{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T19:22:33Z","timestamp":1793906553454,"version":"4.8.0"},"reference-count":98,"publisher":"Japan Endocrine Society","issue":"9","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Endocr J"],"published-print":{"date-parts":[[2016]]},"DOI":"10.1507\/endocrj.ej16-0242","type":"journal-article","created":{"date-parts":[[2016,6,23]],"date-time":"2016-06-23T22:08:28Z","timestamp":1466719708000},"page":"765-784","source":"Crossref","is-referenced-by-count":98,"title":["Diagnosis and treatment of adrenal insufficiency including adrenal crisis: a Japan Endocrine Society clinical practice guideline [Opinion]"],"prefix":"10.1507","volume":"63","author":[{"given":"Toshihiko","family":"Yanase","sequence":"first","affiliation":[{"name":"Department of Endocrinology and Diabetes Mellitus, Faculty of Medicine, Fukuoka University, Fukuoa 814-0180, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshihiro","family":"Tajima","sequence":"additional","affiliation":[{"name":"Jichi Children&rsquo;s Medical Center Tochigi, Pediatrics, Shimotsuke 329-0498, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takuyuki","family":"Katabami","sequence":"additional","affiliation":[{"name":"Division of Metabolism and Endocrinology, Department of Internal Medicine, St Marianna University School of Medicine, Yokohama City Seibu Hospital, Yokohama 241-0811, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasumasa","family":"Iwasaki","sequence":"additional","affiliation":[{"name":"Health Service Center, Kochi University, Nankoku 780-8520, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yusuke","family":"Tanahashi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Asahikawa Medical University, Sapporo 078-8510, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akira","family":"Sugawara","sequence":"additional","affiliation":[{"name":"Department of Advanced Biological Sciences for Regeneration, Tohoku University, Graduate School of Medicine, Sendai 980-8575, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomonobu","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Keio University School of Medicine, Tokyo 160-8582, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomoatsu","family":"Mune","sequence":"additional","affiliation":[{"name":"Division of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School, Kurashiki 701-0192, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yutaka","family":"Oki","sequence":"additional","affiliation":[{"name":"Hamamatsu University School of Medicine, Hamamatsu 431-3192, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuichi","family":"Nakagawa","sequence":"additional","affiliation":[{"name":"Shiraume Toyooka Hospital, Iwata 438-0126, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nobuhiro","family":"Miyamura","sequence":"additional","affiliation":[{"name":"Tamana Central Hospital, Tamana 865-0064, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chikara","family":"Shimizu","sequence":"additional","affiliation":[{"name":"Division of Laboratory and Transfusion Medicine, Hokkaido University Hospital, Sapporo 060-8638, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michio","family":"Otsuki","sequence":"additional","affiliation":[{"name":"Department of Metabolic Medicine, Osaka University Graduate School of Medicine, Suita 565-0871, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masatoshi","family":"Nomura","sequence":"additional","affiliation":[{"name":"Department of Medicine and Bioregulatory Science, Graduate School of Medical Science, Kyushu University, Fukuoka 812-8582, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuko","family":"Akehi","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Diabetes Mellitus, Faculty of Medicine, Fukuoka University, Fukuoa 814-0180, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Makito","family":"Tanabe","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Diabetes Mellitus, Faculty of Medicine, Fukuoka University, Fukuoa 814-0180, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Soji","family":"Kasayama","sequence":"additional","affiliation":[{"name":"Nissay