E Charmandari

Summary

Affiliation: National Institutes of Health
Country: USA

Publications

  1. ncbi A novel point mutation in the ligand-binding domain (LBD) of the human glucocorticoid receptor (hGR) causing generalized glucocorticoid resistance: the importance of the C terminus of hGR LBD in conferring transactivational activity
    Evangelia Charmandari
    Department of Pediatric Endocrinology, Great Ormond Street Hospital for Children, 9th Floor, Southwood Building, Great Ormond Street, London, United Kingdom WC1N 3JH
    J Clin Endocrinol Metab 90:3696-705. 2005
  2. ncbi Glucocorticoids and their actions: an introduction
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892 1583, USA
    Ann N Y Acad Sci 1024:1-8. 2004
  3. ncbi Endocrinology of the stress response
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
    Annu Rev Physiol 67:259-84. 2005
  4. ncbi Pediatric stress: hormonal mediators and human development
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
    Horm Res 59:161-79. 2003
  5. ncbi Adrenocorticotropin hypersecretion and pituitary microadenoma following bilateral adrenalectomy in a patient with classic 21-hydroxylase deficiency
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA
    J Pediatr Endocrinol Metab 18:97-101. 2005
  6. ncbi Classic congenital adrenal hyperplasia and puberty
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892 1583, USA
    Eur J Endocrinol 151:U77-82. 2004
  7. ncbi Sexual dimorphism in the synchrony of joint growth hormone and cortisol dynamics in children with classic 21-hydroxylase deficiency
    E Charmandari
    London Centre for Paediatric Endocrinology, University College London, London, UK
    J Pediatr Endocrinol Metab 16:1119-30. 2003
  8. ncbi The human glucocorticoid receptor (hGR) beta isoform suppresses the transcriptional activity of hGRalpha by interfering with formation of active coactivator complexes
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, 10 Center Drive, Building 10, Room 9D42, Bethesda, Maryland 20892 1583, USA
    Mol Endocrinol 19:52-64. 2005
  9. ncbi Familial/sporadic glucocorticoid resistance: clinical phenotype and molecular mechanisms
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892 1583, USA
    Ann N Y Acad Sci 1024:168-81. 2004
  10. ncbi Functional characterization of the natural human glucocorticoid receptor (hGR) mutants hGRalphaR477H and hGRalphaG679S associated with generalized glucocorticoid resistance
    Evangelia Charmandari
    Pediatric Endocrinology Section, Reproductive Biology and Medicine Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20895, USA
    J Clin Endocrinol Metab 91:1535-43. 2006

