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Corticosteroids in Pediatric Endocrinology

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Systemic Corticosteroids for Inflammatory Disorders in Pediatrics

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Abstract

Glucocorticoids are human steroid hormones secreted by the adrenal cortex that play a critical role in several biologic processes. At “pharmacologic” or “stress-related” doses, glucocorticoids are irreplaceable therapeutic means for many allergic, inflammatory, autoimmune, and lymphoproliferative diseases. Glucocorticoids also remain the cornerstone of treatment for certain life-threatening endocrinopathies in childhood, such as congenital adrenal hyperplasia, Addison disease, and steroid replacement therapy for subjects with secondary hypothalamic–pituitary–adrenal axis deficit. In this chapter the physiology of the hypothalamic–pituitary–adrenal axis and of glucocorticoids is described. In addition, the therapeutic approach to glucocorticoid replacement therapy in the most relevant endocrinopathies in childhood (i.e., congenital adrenal hyperplasia, Addison disease, and Cushing disease) is elucidated.

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References

  1. Allolio B, Hoffmann J, Linton EA, Winkelmann W, Kusche M, Schulte HM (1990) Diurnal salivary cortisol patterns during pregnancy and after delivery: relationship to plasma corticotrophin-releasing-hormone. Clin Endocrinol (Oxf) 33(2):279–289

    CAS  Google Scholar 

  2. Alonso N, Granada ML, Lucas A, Salinas I, Reverter J, Oriol A, Sanmarti A (2004) Evaluation of two replacement regimens in primary adrenal insufficiency patients. Effect on clinical symptoms, health-related quality of life and biochemical parameters. J Endocrinol Invest 27(5):449–454

    CAS  PubMed  Google Scholar 

  3. Anderson JR, Goudie RB, Gray KG, Timbury GC (1957) Auto-antibodies in Addison’s disease. Lancet 272(6979):1123–1124

    CAS  PubMed  Google Scholar 

  4. Arlt W, Allolio B (2003) Adrenal insufficiency. Lancet 361(9372):1881–1893. doi:10.1016/S0140-6736(03)13492-7

    CAS  PubMed  Google Scholar 

  5. Balsamo A, Baldazzi L, Menabo S, Cicognani A (2010) Impact of molecular genetics on congenital adrenal hyperplasia management. Sexual Dev Genet Mol Biol Evol Endocrinol Embryol Pathol Sex Determin Differ 4(4–5):233–248. doi:10.1159/000315959

    CAS  Google Scholar 

  6. Barbetta L, Dall’Asta C, Re T, Libe R, Costa E, Ambrosi B (2005) Comparison of different regimens of glucocorticoid replacement therapy in patients with hypoadrenalism. J Endocrinol Invest 28(7):632–637

    CAS  PubMed  Google Scholar 

  7. Belgorosky A, Baquedano MS, Guercio G, Rivarola MA (2008) Adrenarche: postnatal adrenal zonation and hormonal and metabolic regulation. Horm Res 70(5):257–267. doi:10.1159/000157871

    CAS  PubMed  Google Scholar 

  8. Benvenga S, Campenni A, Ruggeri RM, Trimarchi F (2000) Clinical review 113: hypopituitarism secondary to head trauma. J Clin Endocrinol Metab 85(4):1353–1361. doi:10.1210/jcem.85.4.6506

    CAS  PubMed  Google Scholar 

  9. Bergendahl M, Iranmanesh A, Mulligan T, Veldhuis JD (2000) Impact of age on cortisol secretory dynamics basally and as driven by nutrient-withdrawal stress. J Clin Endocrinol Metab 85(6):2203–2214. doi:10.1210/jcem.85.6.6628

    CAS  PubMed  Google Scholar 

  10. Betterle C, Dal Pra C, Mantero F, Zanchetta R (2002) Autoimmune adrenal insufficiency and autoimmune polyendocrine syndromes: autoantibodies, autoantigens, and their applicability in diagnosis and disease prediction. Endocr Rev 23(3):327–364. doi:10.1210/edrv.23.3.0466

    CAS  PubMed  Google Scholar 

  11. Betterle C, Volpato M (1998) Adrenal and ovarian autoimmunity. Eur J Endocrinol/Eur Fed End Soc 138(1):16–25

    CAS  Google Scholar 

  12. Bonfiglio JJ, Inda C, Refojo D, Holsboer F, Arzt E, Silberstein S (2011) The corticotropin-releasing hormone network and the hypothalamic-pituitary-adrenal axis: molecular and cellular mechanisms involved. Neuroendocrinology 94(1):12–20. doi:10.1159/000328226