Hospital, Osaka 550-0012, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"386","reference":[{"key":"1","doi-asserted-by":"crossref","unstructured":"1. Erturk E, Jaffe CA, Barkan AL (1998) Evaluation of the integrity of the hypothalamic-pituitary-adrenal axis by insulin hypoglycemia test. <i>J Clin Endocrinol Metab<\/i> 83: 2350-2354.","DOI":"10.1210\/jc.83.7.2350"},{"key":"2","doi-asserted-by":"crossref","unstructured":"2. Berg C, Meinel T, Lahner H, Yuece A, Mann K, <i>et al<\/i>. (2010) Diagnostic utility of the glucagon stimulation test in comparison to the insulin tolerance test in patients following pituitary surgery. <i>Eur J Endocrinol<\/i> 162: 477-482.","DOI":"10.1530\/EJE-09-0824"},{"key":"3","doi-asserted-by":"crossref","unstructured":"3. Oelkers W (1996) Adrenal Insufficiency. <i>N Engl J Med<\/i> 335: 1206-1212.","DOI":"10.1056\/NEJM199610173351607"},{"key":"4","doi-asserted-by":"crossref","unstructured":"4. Maghnie M, Uga E, Temporini F, Di Iorgi N, Secco A, <i>et al<\/i>. (2005) Evaluation of adrenal function in patients with growth hormone deficiency and hypothalamic-pituitary disorders: comparison between insulin-induced hypoglycemia, low-dose ACTH, standard ACTH and CRH stimulation tests. <i>Eur J Endcrinol <\/i>152: 735-741.","DOI":"10.1530\/eje.1.01911"},{"key":"5","doi-asserted-by":"crossref","unstructured":"5. Grinspoon SK, Biller BM (1994) Clinical review 62: Laboratory assessment of adrenal insufficiency. <i>J Clin Endocrinol Metab<\/i> 79: 923-931.","DOI":"10.1210\/jcem.79.4.7962298"},{"key":"6","doi-asserted-by":"crossref","unstructured":"6. Kazlauskaite R, Evans AT, Villabona CV, Abdu TA, Ambrosi B, <i>et al<\/i>. (2008) Corticotropin tests for hypothalamic-pituitary- adrenal insufficiency: a metaanalysis.<i> J Clin Endocrinol Metab<\/i> 93: 4245-4253.","DOI":"10.1210\/jc.2008-0710"},{"key":"7","doi-asserted-by":"crossref","unstructured":"7. Laureti S, Arvat E, Candeloro P, Di Vito L, Ghigo E, <i>et al<\/i>. (2000) Low dose (1 microg) ACTH test in the evaluation of adrenal dysfunction in pre-clinical Addison&rsquo;s disease. <i>Clin Endocrinol (Oxf)<\/i> 53: 107-115.","DOI":"10.1046\/j.1365-2265.2000.01050.x"},{"key":"8","doi-asserted-by":"crossref","unstructured":"8. Dorin RI, Qualls CR, Crapo LM (2003) Diagnosis of adrenal insufficiency. <i>Ann<\/i> <i>Intern Med<\/i> 139: 194-204.","DOI":"10.7326\/0003-4819-139-3-200308050-00017"},{"key":"9","doi-asserted-by":"crossref","unstructured":"9. Yamamoto T (2014) History of Stress-related Health Changes: A Cue to Pursue a Diagnosis of Latent Primary Adrenal Insufficiency. <i>Intern Med<\/i> 53: 183-188.","DOI":"10.2169\/internalmedicine.53.1156"},{"key":"10","doi-asserted-by":"crossref","unstructured":"10. Boscaro M, Betterle C, Sonino N, Volpato M, Paoletta A, <i>et al<\/i>. (1994) Early adrenal hypofunction in patients with organ-specific autoantibodies and no clinical adrenal insufficiency. <i>J Cin Endocrinol Metab<\/i> 79: 452-455.","DOI":"10.1210\/jcem.79.2.8045962"},{"key":"11","doi-asserted-by":"crossref","unstructured":"11. Christiansen JJ, Djurhuus CB, Gravholt CH, Iversen P, Christiansen JS, <i>et al<\/i>. (2007) Effects of cortisol on carbohydrate, lipid, and protein metabolism: studies of acute cortisol withdrawal in adrenocortical failure. <i>J Clin Endocrinol Metab<\/i> 92: 3553-3559.","DOI":"10.1210\/jc.2007-0445"},{"key":"12","doi-asserted-by":"crossref","unstructured":"12. Mah PM, Jenkins RC, Rostami-Hodjegan A, Newell-Price J, Doane A, <i>et al<\/i>. (2004) Weight-related dosing, timing and monitoring hydrocortisone replacement therapy in patients with adrenal insufficiency. <i>Clin Endocrinol (Oxf) <\/i>61: 367-375.","DOI":"10.1111\/j.1365-2265.2004.02106.x"},{"key":"13","doi-asserted-by":"crossref","unstructured":"13. Esteban NV, Loughlin T, Yergey AL, Zawadzki JK, Booth JD, <i>et al<\/i>. (1991) Daily cortisol production rate in man determined by stable isotope dilution\/mass spectrometry. <i>J Clin Endocrinol Metab<\/i> 71: 39-45.","DOI":"10.1210\/jcem-72-1-39"},{"key":"14","doi-asserted-by":"crossref","unstructured":"14. 