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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
#1
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
#2
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
#3
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
#4
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/28844704/gallbladder-adenomyomatosis-diagnosis-and-management
#5
N Golse, M Lewin, A Rode, M Sebagh, J-Y Mabrut
Gallbladder (GB) adenomyomatosis (ADM) is a benign, acquired anomaly, characterized by hypertrophy of the mucosal epithelium that invaginates into the interstices of a thickened muscularis forming so-called Rokitansky-Aschoff sinuses. There are three forms of ADM: segmental, fundal and more rarely, diffuse. Etiology and pathogenesis are not well understood but chronic inflammation of the GB is a necessary precursor. Prevalence of ADM in cholecystectomy specimens is estimated between 1% and 9% with a balanced sex ratio; the incidence increases after the age of 50...
August 22, 2017: Journal of Visceral Surgery
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/28815558/sequence-variants-in-esr1-and-oxtr-are-associated-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#7
Sara Yvonne Brucker, Liliane Frank, Simone Eisenbeis, Melanie Henes, Diethelm Wallwiener, Olaf Riess, Barbara Van Eijck, Dorit Schöller, Michael Bonin, Kristin Katharina Rall
INTRODUCTION: Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is characterized by congenital absence of the uterus and the upper two-thirds of the vagina in otherwise phenotypically normal females. It is found isolated or associated with renal, skeletal and other malformations. Despite of ongoing research, the etiology is mainly unknown. For a long time, the hypothesis of deficient hormone receptors as cause for MRKHS exists, and is supported by previous findings of our group. The aim of the present study was to identify unknown genetic causes for MRKHS and to compare them with data banks including a review of the literature...
August 16, 2017: Acta Obstetricia et Gynecologica Scandinavica
https://www.readbyqxmd.com/read/28804623/recent-advances-in-the-molecular-mechanisms-of-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#8
Keiko Watanabe, Yusuke Kobayashi, Kouji Banno, Yusuke Matoba, Haruko Kunitomi, Kanako Nakamura, Masataka Adachi, Kiyoko Umene, Iori Kisu, Eiichiro Tominaga, Daisuke Aoki
Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a disease caused by congenital absence of the uterus and two-thirds of the upper vagina. The pathogenic mechanism of MRKHS may involve gene abnormalities, and there are various case reports associating MRKHS with the Wnt family member 4 (Wnt4) mutation. Analysis of genes mapped to regions in which deletion and duplication are frequently detected in patients with MRKHS has shown involvement of LIM homeobox 1 (LHX1), HNF1 homeobox B (HNF1B) and T-box 6 (TBX6)...
August 2017: Biomedical Reports
https://www.readbyqxmd.com/read/28802954/creation-of-a-neovagina-by-laparoscopic-modified-davydov-vaginoplasty-in-patients-with-partial-androgen-insensitivity-syndrome
#9
Stefano Bianchi, Nicola Berlanda, Federica Brunetti, Alessandro Bulfoni, Cecilia Ferrero Caroggio, Luigi Fedele
STUDY OBJECTIVE: To evaluate the feasibility, safety, and outcome of laparoscopic modified Davydov vaginoplasty in subjects with partial androgen insensitivity syndrome (PAIS). DESIGN: A retrospective cohort study (Canadian Task Force classification III). SETTING: A tertiary referral center. PATIENTS: Ten continuous patients operated on between October 2008 and May 2014. INTERVENTIONS: Laparoscopic modified Davydov vaginoplasty...
August 9, 2017: Journal of Minimally Invasive Gynecology
https://www.readbyqxmd.com/read/28719918/-the-wish-for-a-child-in-the-case-of-permanent-infertility-development-of-the-german-questionnaire-on-attitudes-toward-motherhood
#10
Maike Fliegner, Hertha Richter-Appelt, Kerstin Krupp, Franziska Brunner
Study 1 Development of the questionnaire Most questionnaires on attitudes toward motherhood presume that the subject is fertile and positive and negative attitudes are represented on a one-dimensional scale. Moreover, the questionnaires often do not provide German versions and German norms. The aim of this study is to examine whether the German Questionnaire on Attitudes toward Motherhood ("FEMu") can be used to describe attitudes toward motherhood multi-dimensionally and whether it is applicable independent of a person's fertility status...
