.readbyqxmd.com/read/27678567/vaginal-dilation-in-mayer-rokitansky-kuster-hauser-mrkh-syndrome
#15
A Ketheeswaran, J Morrisey, J Abbott, M Bennett, J Dudley, R Deans
No abstract text is available yet for this article.
November 2015: Journal of Minimally Invasive Gynecology
https://www.readbyqxmd.com/read/27678038/using-the-wharton-sheares-george-method-to-create-a-neovagina-in%C3%A2-patients-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-a-step-by-step-video-tutorial
#16
Lorenz Kuessel, René Wenzl, Marie-Louise Marschalek, Georg Slavka, Daniela Doerfler, Heinrich Husslein
OBJECTIVE: To provide a review of the literature regarding this technique as well as a step-by-step description with the goal of increasing its use as a safe surgical option. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by vaginal agenesis and a variety of mullerian duct anomalies. To date, a variety of procedures have been described for creating a neovagina, but the best treatment remains debated. The Wharton-Sheares-George method, a minimally invasive surgical approach for the creation of a neovagina, is remarkably simple to perform...
December 2016: Fertility and Sterility
https://www.readbyqxmd.com/read/27631471/crp-cyclic-amp-regulates-the-expression-of-type-3-fimbriae-via-cyclic-di-gmp-in-klebsiella-pneumoniae
#17
Ching-Ting Lin, Tien-Huang Lin, Chien-Chen Wu, Lei Wan, Chun-Fa Huang, Hwei-Ling Peng
Klebsiella pneumoniae is the predominant pathogen isolated from liver abscesses of diabetic patients in Asian countries. However, the effects of elevated blood glucose levels on the virulence of this pathogen remain largely unknown. Type 3 fimbriae, encoded by the mrkABCDF genes, are important virulence factors in K. pneumoniae pathogenesis. In this study, the effects of exogenous glucose and the intracellular cyclic AMP (cAMP) signaling pathway on type 3 fimbriae expression regulation were investigated. The production of MrkA, the major subunit of type 3 fimbriae, was increased in glucose-rich medium, whereas cAMP supplementation reversed the effect...
2016: PloS One
https://www.readbyqxmd.com/read/27627107/type-3-fimbriae-encoded-on-plasmids-are-expressed-from-a-unique-promoter-without-affecting-host-motility-facilitating-an-exceptional-phenotype-that-enhances-conjugal-plasmid-transfer
#18
Jonas Stenløkke Madsen, Leise Riber, Witold Kot, Alrun Basfeld, Mette Burmølle, Lars Hestbjerg Hansen, Søren Johannes Sørensen
Horizontal gene transfer (HGT), the transmission of genetic material to a recipient that is not the progeny of the donor, is fundamental in bacterial evolution. HGT is often mediated by mobile genetic elements such as conjugative plasmids, which may be in conflict with the chromosomal elements of the genome because they are independent replicons that may petition their own evolutionary strategy. Here we study differences between type 3 fimbriae encoded on wild type plasmids and in chromosomes. Using known and newly characterized plasmids we show that the expression of type 3 fimbriae encoded on plasmids is systematically different, as MrkH, a c-di-GMP dependent transcriptional activator is not needed for strong expression of the fimbriae...
2016: PloS One
https://www.readbyqxmd.com/read/27625567/mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-with-alopecia-a-rare-case-report-with-review-of-literature
#19
Sanjiv V Choudhary, Uday V Choudhari
A 17-year-old girl presented with alopecia involving lateral margins of the scalp with primary amenorrhea. There was no history of parental consanguinity, and no other siblings were having similar complaints. Her secondary sexual characters were well developed with hypoplastic vagina. Histopathological findings from scalp biopsy showed features of alopecia areata. Ultrasonography of abdomen and pelvis revealed the absence of uterus and the right kidney. Follicle-stimulating hormone, luteinizing hormone, estradiol, testosterone, and thyroid function test was within normal limits...
July 2016: International Journal of Trichology
https://www.readbyqxmd.com/read/27617320/an-unusual-cause-of-urinary-incontinence-urethral-coitus-in-a-case-of-mayer-rokitansky-kuster-hauser-syndrome
#20
Farzaneh Sharifiaghdas, Azar Daneshpajooh, Samira Sohbati, Mahboubeh Mirzaei
Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare anomaly in women and is characterized by congenital aplasia of the uterus and vagina, with normal development of secondary sexual characteristics and a normal karyotype. We report a case of a 38-year-old women with MRKH syndrome that had experienced urethral sex for many years. She presented with urinary incontinence and dyspareunia. The patient's secondary sexual characteristics were normal, and examination revealed a widely open incompetent megalourethra and an absent vagina...
September 2016: Investigative and Clinical Urology
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