Chromosomal disorder (from the presence of only one sex chromosome, X, in all or some of the body's cells) that causes abnormal sexual development in females. The syndrome may include absent or undeveloped ovaries, underdeveloped secondary sex characteristics, low hairline, webbed neck, shield-shaped chest with wide-spaced nipples, and kidney and heart malformations with coarctation (narrowing) of the aorta. It may not be recognized until a girl fails to undergo puberty at a normal age. Estrogen treatment results in puberty, adult appearance, and normal sex drive but not fertility. Surgery can correct malformations.
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