How is adrenal crisis treated in pediatric adrenal insufficiency (Addison disease)?

Answer

Patients with suspected adrenal crisis should undergo immediate treatment with a parenteral injection of 100 mg (50 mg/m² for young children) hydrocortisone, after which, appropriate fluid resuscitation should be administered, as well as 200 mg (50-100 mg/m² for children) of hydrocortisone/24 hours (by way of continuous IV therapy or 6-hourly injection). If hydrocortisone is unavailable, prednisolone may be used. Dexamethasone is least-preferred, as its onset of action is slow.^[46]

After results for the patient's electrolyte, blood sugar, cortisol, and adrenocorticotropic hormone (ACTH) concentrations are obtained, administer glucocorticoids if adrenal insufficiency is suspected. If a cosyntropin stimulation test is chosen, a single dose of dexamethasone may be administered without interfering with the measurement of the cortisol response to cosyntropin.

No surgical management is needed in most cases.

Did this answer your question?

Yes No

Additional feedback? (Optional)

Thank you for your feedback!

Guran T. Latest Insights on the Etiology and Management of Primary Adrenal Insufficiency in Children. J Clin Res Pediatr Endocrinol. 2017 Dec 30. 9 (Suppl 2):9-22. [Medline]. [Full Text].
Lashansky G, Saenger P, Fishman K, Gautier T, Mayes D, Berg G. Normative data for adrenal steroidogenesis in a healthy pediatric population: age- and sex-related changes after adrenocorticotropin stimulation. J Clin Endocrinol Metab. 1991 Sep. 73(3):674-86. [Medline].
Neary N, Nieman L. Adrenal insufficiency: etiology, diagnosis and treatment. Curr Opin Endocrinol Diabetes Obes. 2010 Apr 6. [Medline].
Heckmann M, Hartmann MF, Kampschulte B, Gack H, Bodeker RH, Gortner L. Cortisol production rates in preterm infants in relation to growth and illness: a noninvasive prospective study using gas chromatography-mass spectrometry. J Clin Endocrinol Metab. 2005 Oct. 90(10):5737-42. [Medline]. [Full Text].
Stewart, PM. The adrenal cortex. Kronenberg HM, Melmed S, Polonsky KS, Larsen RP, eds. Williams Textbook of Endocrinology. 11th ed. Philadelphia, PA: Saunders; 2008. Chapter 14.
Allen DB. Effects of inhaled steroids on growth, bone metabolism, and adrenal function. Adv Pediatr. 2006. 53:101-10. [Medline].
Orme LM, Bond JD, Humphrey MS, Zacharin MR, Downie PA, Jamsen KM. Megestrol acetate in pediatric oncology patients may lead to severe, symptomatic adrenal suppression. Cancer. 2003 Jul 15. 98(2):397-405. [Medline].
Gibb FW, Stewart A, Walker BR, Strachan MW. Adrenal insufficiency in patients on long-term opioid analgesia. Clin Endocrinol (Oxf). 2016 Jun 4. [Medline].
Tsigos C, Arai K, Hung W, Chrousos GP. Hereditary isolated glucocorticoid deficiency is associated with abnormalities of the adrenocorticotropin receptor gene. J Clin Invest. 1993 Nov. 92(5):2458-61. [Medline]. [Full Text].
Clark A, Weber A. Molecular insights into inherited ACTH resistance syndromes. Trends Endocrinol Metab. 1994. 5:209-14. [Full Text].
Handschug K, Sperling S, Yoon SJ, Hennig S, Clark AJ, Huebner A. Triple A syndrome is caused by mutations in AAAS, a new WD-repeat protein gene. Hum Mol Genet. 2001 Feb 1. 10(3):283-90. [Medline].
