Pediatric Adrenal Insufficiency (Addison Disease) Questions & Answers

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16. 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. [QxMD MEDLINE Link]. [Full Text].

17. 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. [QxMD MEDLINE Link]. [Full Text].

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19. Nowaczyk MJM, Wassif CA, Adam MP, et al. Smith-Lemli-Opitz Syndrome. GeneReviews. Updated 2020 Jan 30. [QxMD MEDLINE Link]. [Full Text].

20. 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. [QxMD MEDLINE Link]. [Full Text].

21. 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. [QxMD MEDLINE Link]. [Full Text].

22. Storr HL, Mitchell H, Swords FM, et al. Clinical features, diagnosis, treatment and molecular studies in paediatric Cushing's syndrome due to primary nodular adrenocortical hyperplasia. Clin Endocrinol (Oxf). 2004 Nov. 61 (5):553-9. [QxMD MEDLINE Link].

23. 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. [QxMD MEDLINE Link]. [Full Text].

24. Ferraz-de-Souza B, Achermann JC. Disorders of adrenal development. Endocr Dev. 2008. 13:19-32. [QxMD MEDLINE Link].

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

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

27. 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. [QxMD MEDLINE Link].

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29. Miller WL. Minireview: regulation of steroidogenesis by electron transfer. Endocrinology. 2005 Jun. 146(6):2544-50. [QxMD MEDLINE Link].

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31. 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. [QxMD MEDLINE Link].

32. 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. [QxMD MEDLINE Link].

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

34. 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. [QxMD MEDLINE Link].

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

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

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

38. 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. [QxMD MEDLINE Link]. [Full Text].

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

40. 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. [QxMD MEDLINE Link].

41. Kamrath C. Beyond the adrenals: Organ manifestations in inherited primary adrenal insufficiency in children. Eur J Endocrinol. 2020 Mar. 182 (3):C9-C12. [QxMD MEDLINE Link]. [Full Text].

42. Arlt W, Allolio B. Adrenal insufficiency. Lancet. 2003 May 31. 361(9372):1881-93. [QxMD MEDLINE Link].

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

44. 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. [QxMD MEDLINE Link].

45. 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. [QxMD MEDLINE Link].

46. 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. [QxMD MEDLINE Link]. [Full Text].

47. [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. [QxMD MEDLINE Link]. [Full Text].

48. 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. [QxMD MEDLINE Link]. [Full Text].

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50. 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. [QxMD MEDLINE Link].

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52. 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. [QxMD MEDLINE Link].

53. 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. [QxMD MEDLINE Link].

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

58. 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. [QxMD MEDLINE Link].

59. 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. [QxMD MEDLINE Link].

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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.

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.

Sasigarn A Bowden, MD, FAAP Professor of Pediatrics, Section of Pediatric Endocrinology, Metabolism and Diabetes, Department of Pediatrics, Ohio State University College of Medicine; Pediatric Endocrinologist, 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, FAAP 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.

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, The Scientific Research Honor Society, 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.

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