eMedicine Specialties > Pediatrics: General Medicine > Endocrinology

Allgrove (AAA) Syndrome: Follow-up

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
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; Jacalyn Bishop, MD, Pediatric Endocrinologist, Private Practice
Contributor Information and Disclosures

Updated: Feb 20, 2009

Follow-up

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.

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.

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.

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.

Miscellaneous

Medicolegal Pitfalls

  • Careful replacement of glucocorticoids in patients with known adrenal insufficiency is critical to avoid an adrenal crisis and to allow for normal growth in these children.
  • Careful documentation of stress dose education and frequent monitoring of growth are essential.
 


More on Allgrove (AAA) Syndrome

Overview: Allgrove (AAA) Syndrome
Differential Diagnoses & Workup: Allgrove (AAA) Syndrome
Treatment & Medication: Allgrove (AAA) Syndrome
Follow-up: Allgrove (AAA) Syndrome
References
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References

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Further Reading

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Keywords

Allgrove (AAA) syndrome, 4A syndrome, triple-A syndrome, achalasia-addisonianism-alacrima syndrome, achalasia-addisonianism-alacrima-autonomic neuropathy syndrome, addisonian-achalasia syndrome, alacrima-achalasia-addisonianism, glucocorticoid deficiency, achalasia, hypoadrenalism with achalasia, isolated glucocorticoid deficiency, adrenal insufficiency, growth failure, developmental delay, hypoglycemia, microcephaly, hypoglycemia, hyperpigmentation, hyperkeratosis

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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 Lawson-Wilkins 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.

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

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, Lawson-Wilkins 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.

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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