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https://www.readbyqxmd.com/read/28815558/sequence-variants-in-esr1-and-oxtr-are-associated-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#1
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
#2
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
#3
Stefano Bianchi, Nicola Berlanda, Federica Brunetti, Alessandro Bulfoni, Cecilia Ferrero Caroggio, Luigi Fedele
OBJECTIVE: To evaluate the feasibility, safety and outcome of the laparoscopic modified Davydov vaginoplasty in subjects with partial androgen insensitivity syndrome (PAIS). DESIGN: Retrospective cohort study. CANADIAN TASK FORCE CLASSIFICATION OF STUDY DESIGN: III. SETTING: Tertiary referral Center. PATIENTS: Ten continuous patients operated 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
#4
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
#5
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
#6
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
#7
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...
June 14, 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
#8
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
#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
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
#11
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
#12
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
#13
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
#14
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
https://www.readbyqxmd.com/read/28420892/adenomyomatosis-concomitant-with-primary-gallbladder-carcinoma
#15
Tatsuya Morikawa, Takehiro Okabayashi, Yasuo Shima, Tatsuaki Sumiyoshi, Akihito Kozuki, Yuichi Saisaka, Teppei Tokumaru, Kenta Sui, Jun Iwata, Manabu Matsumoto, Sojiro Morita, Yutaka Nishioka
Some clinicians have proposed a relationship between gallbladder (GB) cancer and adenomyomatosis (ADM) of the gallbladder, although the latter condition is not considered to have malignant potential. We retrospectively reviewed the surgical pathology database of patients who underwent resection for ADM of the gallbladder at our institution from March 2005 to May 2015. In total, 624 patients underwent surgical resection of the gallbladder with Rokitansky-Aschoff sinuses. Of these cases, 93 were pathologically diagnosed with ADM of the gallbladder, with 44 (47...
April 2017: Acta Medica Okayama
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
#16
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
#17
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
#18
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/28314972/-rare-diseases-with-clinical-relevance-the-silver-russell-syndrome
#19
C Neissner, C Schepp, W H Rösch
The Silver-Russell syndrome (SRS) is a rare imprinting disease associated with pre- and postnatal growth retardation, craniofacial features, and asymmetry. Genitourinary abnormalities are seen in up to 20% of affected individuals. Apart from structural renal anomalies, cryptorchidism and hypospadias occur frequently in boys, while girls often have anomalies similar to those in Mayer-Rokitansky-Küster-Hauser syndrome with congenital hypoplasia or aplasia of the uterus and upper part of the vagina. Frequently hypospadias repair and orchiopexy are difficult because of lack of buccal mucosa due to facial dysmorphism and intraabdominal position of the testicles, respectively...
July 2017: Der Urologe. Ausg. A
https://www.readbyqxmd.com/read/28274598/sexual-life-of-women-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-after-laparoscopic-vecchietti-vaginoplasty
#20
Zlatko Pastor, Jiří Froněk, Marta Nováčková, Roman Chmel
INTRODUCTION: Adequate anatomic and physiologic functions of the genitalia are fundamental prerequisites for sexual well-being and reproduction. Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) compromises female sexual life and makes reproduction impossible. AIM: To assess the psychosexual effect of vaginal reconstruction using the laparoscopic Vecchietti technique in patients with MRKHS. METHODS: Forty-two patients with MRKHS who underwent laparoscopic Vecchietti vaginoplasty were included...
June 2017: Sexual Medicine
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