How to proceed with a 6-year-4-month-old girl presenting with premature adrenarche, elevated Dehydroepiandrosterone sulfate (DHEAS) levels, and advanced bone age, but normal testosterone, 17-alpha hydroxyprogesterone, and follicle-stimulating hormone (FSH) levels?

Management of Premature Adrenarche in a 6-Year-4-Month-Old Girl

This case represents premature adrenarche and requires careful monitoring without specific treatment at this time, as it is generally a benign condition that does not require immediate intervention.

Diagnostic Assessment

• The diagnosis of premature adrenarche is confirmed by the presence of axillary and pubic hair before age 8 years in girls, along with elevated DHEAS levels (149 μg/dL), but without breast development or clitoromegaly 1, 2
• The advanced bone age (9-10 years) is consistent with premature adrenarche, as these children typically show bone age advancement proportionate to their increased growth rate 3
• Normal testosterone, 17-alpha hydroxyprogesterone, and suppressed gonadotropins (FSH <0.66, LH <0.216) help rule out other causes of androgen excess such as congenital adrenal hyperplasia and precocious puberty 2

Differential Diagnosis Considerations

• Congenital adrenal hyperplasia (CAH) has been ruled out by normal 17-alpha hydroxyprogesterone levels 2
• True precocious puberty is excluded by the absence of breast development and suppressed gonadotropins 4
• Adrenal tumors are unlikely with normal testosterone levels and the absence of rapid progression of virilization 3

Management Plan

1. Regular monitoring is recommended without specific treatment

   • Schedule follow-up visits every 6-12 months to monitor growth velocity, bone age advancement, and pubertal development 2
   • Monitor height, weight, and BMI percentiles, as premature adrenarche is associated with higher rates of overweight/obesity 5

2. Laboratory monitoring

   • Consider periodic reassessment of androgen levels (DHEAS, testosterone) if clinical progression occurs 6
   • No need for immediate repeat hormonal testing if clinical status remains stable 3

3. Lifestyle counseling

   • Emphasize healthy diet and regular physical activity, especially since the child is in the 75-90th percentile for weight 5
   • Weight management is important as obesity can exacerbate androgen levels in children with premature adrenarche 5

4. Bone age monitoring

   • Repeat bone age assessment in 12 months to evaluate the rate of skeletal maturation 3
   • Current bone age advancement (9-10 years vs chronological age of 6.3 years) requires monitoring but is not uncommon in premature adrenarche 3

5. Psychological support

   • Provide age-appropriate explanation to the child and parents about the condition 6
   • Address any concerns about body image or social implications of early body hair development 6

Prognosis and Long-term Considerations

• Premature adrenarche is generally a benign condition but has been associated with:

  • Increased risk for insulin resistance and metabolic syndrome in some populations 2
  • Possible increased risk for polycystic ovarian syndrome (PCOS) in adolescence and adulthood 6
  • These risks are higher in children who are overweight/obese 5

• Consider screening for metabolic abnormalities in follow-up visits, particularly if the child is overweight or obese 2, 5

• No specific medication is recommended at this time, though in cases with significant insulin resistance and low birth weight (not applicable in this case), some clinicians may consider metformin on a case-by-case basis 2

When to Consider Further Evaluation

• If signs of true central puberty develop (breast development, accelerated growth velocity) 4
• If rapid progression of virilization occurs (clitoromegaly, voice deepening, severe acne) 3
• If bone age advancement becomes more pronounced, potentially affecting final height 3