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. <i>J Clin Endocrinol Metab<\/i> 83: 1247-1252.","DOI":"10.1210\/jc.83.4.1247"},{"key":"15","doi-asserted-by":"crossref","unstructured":"15. Bleicken B, Hahner S, Loeffler M, Ventz M, Decker O, <i>et al<\/i>. (2010) Influence of hydrocortisone dosage scheme on health-related quality of life in patients with adrenal insufficiency. <i>Clin Endocrinol<\/i> <i>(Oxf) <\/i>72: 297-304.","DOI":"10.1111\/j.1365-2265.2009.03596.x"},{"key":"16","doi-asserted-by":"crossref","unstructured":"16. Arlt W (2009) The approach to the adult with newly diagnosed adrenal insufficiency. <i>J Clin Endocrinol Metab<\/i> 94: 1059-1067.","DOI":"10.1210\/jc.2009-0032"},{"key":"17","doi-asserted-by":"crossref","unstructured":"17. Maisey DN, Brown RC, Day JL (1974) Letter: Rifampicin and cortisone replacement therapy.<i> Lancet<\/i> 2: 896-897.","DOI":"10.1016\/S0140-6736(74)91230-6"},{"key":"18","doi-asserted-by":"crossref","unstructured":"18. Kyriazopoulou V, Parparousi O, Vagenakis AG (1984) Rifampicin-induced adrenal crisis in addisonian patients receiving corticosteroid replacement therapy.<i> J Clin Endocrinol Metab<\/i> 59: 1204-1206.","DOI":"10.1210\/jcem-59-6-1204"},{"key":"19","doi-asserted-by":"crossref","unstructured":"19. Robinson BG, Hales IB, Henniker AJ, Ho K, Luttrell BM, <i>et al<\/i>. (1987) The effect of o,p&rsquo;-DDD on adrenal steroid replacement therapy requirements. <i>Clin Endocrinol (Oxf) <\/i>27: 437-444.","DOI":"10.1111\/j.1365-2265.1987.tb01171.x"},{"key":"20","doi-asserted-by":"crossref","unstructured":"20. Lacroix A (2010) Approach to the patient with adrenocortical carcinoma.<i> J Clin Endocrinol Metab<\/i> 95: 4812-4822.","DOI":"10.1210\/jc.2010-0990"},{"key":"21","doi-asserted-by":"crossref","unstructured":"21. Weaver JU, Thaventhiran L, Noonan K, Burrin JM, Taylor NF, <i>et al<\/i>. (1994) The effect of growth hormone replacement on cortisol metabolism and glucocorticoid sensitivity in hypopituitary adults. <i>Clin Endocrinol (Oxf) <\/i>41: 639-648.","DOI":"10.1111\/j.1365-2265.1994.tb01830.x"},{"key":"22","doi-asserted-by":"crossref","unstructured":"22. Agha A, Monson JP (2007) Modulation of glucocorticoid metabolism by the growth hormone - IGF-1 axis. <i>Clin Endocrinol (Oxf)<\/i> 66: 459-465.","DOI":"10.1111\/j.1365-2265.2007.02763.x"},{"key":"23","doi-asserted-by":"crossref","unstructured":"23. Bornstein SR (2009) Predisposing factors for adrenal insufficiency. <i>N Engl J Med<\/i> 360: 2328-2339.","DOI":"10.1056\/NEJMra0804635"},{"key":"24","doi-asserted-by":"crossref","unstructured":"24. Debono M, Ross RJ, Newell-Price J (2009) Inadequacies of glucocorticoid replacement and improvements by physiological circadian therapy. <i>Eur J Endocrinol <\/i>160: 719-729.","DOI":"10.1530\/EJE-08-0874"},{"key":"25","doi-asserted-by":"crossref","unstructured":"25. Lamberts SWJ, Bons EG, Bruining HA, de Jong FH (1987) Differential effects of the imidazole derivatives etomidate, ketoconazole and miconazole and of metyrapone on the secretion of cortisol and its precursors by human adrenocortical cells. <i>J Pharmacol Exp Ther<\/i> 240: 259-264.","DOI":"10.1016\/S0022-3565(25)22688-X"},{"key":"26","doi-asserted-by":"crossref","unstructured":"26. Quinkler M, Hahner S (2012) What is the best long-term management strategy for patients with primary adrenal insufficiency? <i>Clin Endocrinol<\/i> <i>(Oxf)<\/i> 76: 21-25.","DOI":"10.1111\/j.1365-2265.2011.04103.x"},{"key":"27","doi-asserted-by":"crossref","unstructured":"27. Davis J, Sheppard M (1986) Acute adrenal crisis precipitated by thyroxine. <i>Br Med J (Clin Res Ed)<\/i> 292: 1595.","DOI":"10.1136\/bmj.292.6535.1595"},{"key":"28","doi-asserted-by":"crossref","unstructured":"28. Hahner S, Loeffler M, Bleicken B, Drechsler C, Milovanovic D, <i>et al<\/i>. (2010) Epidemiology of adrenal crisis in chronic adrenal insufficiency: the need for new prevention strategies.<i> Eur J Endocrinol <\/i>162: 597-602.","DOI":"10.1530\/EJE-09-0884"},{"key":"29","doi-asserted-by":"crossref","unstructured":"29. Huang CH, Chou KJ, Lee PT, Chen CL, Chung HM, <i>et al<\/i>. (2005) A case of lymphocytic hypophysitis with masked diabetes insipidus unveiled by glucocorticoid replacement. <i>Am J Kidney Dis <\/i>45: 197-200.","DOI":"10.1053\/j.ajkd.2004.09.033"},{"key":"30","doi-asserted-by":"crossref","unstructured":"30. Hahner S, Allolio B (2009) Therapeutic management of adrenal insufficiency.