Adrenocorticotropin hypersecretion and pituitary microadenoma following bilateral adrenalectomy in a patient with classic 21-hydroxylase deficiency

Journal of Pediatric Endocrinology and Metabolism

Volumn 18, Issue 1, 2005, Pages 97-101

  Charmandari, Evangelia ^a,c   Chrousos, George P ^a   Merke, Deborah P ^a,b  

^a EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH AND HUMAN DEVELOPMENT   (United States)

^b NATIONAL INSTITUTES OF HEALTH   (United States)

^c GREAT ORMOND STREET HOSPITAL   (United Kingdom)

Author keywords

21 hydroxylase deficiency; Adrenal rest tissue; Adrenalectomy; Congenital adrenal hyperplasia; Pituitary microadenoma

Indexed keywords

CORTICOTROPIN; CORTICOTROPIN RELEASING FACTOR; DEXAMETHASONE; FLUDROCORTISONE; HYDROCORTISONE; HYDROCORTISONE SODIUM PHOSPHATE; STEROID 21 MONOOXYGENASE;

ADRENAL FUNCTION; ADRENAL GLAND; ADRENALECTOMY; ADULT; ARTICLE; CANCER RISK; CARCINOGENESIS; CASE REPORT; CONCENTRATION (PARAMETERS); CONGENITAL ADRENAL HYPERPLASIA; CORTICOTROPIN RELEASE; DISEASE SEVERITY; ECTOPIC TISSUE; FEMALE; HUMAN; HYPERANDROGENISM; HYPERPIGMENTATION; HYPOPHYSIS ADENOMA; HYPOTHALAMUS HYPOPHYSIS ADRENAL SYSTEM; NELSON SYNDROME; SALT LOSING NEPHRITIS; STEROID THERAPY; SURGICAL RISK; TREATMENT FAILURE;

EID: 13644254140     PISSN: 0334018X     EISSN: None     Source Type: Journal    
DOI: 10.1515/JPEM.2005.18.1.97     Document Type: Article

References (15)

1. Merke DP, Bornstein SR, Avila NA, Chrousos GP. NIH conference. Future directions in the study and management of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Ann Intern Med 2002; 136: 320-334.
2. Van Wyk JJ, Ritzen EM. The role of bilateral adrenalectomy in the treatment of congenital adrenal hyperplasia. J Clin Endocrinol Metab 2003; 88: 2993-2998.
3. Speiser PW, Heier L, Serrat J, New MI, Nass R. Failure of steroid replacement to consistently normalize pituitary function in congenital adrenal hyperplasia: hormonal and MRI data. Horm Res 1995; 44: 241-246.
4. Consensus Statement on 21-Hydroxylase Deficiency from the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology. J Clin Endocrinol Metab 2002; 87: 4048-4053.
5. Speiser PW, Dupont J, Zhu D, Serrat J, Buegeleisen M, Tusie-Luna MT, Lesser M, New MI, White PC. Disease expression and molecular genotype in congenital adrenal hyperplasia due to 21-hydroxylase deficiency. J Clin Invest 1992; 90: 584-595.
6. Merke DP, Bornstein SR, Braddock D, Chrousos GP. Adrenal lymphocytic infiltration and adrenocortical tumors in a patient with 21-hydroxylase deficiency. N Engl J Med 1999; 340: 1121-1122.
7. Gunther DF, Bukowski TP, Ritzen EM, Wedell A, Van Wyk JJ. Prophylactic adrenalectomy of a three-year-old girl with congenital adrenal hyperplasia: pre- and postoperative studies. J Clin Endocrinol Metab 1997; 82: 3324-3327.
8. Merke DP, Chrousos GP, Eisenhofer G, Weise M, Keil MF, Rogol AD, Van Wyk JJ, Bornstein SR. Adreno-medullary dysplasia and hypofunction in patients with classic 21-hydroxylase deficiency. N Engl J Med 2000; 343: 1362-1368.
9. Yanase T, Sekiya K, Ando M, Nawata H, Kato K, Ibayashi H. Probable ACTH-secreting pituitary tumour in association with Addison's disease. Acta Endocrinol (Copenh) 1985; 110: 36-41.
10. Carr DB, Fisher JE, Rosenblatt M. Response to low-dose pulsatile cortisol in Addison's disease with suspected corticotropinoma. Horm Metab Res 1986; 18: 569-573.
11. Karl M, Lamberts SW, Koper JW, Katz DA, Huizenga NE, Kino T, Haddad BR, Hughes MR, Chrousos GP. Cushing's disease preceded by generalized glucocorticoid resistance: clinical consequences of a novel, dominant-negative glucocorticoid receptor mutation. Proc Assoc Am Physicians 1996; 108: 296-307.
12. Horrocks PM, Franks S, Hockley AD, Rolfe EB, Van Noorden S, London DR. An ACTH-secreting pituitary tumour arising in a patient with congenital adrenal hyperplasia. Clin Endocrinol (Oxf) 1982; 17: 457-468.
13. Chrousos GP, Schulte HM, Oldfield EH, Gold PW, Cutler GB Jr, Loriaux DL. The corticotropin-releasing factor stimulation test. An aid in the evaluation of patients with Cushing's syndrome. N Engl J Med 1984; 310: 622-626.
14. Oldfield EH, Schulte HM, Chrousos, GP, Gold PW, Benker G, Peterson RE, Cutler GB Jr, Loriaux DL. Corticotropin-releasing hormone (CRH) stimulation in Nelson's syndrome: response of adrenocorticotropin secretion to pulse injection and continuous infusion of CRH. J Clin Endocrinol Metab 1986; 62: 1020-1026.
15. Schulte HM, Chrousos GP, Avgerinos P, Oldfield EH, Gold PW, Cutler GB Jr, Loriaux DL. 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.