    CAS  PubMed  Google Scholar 

  13. Boscaro M, Betterle C, Volpato M, Fallo F, Furmaniak J, Rees Smith B, Sonino N (1996) Hormonal responses during various phases of autoimmune adrenal failure: no evidence for 21-hydroxylase enzyme activity inhibition in vivo. J Clin Endocrinol Metab 81(8):2801–2804. doi:10.1210/jcem.81.8.8768833

    CAS  PubMed  Google Scholar 

  14. Bose HS, Sugawara T, Strauss JF 3rd, Miller WL, International Congenital Lipoid Adrenal Hyperplasia C (1996) The pathophysiology and genetics of congenital lipoid adrenal hyperplasia. N Engl J Med 335(25):1870–1878. doi:10.1056/NEJM199612193352503

    CAS  PubMed  Google Scholar 

  15. Bottner A, Keller E, Kratzsch J, Stobbe H, Weigel JF, Keller A, Hirsch W, Kiess W, Blum WF, Pfaffle RW (2004) PROP1 mutations cause progressive deterioration of anterior pituitary function including adrenal insufficiency: a longitudinal analysis. J Clin Endocrinol Metab 89(10):5256–5265. doi:10.1210/jc.2004-0661

    PubMed  Google Scholar 

  16. Brandon DD, Isabelle LM, Samuels MH, Kendall JW, Loriaux DL (1999) Cortisol production rate measurement by stable isotope dilution using gas chromatography-negative ion chemical ionization mass spectrometry. Steroids 64(6):372–378

    CAS  PubMed  Google Scholar 

  17. Bryan SM, Honour JW, Hindmarsh PC (2009) Management of altered hydrocortisone pharmacokinetics in a boy with congenital adrenal hyperplasia using a continuous subcutaneous hydrocortisone infusion. J Clin Endocrinol Metab 94(9):3477–3480. doi:10.1210/jc.2009-0630

    CAS  PubMed  Google Scholar 

  18. Buttgereit F, Scheffold A (2002) Rapid glucocorticoid effects on immune cells. Steroids 67(6):529–534

    CAS  PubMed  Google Scholar 

  19. Calogero AE, Gallucci WT, Chrousos GP, Gold PW (1988) Interaction between GABAergic neurotransmission and rat hypothalamic corticotropin-releasing hormone secretion in vitro. Brain Res 463(1):28–36

    CAS  PubMed  Google Scholar 

  20. Chrousos GP (1998) Ultradian, circadian, and stress-related hypothalamic-pituitary-adrenal axis activity – a dynamic digital-to-analog modulation. Endocrinology 139(2):437–440. doi:10.1210/endo.139.2.5857

    CAS  PubMed  Google Scholar 

  21. Chrousos GP (2000) The stress response and immune function: clinical implications. The 1999 Novera H. Spector Lecture. Ann N Y Acad Sci 917:38–67

    CAS  PubMed  Google Scholar 

  22. Chrousos GP (2009) Stress and disorders of the stress system. Nat Rev Endocrinol 5(7):374–381. doi:10.1038/nrendo.2009.106

    CAS  PubMed  Google Scholar 

  23. Chrousos GP, Gold PW (1992) The concepts of stress and stress system disorders. Overview of physical and behavioral homeostasis. JAMA 267(9):1244–1252

    CAS  PubMed  Google Scholar 

  24. Chrousos GP, Kino T (2005) Intracellular glucocorticoid signaling: a formerly simple system turns stochastic. Sci STKE Signal Transduct Knowledge Environ (304):pe48. doi:10.1126/stke.3042005pe48

  25. Cooper MS, Stewart PM (2003) Corticosteroid insufficiency in acutely ill patients. N Engl J Med 348(8):727–734. doi:10.1056/NEJMra020529

    CAS  PubMed  Google Scholar 

  26. Coursin DB, Wood KE (2002) Corticosteroid supplementation for adrenal insufficiency. JAMA 287(2):236–240

    CAS  PubMed  Google Scholar 

  27. den Brinker M, Joosten KF, Liem O, de Jong FH, Hop WC, Hazelzet JA, van Dijk M, Hokken-Koelega AC (2005) Adrenal insufficiency in meningococcal sepsis: bioavailable cortisol levels and impact of interleukin-6 levels and intubation with etomidate on adrenal function and mortality. J Clin Endocrinol Metab 90(9):5110–5117. doi:10.1210/jc.2005-1107

    Google Scholar 

  28. Esteban NV, Yergey AL (1990) Cortisol production rates measured by liquid chromatography/mass spectrometry. Steroids 55(4):152–158