<i> Best Pract Res Clin Endocrinol Metab <\/i>23: 167-179.","DOI":"10.1016\/j.beem.2008.09.009"},{"key":"31","doi-asserted-by":"crossref","unstructured":"31. Lekarev O, New MI (2011) Adrenal disease in pregnancy. <i>Best Pract Res Clin Endocrinol Metab<\/i> 25: 959-973.","DOI":"10.1016\/j.beem.2011.08.004"},{"key":"32","doi-asserted-by":"crossref","unstructured":"32. Hochberg Z, Pacak K, Chrousos GP (2003) Endocrine withdrawal syndromes. <i>Endocri Rev<\/i> 24: 523-538.","DOI":"10.1210\/er.2001-0014"},{"key":"33","doi-asserted-by":"crossref","unstructured":"33. 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. <i>N Engl J Med<\/i> 326: 226-230.","DOI":"10.1056\/NEJM199201233260403"},{"key":"34","doi-asserted-by":"crossref","unstructured":"34. Bruni FM, De Luca G, Venturoli V, Boner AL (2009) Intranasal corticosteroids and adrenal suppression. <i>Neuroimmunomodulation<\/i> 16: 353-362.","DOI":"10.1159\/000216193"},{"key":"35","doi-asserted-by":"crossref","unstructured":"35. Tempark T, Phatarakijnirund V, Chatproedprai S, Watcharasindhu S, Supornsilchai V, <i>et al<\/i>. (2010) Exogenous Cushing&rsquo;s syndrome due to topical corticosteroid application: case report and review literature. <i>Endocrine <\/i>38: 328-334.","DOI":"10.1007\/s12020-010-9393-6"},{"key":"36","doi-asserted-by":"crossref","unstructured":"36. Todd GRG, Acerini CL, Ross-Russell R, Zahra S, Warner JT, <i>et al<\/i>. (2002) Survey of adrenal crisis associated with inhaled corticosteroids in the United Kingdom. <i>Arch Dis Child <\/i>87: 457-461.","DOI":"10.1136\/adc.87.6.457"},{"key":"37","doi-asserted-by":"crossref","unstructured":"37. Tamada D, Onodera T, Kitamura T, Yamamoto Y, Hayashi Y, <i>et al<\/i>. (2013) Hyperthyroidism due to thyroid stimulating hormone secretion after surgery for Cushing&rsquo;s syndrome: A novel cause of the syndrome of inappropriate secretion of thyroid stimulating hormone. <i>J Clin Endocrinol Metab <\/i>98: 2656-2662.","DOI":"10.1210\/jc.2013-2135"},{"key":"38","unstructured":"38. Research group (Chair, Yanase T) (2015) Diagnostic Criteria and classification of disease severity, congental adrenal enzyme disorders, congenital adrenal hypoplasia, ACTH insensitivity syndrome, and Addison&rsquo;s disease. <i>Annual report of Intractable Disease Research Grant of Ministry of Health, Labour and Welfare, Japan &ldquo;Research on Adrenal Hormone Disorders&rdquo;<\/i> : 21-48 (In Japanese)."},{"key":"39","doi-asserted-by":"crossref","unstructured":"39. Meimaridou E, Kowalczyk J, Guasti L, Hughes CR, Wagner F, <i>et al<\/i>. (2012) Mutations in NNT encoding nic- otinamide nucleotide transhydrogenase cause familial glucocorticoid deficiency. <i>Nat Genet <\/i>44: 740-742.","DOI":"10.1038\/ng.2299"},{"key":"40","doi-asserted-by":"crossref","unstructured":"40. Prasad R, Chan LF, Hughes CR, Kaski JP, Kowalczyk JC, <i>et al<\/i>. (2014) Thioredoxin reductase 2 (TXNRD2) mutation associated with familial glucocorticoid deficiency (FGD). <i>J Clin Endocrinol Metab<\/i> 99: E1556-1563.","DOI":"10.1210\/jc.2013-3844"},{"key":"41","doi-asserted-by":"crossref","unstructured":"41. Shimatsu A, Oki Y, Fujisawa I, Sano T (2009) Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin G4-related systemic disease: an emerging clinical entity. <i>Endocr J<\/i> 56: 1033-1041.","DOI":"10.1507\/endocrj.K09E-277"},{"key":"42","unstructured":"42. Yanase T, Fujieda K, Kajieda H, Tanahashi Y, Suzuki S (2011) A nation wide epidemiological study of Addison&rsquo;s disease and subclinical Cushing&rsquo;s disease. <i>Annual report of Intractable Disease Research Grant of Ministry of Health, Labour and Welfare, Japan &ldquo;Research on Adrenal Hormone Disorders&rdquo; <\/i>: 117-124 (In Japanese)."