Medscape is available in 5 Language Editions – Choose your Edition here.


Pediatric Adrenal Insufficiency (Addison Disease)

  • Author: Phyllis W Speiser, MD; Chief Editor: Stephen Kemp, MD, PhD  more...
Updated: Jun 19, 2016


Adrenal insufficiency (Addison disease) can be classified as primary, which occurs when the adrenal gland itself is dysfunctional, or secondary, also called central adrenal insufficiency, which occurs when a lack of secretion of corticotropin-releasing hormone (CRH) from the hypothalamus or of adrenocorticotropic hormone (ACTH) from the pituitary leads to hypofunction of the adrenal cortex. See the image below.

Regulation of the adrenal cortex. ACTH = adrenocor Regulation of the adrenal cortex. ACTH = adrenocorticotropic hormone; CRF = corticotropin-releasing factor; neg. = negative.

Adrenal insufficiency (Addison disease) can further be classified as congenital or acquired (see Etiology).

See also Addison Disease (Adrenal Insufficiency).



The adrenal cortex is divided into 3 major anatomic zones. The zona glomerulosa produces aldosterone, and the zonae fasciculata and reticularis together produce cortisol and adrenal androgens. A fetal zone, unique to primates, produces dehydroepiandrosterone (DHEA), a precursor of both androgens and estrogens. This zone involutes within the first few months of postnatal life.

Aldosterone secretion is primarily regulated by the renin-angiotensin system. Increased serum potassium concentrations can also stimulate aldosterone secretion. Cortisol secretion is regulated by adrenocorticotropic hormone (ACTH), which, in turn, is regulated by corticotropin-releasing hormone (CRH) from the hypothalamus. Serum cortisol inhibits the secretion of both CRH and ACTH to prevent excessive secretion of cortisol from the adrenal glands.

ACTH partially regulates adrenal androgen secretion; other unknown factors contribute to this regulation as well. ACTH not only stimulates cortisol secretion but also promotes growth of the adrenal cortex in conjunction with growth factors such as insulinlike growth factor (IGF)-1 and IGF-2.[1]



Iatrogenic central adrenal insufficiency as well as acquired and congenital primary adrenal insufficiency (Addison disease) are briefly discussed in this section.

Iatrogenic central adrenal insufficiency

Most cases of adrenal insufficiency (Addison disease) are iatrogenic, caused by long-term administration of glucocorticoids. A mere 2 weeks' exposure to pharmacologic doses of glucocorticoids can suppress the corticotropin-releasing hormone (CRH)–adrenocorticotropic hormone (ACTH)–adrenal axis. The suppression can be so great that acute withdrawal or stress may prevent the axis from responding with sufficient cortisol production to prevent an acute adrenal crisis.

Treatment with megestrol acetate, an orexigenic agent, has also resulted in iatrogenic adrenal suppression. The mechanism is presumably related to the glucocorticoid properties of megestrol acetate.[2]

A study by Gibb et al found that in four out of 48 patients on long-term opioid analgesia for chronic pain (8.3%), the basal morning plasma cortisol concentration was below 100 nmol/L, indicating that such treatment can suppress the hypothalamic-pituitary-adrenal axis in a clinically significant proportion of patients.[3]

Other causes of central adrenal insufficiency include congenital or acquired hypopituitarism and ACTH unresponsiveness. This unresponsiveness may be isolated (as in Familial Glucocorticoid Deficiency) (Online Mendelian Inheritance in Man database [OMIM] 202200),[4, 5] or it may be associated with achalasia and alacrima (as in achalasia-addisonism-alacrima syndrome, or triple A syndrome [AAAS]) (OMIM 231550).[6, 7]

Acquired primary adrenal insufficiency

In developed countries, the most common cause of adrenal insufficiency (Addison disease) is autoimmune destruction of the adrenal cortex.[8] This disorder may occur in isolation or may be part of a polyglandular autoimmune disorder (PGAD).