Grant DB, Barnes ND, Dumic M, Ginalska-Malinowska M, Milla PJ, von Petrykowski W. Neurological and adrenal dysfunction in the adrenal insufficiency/alacrima/achalasia (3A) syndrome. Arch Dis Child. 1993 Jun. 68(6):779-82. [Medline].
Perry R, Kecha O, Paquette J, Huot C, Van Vliet G, Deal C. Primary adrenal insufficiency in children: twenty years experience at the Sainte-Justine Hospital, Montreal. J Clin Endocrinol Metab. 2005 Jun. 90(6):3243-50. [Medline]. [Full Text].
Purandare A, Godil MA, Prakash D, Parker R, Zerah M, Wilson TA. Spontaneous adrenal hemorrhage associated with transient antiphospholipid antibody in a child. Clin Pediatr (Phila). 2001 Jun. 40(6):347-50. [Medline].
Laureti S, Casucci G, Santeusanio F, Angeletti G, Aubourg P, Brunetti P. X-linked adrenoleukodystrophy is a frequent cause of idiopathic Addison's disease in young adult male patients. J Clin Endocrinol Metab. 1996 Feb. 81(2):470-4. [Medline]. [Full Text].
Korenke GC, Roth C, Krasemann E, Hufner M, Hunneman DH, Hanefeld F. Variability of endocrinological dysfunction in 55 patients with X-linked adrenoleucodystrophy: clinical, laboratory and genetic findings. Eur J Endocrinol. 1997 Jul. 137(1):40-7. [Medline]. [Full Text].
Andersson HC, Frentz J, Martínez JE, Tuck-Muller CM, Bellizaire J. Adrenal insufficiency in Smith-Lemli-Opitz syndrome. Am J Med Genet. 1999 Feb 19. 82(5):382-4. [Medline].
Nowaczyk MJM, Wassif CA, Adam MP, et al. Smith-Lemli-Opitz Syndrome. GeneReviews. Updated 2020 Jan 30. [Medline]. [Full Text].
Loose DS, Kan PB, Hirst MA, Marcus RA, Feldman D. Ketoconazole blocks adrenal steroidogenesis by inhibiting cytochrome P450-dependent enzymes. J Clin Invest. 1983 May. 71 (5):1495-9. [Medline]. [Full Text].
Lavin N, ed. Manual of Endocrinology and Metabolism. 4th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2009.
Reimondo G, Puglisi S, Zaggia B, et al. Effects of mitotane on the hypothalamic-pituitary-adrenal axis in patients with adrenocortical carcinoma. Eur J Endocrinol. 2017 Oct. 177 (4):361-7. [Medline]. [Full Text].
Peter M, Viemann M, Partsch CJ, Sippell WG. Congenital adrenal hypoplasia: clinical spectrum, experience with hormonal diagnosis, and report on new point mutations of the DAX-1 gene. J Clin Endocrinol Metab. 1998 Aug. 83(8):2666-74. [Medline]. [Full Text].
Ferraz-de-Souza B, Achermann JC. Disorders of adrenal development. Endocr Dev. 2008. 13:19-32. [Medline].
Kempna P, Fluck CE. Adrenal gland development and defects. Best Pract Res Clin Endocrinol Metab. 2008 Feb. 22(1):77-93. [Medline].
Lalli E, Sassone-Corsi P. DAX-1 and the adrenal cortex. Curr Opin Endocrinol Diabetes. 1999. 6:185-90. [Full Text].
Baker BY, Lin L, Kim CJ, et al. Nonclassic congenital lipoid adrenal hyperplasia: a new disorder of the steroidogenic acute regulatory protein with very late presentation and normal male genitalia. J Clin Endocrinol Metab. 2006 Dec. 91(12):4781-5. [Medline].
Kim CJ, Lin L, Huang N, et al. Severe combined adrenal and gonadal deficiency caused by novel mutations in the cholesterol side chain cleavage enzyme, P450scc. J Clin Endocrinol Metab. 2008 Mar. 93(3):696-702. [Medline].
Miller WL. Minireview: regulation of steroidogenesis by electron transfer. Endocrinology. 2005 Jun. 146(6):2544-50. [Medline].