July 18, 2017: Psychotherapie, Psychosomatik, Medizinische Psychologie
https://www.readbyqxmd.com/read/28711548/experience-with-specially-designed-pored-polyacetal-mold-dressing-method-used-in-mcindoe-style-vaginoplasty
#11
So-Eun Han, Ju Young Go, Doo Seok Choi, Gi Hong Seo, So Young Lim
INTRODUCTION: Appropriate postoperative care and prolonged use of the mold are prerequisites for achieving successful results in McIndoe-style vaginoplasty. OBJECTIVE: We introduce a specially designed pored polyacetal mold to maintain the reconstructed vagina, with favorable long-term results allowing drainage of the serous and bloody discharge without removal of the mold, and reduction of infection rate and the chance of skin graft shearing in McIndoe vaginoplasty...
June 16, 2017: Journal of Pediatric Urology
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...
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
#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/28622182/reflux-associated-cholecystopathy-analysis-of-76-gallbladders-from-patients-with-supra-oddi-union-of-the-pancreatic-duct-and-common-bile-duct-pancreatobiliary-maljunction-elucidates-a-specific-diagnostic-pattern-of-mucosal-hyperplasia-as-a-prelude-to-carcinoma
#14
Takashi Muraki, Bahar Memis, Michelle D Reid, Takeshi Uehara, Tetsuya Ito, Osamu Hasebe, Shinji Okaniwa, Naoto Horigome, Takeshi Hisa, Pardeep Mittal, Alexa Freedman, Shishir Maithel, Juan M Sarmiento, Alyssa Krasinskas, Jill Koshiol, Volkan Adsay
Pancreaticobiliary maljunction (PBM) is the anomalous union of the main pancreatic duct and common bile duct outside the Oddi-sphincter, allowing the reflux of pancreatic juice to the gallbladder. There is only limited awareness and understanding of the pathologic correlates of this condition, mostly from Japan; this entity is largely unrecognized in the West. In this study, 76 gallbladders from patients with PBM (64 from Japan; 12 from the United States) were analyzed and contrasted with 66 from non-PBM patients...
September 2017: American Journal of Surgical Pathology
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
#15
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
#16
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/28540840/living-donor-kidney-transplant-in-a-patient-with-type-b-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-reconstructed-vagina-and-abnormal-pelvic-vessels-a-case-report
#17
Mariarosaria Campise, Mariano Ferraresso, Evaldo Favi, Claudio Beretta, Caterina Colico, Piergiorgio Messa
Mayer-Rokitansky-Küster-Hauser syndrome is a rare disorder consisting of vaginal aplasia and other müllerian duct abnormalities. Urinary tract malfor-mations possibly leading to renal failure are also common. For these patients, kidney transplant remains the best option. However, aberrant anatomy and scarring from previous operations may actually preclude successful implantation of the graft. In this setting, careful pretransplant evaluation with high-resolution imaging studies and multidisciplinary planning are mandatory...
May 22, 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28479310/-jarcho-levin-and-rokitansky-syndromes-an-excepcional-association
#18
Rebeca Barriga Buján, Alba Muinelo Segade, Ana Prado-Carro, Pedro González Herranz, Rafaela Soler Fernández
No abstract text is available yet for this article.
May 4, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28434104/mullerian-dysgenesis-a-critical-review-of-the-literature
#19
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/28426677/information-ranks-highest-expectations-of-female-adolescents-with-a-rare-genital-malformation-towards-health-care-services
#20
Elisabeth Simoes, Alexander N Sokolov, Andrea Kronenthaler, Hanna Hiltner, Norbert Schaeffeler, Katharina Rall, Esther Ueding, Monika A Rieger, Anke Wagner, Leonie S Poesch, Marie-Christin Baur, Judith Kittel, Sara Y Brucker
BACKGROUND: Access to highly specialized health care services and support to meet the patient's specific needs is critical for health outcome, especially during age-related transitions within the health care system such as with adolescents entering adult medicine. Being affected by an orphan disease complicates the situation in several important respects. Long distances to dedicated institutions and scarcity of knowledge, even among medical doctors, may present major obstacles for proper access to health care services and health chances...
2017: PloS One
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