Detail Information

Publications40

  1. ncbi A novel point mutation in the ligand-binding domain (LBD) of the human glucocorticoid receptor (hGR) causing generalized glucocorticoid resistance: the importance of the C terminus of hGR LBD in conferring transactivational activity
    Evangelia Charmandari
    Department of Pediatric Endocrinology, Great Ormond Street Hospital for Children, 9th Floor, Southwood Building, Great Ormond Street, London, United Kingdom WC1N 3JH
    J Clin Endocrinol Metab 90:3696-705. 2005
    ..We conclude that the carboxyl terminus of the ligand-binding domain of hGRalpha is extremely important in conferring transactivational activity by altering multiple functions of this composite transcription factor...
  2. ncbi Glucocorticoids and their actions: an introduction
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892 1583, USA
    Ann N Y Acad Sci 1024:1-8. 2004
  3. ncbi Endocrinology of the stress response
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
    Annu Rev Physiol 67:259-84. 2005
    ....
  4. ncbi Pediatric stress: hormonal mediators and human development
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
    Horm Res 59:161-79. 2003
    ....
  5. ncbi Adrenocorticotropin hypersecretion and pituitary microadenoma following bilateral adrenalectomy in a patient with classic 21-hydroxylase deficiency
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA
    J Pediatr Endocrinol Metab 18:97-101. 2005
    ..v. bolus) and a low dose (0.5 mg given orally every 6 h for 48 h) dexamethasone suppression test. Patients with CAH have hyperactivity of the hypothalamic-pituitary-adrenal axis and are at risk for pituitary tumor formation...
  6. ncbi Classic congenital adrenal hyperplasia and puberty
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892 1583, USA
    Eur J Endocrinol 151:U77-82. 2004
    ..The latter may further enhance adrenal and/or ovarian androgen secretion, decrease the therapeutic efficacy of glucocorticoids and contribute to later development of the metabolic syndrome and its complications...
  7. ncbi Sexual dimorphism in the synchrony of joint growth hormone and cortisol dynamics in children with classic 21-hydroxylase deficiency
    E Charmandari
    London Centre for Paediatric Endocrinology, University College London, London, UK
    J Pediatr Endocrinol Metab 16:1119-30. 2003
    ..83 +/- 0.20) (p = 0.03). These findings suggest that females with classic 21-hydroxylase deficiency have a more irregular pattern of GH secretion and a more asynchronous joint GH and cortisol dynamics than their male counterparts...
  8. ncbi The human glucocorticoid receptor (hGR) beta isoform suppresses the transcriptional activity of hGRalpha by interfering with formation of active coactivator complexes
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, 10 Center Drive, Building 10, Room 9D42, Bethesda, Maryland 20892 1583, USA
    Mol Endocrinol 19:52-64. 2005
    ..These findings suggest that participation of hGRbeta in the formation of a coactivator complex renders this complex ineffective...
  9. ncbi Familial/sporadic glucocorticoid resistance: clinical phenotype and molecular mechanisms
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892 1583, USA
    Ann N Y Acad Sci 1024:168-81. 2004
    ..The present review focuses on the mechanisms of GR action and the clinical manifestations and molecular mechanisms of familial/sporadic glucocorticoid resistance...
  10. ncbi Functional characterization of the natural human glucocorticoid receptor (hGR) mutants hGRalphaR477H and hGRalphaG679S associated with generalized glucocorticoid resistance
    Evangelia Charmandari
    Pediatric Endocrinology Section, Reproductive Biology and Medicine Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20895, USA
    J Clin Endocrinol Metab 91:1535-43. 2006
    ....
  11. ncbi Metabolic syndrome manifestations in classic congenital adrenal hyperplasia: do they predispose to atherosclerotic cardiovascular disease and secondary polycystic ovary syndrome?
    Evangelia Charmandari
    Section on Pediatric Endocrinology, Reproductive and Molecular Biology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
    Ann N Y Acad Sci 1083:37-53. 2006
    ..Nonpharmacologic and pharmacologic interventions targeting obesity and/or insulin resistance may offer an improved outcome in terms of cardiovascular morbidity...
  12. ncbi Treatment with flutamide decreases cortisol clearance: implications for therapy in congenital adrenal hyperplasia
    E Charmandari
    London Centre for Paediatric Endocrinology, University College London, UK
    J Pediatr Endocrinol Metab 15:435-9. 2002
    ..Anti-androgen therapy with flutamide is an option that allows control of hyperandrogenism without recourse to supraphysiological doses of glucocorticoid...