},{"key":"43","doi-asserted-by":"crossref","unstructured":"43. Howlett TA (1997) An assessment of optimal hydrocortisone replacement therapy. <i>Clin Endocrinol (Oxf) <\/i>46: 263-268.","DOI":"10.1046\/j.1365-2265.1997.1340955.x"},{"key":"44","doi-asserted-by":"crossref","unstructured":"44. Hahner S, Loeffler M, Fassnacht M, Weismann D, Koschker AC, <i>et al<\/i>. (2007) Impaired subjective health status in 256 patients with adrenal insufficiency on standard therapy based on cross-sectional analysis. <i>J Clin Endocrinol Metab <\/i>92: 3912-3922.","DOI":"10.1210\/jc.2007-0685"},{"key":"45","doi-asserted-by":"crossref","unstructured":"45. Hahner S, Allolio B (2005) Management of adrenal insufficiency in different clinical settings. <i>Expert Opin Pharmacother<\/i> 6: 2407-2417.","DOI":"10.1517\/14656566.6.14.2407"},{"key":"46","doi-asserted-by":"crossref","unstructured":"46. Udelsman R, Norton JA, Jelenich SE, Goldstein DS, Linehan WM, <i>et al<\/i>. (1987) Responses of the hypothalamic-pituitary-adrenal and renin-angiotensin axes and the sympathetic system during controlled surgical and anesthetic stress. <i>J Clin Endocrinol Metab<\/i> 64: 986-994.","DOI":"10.1210\/jcem-64-5-986"},{"key":"47","doi-asserted-by":"crossref","unstructured":"47. Donald RA, Perry EG, Wittert GA, Chapman M, Livesey JH, <i>et al<\/i>. (1993) The plasma ACTH, AVP, CRH and catecholamine responses to conventional and laparoscopic cholecystectomy.<i> Clin Endocrinol (Oxf)<\/i> 38: 609-615.","DOI":"10.1111\/j.1365-2265.1993.tb02142.x"},{"key":"48","doi-asserted-by":"crossref","unstructured":"48. Glowniak JV, Loriaux DL (1997) A double-blind study of perioperative steroid requirements in secondary adrenal insufficiency. <i>Surgery<\/i> 121: 123-129.","DOI":"10.1016\/S0039-6060(97)90280-4"},{"key":"49","doi-asserted-by":"crossref","unstructured":"49. Symreng T, Karlberg BE, K&aring;gedal B, Schildt B (1981) Physiological cortisol substitution of long-term steroid-treated patients undergoing major surgery. <i>Br J Anaesth <\/i>53: 949-954.","DOI":"10.1093\/bja\/53.9.949"},{"key":"50","doi-asserted-by":"crossref","unstructured":"50. Nicholson G, Burrin JM, Hall GM (1998) Peri-operative steroid supplementation. <i>Anaesthesia <\/i>53: 1091-1104.","DOI":"10.1046\/j.1365-2044.1998.00578.x"},{"key":"51","doi-asserted-by":"crossref","unstructured":"51. Salem M, Tainsh RE Jr, Bromberg J, Loriaux DL, Chernow B (1994) Perioperative glucocorticoid coverage. A reassessment 42 years after emergence of a problem. <i>Ann Surg<\/i> 219: 416-425.","DOI":"10.1097\/00000658-199404000-00013"},{"key":"52","doi-asserted-by":"crossref","unstructured":"52. 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. <i>J Clin Endocrinol Metab<\/i> 91: 3866-3872.","DOI":"10.1210\/jc.2006-1049"},{"key":"53","doi-asserted-by":"crossref","unstructured":"53. Jung C, Ho JT, Torpy DJ, Rogers A, Doogue M, <i>et al<\/i>. (2011) A longitudinal study of plasma and urinary cortisol in pregnancy and postpartum. <i>J Clin Endocrinol Metab<\/i> 96: 1533-1540.","DOI":"10.1210\/jc.2010-2395"},{"key":"54","doi-asserted-by":"crossref","unstructured":"54. Lekarev O, New MI (2011) Adrenal disease in pregnancy. <i>Best Pract Res Clin Endocrinol Metab <\/i>25: 959-973.","DOI":"10.1016\/j.beem.2011.08.004"},{"key":"55","doi-asserted-by":"crossref","unstructured":"55. Lebbe M, Arlt W (2013) What is the best diagnostic and therapeutic management strategy for an Addison patient during pregnancy? <i>Clin Endocrinol (Oxf)<\/i> 78: 497-502.","DOI":"10.1111\/cen.12097"},{"key":"56","doi-asserted-by":"crossref","unstructured":"56. Ballard PL, Granberg P, Ballard RA (1975) Glucocorticoid levels in maternal and cord serum after prenatal betamethasone therapy to prevent respiratory distress syndrome. <i>J Clin Invest<\/i> 56: 1548-1554.","DOI":"10.1172\/JCI108236"},{"key":"57","doi-asserted-by":"crossref","unstructured":"57. Crown A, Lightman S (2005) Why is the management of glucocorticoid deficiency still controversial: a review of the literature. <i>Clin Endocrinol (Oxf) <\/i>63: 483-492.","DOI":"10.1111\/j.1365-2265.2005.02320.x"},{"key":"58","doi-asserted-by":"crossref","unstructured":"58. Brucato A, Astori MG, Cimaz R, Villa P, Li Destri M, <i>et al<\/i>. (2006) Normal neuropsychological development in children with congenital complete heart block who may or may not be exposed to high-dose dexamethasone in