What are the symptoms of the StAR deficiency (lipoid adrenal hyperplasia) form of congenital adrenal hyperplasia (CAH)?

Updated: Oct 06, 2020
  • Author: Thomas A Wilson, MD; Chief Editor: Sasigarn A Bowden, MD  more...
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Answer

Infants with StAR deficiency (lipoid adrenal hyperplasia) usually have signs of adrenal insufficiency (eg, poor feeding, vomiting, dehydration, hypotension, hyponatremia, hyperkalemia). Some patients do not receive medical attention until late infancy. Male patients with this form of adrenal hyperplasia have female or ambiguous genitalia. Female patients have normal female genitalia. A curious observation is that girls who survive develop breasts and menstruate at puberty, suggesting preservation of ovarian steroidogenesis.


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