Abstract
Untreated, complete glucocorticoid insufficiency leads to circulatory collapse and death. Although uncommon, the possibility of this endocrine emergency engenders vigilance, both for a new diagnosis of adrenal insufficiency as well as for exacerbation of a chronic, known condition. Conversely, overtreatment of adrenal insufficiency with excessive glucocorticoid carries the morbidity associated with iatrogenic Cushing’ s syndrome. Thus, glucocorticoid insufficiency presents two major challenges for clinicians: timely diagnosis and appropriate treatment.
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Ahonen P, Myllarnieini S, Sipila I, et al. Clinical variation of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) in a series of 68 patients. N Engl J Med 1990; 322: 1829–1836.
Betterle C, Volpato M, Greggio AN, et al. Type 2 polyglandular autoimmune disease (Schmidt’s syndrome). J Pediatr Endocrinol Metab 1996; 9: 113–123.
Piedrola G, Casado JL, Lopez E, et al. Clinical features of adrenal insufficiency in patients with acquired immunodeficiency syndrome. Clin Endocrinol (Oxf) 1996; 45: 97–101.
Ihde JK, Turnbull AD, Bajourunas DR. Adrenal insufficiency in the cancer patient: implications for the surgeon. Br J Surg 1990; 77: 1335–1337.
Rao RH, Vagnucci AH, Amico JA. Bilateral massive adrenal hemorrhage: early recognition and treatment. Ann Intern Med 1989; 110: 227–235.
Nieman LK. Cushing’s syndrome treatment. In: C. Wayne Bardin, ed., Current Therapy of Endocrinology and Metabolism. Saunders, Philadephia, 1996, pp. 609–614.
Seminara SB, Achermann JC, Genel M, et al X-linked adrenal hypoplasia congenita: a mutation in DAX1 expands the phenotypic spectrum in males and females. J Clin Endocrinol Metab 1999; 84: 4501–4509.
Rizzo WB. X-linked adrenoleukodystrophy: a cause of primary adrenal insufficeincy in males. The Endocrinologist. 1992; 2: 177–183.
Ten S, New M, Maclaren N. Clinical review 130: Addison’s disease 2001. J Clin Endocrinol Metab. 2001; 86: 2909–2922.
Merke DP, Camacho CA. Novel basic and clinical aspects of congenital adrenal hyperplasia. Rev Endocr Metab Disord 2001; 2: 289–296.
White PC. Congenital adrenal hyperplasias. Best Pract Res Clin Endocrinol Metab. 2001; 15: 17–41.
Porter FD. RSH/Smith-Lemli-Opitz syndrome: a multiple congenital anomaly/mental retardation syndrome due to an inborn error of cholesterol biosynthesis. Mol Genet Metab 2000; 71: 163–174.
Avgerinos PC, Chrousos GP, Nieman LK, et al. The corticotropin-releasing hormone test in the postoperative evaluation of patients with Cushing’s syndrome. J Clin Endocrinol Metab 1987; 65: 906–913.
Graber AL, Ney RL, Nicholson WE, et al. Natural history of pitutiary-adrenal recovery following long-term suppression with corticosteroid. J Clin Endocrinol Metab 1965; 25: 11–16.
Yamamoto T, Fukuyama J, Haasegawa K, et al. Isolated corticotropin deficiency in adults. Report of 10 cases and reveiw of the literature. Arch Intern Med 1992; 152: 1705–1712.
Oelkers H, Diederich S, Bahr V. Diagnosis and therapy surveillance in Addison’ s disease: rapid adrenocorticotropin (ACTH) test and measurement of plasma ACTH, renin activity, and aldosterone. J Clin Endocrinol Metab 1992; 75: 259–264.
Grinspoon SK, Biller BM. Clinical review 62: laboratory assessment of adrenal insufficiency. J Clin Endocrinol Metab 1994; 79: 923–931.
Dickstein G, Schechner C. Low dose ACTH test - a word of caution to the word of caution: when and how to use it. J Clin Endocrinol Metab 1997; 82: 322.
Borst GC, Michenfelder HI, O’Brian JT. Discordant cortisol responses to exogeneous ACTH and insulin-induced hypoglycemia in patients with pituitary diseases. N Engl J Med 1982; 302: 1462–1464.
Tordjman K, Jaffe A, Trostanetsky Y, et al. Low-dose (1 mcg) adrenocorticotrophin (ACTH) stimulation as a screening test for impaired hypothalamo-pituitary-adrenal axis function: sensitivity, specificity and accuracy in comparison with the high-dose (250 mcg) test. Clin Endocrinol (Oxf) 2000; 52: 633–640.
Ammari F, Issa BG, Millward E, Scanion MF. A comparison between short ACTH and insulin stress tests for assessing hypothalamo-pituitary-adrenal function. Clin Endocrinol (Oxf) 1996; 44: 473–476.
Soule SG, Fahie-Wilson M, Tomlinson S. Failure of the short ACTH test to unequivocally diagnose long-standing symptomatic secondary hypoadrenalism. Clin Endocrinol (Oxf) 1996; 44: 137–140.
Rasmuson S, Olsson T, Hagg E. A low dose ACTH test to assess the function of the hypothalamicpituitary-adrenal axis. Clin Endocrinol (Oxf) 1996; 44: 151–156.
Cunningham SK, Moore A, McKenna TJ. Normal cortisol response to corticotropin in patients with secondary adrenal failure. Arch Intern Med 1983; 143: 2276–2279.
Clayton RN. Short Synacthen test versus insulin stress test for assessment of the hypothalamo-pituitary-adrenal axis: controversy revisited. Clin Endocrinol (Oxf) 1996; 44: 147–149.