    CAS  PubMed  Google Scholar 

  29. Falorni A, Laureti S, De Bellis A, Zanchetta R, Tiberti C, Arnaldi G, Bini V, Beck-Peccoz P, Bizzarro A, Dotta F, Mantero F, Bellastella A, Betterle C, Santeusanio F, Group SIEAS (2004) Italian Addison network study: update of diagnostic criteria for the etiological classification of primary adrenal insufficiency. J Clin Endocrinol Metab 89(4):1598–1604. doi:10.1210/jc.2003-030954

    CAS  PubMed  Google Scholar 

  30. Fragoso MC, Domenice S, Latronico AC, Martin RM, Pereira MA, Zerbini MC, Lucon AM, Mendonca BB (2003) Cushing’s syndrome secondary to adrenocorticotropin-independent macronodular adrenocortical hyperplasia due to activating mutations of GNAS1 gene. J Clin Endocrinol Metab 88(5):2147–2151. doi:10.1210/jc.2002-021362

    CAS  PubMed  Google Scholar 

  31. Fuller RW (1992) The involvement of serotonin in regulation of pituitary-adrenocortical function. Front Neuroendocrinol 13(3):250–270

    CAS  PubMed  Google Scholar 

  32. Funder JW (1997) Glucocorticoid and mineralocorticoid receptors: biology and clinical relevance. Annu Rev Med 48:231–240. doi:10.1146/annurev.med.48.1.231

    CAS  PubMed  Google Scholar 

  33. Galon J, Franchimont D, Hiroi N, Frey G, Boettner A, Ehrhart-Bornstein M, O’Shea JJ, Chrousos GP, Bornstein SR (2002) Gene profiling reveals unknown enhancing and suppressive actions of glucocorticoids on immune cells. FASEB J Off Publ Fed Am Soc Exp Biol 16(1):61–71. doi:10.1096/fj.01-0245com

    CAS  Google Scholar 

  34. Gathercole LL, Lavery GG, Morgan SA, Cooper MS, Sinclair AJ, Tomlinson JW, Stewart PM (2013) 11beta-hydroxysteroid dehydrogenase 1: translational and therapeutic aspects. Endocr Rev 34(4):525–555. doi:10.1210/er.2012-1050

    CAS  PubMed  Google Scholar 

  35. Giavoli C, Libe R, Corbetta S, Ferrante E, Lania A, Arosio M, Spada A, Beck-Peccoz P (2004) Effect of recombinant human growth hormone (GH) replacement on the hypothalamic-pituitary-adrenal axis in adult GH-deficient patients. J Clin Endocrinol Metab 89(11):5397–5401. doi:10.1210/jc.2004-1114

    CAS  PubMed  Google Scholar 

  36. Gomez-Sanchez CE (2013) Adrenal dysfunction in critically ill patients. N Engl J Med 368(16):1547–1549. doi:10.1056/NEJMe1302305

    CAS  PubMed  Google Scholar 

  37. Habib KE, Gold PW, Chrousos GP (2001) Neuroendocrinology of stress. Endocrinol Metab Clin North Am 30(3):695–728; vii–viii

    CAS  PubMed  Google Scholar 

  38. Hartmann A, Veldhuis JD, Deuschle M, Standhardt H, Heuser I (1997) Twenty-four hour cortisol release profiles in patients with Alzheimer’s and Parkinson’s disease compared to normal controls: ultradian secretory pulsatility and diurnal variation. Neurobiol Aging 18(3):285–289

    CAS  PubMed  Google Scholar 

  39. Havard CW, Saldanha VF, Bird R, Gardner R (1970) Adrenal function in hypothyroidism. Br Med J 1(5692):337–339

    CAS  PubMed Central  PubMed  Google Scholar 

  40. Henzen C, Suter A, Lerch E, Urbinelli R, Schorno XH, Briner VA (2000) Suppression and recovery of adrenal response after short-term, high-dose glucocorticoid treatment. Lancet 355(9203):542–545. doi:10.1016/S0140-6736(99)06290-X

    CAS  PubMed  Google Scholar 

  41. Heuer H, Visser TJ (2009) Minireview: pathophysiological importance of thyroid hormone transporters. Endocrinology 150(3):1078–1083. doi:10.1210/en.2008-1518