  13. ncbi Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: alterations in cortisol pharmacokinetics at puberty
    E Charmandari
    London Centre for Paediatric Endocrinology, University College London, United Kingdom W1N 8AA
    J Clin Endocrinol Metab 86:2701-8. 2001
    ..These alterations probably reflect changes in the endocrine milieu at puberty and may have implications for therapy of CAH and other conditions requiring cortisol substitution in the adolescent years...
  14. pmc Congenital adrenal hyperplasia: management during critical illness
    E Charmandari
    London Centre for Paediatric Endocrinology, Great Ormond Street Hospital and The Institute of Child Health, University College London, London WC1N 3JH, UK
    Arch Dis Child 85:26-8. 2001
    ..Little is known of the optimal dose and administration schedule of hydrocortisone in critically ill patients with congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency...
  15. ncbi Serum cortisol and 17-hydroxyprogesterone interrelation in classic 21-hydroxylase deficiency: is current replacement therapy satisfactory?
    E Charmandari
    London Center for Pediatric Endocrinology, University College London, London W1T 3AA, United Kingdom
    J Clin Endocrinol Metab 86:4679-85. 2001
    ..It should also be emphasized that blood investigations are only complementary to the overall assessment of these patients, which is primarily based on the evaluation of growth and pubertal progress...
  16. ncbi Kinetics and effect of percutaneous administration of dihydrotestosterone in children
    E Charmandari
    London Centre for Paediatric Endocrinology, University College London, London, UK
    Horm Res 56:177-81. 2001
    ..We investigated whether percutaneous administration of DHT is similarly effective in patients with micropenis due to alternative diagnoses...
  17. pmc Why is management of patients with classical congenital adrenal hyperplasia more difficult at puberty?
    E Charmandari
    London Centre for Paediatric Endocrinology, University College London, London, UK
    Arch Dis Child 86:266-9. 2002
    ....
  18. ncbi Children with classic congenital adrenal hyperplasia have elevated serum leptin concentrations and insulin resistance: potential clinical implications
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Clin Endocrinol Metab 87:2114-20. 2002
    ....
  19. ncbi Oral hydrocortisone administration in children with classic 21-hydroxylase deficiency leads to more synchronous joint GH and cortisol secretion
    Evangelia Charmandari
    London Center for Pediatric Endocrinology, University College London, London, W1T 3AA, United Kingdom
    J Clin Endocrinol Metab 87:2238-44. 2002
    ....
  20. ncbi Natural glucocorticoid receptor mutants causing generalized glucocorticoid resistance: molecular genotype, genetic transmission, and clinical phenotype
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Clin Endocrinol Metab 89:1939-49. 2004
    ....
  21. ncbi Joint growth hormone and cortisol spontaneous secretion is more asynchronous in older females than in their male counterparts
    E Charmandari
    London Center for Pediatric Endocrinology, University College London, London W1T 3AA, United Kingdom
    J Clin Endocrinol Metab 86:3393-9. 2001
    ....
  22. ncbi Bioavailability of oral hydrocortisone in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency
    E Charmandari
    London Centre for Paediatric Endocrinology, University College London, London, UK
    J Endocrinol 169:65-70. 2001
    ....
  23. ncbi A novel point mutation in helix 11 of the ligand-binding domain of the human glucocorticoid receptor gene causing generalized glucocorticoid resistance
    Evangelia Charmandari
    Section on Endocrinology, Reproductive Biology and Medicine Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Clin Endocrinol Metab 92:3986-90. 2007
    ....
  24. ncbi Familial/sporadic glucocorticoid resistance syndrome and hypertension
    Tomoshige Kino
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development NIH, Building 10, Room 9D42, 10 Center Drive MSC 1583, Bethesda, MD 20892, USA
    Ann N Y Acad Sci 970:101-11. 2002
    ..These results suggest that the pathologic mechanisms of glucocorticoid resistance is quite broad, and this is reflected in the wide variability of the clinical picture in patients with the syndrome...
  25. ncbi Rho family Guanine nucleotide exchange factor Brx couples extracellular signals to the glucocorticoid signaling system
    Tomoshige Kino
    Pediatric Endocrinology Section, Reproductive Biology and Medicine Branch, NICHD, National Institutes of Health, Bethesda Maryland 20892, USA
    J Biol Chem 281:9118-26. 2006
    ..Nuclear Brx might act as a local GRE-GR-transcriptosome activator by mediating the effect of small G-proteins on glucocorticoid-regulated genes...
