Adrenomedullary function in patients with nonclassic congenital adrenal hyperplasia

Hormone and Metabolic Research

Volumn 42, Issue 8, 2010, Pages 607-612

  Verma, S ^a,e   Green Golan, L ^b   Vanryzin, C ^b   Drinkard, B ^b   Mehta, S P ^b   Weise, M ^c   Eisenhofer, G ^d   Merke, D P ^a,b,e  

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

^b NATIONAL INSTITUTES OF HEALTH   (United States)

^c FEDERAL INSTITUTE FOR DRUGS AND MEDICAL DEVICES   (Germany)

^d UNIVERSITY HOSPITAL CARL GUSTAV CARUS   (Germany)

^e US Dept of Hlth and Hum Serv   (United States)

Author keywords

adrenal cortex; adrenal medulla; epinephrine; glucocorticoids

Indexed keywords

ADRENALIN; FLUDROCORTISONE; GLUCOCORTICOID; GLUCOSE; HYDROCORTISONE; GLUCOSE BLOOD LEVEL;

ADOLESCENT; ADRENAL CORTEX INSUFFICIENCY; ADRENAL FUNCTION; ADRENAL MEDULLA; ADRENALIN BLOOD LEVEL; ADULT; ARTICLE; CHILD; CLINICAL ARTICLE; CONGENITAL ADRENAL HYPERPLASIA; CONTROLLED STUDY; DISEASE ASSOCIATION; DISEASE SEVERITY; FEMALE; GLUCOSE BLOOD LEVEL; HUMAN; MALE; PRIORITY JOURNAL; SALT WASTING; SCHOOL CHILD; VIRILIZATION; BLOOD; CASE CONTROL STUDY; CLINICAL TRIAL; EXERCISE TEST; METABOLISM; PATHOPHYSIOLOGY; YOUNG ADULT;

ADOLESCENT; ADRENAL HYPERPLASIA, CONGENITAL; ADRENAL MEDULLA; ADULT; BLOOD GLUCOSE; CASE-CONTROL STUDIES; CHILD; EPINEPHRINE; EXERCISE TEST; FEMALE; HUMANS; HYDROCORTISONE; MALE; YOUNG ADULT;

EID: 77954221575     PISSN: 00185043     EISSN: 14394286     Source Type: Journal    
DOI: 10.1055/s-0030-1253385     Document Type: Article

References (34)

