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https://www.readbyqxmd.com/read/28314972/-rare-diseases-with-clinical-relevance-the-silver-russell-syndrome
#1
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...
March 17, 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
#2
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...
March 5, 2017: Sexual Medicine
https://www.readbyqxmd.com/read/28263220/ectopic-pelvic-fibroid-in-a-woman-with-uterine-agenesis-and-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#3
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
#4
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/28245469/decidualization-is-impaired-in-endometrial-stromal-cells-from-uterine-rudiments-in-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#5
Sara Y Brucker, Simone Eisenbeis, Juliana König, Melanie Lamy, Madhuri S Salker, Ni Zeng, Harald Seeger, Melanie Henes, Dorit Schöller, Birgitt Schönfisch, Annette Staebler, Florin-Andrei Taran, Diethelm Wallwiener, Katharina Rall
BACKGROUND/AIMS: Uterine rudiments from patients with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) contain all tissues typically found in the uterus. Endometrium from the rudiments predominantly exhibits basalis-like features, and endometrial proliferative capacity in patients' epithelium and stroma is significantly lower. METHODS: This single-center, prospective study conducted at a major German university hospital compared in-vitro decidualization in cultured ESCs from MRKHS patients and hysterectomy controls...
February 27, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28216916/a-rare-case-of-46-xx-gonadal-dysgenesis-and-mayer-rokitansky-kuster-hauser-syndrome
#6
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/28216908/role-of-progenitor-cell-producing-normal-vagina-by-metaplasia-in-laparoscopic-peritoneal-vaginoplasty
#7
Pravin N Mhatre, Hemraj R Narkhede, P Amol Pawar, P Jyoti Mhatre, Das Dhanjit Kumar
CONTEXT: Host of vaginoplasty techniques have been described. None has been successful in developing normal vagina. Laparoscopic peritoneal vaginoplasty (LPV) is performed in Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) culminating in normal vagina. AIMS: This study aims to confirm normal development of neovagina by anatomical and functional parameters of histology, cytology, and ultrasonography (USG) in LPV. To identify peritoneal progenitor cell by OCT4/SOX2 markers...
October 2016: Journal of Human Reproductive Sciences
https://www.readbyqxmd.com/read/28216131/assessing-the-experience-of-vaginal-dilator-use-and-potential-barrier-to-ongoing-use-among-a-focus-group-of-women-with-mrkh
#8
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 Kuster Hauser (MRKH) Syndrome and identify barriers affecting patient compliance with dilator use. DESIGN: and Setting: Qualitative study using a structured questionnaire to investigate perception and identify barriers regarding vaginal dilator use. Questionnaire was administered following viewing of 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
#9
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/28163292/a-case-of-intracystic-papillary-neoplasm-of-the-gallbladder-that-exhibited-findings-similar-to-gallbladder-adenomyomatosis-with-the-formation-of-intramural-cysts-because-of-rokitansky-aschoff-sinus-infiltration
#10
Jun Unno, Takehiro Akahane, Kenichiro Nakagawa, Keiichiro Hiramoto, Yasuchika Yamamoto, Gen Tominaga, Fumiyoshi Fujishima, Yuko Itakura
A 74-year-old man underwent regular follow-up observations after being diagnosed with gallbladder adenomyomatosis based on findings, such as the thickening of the wall of the gallbladder fundus and the presence of intramural cysts. Over the course of 3 years, a papillary tumor located on the thickened wall of the gallbladder had increased in size and extended into the lumen. Consequently, the patient was diagnosed with gallbladder cancer and underwent extended cholecystectomy. The histological diagnosis was intracystic papillary neoplasm (ICPN) of the gallbladder...