    CAS  PubMed  Google Scholar 

  42. Hiatt JR, Hiatt N (1997) The conquest of Addison’s disease. Am J Surg 174(3):280–283

    CAS  PubMed  Google Scholar 

  43. Howlett TA (1997) An assessment of optimal hydrocortisone replacement therapy. Clin Endocrinol (Oxf) 46(3):263–268

    CAS  Google Scholar 

  44. Hughes I (2002) Congenital adrenal hyperplasia: phenotype and genotype. J Pediatr Endocrinol Metabol JPEM 15(Suppl 5):1329–1340

    CAS  Google Scholar 

  45. Husebye ES, Allolio B, Arlt W, Badenhoop K, Bensing S, Betterle C, Falorni A, Gan EH, Hulting AL, Kasperlik-Zaluska A, Kampe O, Lovas K, Meyer G, Pearce SH (2014) Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. J Intern Med 275(2):104–115. doi:10.1111/joim.12162

    CAS  PubMed  Google Scholar 

  46. Invitti C, De Martin M, Delitala G, Veldhuis JD, Cavagnini F (1998) Altered morning and nighttime pulsatile corticotropin and cortisol release in polycystic ovary syndrome. Metabolism Clin Exp 47(2):143–148

    CAS  Google Scholar 

  47. Jansen M, Wit JM, van den Brande JL (1981) Reinstitution of mineralocorticoid therapy in congenital adrenal hyperplasia. Effects on control and growth. Acta Paediatr Scand 70(2):229–233

    CAS  PubMed  Google Scholar 

  48. Joint LECAHWG (2002) Consensus statement on 21-hydroxylase deficiency from the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology. J Clin Endocrinol Metab 87(9):4048–4053. doi:10.1210/jc.2002-020611

    Google Scholar 

  49. Juszczak A, Ertorer ME, Grossman A (2013) The therapy of Cushing’s disease in adults and children: an update. Horm Metab Res 45(2):109–117. doi:10.1055/s-0032-1330009

    CAS  PubMed  Google Scholar 

  50. Kendall EC (1950) Cortisone. Ann Intern Med 33(4):787–796

    CAS  PubMed  Google Scholar 

  51. Kerrigan JR, Veldhuis JD, Leyo SA, Iranmanesh A, Rogol AD (1993) Estimation of daily cortisol production and clearance rates in normal pubertal males by deconvolution analysis. J Clin Endocrinol Metab 76(6):1505–1510. doi:10.1210/jcem.76.6.8501158

    CAS  PubMed  Google Scholar 

  52. Kino T, Chrousos GP (2001) Glucocorticoid and mineralocorticoid resistance/hypersensitivity syndromes. J Endocrinol 169(3):437–445

    CAS  PubMed  Google Scholar 

  53. Kirk JM, Brain CE, Carson DJ, Hyde JC, Grant DB (1999) Cushing’s syndrome caused by nodular adrenal hyperplasia in children with McCune-Albright syndrome. J Pediatr 134(6):789–792

    CAS  PubMed  Google Scholar 

  54. Kong MF, Jeffcoate W (1994) Eighty-six cases of Addison’s disease. Clin Endocrinol (Oxf) 41(6):757–761

    CAS  Google Scholar 

  55. Kraan GP, Dullaart RP, Pratt JJ, Wolthers BG, Drayer NM, De Bruin R (1998) The daily cortisol production reinvestigated in healthy men. The serum and urinary cortisol production rates are not significantly different. J Clin Endocrinol Metab 83(4):1247–1252. doi:10.1210/jcem.83.4.4694

    CAS  PubMed  Google Scholar 

  56. Krone N, Braun A, Roscher AA, Knorr D, Schwarz HP (2000) Predicting phenotype in steroid 21-hydroxylase deficiency? Comprehensive genotyping in 155 unrelated, well defined patients from southern Germany. J Clin Endocrinol Metab 85(3):1059–1065. doi:10.1210/jcem.85.3.6441

    CAS  PubMed  Google Scholar 

  57. Kyriazopoulou V (2007) Glucocorticoid replacement therapy in patients with Addison’s disease. Expert Opin Pharmacother 8(6):725–729. doi:10.1517/14656566.8.6.725

    CAS  PubMed  Google Scholar 

  58. Lamberts SW, Bruining HA, de Jong FH (1997) Corticosteroid therapy in severe illness. N Engl J Med 337(18):1285–1292. doi:10.1056/NEJM199710303371807

    CAS  PubMed  Google Scholar 

  59. Laureti S, Falorni A, Santeusanio F (2003) Improvement of treatment of primary adrenal insufficiency by administration of cortisone acetate in three daily doses. J Endocrinol Invest 26(11):1071–1075

    CAS  PubMed  Google Scholar 

  60. Levine A, Cohen D, Zadik Z (1994) Urinary free cortisol values in children under stress. J Pediatr 125(6 Pt 1):853–857

