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https://www.readbyqxmd.com/read/29133152/laparoscopic-assisted-uterus-retrieval-from-live-organ-donors-for-uterine-transplant
#1
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/29068465/array-cgh-analysis-in-patients-with-m%C3%A3-llerian-fusion-anomalies
#2
S Ledig, A C Tewes, J Hucke, T Römer, K Kapczuk, C Schippert, P Hillemanns, P Wieacker
Fusion anomalies of the Müllerian ducts are associated with an increased risk for miscarriage and premature labor. In most cases polygenic-multifactorial inheritance can be assumed but autosomal-dominant inheritance with reduced penetrance and variable manifestation should be considered. We performed array-CGH (comparative genomic hybridization) analysis in a cohort of 103 patients with Müllerian fusion anomalies. In eight patients we detected microdeletions and microduplications in chromosomal regions 17q12, 22q11...
October 25, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/29056568/anatomic-and-functional-outcomes-of-paramesonephric-remnant-supported-laparoscopic-double-layer-peritoneal-pull-down-vaginoplasty-technique-in-patients-with-mayer-rokitansky-kuster-hauser-syndrome-uncu-modification
#3
Gürkan Uncu, Kemal Özerkan, Barış Ata, Işıl Kasapoğlu, Mehmet Aral Atalay, Adnan Orhan, Kiper Aslan
OBJECTIVE: To describe modifications to the double layer peritoneal pull-down laparoscopic vaginoplasty technique (Davydov operation). To evaluate anatomic and functional outcomes of the new technique named Uncu Modification. DESIGN: Case series. TASK FORCE CLASSIFICATION OF STUDY DESIGN: III SETTING: Tertiary Care University Hospital PATIENTS: Women with Mayer-Rokitansky-Küster Hauser Syndrome (MRKHS) who were operated on between years 2010-2016...
October 19, 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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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...
October 2017: Journal of Visceral Surgery
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/28815558/sequence-variants-in-esr1-and-oxtr-are-associated-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#12
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 ongoing research, the etiology is mainly unknown. For a long time, the hypothesis of deficient hormone receptors as the cause for MRKHS has existed, 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...
November 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
#13
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
#14
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...
November 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
#15
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...
September 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
#16
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
#17
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
#18
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
#19
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
#20
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
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