1. Merke DP, Bornstein SR. Congenital adrenal hyperplasia. Lancet 2005 365 2125-2136
2. Speiser PW, Dupont J, Zhu D, Serrat J, Buegeleisen M, Tusie-Luna 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
3. Boikos SA, Horvath A, Heyerdahl S, Stein E, Robinson-White A, Bossis I, Bertherat J, Carney JA, Stratakis CA. Phosphodiesterase 11A expression in the adrenal cortex, primary pigmented nodular adrenocortical disease, and other corticotropin-independent lesions. Horm Metab Res 2008 40 347-353 (Pubitemid 351859447)
4. Kuulasmaa T, Jaaskelainen J, Suppola S, Pietilainen T, Heikkila P, Aaltomaa S, Kosma VM, Voutilainen R. WNT-4 mRNA expression in human adrenocortical tumors and cultured adrenal cells. Horm Metab Res 2008 40 668-673
5. Powers JF, Picard KL, Tischler AS. RET expression and neuron-like differentiation of pheochromocytoma and normal chromaffin cells. Horm Metab Res 2009 41 710-714
6. Merke DP, Chrousos GP, Eisenhofer G, Weise M, Keil MF, Rogol AD, Van Wyk JJ, Bornstein SR. Adrenomedullary dysplasia and hypofunction in patients with classic 21-hydroxylase deficiency. N Engl J Med 2000 343 1362-1368
7. Weise M, Drinkard B, Mehlinger SL, Holzer SM, Eisenhofer G, Charmandari E, Chrousos GP, Merke DP. Stress dose of hydrocortisone is not beneficial in patients with classic congenital adrenal hyperplasia undergoing short-term, high-intensity exercise. J Clin Endocrinol Metab 2004 89 3679-3684
8. Weise M, Mehlinger SL, Drinkard B, Rawson E, Charmandari E, Hiroi M, Eisenhofer G, Yanovski JA, Chrousos GP, Merke DP. Patients with classic congenital adrenal hyperplasia have decreased epinephrine reserve and defective glucose elevation in response to high-intensity exercise. J Clin Endocrinol Metab 2004 89 591-597
9. Green-Golan L, Yates C, Drinkard B, VanRyzin C, Eisenhofer G, Weise M, Merke DP. Patients with classic congenital adrenal hyperplasia have decreased epinephrine reserve and defective glycemic control during prolonged moderate-intensity exercise. J Clin Endocrinol Metab 2007 92 3019-3024
10. Riepe FG, Krone N, Kruger SN, Sweep FC, Lenders JW, Dotsch J, Monig H, Sippell WG, Partsch CJ. Absence of exercise-induced leptin suppression associated with insufficient epinephrine reserve in patients with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Exp Clin Endocrinol Diabetes 2006 114 105-110
11. Borg GA. Psychophysical bases of perceived exertion. Med Sci Sports Exerc 1982 14 377-381
12. Eisenhofer G, Goldstein DS, Stull R, Keiser HR, Sunderland T, Murphy DL, Kopin IJ. Simultaneous liquid-chromatographic determination of 3,4-dihydroxyphenylglycol, catecholamines, and 3,4-dihydroxyphenylalanine in plasma, and their responses to inhibition of monoamine oxidase. Clin Chem 1986 32 2030-2033
13. Center for Disease Control. Epi Info. In. 3.3.2 ed 2000
14. th week human fetal adrenals. Horm Metab Res 1997 29 30-32
15. Ehrhart-Bornstein M, Bornstein SR. Cross-talk between adrenal medulla and adrenal cortex in stress. Ann N Y Acad Sci 2008 1148 112-117
16. Wurtman RJ, Pohorecky LA. Adrenocortical control of epinephrine synthesis in health and disease. Adv Metab Disord 1971 5 53-76
17. Linder BL, Esteban NV, Yergey AL, Winterer JC, Loriaux DL, Cassorla F. Cortisol production rate in childhood and adolescence. J Pediatr 1990 117 892-896
18. Bidet M, Bellanne-Chantelot C, Galand-Portier M-B, Tardy V, Billaud L, Laborde K, Coussieu C, Morel Y, Vaury C, Golmard J-L, Claustre A, Mornet E, Chakhtoura Z, Mowszowicz I, Bachelot A, Touraine P, Kutten F. Clinical and molecular characterization of a cohort of 161 unrelated women with nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency and 330 family members. J Clin Endocrinol Metab 2009 94 1570-1578
19. Charmandari E, Eisenhofer G, Mehlinger SL, Carlson A, Wesley R, Keil MF, Chrousos GP, New MI, Merke DP. Adrenomedullary function may predict phenotype and genotype in classic 21-hydroxylase deficiency. J Clin Endocrinol Metab 2002 87 3031-3037
20. Marker JC, Hirsch IB, Smith LJ, Parvin CA, Holloszy J O, Cryer PE. Catecholamines in prevention of hypoglycemia during exercise in humans. Am J Physiol 1991 260 E705-E712
21. Galbo H, Christensen NJ, Mikines KJ, Sonne B, Hilsted J, Hagen C, Fahrenkrug J. The effect of fasting on the hormonal response to graded exercise. J Clin Endocrinol Metab 1981 52 1106-1112
22. Fellmann N, Coudert J. [Physiology of muscular exercise in children (author's transl)]. Arch Pediatr 1994 1 827-840
23. Hansen IL, Levy MM, Kerr DS. Differential diagnosis of hypoglycemia in children by responses to fasting and 2-deoxyglucose. Metabolism 1983 32 960-970
24. Kruse K, Bartels H. [Significance of the 2-deoxy-d-glucose-test on the differentiation of hypoglycemia in childhood (author's transl)]. Klin Padiatr 1975 187 308-313
25. Sizonenko PC, Paunier L, Vallotton B, Cuendet GS, Zahnd G, Marliss EB. Response to 2-deoxy-D-glucose and to glucagon in ketotic hypoglycemia of childhood: evidence for epinephrine deficiency and altered alanine availability. Pediatr Res 1973 7 983-993
26. Tietze HU, Zurbrugg RP, Zuppinger KA, Joss EE, Kaser H. Occurrence of impaired cortisol regulation in children with hypoglycemia associated with adrenal medullary hyporesponsiveness. J Clin Endocrinol Metab 1972 34 948-958
27. Bao S, Briscoe VJ, Tate DB, Davis SN. Effects of differing antecedent increases of plasma cortisol on counterregulatory responses during subsequent exercise in type 1 diabetes. Diabetes 2009 58 2100-2108
28. Zuckerman-Levin N, Tiosano D, Eisenhofer G, Bornstein S, Hochberg Z. The importance of adrenocortical glucocorticoids for adrenomedullary and physiological response to stress: a study in isolated glucocorticoid deficiency. J Clin Endocrinol Metab 2001 86 5920-5924
29. Artavia-Loria E, Chaussain JL, Bougneres PF, Job JC. Frequency of hypoglycemia in children with adrenal insufficiency. Acta Endocrinol Suppl (Copenh) 1986 279 275-278
30. Hinde FR, Johnston DI. Hypoglycaemia during illness in children with congenital adrenal hyperplasia. Br Med J (Clin Res Ed) 1984 289 1603-1604
31. Donaldson MD, Thomas PH, Love JG, Murray JD, McNinch AW, Savage DC. Presentation, acute illness, and learning difficulties in salt wasting 21-hydroxylase deficiency. Arch Dis Child 1994 70 214-218
32. Mackinnon J, Grant DB. Hypoglycaemia in congenital adrenal hyperplasia. Arch Dis Child 1977 52 591-593
33. Charmandari E, Weise M, Bornstein SR, Eisenhofer G, Keil MF, Chrousos G, Merke DP. Children with classic congenital adrenal hyperplasia have elevated serum leptin concentrations and insulin resistance: potential clinical implications. J Clin Endocrinol Metab 2002 87 2114-2120
34. Bidet M, Bellanne-Chantelot C, Galand-Portier MB, Golmard J-L, Tardy V, Morel Y, Clauin S, Coussieu C, Boudou P, Mowzowicz I, Bachelot A, Touraine P, Kutten F. Fertility in women with nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency. J Clin Endocrinol Metab 2010 95 1182-1190