Allgrove (AAA) Syndrome Medication

  • Author: Bruce A Boston, MD; Chief Editor: Stephen Kemp, MD, PhD   more...
 
Updated: Mar 1, 2012
 

Corticosteroids

Class Summary

Careful replacement of glucocorticoids in patients with known adrenal insufficiency is critical to avoid adrenal crisis and to allow for normal growth in children. Growth must be monitored closely, as overtreatment with glucocorticoids impairs linear growth.

Providing stress doses of hydrocortisone during illness or injury is another important feature of medical management. Typically, a doubling or tripling of the oral dose is sufficient for routine illnesses. A larger increase in dose (provided IV if necessary) is required for severe illness and major trauma (see Adrenal Insufficiency).

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Hydrocortisone (Hydrocortone, Cortef)

 

Has mineralocorticoid and glucocorticoid effects. Useful in management of inflammation caused by immune response.

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Prednisone (Deltasone)

 

Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production.

In patients who have difficulty complying, it is acceptable to replace hydrocortisone with an equipotent dose of prednisone (prednisone is 4-5 times as potent as hydrocortisone).

Doses can be adjusted based on symptoms and monitoring linear growth and weight gain.

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Fludrocortisone (Florinef)

 

Provides physiologic replacement of mineralocorticoid deficiency.

Dose must be sufficient to lower plasma renin activity to normal without inducing hypertension.

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Dexamethasone (Decadron)

 

For various allergic and inflammatory diseases. Decreases inflammation by suppressing migration of PMN leukocytes and reducing capillary permeability.

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Contributor Information and Disclosures
Author

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

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 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, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

References

  1. Allgrove J, Clayden GS, Grant DB. Familial glucocorticoid deficiency with achalasia of the cardia and deficient tear production. Lancet. Jun 17 1978;1(8077):1284-6. [Medline].

  2. Kasar PA, Khadilkar VV, Tibrewala VN. Allgrove syndrome. Indian J Pediatr. Oct 2007;74(10):959-61. [Medline].

  3. Kelch RP, Kaplan SL, Biglieri EG. Hereditary adrenocortical unresponsiveness to adrenocorticotropic hormone. J Pediatr. Oct 1972;81(4):726-36. [Medline].

  4. Counahan R, West R. Ocular and fingertip abnormalities in isolated glucocorticoid deficiency. J Pediatr. Oct 1974;85(4):580-1. [Medline].

  5. Gazarian M, Cowell CT, Bonney M. The "4A" syndrome: adrenocortical insufficiency associated with achalasia, alacrima, autonomic and other neurological abnormalities. Eur J Pediatr. Jan 1995;154(1):18-23. [Medline].

  6. Sarathi V, Shah NS. Triple-A syndrome. Adv Exp Med Biol. 2010;685:1-8. [Medline].

  7. Alhussaini B, Gottrand F, Goutet JM, Scaillon M, Michaud L, Spyckerelle C, et al. Clinical and manometric characteristics of Allgrove syndrome. J Pediatr Gastroenterol Nutr. Sep 2011;53(3):271-4. [Medline].

  8. Moschos MM, Margetis I, Koehler K, Gatzioufas Z, Huebner A. New ophthalmic features in a family with triple A syndrome. Int Ophthalmol. Jun 2011;31(3):239-43. [Medline].

  9. Vallet AE, Verschueren A, Petiot P, Vandenberghe N, Nicolino M, Roman S, et al. Neurological features in adult Triple-A (Allgrove) syndrome. J Neurol. Jan 2012;259(1):39-46. [Medline].

  10. Chen W, Kelly MA, Opitz-Araya X. Exocrine gland dysfunction in MC5-R-deficient mice: evidence for coordinated regulation of exocrine gland function by melanocortin peptides. Cell. Dec 12 1997;91(6):789-98. [Medline].

  11. Chu ML, Berlin D, Axelrod FB. Allgrove syndrome: documenting cholinergic dysfunction by autonomic tests. J Pediatr. Jul 1996;129(1):156-9. [Medline].

  12. Clark AJ, Weber A. Adrenocorticotropin insensitivity syndromes. Endocr Rev. Dec 1998;19(6):828-43. [Medline].

  13. Dumic M, Radica A, Jusic A. Selective ACTH insensitivity associated with autonomic nervous system disorders and sensory polyneuropathy. Eur J Pediatr. Nov 1987;146(6):592-4. [Medline].

  14. Ehrich E, Aranoff G, Johnson WG. Familial achalasia associated with adrenocortical insufficiency, alacrima, and neurological abnormalities. Am J Med Genet. Mar 1987;26(3):637-44. [Medline].

  15. Fagan JE, McArthur RG, Machida H. Palatopharyngeal incompetence in association with esophageal dysmotility, acquired glucocorticoid deficiency, and deficient tear production. Clin Invest Med. Jul 1987;10(4):345-9. [Medline].

  16. Grant DB, Barnes ND, Dumic M. Neurological and adrenal dysfunction in the adrenal insufficiency/alacrima/achalasia (3A) syndrome. Arch Dis Child. Jun 1993;68(6):779-82. [Medline].