utero. <i>Ann Rheum Dis <\/i>65: 1422-1426.","DOI":"10.1136\/ard.2005.049866"},{"key":"59","doi-asserted-by":"crossref","unstructured":"59. Rodr&iacute;guez-Pinilla E, Mart&iacute;nez-Fr&iacute;as ML (1998) Corticosteroids during pregnancy and oral clefts: a case-control study. <i>Teratology<\/i> 58: 2-5.","DOI":"10.1002\/(SICI)1096-9926(199807)58:1<2::AID-TERA2>3.0.CO;2-4"},{"key":"60","doi-asserted-by":"crossref","unstructured":"60. Park-Wyllie L, Mazzotta P, Pastuszak A, Moretti ME, Beique L, <i>et al<\/i>. (2000) Birth defects after maternal exposure to corticosteroids: prospective cohort study and meta-analysis of epidemiological studies. <i>Teratology<\/i> 62: 385-392.","DOI":"10.1002\/1096-9926(200012)62:6<385::AID-TERA5>3.0.CO;2-Z"},{"key":"61","doi-asserted-by":"crossref","unstructured":"61. Shen WT, Lee J, Kebebew E, Clark OH, Duh QY (2006) Selective use of steroid replacement after adrenalectomy. <i>Arch Surg<\/i> 141: 771-774.","DOI":"10.1001\/archsurg.141.8.771"},{"key":"62","doi-asserted-by":"crossref","unstructured":"62. Saiko Y, Minowada S, Shinohara M, Nutahara K, Homma K, <i>et al<\/i>. (1993) A survey on postoperative steroid replacement in 40 patients with Cushing&rsquo;s syndrome. <i>Nihon Hinyokika Gakkai Zasshi <\/i>84: 313-321 (In Japanese).","DOI":"10.5980\/jpnjurol1989.84.313"},{"key":"63","doi-asserted-by":"crossref","unstructured":"63. Biller BM, Grossman AB, Stewart PM, Melmed S, Bertagna X, <i>et al<\/i>. (2008) Treatment of Adrenocorticotropin-Dependent Cushing&rsquo;s Syndrome: A Consensus Statement. <i>J Clin Endocrinol Metab <\/i>93: 2454-2462.","DOI":"10.1210\/jc.2007-2734"},{"key":"64","doi-asserted-by":"crossref","unstructured":"64. Bhattacharyya A, Kaushal K, Tymms DJ, Davis JR (2005) Steroid withdrawal syndrome after successful treatment of Cushing&rsquo;s syndrome: a reminder. <i>Eur J Endocrinol<\/i> 153: 207-210.","DOI":"10.1530\/eje.1.01953"},{"key":"65","doi-asserted-by":"crossref","unstructured":"65. Lamberts SWJ, Bruining HA, de Jong FH (1997) Corticosteroid therapy in severe illness. <i>N Engl J Med<\/i> 337: 1285-1292.","DOI":"10.1056\/NEJM199710303371807"},{"key":"66","doi-asserted-by":"crossref","unstructured":"66. Jung C, Inder WJ (2008) Management of adrenal insufficiency during the stress of medical illness and surgery.<i> Med J Aust <\/i>188: 409-413.","DOI":"10.5694\/j.1326-5377.2008.tb01686.x"},{"key":"67","doi-asserted-by":"crossref","unstructured":"67. Debono M, Ghobadi C, Rostami-Hodjegan A, Huatan H, Campbell MJ, <i>et al<\/i>. (2009) Modified-release hydrocortisone to provide circadian cortisol profiles.<i> J Clin Endocrinol Metab<\/i> 94: 1548-1554.","DOI":"10.1210\/jc.2008-2380"},{"key":"68","doi-asserted-by":"crossref","unstructured":"68. Verma S, Vanryzin C, Sinaii N, Kim MS, Nieman LK, <i>et al<\/i>. (2010) A pharmacokinetic and pharmacodynamic study of delayed- and extended-release hydrocortisone (Chronocort) vs. conventional hydrocortisone (Cortef) in the treatment of congenital adrenal hyperplasia. <i>Clin Endocrinol (Oxf) <\/i>72: 441-447.","DOI":"10.1111\/j.1365-2265.2009.03636.x"},{"key":"69","doi-asserted-by":"crossref","unstructured":"69. Johannsson G, Nilsson AG, Bergthorsdottir R, Burman P, Dahlqvist P, <i>et al<\/i>. (2012) Improved cortisol exposure-time profile and outcome in patients with adrenal insufficiency: a prospective randomized trial of a novel hydrocortisone dual-release formulation.<i> J Clin Endocrinol Metab<\/i> 97: 473-481.","DOI":"10.1210\/jc.2011-1926"},{"key":"70","doi-asserted-by":"crossref","unstructured":"70. Bergthorsdottir R, Leonsson-Zachrisson M, Od&eacute;n A, Johannsson G (2006) Premature mortality in patients with Addison&rsquo;s disease: a population-based study. <i>J Clin Endocrinol Metab<\/i> 91: 4849-4853.","DOI":"10.1210\/jc.2006-0076"},{"key":"71","unstructured":"71. Erichsen MM, L&oslash;v&aring;s K, Fougner KJ, Svartberg J, Hauge ER, <i>et al<\/i>. (2009) Normal overall mortality rate in Addison&rsquo;s disease, but young patients are at risk of premature death. <i>Eur J Endocrinol <\/i>160: 233-237."