    CAS  PubMed  Google Scholar 

  61. Linder BL, Esteban NV, Yergey AL, Winterer JC, Loriaux DL, Cassorla F (1990) Cortisol production rate in childhood and adolescence. J Pediatr 117(6):892–896

    CAS  PubMed  Google Scholar 

  62. Lindsay JR, Nieman LK (2005) The hypothalamic-pituitary-adrenal axis in pregnancy: challenges in disease detection and treatment. Endocr Rev 26(6):775–799. doi:10.1210/er.2004-0025

    CAS  PubMed  Google Scholar 

  63. Lodish M, Dunn SV, Sinaii N, Keil MF, Stratakis CA (2012) Recovery of the hypothalamic-pituitary-adrenal axis in children and adolescents after surgical cure of Cushing’s disease. J Clin Endocrinol Metab 97(5):1483–1491. doi:10.1210/jc.2011-2325

    CAS  PubMed Central  PubMed  Google Scholar 

  64. Lodish MB, Sinaii N, Patronas N, Batista DL, Keil M, Samuel J, Moran J, Verma S, Popovic J, Stratakis CA (2009) Blood pressure in pediatric patients with Cushing syndrome. J Clin Endocrinol Metab 94(6):2002–2008. doi:10.1210/jc.2008-2694

    CAS  PubMed Central  PubMed  Google Scholar 

  65. Lovas K, Husebye ES (2007) Continuous subcutaneous hydrocortisone infusion in Addison’s disease. Eur J Endocrinol/Eur Fed End Soc 157(1):109–112. doi:10.1530/EJE-07-0052

    Google Scholar 

  66. Lovas K, Husebye ES (2008) Replacement therapy for Addison’s disease: recent developments. Expert Opin Investig Drugs 17(4):497–509. doi:10.1517/13543784.17.4.497

    PubMed  Google Scholar 

  67. Magiakou MA, Mastorakos G, Oldfield EH, Gomez MT, Doppman JL, Cutler GB Jr, Nieman LK, Chrousos GP (1994) Cushing’s syndrome in children and adolescents. Presentation, diagnosis, and therapy. N Engl J Med 331(10):629–636. doi:10.1056/NEJM199409083311002

    CAS  PubMed  Google Scholar 

  68. Marik PE, Pastores SM, Annane D, Meduri GU, Sprung CL, Arlt W, Keh D, Briegel J, Beishuizen A, Dimopoulou I, Tsagarakis S, Singer M, Chrousos GP, Zaloga G, Bokhari F, Vogeser M, American College of Critical Care M (2008) Recommendations for the diagnosis and management of corticosteroid insufficiency in critically ill adult patients: consensus statements from an international task force by the American College of Critical Care Medicine. Crit Care Med 36(6):1937–1949. doi:10.1097/CCM.0b013e31817603ba

    CAS  PubMed  Google Scholar 

  69. McEwen BS (2007) Physiology and neurobiology of stress and adaptation: central role of the brain. Physiol Rev 87(3):873–904. doi:10.1152/physrev.00041.2006

    PubMed  Google Scholar 

  70. McEwen BS, Biron CA, Brunson KW, Bulloch K, Chambers WH, Dhabhar FS, Goldfarb RH, Kitson RP, Miller AH, Spencer RL, Weiss JM (1997) The role of adrenocorticoids as modulators of immune function in health and disease: neural, endocrine and immune interactions. Brain Res Brain Res Rev 23(1–2):79–133

    CAS  PubMed  Google Scholar 

  71. Menon K, Lawson M (2007) Identification of adrenal insufficiency in pediatric critical illness. Pediatr Crit Care Med J Soc Crit Care Med World Fed Pediatr Intensive Crit Care Soc 8(3):276–278. doi:10.1097/01.PCC.0000262796.38637.15

    Google Scholar 

  72. Merke DP, Bornstein SR (2005) Congenital adrenal hyperplasia. Lancet 365(9477):2125–2136. doi:10.1016/S0140-6736(05)66736-0

    PubMed  Google Scholar 

  73. Merke DP, Cho D, Calis KA, Keil MF, Chrousos GP (2001) Hydrocortisone suspension and hydrocortisone tablets are not bioequivalent in the treatment of children with congenital adrenal hyperplasia. J Clin Endocrinol Metab 86(1):441–445. doi:10.1210/jcem.86.1.7275

