Premature Adrenarche
Premature Adrenarche: Excerpt from The 5-Minute Pediatric Consult
J. Nina Ham, MDAndrea Kelly, MD
Premature Adrenarche - BASICS
Premature Adrenarche - description
- Appearance of small amounts of pubic hair before age 8 in girls and age 9 in boys
- Recent data suggest that the onset of normal sexual development in girls is younger than previously recognized, but lowering of the traditionally accepted limits is subject to debate.
- With premature adrenarche, axillary hair, acne, and apocrine sweat gland secretion are not always present.
- No other signs of sexual development are exhibited. Presence of breast development suggests precocious puberty and not premature adrenarche.
Premature Adrenarche - risk factors
Premature Adrenarche - genetics
A familial pattern suggesting either recessive or dominant inheritance has been described.
Premature Adrenarche - pathophysiology
- Levels of dehydroepiandrosterone (DHEA) and dehydroepiandrosterone-sulfate (DHEAS) from the adrenal glands increase earlier than typically seen in normal puberty.
- Zona reticularis of the adrenals normally begins to increase androgen secretion at age 7–8 years.
Premature Adrenarche - DIAGNOSIS
Premature Adrenarche - signs & symptoms
Premature Adrenarche - history
- Careful attention to presence of any other signs of sexual precocity as well as rate of progression
- Family history of pubertal development, infertility, irregular menses, hirsutism, premature male pattern balding
- Birth weight that is small for gestational age (SGA) may predispose children to development of premature adrenarche.
Premature Adrenarche - physical exam
- The presence of pigmented, curly hairs in the pubic area is consistent with the androgen effect.
- In girls, clitoromegaly suggests congenital adrenal hyperplasia or androgen-secreting tumors.
- The finding of acanthosis nigricans suggests that insulin resistance and the risk of developing ovarian hyperandrogenism are present.
Do not fail to differentiate between true pubic hair (curly and short) and dark lanugo hair (straight and long).
Premature Adrenarche - tests
Premature Adrenarche - lab
- Adrenal steroids: DHEA and DHEAS are often in the early pubertal range, but testosterone and 17α-hydroxyprogesterone (17-OHP) should be in the prepubertal range.
- Gonadotropin-releasing hormone stimulation test: Not routinely recommended but would have a normal prepubertal response
- Children with systemic signs of virilization (such as a significantly advanced bone age) or elevated adrenal steroids (17-OHP or DHEA) should have adrenocorticotropic hormone (ACTH) stimulation testing to exclude congenital adrenal hyperplasia and other hyperandrogen syndromes.
Premature Adrenarche - imaging
- Bone age usually is normal (not significantly advanced).
- Abdominal ultrasound, CT scan, or MRI should be considered if signs of significant virilization are present or if rapid progression has occurred; look for intracranial or intra-abdominal masses, especially if androgens are markedly elevated.
Premature Adrenarche - differencial diagnosis
- Tumors: Androgen-secreting tumors can arise in the gonads or adrenal glands.
- Congenital: Nonclassic congenital adrenal hyperplasia
- Miscellaneous:
- Central precocious puberty
- Familial male precocious puberty (testotoxicosis)
- Exogenous male hormone exposure
Premature Adrenarche - TREATMENT
Premature Adrenarche - general measures
- No treatment
- Reassure parents and children that this is a benign process.
- Reassess every 6 months to look for signs of virilization and pubertal progression.
Premature Adrenarche - FOLLOW UP
- Regression does not occur.
- Watch for other signs of puberty, such as breast development, testicular enlargement (>4 mL) or growth acceleration, that suggest onset of true precocious puberty.
- Increasing virilization suggests nonclassic congenital adrenal hyperplasia or early polycystic ovarian syndrome.
- Acanthosis nigricans or signs of insulin resistance have been reported in girls with a history of premature adrenarche.
- Monitor for glucose intolerance or early type 2 diabetes. Can check fasting glucose and insulin levels or oral glucose tolerance test if suspicion is high (e.g., obesity, acanthosis nigricans, polyuria, polydipsia).
Premature Adrenarche - prognosis
- Undergo puberty appropriately with normal fertility.
- Development of ovarian or adrenal hyperandrogenism during adolescence (also known as “polycystic ovarian syndrome”) is more common in some girls with premature adrenarche. Insulin resistance, a common finding in ovarian hyperandrogenism, has been reported in some children and adolescents with a history of premature adrenarche.
- Final adult height is normal.
Premature Adrenarche - complications
- Can be the 1st sign of true precocious puberty (i.e., development of breast tissue and advancement of bone age) and thus warrants careful observation.
- Boys with premature adrenarche and precocious puberty are more likely than girls to have an underlying CNS disorder.
Premature Adrenarche - bibliography
- Auchus RJ, Fainey WE. Adrenarche–physiology, biochemistry and human disease. Clin Endocrinol. 2004;60:288–296.
- Herman-Giddens ME, Slora EJ, Wasserman RC, et al. Secondary sexual characteristics and menses in young girls seen in office practice: A study from the pediatric research office in settings network. Pediatrics. 1997;99:505–512.
- Ibanez L, Jimenes R, de Zegher F. Early puberty-menarche after precocious pubarche: Relation to prenatal growth. Pediatrics. 2006;117:117–121.
- Kaplowitz P. Clinical characteristics of 104 children referred for evaluation of precocious puberty. J Clin Endocrinol Metab. 2004;89:3644–3650.
- Kaplowitz P, Oberfield SE, Drug and Therapeutics and Executive Committees of the Lawson Wilkins Pediatric Endocrine Society. Reexamination of the age limit for defining when puberty is precocious in girls in the United States: Implications for evaluation and treatment. Pediatrics. 1999;104:936–941.
- Midyett LK, Moore WV, Jacobson JD. Are pubertal changes in girls before age 8 benign? Pediatrics. 2003;111:47–51.
- Nebesio TD, Eugster EA. Pubic hair of infancy: Endocrinopathy or enigma? Pediatrics. 2006;117:951–954.
- Neville KA, Walker JL. Precocious pubarche is associated with SGA, prematurity, weight gain, and obesity. Arch Dis Child. 2005;90:258–261.
- Pere A, Perheentupa J, Peter M, et al. Follow up of growth and steroids in premature adrenarche. Eur J Pediatr. 1995;154:346–352.
- Vuguin P, Saenger P, Dimartino-Mardi J. Fasting glucose insulin ratio: A useful measure of insulin resistance in girls with premature adrenarche. J Clin Endocrinol Metab. 2001;86:4618–4621.
Premature Adrenarche - CODES
Premature Adrenarche - icd9
259.1 Adrenorche precocious
Premature Adrenarche - FAQ
- Q: Is there a dietary cause of excess adrenal hormones?
- A: No.
- Q: Does premature adrenarche mean puberty will be early?
- A: The onset of puberty in these children is within the normal range and should follow the familial pattern.
- Q: Can anything be done to reverse the changes?
- A: This is a benign process that does not have long-term sequelae. Antiandrogen drugs are available but are not recommended.
Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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