Pandey AV, Fluck CE, Huang N, Tajima T, Fujieda K, Miller WL. P450 oxidoreductase deficiency: a new disorder of steroidogenesis affecting all microsomal P450 enzymes. Endocr Res. 2004 Nov. 30(4):881-8. [Medline].
Fluck CE, Tajima T, Pandey AV, Arlt W, Okuhara K, Verge CF. Mutant P450 oxidoreductase causes disordered steroidogenesis with and without Antley-Bixler syndrome. Nat Genet. 2004 Mar. 36(3):228-30. [Medline].
Vinclair M, Broux C, Faure P, et al. Duration of adrenal inhibition following a single dose of etomidate in critically ill patients. Intensive Care Med. 2008 Apr. 34(4):714-9. [Medline].
Lamberts SW, Bruining HA, de Jong FH. Corticosteroid therapy in severe illness. N Engl J Med. 1997 Oct 30. 337(18):1285-92. [Medline].
Pizarro CF, Troster EJ, Damiani D, Carcillo JA. Absolute and relative adrenal insufficiency in children with septic shock. Crit Care Med. 2005 Apr. 33(4):855-9. [Medline].
Fleseriu M, Loriaux DL. "Relative" adrenal insufficiency in critical illness. Endocr Pract. 2009 Sep-Oct. 15(6):632-40. [Medline].
Hsieh S, White PC. Presentation of primary adrenal insufficiency in childhood. J Clin Endocrinol Metab. 2011 Jun. 96(6):E925-8. [Medline].
Willis AC, Vince FP. The prevalence of Addison's disease in Coventry, UK. Postgrad Med J. 1997 May. 73(859):286-8. [Medline].
Laureti S, Vecchi L, Santeusanio F, Falorni A. Is the prevalence of Addison's disease underestimated? [letter]. J Clin Endocrinol Metab. 1999 May. 84(5):1762. [Medline]. [Full Text].
Olafsson AS, Sigurjonsdottir HA. Increasing prevalence of Addison disease: results from a nationwide study. Endocr Pract. 2016 Jan. 22 (1):30-5. [Medline].
Chantzichristos D, Persson A, Eliasson B, et al. Mortality in patients with diabetes mellitus and Addison's disease: a nationwide, matched, observational cohort study. Eur J Endocrinol. 2017 Jan. 176 (1):31-39. [Medline].
Kamrath C. Beyond the adrenals: Organ manifestations in inherited primary adrenal insufficiency in children. Eur J Endocrinol. 2020 Mar. 182 (3):C9-C12. [Medline]. [Full Text].
Arlt W, Allolio B. Adrenal insufficiency. Lancet. 2003 May 31. 361(9372):1881-93. [Medline].
Besser GM, Thorner MO. Adrenal insufficiency. Clinical Endocrinology. St Louis, Mo: Mosby-Year Book; 1996. [CD-ROM]:
Dellinger RP, Levy MM, Carlet JM, et al. Surviving Sepsis Campaign: international guidelines for management of severe sepsis and septic shock: 2008. Crit Care Med. 2008 Jan. 36(1):296-327. [Medline].
Clark L, Preissig C, Rigby MR, Bowyer F. Endocrine issues in the pediatric intensive care unit. Pediatr Clin North Am. 2008 Jun. 55(3):805-33, xiii. [Medline].
Kamoi K, Tamura T, Tanaka K, Ishibashi M, Yamaji T. Hyponatremia and osmoregulation of thirst and vasopressin secretion in patients with adrenal insufficiency. J Clin Endocrinol Metab. 1993 Dec. 77(6):1584-8. [Medline]. [Full Text].
[Guideline] Bornstein SR, Allolio B, Arlt W, et al. Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2016 Feb 10. 101 (2):364-89. [Medline]. [Full Text].
Chao CS, Shi RZ, Kumar RB, Aye T. Salivary cortisol levels by tandem mass spectrometry during high dose ACTH stimulation test for adrenal insufficiency in children. Endocrine. 2020 Jan. 67 (1):190-7. [Medline].