Patients with type 1 PGAD (OMIM 240300) usually present in the first decade of life with mucocutaneous candidiasis or hypoparathyroidism. This is an autosomal recessive disorder that involves the AIRE gene on chromosome 21 and presents with all or some of the following features:

  • Chronic mucocutaneous candidiasis
  • Hypoparathyroidism
  • Adrenal failure
  • Gonadal failure
  • Vitiligo
  • Alopecia
  • Hypothyroidism
  • Type 1 diabetes mellitus
  • Pernicious anemia
  • Steatorrhea

Type 2 PGAD (Schmidt syndrome; OMIM 269200) consists of type 1 diabetes mellitus, autoimmune thyroid disease, and adrenal failure. Individuals with this condition generally present in the second or third decades of life, although some components of the syndrome may be present in the pediatric age group. Type 2 PGAD is transmitted as an autosomal disorder with variable penetrance. Addison disease should be considered in patients with type 1 diabetes and unexplained fatigue, hypotension, hypoglycemia, hyponatremia and hyperkalemia.

Other acquired causes of adrenal failure include the following:

  • Adrenal hemorrhage [9]
  • Infections (eg, tuberculosis [TB], human immunodeficiency virus [HIV] infection)
  • Neoplastic destruction
  • Metabolic disorders (eg, various forms of adrenal leukodystrophy [OMIM 300100], [10, 11] Wolman disease [OMIM 278000], Smith-Lemli-Opitz syndrome [OMIM 270400] [12]
  • Administration of the anesthetic agent etomidate [13]

Hemochromatosis may cause either primary (hereditary form OMIM 235200) or secondary adrenal insufficiency. Among patients with thalassemia or other forms of anemia who have received multiple transfusions, iron deposition in the pituitary and/or adrenal glands may also cause adrenal insufficiency.

Congenital primary adrenal insufficiency

Congenital Addison disease may occur as a result of adrenal hypoplasia[14, 15, 16] or hyperplasia.

Inherited as an X-linked disorder, adrenal hypoplasia congenita (OMIM 300200) is caused by deletion or mutation of the DAX1/NR0B1 gene on chromosome Xp21.2, and is additionally associated with hypogonadotrophic hypogonadism and primary defects in sperm production.[17] This is often part of a contiguous gene deletion that also involves the genes for glycerol kinase deficiency and dystrophin, resulting in elevations in serum glycerol (often measured using a triglyceride assay) and Duchenne muscular dystrophy. An alternate form, non-X linked, is characterized by intrauterine growth retardation and skeletal and genital anomalies (ie, IMAGe syndrome) (OMIM 300290). A third type of familial of adrenal hypoplasia congenita of uncertain etiology has been described (OMIM 240200).

Congenital adrenal hyperplasia results from a deficiency of one of several enzymes required for adrenal synthesis of cortisol. Symptoms of adrenal insufficiency (Addison disease) most often develop with combined deficiencies of cortisol and aldosterone. The most prevalent form of congenital adrenal hyperplasia is caused by a deficiency in steroid 21-hydroxylase (OMIM 201910).

Lipoid adrenal hyperplasia is another rare form of adrenal insufficiency (Addison disease) caused by a mutation in the steroid acute regulatory protein (ie, STAR protein) (OMIM 201710)[18] or a mutation in the cholesterol side-chain cleavage gene (at the cytochrome P450 [CYP] 11A locus) (OMIM 118485).[19] This disease causes a defective synthesis of all adrenocortical hormones. In its complete form, the disease is lethal.

Mutations or deletions involving CYP oxidoreductase, a flavoprotein that provides electrons to various enzyme systems, results in combined deficiencies of 17-hydroxylase, 21-hydroxylase, and 17-20 lyase activities. The result is adrenal insufficiency (Addison disease), which is often accompanied by skeletal dysplasia, genital anomalies, and primary hypogonadism (OMIM 613571).[20, 21, 22]

Relative adrenal insufficiency

The term relative adrenal insufficiency (Addison disease) has been coined to describe patients with critical illness who do not appear to mount the cortisol response expected given the severity of their illness.

Some patients developed adrenal insufficiency (Addison disease) after exposure to etomidate, an agent known to interfere with cortisol synthesis.[13] Early reports indicated improvements in outcome when such patients were provided with glucocorticoids at stress doses. Subsequent studies have clearly confirmed the fact that a substantial number of patients with critical illness who have not been exposed to etomidate have low serum cortisol concentrations.[23] Some studies have found that those with very high concentrations of cortisol have a worse prognosis and a higher complication rate of secondary sepsis or intestinal perforation. Controlled trials in adults have failed to confirm the benefit of glucocorticoid replacement therapy.