2017: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
https://www.readbyqxmd.com/read/28127678/gallbladder-adenomyomatosis-imaging-findings-tricks-and-pitfalls
#11
REVIEW
Matteo Bonatti, Norberto Vezzali, Fabio Lombardo, Federica Ferro, Giulia Zamboni, Martina Tauber, Giampietro Bonatti
Gallbladder adenomyomatosis (GA) is a benign alteration of the gallbladder wall that can be found in up to 9% of patients. GA is characterized by a gallbladder wall thickening containing small bile-filled cystic spaces (i.e., the Rokitansky-Aschoff sinuses, RAS). The bile contained in RAS may undergo a progressive concentration process leading to crystal precipitation and calcification development. A correct characterization of GA is fundamental in order to avoid unnecessary cholecystectomies. Ultrasound (US) is the imaging modality of choice for diagnosing GA; the use of high-frequency probes and a precise focal depth adjustment enable correct identification and characterization of GA in the majority of cases...
January 26, 2017: Insights Into Imaging
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
#12
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/27987200/metastatic-papillary-adenocarcinoma-in-a-22-year-old-is-her-history-of-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-causative-or-incidental
#13
Regina J Brown, Joshua H Petit, Lars Widdel, Patricia S Huguelet, Colin Weekes
No abstract text is available yet for this article.
December 15, 2016: Oncology (Williston Park, NY)
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
#14
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/27852280/treatment-management-during-the-adolescent-transition-period-of-girls-and-young-women-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-mrkhs-a-systematic-literature-review
#15
REVIEW
Anke Wagner, Sara Yvonne Brucker, Esther Ueding, Dagmar Gröber-Grätz, Elisabeth Simoes, Katharina Rall, Andrea Kronenthaler, Norbert Schäffeler, Monika A Rieger
INTRODUCTION: In health services research, there is a special emphasis on the transition from adolescence into adulthood. During this transition period, adolescents change from pediatric to adult medical care. This process must be carefully structured, particularly when special medical care is required. Challenges and difficulties become apparent particularly in the case of rare diseases. This is increasingly so when the rare disease affects the adolescence-specific development of patients, such as Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS), also known as Müllerian agenesis...
November 16, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27843659/rare-case-of-leiomyoma-and-adenomyosis-in-mayer-rokitansky-kuster-hauser-syndrome
#16
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/27825026/congenital-malformations-and-other-comorbidities-in-125-women-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#17
Karina Kapczuk, Kinga Iwaniec, Zbigniew Friebe, Witold Kędzia
OBJECTIVE: To describe congenital malformations and coexisting disorders occurring in 125 Polish women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS). The syndrome is defined as uterovaginal aplasia in female with normal 46,XX karyotype. STUDY DESIGN: A retrospective analysis of the clinical data of MRKHS patients diagnosed or treated at the Gynecology and Obstetrics Clinical Hospital of Poznan University of Medical Sciences between 2010 and 2015. RESULTS: Sixty-eight patients (54,4%) were found to have one or more coexisting anomalies...
December 2016: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/27788530/creation-of-a-neovagina-by-laparoscopic-modified-vecchietti-technique-anatomic-and-functional-results
#18
Eduardo Baptista, Giselda Carvalho, Carlos Nobre, Isabel Dias, Isabel Torgal
Purpose To evaluate the anatomic and functional results of a laparoscopic modified Vecchietti technique for the creation of a neovagina in patients with congenital vaginal aplasia. Methods Retrospective study of nine patients with congenital vaginal aplasia submitted to the laparoscopic Vecchietti procedure, in our department, between 2006 and 2013. The anatomical results were evaluated by assessing the length, width and epithelialization of the neovagina at the postoperative visits. The functional outcome was evaluated using the Rosen Female Sexual Function Index (FSFI) questionnaire and comparing the patients' results to those of a control group of 20 healthy women...
September 2016: Revista Brasileira de Ginecologia e Obstetrícia
https://www.readbyqxmd.com/read/27716927/genetics-of-mayer-rokitansky-k%C3%A3-ster-hauser-mrkh-syndrome
#19
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
#20
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
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