Schulte HM, Chrousos GP, Avgerinos P, et al. The corticotropin-releasing hormone stimulation test: a possible aid in the evaluation of patients with adrenal insufficiency. J Clin Endocrinol Metab 1984; 58: 1064–1067.
Dluhy RG, Himathongkam T, Greenfield M. Rapid ACTH test with plasma aldosterone levels. Ann Intern Med 1974; 80: 693–696.
New MI. Minireview: 21-hydroxylase deficiency congenital adrenal hyperplasia. J Steroid Biochem Molec Biol 1994; 48: 15–22.
Miller WL. Genetics, diagnosis, and management of 21-hydroxylase deficiency. J Clin Endocrinol Metab 1994; 78: 241–246.
Speiser PW, White PC, Dupont J, et al. Molecular genetic prenatal diagnosis of congenital adrenal hyperplasia due to 21-hydroxylase deficiency by allele-specific hybridization. Recent Prog Horm Res 1994; 49: 367–371.
Barbat B, Bogyo A, Raux-Dermay MC, et al. Screening of CYP21 gene mutations in 120 French patients affected by steroid 21-hydroxylase deficiency. Hum Mutat 1995; 5: 126–130.
Couillin P, Nicolas H, Boue J, et al. HLA typing of amniotic-fluid cells applied to prenatal diagnosis of congenital adrenal hyperplasia. Lancet 1979; 1: 1076.
Coursin DB, Wood ICE. Corticosteroid supplementation for adrenal insufficiency. JAMA. 2002; 287: 236–240.
Merke DP, Cutler GB. New ideas for medical treatment of congenital adrenal hyperplasia. Endocrinol Metab Clin North Am 2001; 30: 121–135.
David M, Forest MG. Prenatal treatment of congenital adrenal hyperplasia resulting from 21-hydroxylase deficiency. J Pediatr 1984; 105: 799–803.
Speiser PW. First trimester prenatal treatment and molecular genetic diagnosis of congenital adrenal hyperplasia (21-hydroxylase deficiency). J Clin Endocrinol Metab. 1990; 70: 838–848.
Speiser PW, New MI. Prenatal diagnosis and treatment of congenital adrenal hyperplasia. J Ped Endocrinol 1994; 7: 183–191.
New MI, Carlson A, Obeid J, et al. Prenatal diagnosis for congenital adrenal hyperplasia in 532 pregnancies. J Clin Endocrinol Metab. 2001; 86: 5651–5657
Lajic S, Wedell A, Bui TH, et al. Long-term somatic follow-up of prenatally treated children with congenital adrenal hyperplasia. J Clin Endocrinol Metab. 1998; 83: 3872–3880.
Mulaikal RM, Migeon CJ, Rock JA. Fertility rates in female patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. N Engl J Med 1987; 316: 178–188.
Dittmann RW, Kappes ME, Kappes MH. Sexual behavior in adolescent and adult females with congenital adrenal hyperplasia. Psychoneuroendocrinology 1992; 17: 153–170.
Kuhnle U, Bollinger M, Schwarz HP, et al. Partnership and sexuality in adult female patients with congenital adrenal hyperplasia: first results of a cross-sectional qualtiy-of-life evaluation. J Steroid Biochem Mol Biol 1993; 45: 123–126.
Lajic S, Wedell A, Ritzen EM, et al. Scandinavian experience of prenatal treatment of congenital adrenal hyperplasia. Horm Res 1997; 48: 22.
Buescher MA, McClamrock HD, Adashi EY. Cushing’s syndrome in pregnancy. Obstet Gynecol 1992; 79: 130–137.
Forest MG, Betuel H, David M. Prenatal treatment in congenital adrenal hyperplasia due to 21hydroxylase deficiency: update 88 of the French multicentric study. Endocr Res 1989; 15: 277–301.
Don HG, Sippell WG, Willig RP. Praenatale Diagnostik and Therapie des Adrenogenitalen Syndroms (AGS) mit 21-Hydroxylase Defekt. Monatsschr Kinderheilkd 1992; 140: 661–663.
Forest MG, David M, Morel Y. Prenatal diagnosis and treatment of 21-hydroxylase deficiency. J Steroid Biochem Mol Biol 1993; 45: 75–82.
Pang S, Clark AT, Freeman LC, et al. Maternal side-effects of prenatal dexamethasone therapy for fetal congenital adrenal hyperplasia. J Clin Endocrinol Metab 1992; 75: 249–253.
Mercado AB, Wilson RC, Cheng KC, et al. Prenatal treatment and diagnosis of congenital adrenal hyperplasia owing to 21-hydroxylase deficiency. J Clin Endocrinol Metab1995; 80: 2014–2020.
Seckl JR, Miller WL. Commentary: how safe is long-term prenatal glucocorticoid treatment? J Am Med Assoc 1997; 277: 1077–1079.
Uno H, Lohmiller L, Thieme C, et al. Brain damage induced by prenatal exposure to dexamethasone in fetal rhesus macaques. I. Hippocampus. Brain Res Dev 1990; 53: 157–167.
Trautman PD, Meyer-Bahlburg HFL, Postelnek J, New MI. Effects of early prenatal dexamethasone on the cognitive and behavioral development of young children: results of a pilot study. Psychoneuroendocrinology 1995; 20: 439–449.
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Nieman, L.K., Rother, K.I. (2003). Glucocorticoid Treatment in Prenatal and Postnatal Life. In: Meikle, A.W. (eds) Endocrine Replacement Therapy in Clinical Practice. Contemporary Endocrinology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-375-0_16
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DOI: https://doi.org/10.1007/978-1-59259-375-0_16
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