  17. Grant DB, Dunger DB, Smith I. Familial glucocorticoid deficiency with achalasia of the cardia associated with mixed neuropathy, long-tract degeneration and mild dementia. Eur J Pediatr. Feb 1992;151(2):85-9. [Medline].

  18. Handschug K, Sperling S, Kim Yoon S. Triple A syndrome is caused by mutations in AAAS, a new WD-repeat protein gene. Human Molecular Genetics. 2001;10:283-290. [Medline]. [Full Text].

  19. Heinrichs C, Tsigos C, Deschepper J. Familial adrenocorticotropin unresponsiveness associated with alacrima and achalasia: biochemical and molecular studies in two siblings with clinical heterogeneity. Eur J Pediatr. Mar 1995;154(3):191-6. [Medline].

  20. Kimber J, McLean BN, Hammans SR. Allgrove or 4 "A" syndrome: an autosomal recessive syndrome causing multisystem neurological disease. J Neurol Neurosurg Psychiatry. 2003;74:654-657. [Medline]. [Full Text].

  21. Lanes R, Plotnick LP, Bynum TE. Glucocorticoid and partial mineralocorticoid deficiency associated with achalasia. J Clin Endocrinol Metab. Feb 1980;50(2):268-70. [Medline].

  22. Makari G, Hoffman WH, Carroll JE. Autonomic dysfunction and adrenocortical unresponsiveness to ACTH. J Child Neurol. Jul 1988;3(3):174-6. [Medline].

  23. Moore PS, Couch RM, Perry YS. Allgrove syndrome: an autosomal recessive syndrome of ACTH insensitivity, achalasia and alacrima. Clin Endocrinol (Oxf). Feb 1991;34(2):107-14. [Medline].

  24. Moser HW, Moser AB, Frayer KK. Adrenoleukodystrophy: increased plasma content of saturated very long chain fatty acids. Neurology. Oct 1981;31(10):1241-9. [Medline].

  25. Mountjoy KG, Robbins LS, Mortrud MT. The cloning of a family of genes that encode the melanocortin receptors. Science. Aug 28 1992;257(5074):1248-51. [Medline].

  26. Mullaney PB, Weatherhead R, Millar L. Keratoconjunctivitis sicca associated with achalasia of the cardia, adrenocortical insufficiency, and lacrimal gland degeneration: Keratoconjunctivitis sicca secondary to lacrimal gland degeneration may parallel degenerative changes in esophageal and. Ophthalmology. Apr 1998;105(4):643-50. [Medline].

  27. Nihoul-Fekete C, Bawab F, Lortat-Jacob S. Achalasia of the esophagus in childhood: surgical treatment in 35 cases with special reference to familial cases and glucocorticoid deficiency association. J Pediatr Surg. Oct 1989;24(10):1060-3. [Medline].

  28. Prpic I, Huebner A, Persic M, et al. Triple A syndrome: genotype-phenotype assessment. Clin Genet. May 2003;63(5):415-7. [Medline].

  29. Stratakis CA, Lin JP, Pras E. Segregation of Allgrove (triple-A) syndrome in Puerto Rican kindreds with chromosome 12 (12q13) polymorphic markers. Proc Assoc Am Physicians. Sep 1997;109(5):478-82. [Medline].

  30. Stuckey BG, Mastaglia FL, Reed WD. Glucocorticoid insufficiency, achalasia, alacrima with autonomic motor neuropathy. Ann Intern Med. Jan 1987;106(1):61-3. [Medline].

  31. Thomas RJ, Sen S, Zachariah N. Achalasia cardia in infancy and childhood: an Indian experience. J R Coll Surg Edinb. Apr 1998;43(2):103-4. [Medline].

  32. Tsao CY, Romshe CA, Lo WD. Familial adrenal insufficiency, achalasia, alacrima, peripheral neuropathy, microcephaly, normal plasma very long chain fatty acids, and normal muscle mitochondrial respiratory chain enzymes. J Child Neurol. Apr 1994;9(2):135-8. [Medline].

  33. Tsigos C, Arai K, Latronico AC. A novel mutation of the adrenocorticotropin receptor (ACTH-R) gene in a family with the syndrome of isolated glucocorticoid deficiency, but no ACTH-R abnormalities in two families with the triple A syndrome. J Clin Endocrinol Metab. Jul 1995;80(7):2186-9. [Medline].

  34. Tuck JS, Bisset RA, Doig CM. Achalasia of the cardia in childhood and the syndrome of achalasia alacrima and ACTH insensitivity. Clin Radiol. Oct 1991;44(4):260-4. [Medline].

  35. Vaughan WH, Williams JL. Familial achalasia with pulmonary complications in children. Radiology. May 1973;107(2):407-9. [Medline].

  36. Verma S, Brown S, Dakkak M. Association of adult achalasia and alacrima. Dig Dis Sci. May 1999;44(5):876-8. [Medline].

  37. Weber A, Wienker TF, Jung M. Linkage of the gene for the triple A syndrome to chromosome 12q13 near the type II keratin gene cluster. Hum Mol Genet. Dec 1996;5(12):2061-6. [Medline].

 
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