Kronenberg HM, Melmed S, Polonsky KS, Larson PR, eds. Williams Textbook of Endocrinology. 11th ed. Philadelphia, PA: Saunders; 2008. 229.
Iwanaga K, Yamamoto A, Matsukura T, Niwa F, Kawai M. Corticotrophin-releasing hormone stimulation tests for the infants with relative adrenal insufficiency. Clin Endocrinol (Oxf). 2017 Dec. 87 (6):660-4. [Medline].
Kazlauskaite R, Evans AT, Villabona CV, et al. Corticotropin tests for hypothalamic-pituitary- adrenal insufficiency: a metaanalysis. J Clin Endocrinol Metab. 2008 Nov. 93(11):4245-53. [Medline].
Thaler LM. Comment on the low-dose corticotropin stimulation test is more sensitive than the high-dose test. [letter]. J Clin Endocrinol Metab. 1998 Dec. 83(12):4530-1; author reply 4532-3. [Medline].
Tordjman K, Jaffe A, Greenman Y, Stern N. Comments on the comparison of low and high dose corticotropin stimulation tests in patients with pituitary disease. J Clin Endocrinol Metab. 1998 Dec. 83(12):4530; author reply 4532-3. [Medline].
Mayenknecht J, Diederich S, Bahr V, Plockinger U, Oelkers W. Comparison of low and high dose corticotropin stimulation tests in patients with pituitary disease. J Clin Endocrinol Metab. 1998 May. 83(5):1558-62. [Medline]. [Full Text].
Dickstein G. Commentary to the article: Comparison of low and high dose corticotropin stimulation tests in patients with pituitary disease [letter]. J Clin Endocrinol Metab. 1998 Dec. 83(12):4531-3. [Medline]. [Full Text].
Neary N, Nieman L. Adrenal insufficiency: etiology, diagnosis and treatment. Curr Opin Endocrinol Diabetes Obes. 2010 Jun. 17(3):217-23. [Medline]. [Full Text].
Libe R, Barbetta L, Dall'Asta C, et al. Effects of dehydroepiandrosterone (DHEA) supplementation on hormonal, metabolic and behavioral status in patients with hypoadrenalism. J Endocrinol Invest. 2004 Sep. 27(8):736-41. [Medline].
van Thiel SW, Romijn JA, Pereira AM, et al. Effects of dehydroepiandrostenedione, superimposed on growth hormone substitution, on quality of life and insulin-like growth factor I in patients with secondary adrenal insufficiency: a randomized, placebo-controlled, cross-over trial. J Clin Endocrinol Metab. 2005 Jun. 90(6):3295-303. [Medline].
Quinkler M, Ekman B, Marelli C, et al. Prednisolone is associated with a worse lipid profile than hydrocortisone in patients with adrenal insufficiency. Endocr Connect. 2017 Jan. 6 (1):1-8. [Medline]. [Full Text].
Chandy DD, Bhatia E. Bone mineral density in patients with Addison disease on replacement therapy with prednisolone. Endocr Pract. 2016 Apr. 22 (4):434-9. [Medline].
Merke DP, Chrousos GP, Eisenhofer G, et al. Adrenomedullary dysplasia and hypofunction in patients with classic 21-hydroxylase deficiency. N Engl J Med. 2000 Nov 9. 343(19):1362-8. [Medline].
Coutant R, Maurey H, Rouleau S, et al. Defect in epinephrine production in children with craniopharyngioma: functional or organic origin?. J Clin Endocrinol Metab. 2003 Dec. 88(12):5969-75. [Medline].
Frank GR, Speiser PW, Griffin KJ, Stratakis CA. Safety of medications and hormones used in pediatric endocrinology: adrenal. Pediatr Endocrinol Rev. 2004 Nov. 2 Suppl 1:134-45. [Medline].
[Guideline] Reznik Y, Barat P, Bertherat J, et al. SFE/SFEDP adrenal insufficiency French consensus: Introduction and handbook. Ann Endocrinol (Paris). 2018 Jan 12. [Medline].