    CAS  PubMed  Google Scholar 

  74. Migeon CJ, Green OC, Eckert JP (1963) Study of adrenocortical function in obesity. Metabolism Clin Exp 12:718–739

    CAS  Google Scholar 

  75. Miller WL (2007) StAR search – what we know about how the steroidogenic acute regulatory protein mediates mitochondrial cholesterol import. Mol Endocrinol 21(3):589–601. doi:10.1210/me.2006-0303

    CAS  PubMed  Google Scholar 

  76. Mullis PE, Hindmarsh PC, Brook CG (1990) Sodium chloride supplement at diagnosis and during infancy in children with salt-losing 21-hydroxylase deficiency. Eur J Pediatr 150(1):22–25

    CAS  PubMed  Google Scholar 

  77. Munck A, Guyre PM, Holbrook NJ (1984) Physiological functions of glucocorticoids in stress and their relation to pharmacological actions. Endocr Rev 5(1):25–44. doi:10.1210/edrv-5-1-25

    CAS  PubMed  Google Scholar 

  78. Munck A, Naray-Fejes-Toth A (1992) The ups and downs of glucocorticoid physiology. Permissive and suppressive effects revisited. Mol Cell Endocrinol 90(1):C1–C4

    CAS  PubMed  Google Scholar 

  79. Nerup J (1974) Addison’s disease – a review of some clinical, pathological and immunological features. Dan Med Bull 21(6):201–217

    CAS  PubMed  Google Scholar 

  80. Nicholson G, Burrin JM, Hall GM (1998) Peri-operative steroid supplementation. Anaesthesia 53(11):1091–1104

    CAS  PubMed  Google Scholar 

  81. Nicolaides NC, Galata Z, Kino T, Chrousos GP, Charmandari E (2010) The human glucocorticoid receptor: molecular basis of biologic function. Steroids 75(1):1–12. doi:10.1016/j.steroids.2009.09.002

    CAS  PubMed Central  PubMed  Google Scholar 

  82. Nordenstrom A, Marcus C, Axelson M, Wedell A, Ritzen EM (1999) Failure of cortisone acetate treatment in congenital adrenal hyperplasia because of defective 11beta-hydroxysteroid dehydrogenase reductase activity. J Clin Endocrinol Metab 84(4):1210–1213. doi:10.1210/jcem.84.4.5584

    CAS  PubMed  Google Scholar 

  83. Oberfield SE, Chin D, Uli N, David R, Sklar C (1997) Endocrine late effects of childhood cancers. J Pediatr 131(1 Pt 2):S37–S41

    CAS  PubMed  Google Scholar 

  84. Oelkers W (1996) Adrenal insufficiency. N Engl J Med 335(16):1206–1212. doi:10.1056/NEJM199610173351607

    CAS  PubMed  Google Scholar 

  85. Orth DN (1995) Cushing’s syndrome. N Engl J Med 332(12):791–803. doi:10.1056/NEJM199503233321207

    CAS  PubMed  Google Scholar 

  86. Payne AH, Hales DB (2004) Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones. Endocr Rev 25(6):947–970. doi:10.1210/er.2003-0030

    CAS  PubMed  Google Scholar 

  87. Peacey SR, Guo CY, Robinson AM, Price A, Giles MA, Eastell R, Weetman AP (1997) Glucocorticoid replacement therapy: are patients over treated and does it matter? Clin Endocrinol (Oxf) 46(3):255–261

    CAS  Google Scholar 

  88. Ramakrishnan R, DuBois DC, Almon RR, Pyszczynski NA, Jusko WJ (2002) Pharmacodynamics and pharmacogenomics of methylprednisolone during 7-day infusions in rats. J Pharmacol Exp Ther 300(1):245–256

    CAS  PubMed  Google Scholar 

  89. Reddy TE, Pauli F, Sprouse RO, Neff NF, Newberry KM, Garabedian MJ, Myers RM (2009) Genomic determination of the glucocorticoid response reveals unexpected mechanisms of gene regulation. Genome Res 19(12):2163–2171. doi:10.1101/gr.097022.109

    CAS  PubMed Central  PubMed  Google Scholar 

  90. Rhen T, Cidlowski JA (2005) Antiinflammatory action of glucocorticoids – new mechanisms for old drugs. N Engl J Med 353(16):1711–1723. doi:10.1056/NEJMra050541

    CAS  PubMed  Google Scholar 

  91. Rivkees SA, Crawford JD (2000) Dexamethasone treatment of virilizing congenital adrenal hyperplasia: the ability to achieve normal growth. Pediatrics 106(4):767–773

