Benign idiopathic premature adrenarche

7 Interesting Facts of Benign idiopathic premature adrenarche

  1. Condition in which there is early onset of pubic hair (pubarche) and/or body odor that occurs before age 8 years in girls and before age 9 years in boys
  2. No true pubertal development (ie, no breast development in girls, no testicular growth in boys), nor evidence of clitoromegaly or penile growth in idiopathic premature pubarche or premature adrenarche 
  3. In both idiopathic premature adrenarche and nonclassic congenital adrenal hyperplasia, DHEA-S levels are mildly increased for age 
  4. In contrast to various forms of nonclassic congenital adrenal hyperplasia, which often show rapid linear growth acceleration, growth rate is normal in idiopathic premature adrenarche (ie, the child tracks along the same percentile on the linear growth curve)
    • Bone age is often obtained in the evaluation of both, but caution must be exercised in interpretation because of the skill and expertise involved in readings 
      • In taller children, bone age is advanced by up to 2 years in approximately 30% of cases of idiopathic (benign) premature adrenarche
  5. Idiopathic premature adrenarche occurs more commonly in black girls than white girls, whereas nonclassic congenital adrenal hyperplasia is most often seen in patients of Ashkenazi Jewish and Hispanic descent and is uncommon in black children 
  6. To distinguish from nonclassic congenital adrenal hyperplasia due to 21-hydroxylase deficiency, measure basal and corticotropin-stimulated 17-hydroxyprogesterone levels
  7. Benign idiopathic premature adrenarche is a diagnosis of exclusion as certain pathologic processes (eg, androgen-secreting tumors, nonclassic variants of congenital adrenal hyperplasia) must be excluded if child exhibits premature signs of androgen excess on physical examination 
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