Among critically ill children, a low incremental cortisol response to ACTH does not predict mortality.[24] There is still much controversy regarding how to best diagnose adrenal insufficiency in hospitalized children and adults, as well as whether and when to treat. Thus, the decision to treat a critically ill patient with glucocorticoids must be made on a case-by-case basis until further definitive evidence is available.[25]



Primary adrenal insufficiency (Addison disease) is uncommon in the United States. By comparison, iatrogenic central adrenal insufficiency is a more frequent cause of morbidity and mortality, although its exact incidence is unknown. Retrospective case review in one US urban center suggests that the prevalence of adrenal insufficiency in childhood is higher than previously suspected, approximately equivalent to that of congenital adrenal hyperplasia.[26] Adrenal insufficiency (Addison disease) secondary to congenital adrenal hyperplasia occurs in approximately 1 per 16,000 infants.

Willis and Vince collected data from Coventry County, Great Britain, where the prevalence of adrenal insufficiency (Addison disease) was similarly reported as 110 cases per million persons of all ages.[27] More than 90% of cases have been attributed to autoimmune disease. An Italian study provided statistics comparable to those observed in Great Britain:[28]  an estimated 117 cases per million persons. A study by Olafsson and Sigurjonsdottir estimated the prevalence of primary adrenal insufficiency in Iceland to be 22.1 per 100,000 population.[29]

Worldwide, the most common cause of adrenal insufficiency (Addison disease) is tuberculosis (TB), with a calculated incidence of this condition caused by TB at approximately 5-6 cases per million persons per year.

Although there does not appear to be a racial predilection, sex and age-related differences have been observed. Autoimmune adrenal insufficiency (Addison disease) is more common in female individuals than in male individuals and in adults than children, whereas adrenal insufficiency due to adrenoleukodystrophy is limited to male individuals, because it is X linked.

A form of congenital adrenal hypoplasia due to a defect in DAX1/NR0B1 is also X-linked and, therefore, is confined to males. Secondary forms of adrenal insufficiency (Addison disease) such as those due to a deficiency of adrenocorticotropic hormone (ACTH) or corticotropin-releasing hormone (CRH), or a defect in the ACTH receptor, are equally common among male and female individuals.

Congenital causes, such as congenital adrenal hyperplasia, congenital adrenal hypoplasia, and defects in the ACTH receptor, most commonly become apparent in childhood.



With proper treatment and compliance, patients with adrenal insufficiency (Addison disease) can live a normal life span without limitations. However, the prognosis for an untreated patient with adrenal insufficiency (Addison disease) is poor. Some studies have found that those with very high concentrations of cortisol have a worse prognosis and a higher complication rate of secondary sepsis or intestinal perforation.

Death is a common outcome, usually from hypotension or cardiac arrhythmia secondary to hyperkalemia, unless replacement steroid therapy is begun.


Hypotension, shock, hypoglycemia, and death are the primary complications of adrenal insufficiency. In addition, daily oral glucocorticoid therapy may provide iatrogenic suppression of the hypothalamic-pituitary-adrenal (HPA) axis within 2 weeks. Effects can last for weeks to months, depending on the duration of exposure to pharmacologic doses of glucocorticoids. Complications of excessive glucocorticoids include the following:

  • Growth failure
  • Obesity
  • Striae
  • Osteoporosis
  • Muscle weakness
  • Hypertension
  • Hyperglycemia
  • Cataracts

Complications of excessive administration of mineralocorticoids include hypertension and hypokalemia.


Patient Education

Educate patients with adrenal insufficiency (Addison disease) and their caretakers about the consequences and potential for death if adequate replacement therapy is not provided.

Advise patients and their caretakers to immediately seek medical help if the patient becomes ill. Patients should wear or carry a medical alert tag or card at all times to help them receive appropriate emergency care if they are found unconscious.

