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         Mayer Rokitansky Kuster Hauser Syndrome:     more detail

81. The Hammersmith Hosptials NHS Trust
You are of course entirely female. This is known as the mayer rokitanskyKuster hauser syndrome often shortened to rokitansky syndrome.
http://www.femgenab.org.uk/absent.htm
The Absent Vagina Being born without a vagina is very distressing, and it is almost always associated with an absence of the uterus. The ovaries are normally present and function as anybody else's ovaries would do, producing eggs and female hormones which keep you healthy. You are of course entirely female. This is known as the Mayer Rokitansky Kuster Hauser Syndrome often shortened to Rokitansky Syndrome. How will I know if I have this problem? You discover that you have no vagina when you fail to start having periods at the age that you would expect. You develop breasts and pubic hair like any other woman, but you will not have a period. This is because your ovaries produce the normal female hormones which make normal development occur, but the absence of the uterus and vagina means there is no period. Most girls report this at the ages of 15 or 16 years. Alternatively, some may have difficulties with sexual intercourse as their first problem. What investigations or tests will I have?

82. PATIENT PLATFORM
Ghirardini, 1982 Vaginal agenesis (mayer rokitansky - kuster - hausersyndrome) recent etiopathogenetical and anatomical views.
http://www.bioscience.org/patient/rokitans.htm
FRONTIERS IN BIOSCIENCE;
PATIENT PLATFORM
Each question will be responded to after the subject has been carefully investigated. However, Frontiers in Bioscience merely provides the information as a general guide to help patients and with the understanding that the contributors can not accept any type of liability for the use of the information. The information can not substitute expert medical advice and should be regarded as the starting point for finding the proper medial treatment. Questions are subject to editorial corrections. Question: I am seeking information regarding the treatment and postoperative management of Rokitansky syndrome. My 17 year-old daughter was recently diagnosed with this disorder, and we need help deciding what the treatment options, and the postoperative outcome may be. References from Medline MEDLINE reports MEDLINE abstracts MEDLARS reports ASN.1 reports MEDLINE neighbors protein links nucleotide links structure links genome links Back into Entrez for All Selected articles below.
van Bever, 1992

83. Androgen Insensitivity Syndrome (AIS) Support Group - Patient UK
Drash syndrome, SmithLemli-Opitz syndrome. XX conditions mayer RokitanskyKuster hauser (MRKH) syndrome, Mullerian dysgenesis.
http://www.patient.co.uk/showdoc.asp?doc=26739699

84. ScienceDaily -- Browse Topics: Health/Conditions_and_Diseases/M
Sjogren syndrome@ (5); Master s Disease@ (4); mayer rokitansky KusterHauser syndrome@ (8); McArdle s Disease@ (2); Measles, German@ (3
http://www.sciencedaily.com/directory/Health/Conditions_and_Diseases/M
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85. ASRM: HIGHLIGHTS IN FERTILITY AND STERILITY
(Communal, et al, Sexuality after sigmoid colpopoiesis in patients with mayerrokitansky-kuster-Hausersyndrome, Fertility and Sterility, Vol.80, No.3
http://www.asrm.org/Media/Press/09-03f&s.html
MEMBERS-ONLY LOGIN About ASRM Home Page ASRM Annual Meeting ASRM Office of Public Affairs Contact Us ... Site Awards For All Users TOPIC INDEX Headline News ASRM Literature Links to Prof. Orgs. ... Downloading PDF Files Selected ASRM Publications: Fertility and Sterility Menopausal Medicine ASRM News Practice Guidelines ... ASRM Bulletins For Patients Home FAQ - Infertility FAQ - Psychology Patient Fact Sheets ... Find a Doctor For Professionals Home Upcoming Meetings Members-Only Area Membership (Services, Benefits, Sign-Up) ... Research Grants For the Media Home Press Releases ASRM Office of Public Affairs
For Immediate Release:
September 11, 2003
CONTACT:
Sean Tipton at 202/863-2494 or stipton@asrm-dc.org
Eleanor Nicoll at 202/863-2439 or enicoll@asrm-dc.org
HIGHLIGHTS IN FERTILITY AND STERILITY
Vol. 80, No. 3, September 2003
National Survey Reveals Increased Risk Of Death To Turner Syndrome Patients Who Become Pregnant
Turner syndrome, a genetic disorder involving a female having only one X chromosome, rather than the normal two, is associated with lack of development of the ovaries. This results in infertility, which can be overcome through oocyte donation.