},{"key":"72","doi-asserted-by":"crossref","unstructured":"72. Bouillon R (2006) Acute adrenal insufficiency. <i>Endocrinol Metab Clin North Am <\/i>35: 767-775.","DOI":"10.1016\/j.ecl.2006.09.004"},{"key":"73","doi-asserted-by":"crossref","unstructured":"73. White K, Arlt W (2010) Adrenal crisis in treated Addison&rsquo;s disease: a predictable but under-managed event. <i>Eur J Endocrinol<\/i> 162: 115-120.","DOI":"10.1530\/EJE-09-0559"},{"key":"74","doi-asserted-by":"crossref","unstructured":"74. Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, <i>et al<\/i>. (2010) Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline.<i> J Clin Endocrinol Metab <\/i>95: 4133-4160.","DOI":"10.1210\/jc.2009-2631"},{"key":"75","doi-asserted-by":"crossref","unstructured":"75. Charmandari E, Lichtarowicz-Krynska EJ, Hindmarsh PC, Johnston A, Aynsley-Green A, <i>et al<\/i>. (2001) Congenital adrenal hyperplasia: management during critical illness. <i>Arch Dis Child<\/i> 85: 26-28.","DOI":"10.1136\/adc.85.1.26"},{"key":"76","doi-asserted-by":"crossref","unstructured":"76. Mass Screening Committee; Japanese Society for Pediatric Endocrinology; Japanese Society for Mass Screening (2015) Guidelines for diagnosis and treatment of 21-hydroxylase deficiency (2014 revision). <i>Clin Pediatr Endocrinol <\/i>24: 77-105.","DOI":"10.1297\/cpe.24.77"},{"key":"77","unstructured":"77. Speiser PW, Pediatric adrenal insufficiency. Available at: http:\/\/emedicine.medscape.com\/article\/919077-overview (Medscape)"},{"key":"78","unstructured":"78. Fujieda K, Kajino H, Tanahshi Y, Suzuki S (2012) A nation wide epidemiological study of Adrenal hormone disorders. <i>Annual report of Intractable Disease Research Grant of Ministry of Health, Labour and Welfare, Japan &ldquo;Research on Adrenal Hormone Disorders&rdquo; <\/i>: 89-102 (in Japanese)."},{"key":"79","doi-asserted-by":"crossref","unstructured":"79. Perry R, Kecha O, Paquette J, Huot C, Van Vliet G, <i>et al<\/i>. (2005) Primary adrenal insufficiency in children: twenty years experienceat the Sainte-Justine Hospital, Montreal. <i>J Clin Endocrinol Metab<\/i> 90: 3243-3250.","DOI":"10.1210\/jc.2004-0016"},{"key":"80","doi-asserted-by":"crossref","unstructured":"80. Buchanan CR, Preece MA, Milner RD (1991) Mortality, neoplasia, and Creutzfeldt-Jakob disease in patients treated with human pituitary growth hormone in the United Kingdom. <i>BMJ<\/i> 302: 824-828.","DOI":"10.1136\/bmj.302.6780.824"},{"key":"81","doi-asserted-by":"crossref","unstructured":"81. Taback SP, Dean HJ (1996) Mortality in Canadian children with growth hormone (GH) deficiency receiving GH therapy 1967-1992. The Canadian Growth Hormone Advisory Committee. <i>J Clin Endocrinol Metab<\/i> 81: 1693-1696.","DOI":"10.1210\/jcem.81.5.8626817"},{"key":"82","doi-asserted-by":"crossref","unstructured":"82. Mills JL, Schonberger LB, Wysowski DK, Brown P, Durako SJ, <i>et al<\/i>. (2004) Long-term mortality in the United States cohort of pituitary-derived growth hormone recipients.<i> J Pediatr <\/i>144: 430-436.","DOI":"10.1016\/j.jpeds.2003.12.036"},{"key":"83","doi-asserted-by":"crossref","unstructured":"83. Merke DP, Bornstein SR, Avila NA, Chrousos GP (2002) NIH conference. Future directions in the study and management of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. <i>Ann Intern Med <\/i>136: 320-334.","DOI":"10.7326\/0003-4819-136-4-200202190-00012"},{"key":"84","doi-asserted-by":"crossref","unstructured":"84. Bonfig W, Schmidt H, Schwarz HP (2011) Growth patterns in the first three years of life in children with classical congenital adrenal hyperplasia diagnosed by newborn screening and treated with low doses of hydrocortisone. <i>Horm Res Paediatr<\/i> 275: 32-37.","DOI":"10.1159\/000316973"},{"key":"85","doi-asserted-by":"crossref","unstructured":"85. Punthakee Z, Legault L, Polychronakos C (2003) Prednisolone in the treatment of adrenal insufficiency: a re-evaluation of relative potency. <i>J Pediatr<\/i> 143: 402-405.","DOI":"10.1067\/S0022-3476(03)00294-4"},{"key":"86","doi-asserted-by":"crossref","unstructured":"86. Rivkees SA, Crawford JD (2000) Dexamethasone treatment of virilizing congenital adrenal hyperplasia: the ability to achieve normal growth. <i>Pediatrics<\/i> 106: 