Media Gallery

Regulation of the adrenal cortex. ACTH = adrenocorticotropic hormone; CRF = corticotropin-releasing factor; neg. = negative.
Left photograph shows hyperpigmentation on the dorsum of a patient's hand before the treatment of primary adrenal insufficiency. Right photograph shows normal pigmentation after treatment.
Left photograph shows a patient with Addison disease who has prominent pigmentation in areas not exposed to the sun, such as the palmar creases. Right photograph shows normal pigmentation after treatment.
Left photograph shows vitiligo in a patient with autoimmune adrenalitis. Right photograph shows an area of hyperpigmentation surrounding the vitiligo.
Left photomicrograph shows autoimmune adrenalitis. Right photomicrograph shows tuberculous adrenalitis. Note the caseous granuloma.
Computed tomography scan shows enlarged adrenal glands in a patient with early active autoimmune adrenalitis. Patients with chronic disease present with the opposite picture of hypotrophic adrenals.

of 6

Author

Kimberly Tafuri, DO Assistant Professor, Department of Pediatrics, Division of Pediatric Endocrinology, Stony Brook Children's Hospital

Kimberly Tafuri, DO 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.

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 Medical Association, American Pediatric Society, Association of Clinical Scientists, Endocrine Society, Florida Medical Association, Pediatric Endocrine Society, Society for Pediatric Research, Southern Society for Pediatric Research, Society for the Study of Reproduction, American Federation for Clinical Research, Pituitary Society

Disclosure: Nothing to disclose.

Chief Editor

Sasigarn A Bowden, MD Associate Professor of Pediatrics, Section of Pediatric Endocrinology, Metabolism and Diabetes, Department of Pediatrics, Ohio State University College of Medicine; Pediatric Endocrinologist, Associate Fellowship Program Director, Division of Endocrinology, Nationwide Children’s Hospital; Affiliate Faculty/Principal Investigator, Center for Clinical Translational Research, Research Institute at Nationwide Children’s Hospital

Sasigarn A Bowden, MD is a member of the following medical societies: American Society for Bone and Mineral Research, Central Ohio Pediatric Society, Endocrine Society, International Society for Pediatric and Adolescent Diabetes, Pediatric Endocrine Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Karl S Roth, MD Retired Professor and Chair, Department of Pediatrics, Creighton University School of Medicine

Karl S Roth, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Nutrition, American Pediatric Society, American Society for Nutrition, American Society of Nephrology, Association of American Medical Colleges, Medical Society of Virginia, New York Academy of Sciences, Sigma Xi, Society for Pediatric Research, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Phyllis W Speiser, MD Chief, Division of Pediatric Endocrinology, Steven and Alexandra Cohen Children's Medical Center of New York; Professor of Pediatrics, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell

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.

Thomas A Wilson, MD Professor of Clinical Pediatrics, Chief and Program Director, Division of Pediatric Endocrinology, Department of Pediatrics, The School of Medicine at Stony Brook University Medical Center

Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Pediatric Endocrine Society, Phi Beta Kappa

Disclosure: Nothing to disclose.

How is adrenal crisis treated in pediatric adrenal insufficiency (Addison disease)?

Related Questions:

About

Membership

Apps

WebMD Network

Editions

What to Read Next on Medscape

How is adrenal crisis treated in pediatric adrenal insufficiency (Addison disease)?

Related Questions:

References

Tables

Contributor Information and Disclosures

Find Us On

About

Membership

Apps

WebMD Network

Editions

What to Read Next on Medscape

Related Conditions and Diseases

Medscape Consult

News & Perspective

Tools

Most Popular Articles

Recommended