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Article |

The XX Turner Phenotype With Unilateral Streak Gonad and Absent Uterus

Shiu-Loong R. Wong, MD; Barbara M. Lippe, MD; Solomon A. Kaplan, MD
Am J Dis Child. 1971;122(5):449-451. doi:10.1001/archpedi.1971.02110050119020.
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Absence of the uterus, right ovary, and right fallopian tube were demonstrated by pelvic pneumography and confirmed on laparoscopic examination of a patient with primary amenorrhea and the Turner phenotype. A small rudimentary ovary and fallopian tube were present on the left. Chromosomal constitution was 46,XX. This is a rare example of the association of absence of the uterus with the Turner phenotype in a female with apparently normal chromosomal constitution. Other cases with similar findings may help resolve the question as to whether the association was a chance occurrence or due to a genetic anomaly responsible for both the Turner phenotype and regression of the Müllerian structures.


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