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Precocious Pseudopuberty Follow-up

  • Author: Sunil Sinha, MD; Chief Editor: Stephen Kemp, MD, PhD  more...
 
Updated: Oct 30, 2015
 

Further Outpatient Care

After the initial diagnosis of peripheral precocious puberty and the determination of its etiology, most patients require continued monitoring.

  • McCune-Albright syndrome (MAS): Closely follow the cases of patients with MAS for the occurrence of other endocrinopathies or associated pathology.
  • Testotoxicosis: Treatment decisions of these patients are complex. The most extensive report followed 10 boys during 6 years of treatment, but none had reached final height.[7] Thus, no firm recommendations for one particular form of therapy are currently available. Offer genetic counseling for families affected with this condition.
  • Congenital adrenal hyperplasia (CAH): Classic simple virilizing CAH requires lifelong therapy with glucocorticoids in both males and females. Most males with late-onset nonclassic CAH do not require glucocorticoid treatment. Treat symptomatic girls with nonclassic CAH with low-dose glucocorticoids throughout their reproductive years.
  • Testicular, ovarian, and adrenal tumors: These tumors are typically unilateral and should be surgically removed. Once removed, the remaining contralateral testis, ovary, or adrenal gland should recover from suppression and be adequate for normal function.
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Further Inpatient Care

See the list below:

  • No further inpatient care should be required in patients with precocious pseudopuberty.
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Inpatient & Outpatient Medications

The treatment of peripheral precocious puberty depends on its etiology. Although therapy is rarely carried out by anyone other than a pediatric endocrinologist, the following list is included for completeness:

  • MAS may be mild and slowly progressive; thus, no outpatient medical management is required. If the puberty is rapidly progressive or adult height is severely compromised, treatment may be indicated. Testolactone, an aromatase inhibitor, is the most commonly used medication for the treatment of MAS. Other drugs have included medroxyprogesterone acetate and, more recently, tamoxifen. A gonadotropin agonist may have an additional benefit but only if the bone age is so advanced that central precocious puberty has begun.
  • The management of testotoxicosis is difficult and controversial. Testotoxicosis is often treated with ketoconazole, a nonspecific inhibitor of steroidogenesis. An alternative regimen has included spironolactone and testolactone. Gonadotropin agonists may be added as noted in the management of MAS.
  • Treatment of CAH with near-physiologic replacement doses of hydrocortisone is used to suppress adrenal androgen production.
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Transfer

See the list below:

  • If an experienced pediatric endocrinologist or appropriate surgical subspecialty support is not available, transferring the patient to another facility with experienced staff is highly encouraged.
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Complications

See the list below:

  • MAS: Long-term complications stem from the multiple endocrinopathies that these patients may develop. Patients may also develop extremely deforming and disabling polyostotic bone changes.
  • Testotoxicosis: Complications are related to early sexual and physical maturation. Other complications are psychological and related to the early sexual and physical maturation.
  • CAH: Complications from overtreatment with hydrocortisone (eg, poor growth, adrenal suppression, features of Cushing syndrome) may be observed. Undertreatment of females may result in irreversible virilization and polycystic ovarian syndrome. Young men with untreated or poorly treated classic CAH may develop testicular adrenal rests, responsive to glucocorticoid suppression. Subfertility may be associated with CAH in both men and women. Adrenal tumors are more common in patients with CAH than in the general population.
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Prognosis

Prognosis varies with etiology.

  • MAS: Prognosis varies with the number of endocrinopathies and the extent of the bone disease. Most girls have an excellent prognosis.
  • Testotoxicosis: Prognosis is excellent with proper treatment.
  • CAH: Prognosis is excellent with proper treatment.
  • Ovarian granulosa cell tumors: Early recognition and diagnosis of ovarian granulosa cell tumors leads to improved cure rates and disease-free survival rates.
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Patient Education

See the list below:

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Contributor Information and Disclosures
Author

Sunil Sinha, MD Assistant Professor, Division of Pediatric Endocrinology and Metabolism, Department of Pediatrics, University of Tennessee Health Science Center

Sunil Sinha, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, Endocrine Society, Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Coauthor(s)

Cydney L Fenton, MD Director, Center for Diabetes and Endocrinology, Akron Children's Hospital

Cydney L Fenton, MD is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, Endocrine Society, Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Merrily P M Poth, MD Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences

Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Lynne Lipton Levitsky, MD Chief, Pediatric Endocrine Unit, Massachusetts General Hospital; Associate Professor of Pediatrics, Harvard Medical School

Lynne Lipton Levitsky, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Diabetes Association, American Pediatric Society, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research

Disclosure: Received grant/research funds from Eli Lilly for pi; Received grant/research funds from NovoNordisk for pi; Received consulting fee from NovoNordisk for consulting; Partner received consulting fee from Onyx Heart Valve for consulting.

Chief Editor

Stephen Kemp, MD, PhD Former Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas for Medical Sciences College of Medicine, Arkansas Children's Hospital

Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Phyllis W Speiser, MD Chief, Division of Pediatric Endocrinology, Steven and Alexandra Cohen Children's Medical Center of New York; Professor of Pediatrics, Hofstra-North Shore LIJ School of Medicine at Hofstra University

Phyllis W Speiser, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

Acknowledgements

Robert J Ferry Jr, MD Le Bonheur Chair of Excellence in Endocrinology, Professor and Chief, Division of Pediatric Endocrinology and Metabolism, Department of Pediatrics, University of Tennessee Health Science Center

Robert J Ferry Jr, MD is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, American Medical Association, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research, and Texas Pediatric Society

Disclosure: Eli Lilly & Co Grant/research funds Investigator; MacroGenics, Inc Grant/research funds Investigator; Ipsen, SA (formerly Tercica, Inc) Grant/research funds Investigator; NovoNordisk SA Grant/research funds Investigator; Diamyd Grant/research funds Investigator; Bristol-Myers-Squibb Grant/research funds Other; Amylin Other; Pfizer Grant/research funds Other; Takeda Grant/research funds Other

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Graph represents the prevalence of breast development at Tanner stage 2 or greater by age and race.
Graph represents the prevalence of pubic hair at Tanner stage 2 or greater by age and race.
 
 
 
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