    CAS  PubMed  Google Scholar 

  92. Rubin RP (2007) A brief history of great discoveries in pharmacology: in celebration of the centennial anniversary of the founding of the American Society of Pharmacology and Experimental Therapeutics. Pharmacol Rev 59(4):289–359. doi:10.1124/pr.107.70102

    PubMed  Google Scholar 

  93. Sapolsky RM, Romero LM, Munck AU (2000) How do glucocorticoids influence stress responses? Integrating permissive, suppressive, stimulatory, and preparative actions. Endocr Rev 21(1):55–89. doi:10.1210/edrv.21.1.0389

    CAS  PubMed  Google Scholar 

  94. Sarnyai Z, Veldhuis JD, Mello NK, Mendelson JH, Eros-Sarnyai M, Mercer G, Gelles H, Kelly M (1995) The concordance of pulsatile ultradian release of adrenocorticotropin and cortisol in male rhesus monkeys. J Clin Endocrinol Metab 80(1):54–59. doi:10.1210/jcem.80.1.7829639

    CAS  PubMed  Google Scholar 

  95. Schlaghecke R, Kornely E, Santen RT, Ridderskamp P (1992) The effect of long-term glucocorticoid therapy on pituitary-adrenal responses to exogenous corticotropin-releasing hormone. N Engl J Med 326(4):226–230. doi:10.1056/NEJM199201233260403

    CAS  PubMed  Google Scholar 

  96. Seckl JR, Walker BR (2001) Minireview: 11beta-hydroxysteroid dehydrogenase type 1- a tissue-specific amplifier of glucocorticoid action. Endocrinology 142(4):1371–1376. doi:10.1210/endo.142.4.8114

    CAS  PubMed  Google Scholar 

  97. Seckl JR, Walker BR (2004) 11beta-hydroxysteroid dehydrogenase type 1 as a modulator of glucocorticoid action: from metabolism to memory. Trends Endocrinol Metab TEM 15(9):418–424. doi:10.1016/j.tem.2004.09.007

    CAS  Google Scholar 

  98. Shaikh MG, Lewis P, Kirk JM (2004) Thyroxine unmasks Addison’s disease. Acta Paediatr 93(12):1663–1665

    CAS  PubMed  Google Scholar 

  99. Shulman DI, Palmert MR, Kemp SF, Lawson Wilkins D, Therapeutics C (2007) Adrenal insufficiency: still a cause of morbidity and death in childhood. Pediatrics 119(2):e484–e494. doi:10.1542/peds.2006-1612

    PubMed  Google Scholar 

  100. Silva Rdo C, Castro M, Kater CE, Cunha AA, Moraes AM, Alvarenga DB, Moreira AC, Elias LL (2004) Primary adrenal insufficiency in adults: 150 years after Addison. Arq Bras Endocrinol Metabol 48(5):724–738. doi:http://dx.doi.org/10.1590/S0004-27302004000500019

  101. Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP, Meyer-Bahlburg HF, Miller WL, Montori VM, Oberfield SE, Ritzen M, White PC, Endocrine S (2010) Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab 95(9):4133–4160. doi:10.1210/jc.2009-2631

    CAS  PubMed Central  PubMed  Google Scholar 

  102. Spinner MW, Blizzard RM, Childs B (1968) Clinical and genetic heterogeneity in idiopathic Addison’s disease and hypoparathyroidism. J Clin Endocrinol Metab 28(6):795–804. doi:10.1210/jcem-28-6-795

    CAS  PubMed  Google Scholar 

  103. Stikkelbroeck NM, Hoefsloot LH, de Wijs IJ, Otten BJ, Hermus AR, Sistermans EA (2003) CYP21 gene mutation analysis in 198 patients with 21-hydroxylase deficiency in The Netherlands: six novel mutations and a specific cluster of four mutations. J Clin Endocrinol Metab 88(8):3852–3859. doi:10.1210/jc.2002-021681

    CAS  PubMed  Google Scholar 

  104. Stratakis CA (2008) Cushing syndrome caused by adrenocortical tumors and hyperplasias (corticotropin- independent Cushing syndrome). Endocr Dev 13:117–132. doi:10.1159/000134829

    CAS  PubMed Central  PubMed  Google Scholar 

  105. Stratakis CA, Kirschner LS (1998) Clinical and genetic analysis of primary bilateral adrenal diseases (micro- and macronodular disease) leading to Cushing syndrome. Horm Metab Res 30(6–7):456–463. doi:10.1055/s-2007-978914

    CAS  PubMed  Google Scholar 

  106. Suri D, Moran J, Hibbard JU, Kasza K, Weiss RE (2006) Assessment of adrenal reserve in pregnancy: defining the normal response to the adrenocorticotropin stimulation test. J Clin Endocrinol Metab 91(10):3866–3872. doi:10.1210/jc.2006-1049

