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MURCS (Müllerian duct aplasia-renal agenesis-cervicothoracic somite dysplasia): a
rare cause of primary amenorrhoea
#MMPMID27099773
Kumar S
; Sharma S
Oxf Med Case Reports
2016[Apr]; 2016
(4
): 73-5
PMID27099773
show ga
The agenesis of the Müllerian duct is the second most common cause of primary
amenorrhoea after Turner syndrome. The abnormal development of Müllerian duct
often associates with the urinary tract and skeletal abnormalities. MURCS
(Müllerian duct aplasia-renal agenesis-cervicothoracic somite dysplasia)
association is a unique and rare developmental disorder with four common features
of uterine hypoplasia or aplasia, renal agenesis or ectopy, vertebral anomalies
and short stature. We report a case of young female with primary amenorrhoea. She
had well-developed secondary sexual characteristics along with multiple
congenital developmental abnormalities such as the absence of uterus, ectopic
kidney, cervical vertebral fusion, hemivertebrae, scoliosis, cervical rib, facial
asymmetry and growth retardation. Our case highlights the rarity and clinical
importance of this syndrome. For the evaluation of primary amenorrhoea in a
female with well-developed secondary sexual characteristics, congenital anomalies
should be ruled out before hormone and karyotype analyses.
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