767-773.","DOI":"10.1542\/peds.106.4.767"},{"key":"87","doi-asserted-by":"crossref","unstructured":"87. Walker EA, Stewart PM (2003) 11&beta;-Hydroxysteroid dehydrogenase: unexpected connections. <i>Trends Endocrinol Metab<\/i> 14: 334-339.","DOI":"10.1016\/S1043-2760(03)00134-6"},{"key":"88","doi-asserted-by":"crossref","unstructured":"88. Bonfig W, Pozza SB, Schmidt H, Pagel P, Knorr D, <i>et al<\/i>. (2009) Hydrocortisone dosing during puberty in patients with classical congenital adrenal hyperplasia: an evidence based recommendation. <i>J Clin Endocrinol Metab <\/i>94: 3882-3888.","DOI":"10.1210\/jc.2009-0942"},{"key":"89","doi-asserted-by":"crossref","unstructured":"89. Merke DP, Bornstein SR (2005) Congenital adrenal hyperplasia. <i>Lancet<\/i> 365: 2125-2136.","DOI":"10.1016\/S0140-6736(05)66736-0"},{"key":"90","doi-asserted-by":"crossref","unstructured":"90. Merke DP (2008) Approach to the adult with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. <i>J Clin Endocrinol Metab<\/i> 93: 653-660.","DOI":"10.1210\/jc.2007-2417"},{"key":"91","doi-asserted-by":"crossref","unstructured":"91. Izawa M, Aso K, Higuchi A, Ariyasu D, Hasegawa Y (2007) Pregnanetriol in the range of 1.2-2.1 mg\/m<sup>2<\/sup>\/day as an index of optimal control in CYP21A2 deficiency. <i>Clin Pediatr Endocrinol<\/i> 16: 45-52.","DOI":"10.1297\/cpe.16.45"},{"key":"92","doi-asserted-by":"crossref","unstructured":"92. Riepe FG, Krone N, Viemann M, Partsch CJ, Sippell WG (2002) Management of congenital adrenal hyperplasia: results of the ESPE questionnaire. <i>Horm Res<\/i> 58: 196-205.","DOI":"10.1159\/000065492"},{"key":"93","doi-asserted-by":"crossref","unstructured":"93. Arlt W, Willis DS, Wild SH, Krone N, Doherty EJ, <i>et al<\/i>. (2010) United Kingdom Congenital Adrenal Hyperplasia Adult Study Executive (CaHASE). Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients. <i>J Clin Endocrinol Metab <\/i>95: 5110-5121.","DOI":"10.1210\/jc.2010-0917"},{"key":"94","doi-asserted-by":"crossref","unstructured":"94. Finkielstain GP, Kim MS, Sinaii N, Nishitani M, Van Ryzin C, <i>et al<\/i>. (2012) Clinical characteristics of a cohort of 244 patients with congenital adrenal hyperplasia. <i>J Clin Endocrinol Metab<\/i> 97: 4429-4438.","DOI":"10.1210\/jc.2012-2102"},{"key":"95","doi-asserted-by":"crossref","unstructured":"95. Falhammar H, Filipsson H, Holmdahl G, Janson PO, Nordenskj&ouml;ld A, <i>et al<\/i>. (2007) Fractures and bone mineral density in adult women with 21-hydroxylase deficiency. <i>J Clin Endocrinol Metab<\/i> 92: 4643-4649.","DOI":"10.1210\/jc.2007-0744"},{"key":"96","doi-asserted-by":"crossref","unstructured":"96. Reisch N, Arlt W, Krone N (2011) Health problems in congenital adrenal hyperplasia due to 21-hydroxylase deficiency. <i>Horm Res Paediatr<\/i> 76: 73-85.","DOI":"10.1159\/000327794"},{"key":"97","doi-asserted-by":"crossref","unstructured":"97. Bornstein SR, Allolio B, Arlt W, Barthe Al, Don-Wauchope A, <i>et al<\/i>. (2016) Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. <i>J Clin Endocrinol Metab<\/i> 101: 364-389.","DOI":"10.1210\/jc.2015-1710"},{"key":"98","doi-asserted-by":"crossref","unstructured":"98. Narumi S, Amano N, Ishii T, Katsumata N, Muroya K, <i>et al<\/i>. (2016) SAMD9 mutations cause a novel multisystem disorder, MIRAGE syndrome, and are associated with loss of chromosome 7. <i>Nat Genet<\/i> 48: 792-797.","DOI":"10.1038\/ng.3569"}],"container-title":["Endocrine Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.jstage.jst.go.jp\/article\/endocrj\/63\/9\/63_EJ16-0242\/_pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,6,3]],"date-time":"2025-06-03T22:36:12Z","timestamp":1748990172000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.jstage.jst.go.jp\/article\/endocrj\/63\/9\/63_EJ16-0242\/_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016]]},"references-count":98,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2016]]}},"URL":"https:\/\/doi.org\/10.1507\/endocrj.ej16-0242","relation":{},"ISSN":["0918-8959","1348-4540"],"issn-type":[{"value":"0918-8959","type":"print"},{"value":"1348-4540","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016]]}}}