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Rokitansky sequence (disorder)
Rokitansky sequence
Congenital absence of uterus and vagina
Mayer-Rokitansky-Kuster syndrome
Describes a spectrum of Mullerian duct anomalies with congenital aplasia of the uterus and upper two thirds of the vagina in otherwise phenotypically normal females. It can be classified as either MRKH syndrome type 1 (corresponding to isolated utero-vaginal aplasia) or MRKH syndrome type 2 (utero-vaginal aplasia associated with other malformations). MRKH syndrome was thought to be purely sporadic but familial cases seem to be inherited autosomal dominantly with incomplete penetrance and variable expressivity.
Mayer-Rokitansky-Küster-Hauser-syndroom type 1
congenitale afwezigheid van baarmoeder en vagina
syndroom van Mayer-Rokitansky-Küster-Hauser type 1
Rokitansky-sequentie
congenitale afwezigheid van uterus en vagina
MRKH-syndroom type 1
syndroom van Mayer-Rokitansky-Küster
MRKH-syndroom type 1
Aangeboren afwijking waarbij een vrouw geen vagina en geen baarmoeder heeft en er soms ook afwijkingen aan nieren, skelet, hart of gehoor bestaan.
Id8793008
StatusPrimitive
Associated morphologyAbsence
Finding siteUterine structure
OccurrenceCongenital
Pathological processPathological developmental process
Associated morphologyAbsence
Finding siteStructure of upper third of vagina
OccurrenceCongenital
Pathological processPathological developmental process
Associated morphologyAbsence
Finding siteStructure of middle third of vagina
OccurrenceCongenital
Pathological processPathological developmental process
ICD-10 complex map reference set
TargetQ52.0
RuleTRUE
AdviceALWAYS Q52.0
CorrelationSNOMED CT source code to target map code correlation not specified
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