    CAS  PubMed  Google Scholar 

  107. Thompson EB, Tomkins GM, Curran JF (1966) Induction of tyrosine alpha-ketoglutarate transaminase by steroid hormones in a newly established tissue culture cell line. Proc Natl Acad Sci U S A 56(1):296–303

    CAS  PubMed Central  PubMed  Google Scholar 

  108. Tomlinson JW, Walker EA, Bujalska IJ, Draper N, Lavery GG, Cooper MS, Hewison M, Stewart PM (2004) 11beta-hydroxysteroid dehydrogenase type 1: a tissue-specific regulator of glucocorticoid response. Endocr Rev 25(5):831–866. doi:10.1210/er.2003-0031

    CAS  PubMed  Google Scholar 

  109. Tsigos C, Chrousos GP (1996) Differential diagnosis and management of Cushing’s syndrome. Annu Rev Med 47:443–461. doi:10.1146/annurev.med.47.1.443

    CAS  PubMed  Google Scholar 

  110. Turnbull AV, Rivier CL (1999) Regulation of the hypothalamic-pituitary-adrenal axis by cytokines: actions and mechanisms of action. Physiol Rev 79(1):1–71

    CAS  PubMed  Google Scholar 

  111. Valenzuela GA, Smalley WE, Schain DC, Vance ML, McCallum RW (1987) Reversibility of gastric dysmotility in cortisol deficiency. Am J Gastroenterol 82(10):1066–1068

    CAS  PubMed  Google Scholar 

  112. Veldhuis JD (1997) How does one get at glandular secretion, when only hormone concentrations are measured? Clin Endocrinol (Oxf) 46(4):397–400

    CAS  Google Scholar 

  113. Verma S, Vanryzin C, Sinaii N, Kim MS, Nieman LK, Ravindran S, Calis KA, Arlt W, Ross RJ, Merke DP (2010) A pharmacokinetic and pharmacodynamic study of delayed- and extended-release hydrocortisone (Chronocort) vs. conventional hydrocortisone (Cortef) in the treatment of congenital adrenal hyperplasia. Clin Endocrinol (Oxf) 72(4):441–447. doi:10.1111/j.1365-2265.2009.03636.x

    CAS  Google Scholar 

  114. Weise M, Drinkard B, Mehlinger SL, Holzer SM, Eisenhofer G, Charmandari E, Chrousos GP, Merke DP (2004) Stress dose of hydrocortisone is not beneficial in patients with classic congenital adrenal hyperplasia undergoing short-term, high-intensity exercise. J Clin Endocrinol Metab 89(8):3679–3684. doi:10.1210/jc.2003-032051

    CAS  PubMed  Google Scholar 

  115. White PC, Speiser PW (2000) Congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Endocr Rev 21(3):245–291. doi:10.1210/edrv.21.3.0398

    CAS  PubMed  Google Scholar 

  116. Windle RJ, Wood SA, Shanks N, Lightman SL, Ingram CD (1998) Ultradian rhythm of basal corticosterone release in the female rat: dynamic interaction with the response to acute stress. Endocrinology 139(2):443–450. doi:10.1210/endo.139.2.5721

    CAS  PubMed  Google Scholar 

  117. Winqvist O, Karlsson FA, Kampe O (1992) 21-hydroxylase, a major autoantigen in idiopathic Addison’s disease. Lancet 339(8809):1559–1562

    CAS  PubMed  Google Scholar 

  118. Young EA, Abelson J, Lightman SL (2004) Cortisol pulsatility and its role in stress regulation and health. Front Neuroendocrinol 25(2):69–76. doi:10.1016/j.yfrne.2004.07.001

    CAS  PubMed  Google Scholar 

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  1. Department of Pediatrics, University of Chieti, Via dei Vestini 5, I, Chieti, 66100, Italy

    Cosimo Giannini MD, PhD & Angelika Mohn MD

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  1. Cosimo Giannini MD, PhD
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  2. Angelika Mohn MD
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Correspondence to Angelika Mohn MD .

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  1. AOU Meyer Pediatric Rheumatology, Firenze, Italy

    Rolando Cimaz

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Giannini, C., Mohn, A. (2015). Corticosteroids in Pediatric Endocrinology. In: Cimaz, R. (eds) Systemic Corticosteroids for Inflammatory Disorders in Pediatrics. Adis, Cham. https://doi.org/10.1007/978-3-319-16056-6_11

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