Supplemental and injectable glucocorticoid

Patients and their caretakers should know how to administer supplemental glucocorticoid in times of illness or traumatic stress. Include education about how to administer an injectable glucocorticoid when the patient is vomiting or unable to take oral stress doses. Periodically reinforce this information, because caretakers are often reluctant to inject medications.

An intramuscular injection of hydrocortisone (eg, 25 mg for infants, 50 mg for children, 100 mg for adults) can be lifesaving in the interval before the patient receives professional medical care. If this injection is not possible, rectal hydrocortisone can be used until systemic glucocorticoids can be administered.

Contributor Information and Disclosures

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, 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.

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

Stephen Kemp, MD, PhD Former 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, Southern 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.

  1. 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.

  2. 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].

  3. Gibb FW, Stewart A, Walker BR, Strachan MW. Adrenal insufficiency in patients on long-term opioid analgesia. Clin Endocrinol (Oxf). 2016 Jun 4. [Medline].

  4. 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].

  5. Clark A, Weber A. Molecular insights into inherited ACTH resistance syndromes. Trends Endocrinol Metab. 1994. 5:209-14. [Full Text].

  6. 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].

  7. 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].

  8. 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].

  9. 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].

  10. 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].

  11. 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].

  12. 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].

  13. 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].

  14. 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].

  15. Ferraz-de-Souza B, Achermann JC. Disorders of adrenal development. Endocr Dev. 2008. 13:19-32. [Medline].

  16. Kempna P, Fluck CE. Adrenal gland development and defects. Best Pract Res Clin Endocrinol Metab. 2008 Feb. 22(1):77-93. [Medline].

  17. Lalli E, Sassone-Corsi P. DAX-1 and the adrenal cortex. Curr Opin Endocrinol Diabetes. 1999. 6:185-90. [Full Text].

  18. 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].

  19. 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].

  20. Miller WL. Minireview: regulation of steroidogenesis by electron transfer. Endocrinology. 2005 Jun. 146(6):2544-50. [Medline].

  21. 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].

  22. 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].

  23. Lamberts SW, Bruining HA, de Jong FH. Corticosteroid therapy in severe illness. N Engl J Med. 1997 Oct 30. 337(18):1285-92. [Medline].

  24. 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].

  25. Fleseriu M, Loriaux DL. "Relative" adrenal insufficiency in critical illness. Endocr Pract. 2009 Sep-Oct. 15(6):632-40. [Medline].

  26. Hsieh S, White PC. Presentation of primary adrenal insufficiency in childhood. J Clin Endocrinol Metab. 2011 Jun. 96(6):E925-8. [Medline].

  27. Willis AC, Vince FP. The prevalence of Addison's disease in Coventry, UK. Postgrad Med J. 1997 May. 73(859):286-8. [Medline].

  28. 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].

  29. Olafsson AS, Sigurjonsdottir HA. Increasing prevalence of Addison disease: results from a nationwide study. Endocr Pract. 2016 Jan. 22 (1):30-5. [Medline].

  30. Arlt W, Allolio B. Adrenal insufficiency. Lancet. 2003 May 31. 361(9372):1881-93. [Medline].

  31. Besser GM, Thorner MO. Adrenal insufficiency. Clinical Endocrinology. St Louis, Mo: Mosby-Year Book; 1996. [CD-ROM]:

  32. 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].

  33. 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].

  34. 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].

  35. 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].

  36. Neary N, Nieman L. Adrenal insufficiency: etiology, diagnosis and treatment. Curr Opin Endocrinol Diabetes Obes. 2010 Apr 6. [Medline].

  37. 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].

  38. Kronenberg HM, Melmed S, Polonsky KS, Larson PR, eds. Williams Textbook of Endocrinology. 11th ed. Philadelphia, PA: Saunders; 2008. 229.

  39. 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].

  40. 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].

  41. 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].

  42. 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].

  43. 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].

  44. Neary N, Nieman L. Adrenal insufficiency: etiology, diagnosis and treatment. Curr Opin Endocrinol Diabetes Obes. 2010 Jun. 17(3):217-23. [Medline]. [Full Text].

  45. 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].

  46. 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].

  47. 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].

  48. 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].

  49. 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].

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.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.