86. Hermafrodytyzm - Linki
congenitaladrenalhyperplasia.org/. Wrodzony brak pochwy i macicy MayerRokitansky kuster hauser syndrome (MRKH) http//www.mrkh.org. Dysgenezja
http://hermafrodytyzm.org/linki.htm
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l i n k i : : Jeden z najlepszych serwisów po¶wiêconych interseksualizmowi /obojnactwu/
ISNA - Intersex Society of North America

http://www.isna.org/
Grupa wsparcia - Intersex Support Group International
http://www.isgi.org/
Angielskie stowarzyszenie osób hermafrodytycznych - United Kingdom Intersex Association
http://www.ukia.co.uk/
Serwis informacyjny - Grupa Wsparcia dla osób, których cia³o jest inne.
Peer Support and Information for people born with a body that is different.

http://www.bodieslikeours.org/

Serwis dla osób dotkniêtych zespó³em niewra¿liwo¶ci na androgeny - Androgen Insensitivity Syndrome (AIS)
http://www.medhelp.org/www/ais/
Serwis dla osób z zespo³em Klinefeltera - Klinefelter Syndrome Support Group http://klinefeltersyndrome.org/ Serwis dla osób z zespo³em Turnera - Turner Syndrome Support Group http://www.turner-syndrome-us.org/

