eMedicine Specialties > Pediatrics: General Medicine > Endocrinology

Precocious Pseudopuberty: Follow-up

Author: Robert J Ferry Jr, MD,, Chief, Division of Pediatric Endocrinology and Metabolism, Le Bonheur Children's Medical Center, University of Tennessee Health Science Center at Memphis, and St. Jude Children's Research Hospital; Brigade Surgeon, 36th Sustainment Brigade, 13th Expeditionary Sustainment Command, U.S. Army
Coauthor(s): Cydney L Fenton, MD, FAAP, Consulting Staff, Department of Pediatric Endocrinology, Children's Hospital Medical Center of Akron; Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences
Contributor Information and Disclosures

Updated: Jun 15, 2009

Follow-up

Further Inpatient Care

  • No further inpatient care should be required in patients with precocious pseudopuberty.

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.3 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.

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.

Transfer

  • 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.

Complications

  • 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.

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.

Patient Education

Miscellaneous

Medicolegal Pitfalls

Because these diseases are rare, involving medical subspecialists with experience in the evaluation, treatment, and follow-up of these diseases probably is always advisable. Legal retribution is possible should the medical care provider fail to recognize precocious pseudopuberty and fail to refer the patient to the appropriate subspecialist. For example, if a patient presents with rapid virilization and the medical care provider fails to diagnose the adrenal tumor and tells the parents that this is a normal process, the provider opens the door for legal recourse.

 


More on Precocious Pseudopuberty

Overview: Precocious Pseudopuberty
Differential Diagnoses & Workup: Precocious Pseudopuberty
Treatment & Medication: Precocious Pseudopuberty
Follow-up: Precocious Pseudopuberty
Multimedia: Precocious Pseudopuberty
References
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References

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Further Reading

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Keywords

precocious pseudopuberty, precocious puberty, gonadotropin-independent precocious pseudopuberty, gonadotropin-independent precocious puberty, gonadotropin-dependent precocious pseudopuberty, gonadotropin-dependent precocious puberty, incomplete precocious pseudopuberty, incomplete precocious puberty, peripheral precocious pseudopuberty, peripheral precocious puberty, secondary sexual characteristics, congenital adrenal hyperplasia, CAH, human chorionic gonadotropin, HCG, McCune-Albright syndrome, MAS, aromatase excess syndromes, Cushing syndrome, acromegaly, hyperprolactinemia, ovarian cysts, hyperparathyroidism, bone cysts, polyostotic fibrous dysplasia, hepatobiliary dysfunction, pancreatitis, gastrointestinal polyps, abnormal cardiac muscle cells, 21-hydroxylase deficiency, testotoxicosis, familial male precocious puberty, FMPP, polycystic ovarian disease, ambiguous genitalia, salt-wasting adrenal crisis, treatment, diagnosis

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Contributor Information and Disclosures

Author

Robert J Ferry Jr, MD,, Chief, Division of Pediatric Endocrinology and Metabolism, Le Bonheur Children's Medical Center, University of Tennessee Health Science Center at Memphis, and St. Jude Children's Research Hospital; Brigade Surgeon, 36th Sustainment Brigade, 13th Expeditionary Sustainment Command, U.S. Army
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, Lawson-Wilkins Pediatric Endocrine Society, Society for Pediatric Research, and Texas Pediatric Society
Disclosure: Nutropin Speakers Bureau Honoraria Speaking and teaching; Genotropin Speakers Bureau Honoraria Speaking and teaching; Eli Lilly & Co. Grant/research funds Independent contractor; MacroGenics, Inc. Grant/research funds Independent contractor; Ipsen, S.A. (formerly Tercica, Inc.) Grant/research funds Independent contractor

Coauthor(s)

Cydney L Fenton, MD, FAAP, Consulting Staff, Department of Pediatric Endocrinology, Children's Hospital Medical Center of Akron
Cydney L Fenton, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, Endocrine Society, and Lawson-Wilkins 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, and Lawson-Wilkins Pediatric Endocrine Society
Disclosure: Nothing to disclose.

Medical Editor

Phyllis W Speiser, MD, Chief of Pediatric Endocrinology, Schneider Children's Hospital; Professor of Pediatrics, New York University School of Medicine
Phyllis W Speiser, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Lynne Lipton Levitsky, MD, Chief, Pediatric Endocrine Unit, Massachusetts General Hospital; Associate Professor, Department of Pediatrics, Harvard University 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, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research
Disclosure: Pfizer Grant/research funds P.I.; Tercica Grant/research funds PI, also occasional consultant

CME Editor

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, and Lawson-Wilkins Pediatric Endocrine Society
Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD, Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas and 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, and Southern Society for Pediatric Research
Disclosure: Genentech, Inc. Honoraria Speaking and teaching; Pfizer, Inc. Honoraria Consulting

 
 
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