Allgrove (AAA) Syndrome Follow-up

  • Author: Bruce A Boston, MD; Chief Editor: Stephen Kemp, MD, PhD   more...
 
Updated: Feb 20, 2009
 

Further Inpatient Care

  • Glucocorticoid replacement: Inpatient care of Allgrove (AAA) syndrome is primarily directed toward maintaining adequate glucocorticoid replacement.
  • Reflux precautions
    • Patients with achalasia and patients who have undergone esophageal pneumatic dilatation or myotomy are at risk for reflux with recurrent aspiration.
    • Standard reflux precautions are warranted (eg, elevating head of bed, careful feeding of infants).
    • Acid reduction therapy is often warranted.
  • Frequent application of topical eye lubrication is warranted, as patients with alacrima are at risk for developing severe keratopathy due to excessive ocular dehydration.
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Complications

  • Glucocorticoid therapy
    • Overtreatment with glucocorticoids leads to growth failure and features of Cushing syndrome.
    • Undertreatment, particularly during illness, can lead to adrenal crisis with hypotension, hypoglycemia, and possibly death.
  • Achalasia
    • Recurrent aspiration, documented in many patients with achalasia, can lead to acute pneumonitis, choking, and death.
    • Achalasia is also associated with chronic lung disease, as indicated through radiographic studies and pulmonary function tests.
  • Alacrima: Patients with reduced lacrimation are at high risk for developing keratoconjunctivitis sicca and other keratopathy associated with dehydration-induced ocular tissue damage.
  • Autonomic neuropathy and other neurologic disturbance
    • Slow neurologic deterioration occurs in many patients. This most frequently includes mild mental retardation and autonomic neuropathy but may include ataxia and muscle weakness as well.
    • Pediatric patients commonly show developmental delay. Determining if this impairment is a primary feature of the syndrome or simply a reflection of the episodic hypoglycemia that occurs in association with glucocorticoid deficiency is difficult.
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Prognosis

  • Provided the patient is effectively managed, a normal lifespan and childbirth are possible.
  • Cases of parkinsonism, peripheral neuropathy, and seizures developing in patients have been reported, but whether this also occurs in patients who received an early diagnosis and long-term effective medical and surgical management is unclear.
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Patient Education

  • Glucocorticoid therapy
    • Patients must be instructed on the appropriate management of stress dosing of glucocorticoids.
    • A medical alert bracelet or necklace should be worn at all times.
    • Because of the possibility of severe stress or trauma in a situation where medical assistance is not immediately available, the patient and his or her family members should be instructed to inject hydrocortisone or dexamethasone intramuscularly in a dose appropriate for the size of the patient, typically 100 mg hydrocortisone or 2 mg dexamethasone for adolescents and adults.
  • Gastroesophageal reflux
    • Families with an affected infant should be provided with instructions for reflux precautions for eating and sleeping.
    • Recurrent vomiting and eating difficulties should be evaluated by a physician.
  • Alacrima: The importance of maintaining a regular schedule of topical ocular lubrication to prevent dehydration-induced keratopathy and opportunistic ocular infection should be emphasized to patients and their families.
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Contributor Information and Disclosures
Author

Bruce A Boston, MD  Chief, Division of Pediatric Endocrinology, Director, Pediatric Endocrine Training Program, Associate Professor, Department of Pediatrics, Division of Pediatric Endocrinology, Oregon Health Sciences University and Doernbecher Children's Hospital

Bruce A Boston, MD is a member of the following medical societies: Alpha Omega Alpha, American Diabetes Association, Endocrine Society, and Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Coauthor(s)

Daniel L Marks, MD, PhD  Assistant Professor, Department of Pediatrics, Division of Pediatric Endocrinology, Oregon Health Sciences University and Doerenbecher Children's Hospital

Daniel L Marks, MD, PhD is a member of the following medical societies: American Academy of Pediatrics and Oregon Medical Association

Disclosure: Nothing to disclose.

Jacalyn Bishop, MD  Pediatric Endocrinologist, Private Practice

Disclosure: Nothing to disclose.

Specialty Editor Board

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, and Society for Pediatric Research

Disclosure: Nothing to disclose.

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.

Barry B Bercu, MD  Professor, Departments of Pediatrics, Molecular Pharmacology and Physiology, University of South Florida College of Medicine, All Children's Hospital

Barry B Bercu, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Federation for Clinical Research, American Medical Association, American Pediatric Society, Association of Clinical Scientists, Endocrine Society, Florida Medical Association, Pediatric Endocrine Society, Pituitary Society, Society for Pediatric Research, Society for the Study of Reproduction, and Southern Society for Pediatric Research

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 Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD  Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas College of Medicine 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: Nothing to disclose.

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