87. Primary Amenorrhea
Genetic Male (10%). Axis 4 Uterus Mullerian Agenesis (20%) mayerrokitansky-kuster-HauserSyndrome. Primary Amenorrhea Algorithm
http://www.fpnotebook.com/GYN71.htm
Home About Links Index ... Editor's Choice document.write(code); Advertisement Gynecology Menses Assorted Pages Primary Amenorrhea Secondary Amenorrhea Abnormal Uterine Bleeding Causes Anovulatory Bleeding ... Uterine Fibroid Primary Amenorrhea Book Home Page Cardiovascular Medicine Dentistry Dermatology Emergency Medicine Endocrinology Gastroenterology General Medicine Geriatric Medicine Gynecology Hematology and Oncology HIV Infectious Disease Jokes Laboratory Neonatology Nephrology Neurology Obstetrics Ophthalmology Orthopedics Otolaryngology Pediatrics Pharmacology Prevention Psychiatry Pulmonology Radiology Rheumatology Sports Medicine Surgery Urology Chapter Gynecology Index Breast Cervix Contraception Dermatology Endocrinology Examination Hematology and Oncology Infectious Disease Laboratory General Menses Obstetrics Pharmacology Prevention Procedure Psychiatry Radiology Surgery Symptom Evaluation Uterus Vagina Vulva Page Menses Index Amenorrhea Primary Amenorrhea Secondary DUB Causes DUB Management Anovulatory DUB Management Anovulatory Metrorrhagia DUB Management Ovulatory DUB Management Ovulatory Menorrhagia DUB Postmenopause Dysmenorrhea Primary Dysmenorrhea Secondary
  • Definition No menstrual period by: Sixteen years old or One year beyond Family History No secondary sexual characteristics by 14 years old Causes Axis 1: Hypothalamus or Central Anovulation (10%) Post-hormonal contraceptive Amenorrhea Constitutional (6%): Family history Kallmann's Syndrome (Rare): Associated with Anosmia Axis 2: Pituitary Pituitary Tumor (8%) Pituitary Adenoma (hormone producing)
  • 88. Menstruation: No Period By Age 15?
    Even more rarely, a young woman may not have a vagina or uterus at all (mayerrokitansky-kuster-Hausersyndrome); additional testing and extensive counseling
    http://www.ivillagehealth.com/experts/womens/qas/0,,229709_128366-2,00.html
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    89. REF.BIB.
    17 STRUBBE EH, WILLEMSEN WNP, LEMMENS JAM et al-mayer-rokitansky-kuster-Hausersyndrome distinction between two forms based on excretory urographic
    http://www.med.univ-rennes1.fr/cerf/edicerf/PEDIATRIE/rb/8_MASSE_ABDOMINALE_cv_r
    1 - BECKWITH J.B. -Wilms'tumor and other renal tumors of childhood- Hum Pathol,1983, 14, 481-492
    2 - BONDJ.V.-Bilateral Wilms' tumor. Age at diagnosis, associated congenitalanomalies and possible pattern of inheritance .-Lancet, 1975, 2: 482
    3 - BOURLIERE-NAJEAN B, SILES S, PANUEL M et al- Value of MRI and MIBG-I 123scintigraphy in the diagnosis of spinal bone marrow involvement in neuroblastoma in children. Pediatr. Radiol. 1992, 22:443-446
    4 - COHEN H.L, HALLER J.O.-Pediatric and adolescent genital abnormalities.-in"Neonatal and Pediatric Ultrasound" edited by D.S.Babcok, -Clinics in Diagnostic Ultrasound ndeg.24, Churchill Livingstone, 1989, 187-215
    5 - COHEN HL, SHAPIRO MA, MANDEL FS et al- Normal ovaries in neonates andinfants: a sonographic study of 77 patients 1 day to 24 months old.- AJR 1993, 160:583-586
    6 - COHEN M.D.-Radiology of pediatric abdominal masses.-in Syllabus ofPediatric Radiology, Ed Poznanski A et Kirkpatrick J, -75 Th Congress of RSNA, Chicago, 1989, 197-211
    8 - DANEMAN A.-in "Pediatric Body CT", A.Daneman ed, Springer -Verlag, 1987,pp 145-171
    10 - KIRKS D.R., KAUFMAN R.A., BABCOCK D.S.-Renal Neoplasms in Infants andChildren-Seminars in Roentgenology, 1987, 12, 292-302

    90. AIS Fact Sheet
    Smith Lemli-Opitz syndrome. XX conditions with some AIS-like features MayerRokitansky kuster hauser (MRKH) syndrome,. Mullerian dysgenesis. Incidence.
    http://home.vicnet.net.au/~aissg/ais_fact_sheet.htm
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    This Fact Sheet is an AIS Support Group Australia adaptation of the AISSG(UK) Fact Sheet. The AIS Support Group Australia is a peer support group providing information and support to people with AIS and related intersex conditions and their families.
    Introduction
    Up to 8 weeks gestation every foetus, whether of typical male or female chromosomal sex, has the capacity to develop either a male or female reproductive system, and in a typical male (XY) foetus the active intervention of male hormones (androgens) is needed to produce a fully male system. A female body type with female external genitalia is the basic underlying human form.
    What is AIS?
    Androgen Insensitivity Syndrome (old name Testicular Feminisation Syndrome) causes an interruption of the foetal development of the reproductive system. In AIS the child is conceived with typical male (XY) sex chromosomes. Embryonic testes develop and start to produce androgens but the body is unable to respond to these androgens to a varying degree. This means that the genitals can vary from completely female if there is no response through to almost completely male if there is a slight insensitivity to androgens or anywhere in between.

    91. SYNDROME DE ROKYTANSKY KUSTER (HEMI-)
    MASSE ABDOMINALE ET/OU PELVIENNE CHEZ L ENFANT (edicerf/PEDIATRIE)
    http://www.med.univ-rennes1.fr/cgi-bin/iw/fichiers_de_codeadm.pl?code=M11017

    92. Karger Publishers

    http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowFulltext&ProduktNr=

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