  26. ncbi Blood pressure in children and adolescents with congenital adrenal hyperplasia (21-hydroxylase deficiency): a preliminary report
    Edna F Roche
    Centre for Human Growth and Maturation, London Centre for Paediatric Endocrinology, University College London, UK
    Clin Endocrinol (Oxf) 58:589-96. 2003
    ..The latter along with the increased tendency towards obesity raises questions whether other cardiovascular risk factors are altered in CAH...
  27. ncbi HIV-1 accessory protein Vpr inhibits the effect of insulin on the Foxo subfamily of forkhead transcription factors by interfering with their binding to 14-3-3 proteins: potential clinical implications regarding the insulin resistance of HIV-1-infected pat
    Tomoshige Kino
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, NIH, 10 Center Dr MSC 1109, Building 10, Clinical Research Center, Room 1 3140, Bethesda, MD 20892 1109, USA
    Diabetes 54:23-31. 2005
    ..Vpr thus may contribute to the tissue-selective insulin resistance often observed in HIV-1-infected individuals...
  28. ncbi Tissue glucocorticoid resistance/hypersensitivity syndromes
    Tomoshige Kino
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892 1583, USA
    J Steroid Biochem Mol Biol 85:457-67. 2003
    ....
  29. pmc Glucocorticoid receptor mutants demonstrate increased motility inside the nucleus of living cells: time of fluorescence recovery after photobleaching (FRAP) is an integrated measure of receptor function
    Tomoshige Kino
    Pediatric Endocrinology Section, Reproductive Biology and Medicine Branch, National Institute of Child Health and Human Development National Institutes of Health, Bethesda, MD 20892 1109, USA
    Mol Med 10:80-8. 2004
    ....
  30. ncbi Hypothalamo-pituitary-adrenal axis integrity after cranial irradiation for childhood posterior fossa tumours
    H A Spoudeas
    London Centre for Paediatric Endocrinology, University College London, London, United Kingdom
    Med Pediatr Oncol 40:224-9. 2003
    ..The aim of this study was to investigate the hypothalamic-pituitary-adrenal (HPA) axis in long-term survivors of cranial irradiation for childhood posterior fossa tumours...
  31. ncbi Novel causes of generalized glucocorticoid resistance
    E Charmandari
    Section on Pediatric Endocrinology, Reproductive Biology and Medicine Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA
    Horm Metab Res 39:445-50. 2007
    ....
  32. ncbi Adrenomedullary function may predict phenotype and genotype in classic 21-hydroxylase deficiency
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
    J Clin Endocrinol Metab 87:3031-7. 2002
    ..Molecular genotype and plasma free metanephrine concentration predict phenotype with similar accuracy. Both methods are more accurate in the most severe forms of the disease...
  33. ncbi Endocrinologic and psychologic evaluation of 21-hydroxylase deficiency carriers and matched normal subjects: evidence for physical and/or psychologic vulnerability to stress
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, The Warren Grant Magnuson Clinical Center, National Institute of Mental Health, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Clin Endocrinol Metab 89:2228-36. 2004
    ..These changes might predict mild predisposition of these subjects to physical and psychologic pathology, suggesting that larger studies are necessary...
  34. ncbi Stress dose of hydrocortisone is not beneficial in patients with classic congenital adrenal hyperplasia undergoing short-term, high-intensity exercise
    Martina Weise
    Developmental Endocrinology Branch, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Clin Endocrinol Metab 89:3679-84. 2004
    ....
  35. pmc Glucocorticoid receptor: implications for rheumatic diseases
    T Kino
    Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
    Clin Exp Rheumatol 29:S32-41. 2011
    ....
  36. ncbi Flutamide decreases cortisol clearance in patients with congenital adrenal hyperplasia
    Evangelia Charmandari
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development NIH, 10 Center Drive, Building 10 Suite 9D42, Bethesda, MD 20892, USA
    J Clin Endocrinol Metab 87:3197-200. 2002
    ..Glucocorticoid replacement doses should be reduced when flutamide is added to the treatment regimen of patients receiving hydrocortisone...
  37. ncbi Patients with classic congenital adrenal hyperplasia have decreased epinephrine reserve and defective glucose elevation in response to high-intensity exercise
    Martina Weise
    Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Clin Endocrinol Metab 89:591-7. 2004
    ..Whether the combination of epinephrine and cortisol deficiency poses a risk for hypoglycemia and/or decreased endurance during long-term physical stress has to be determined...
  38. ncbi Effects of child- and adolescent-onset endogenous Cushing syndrome on bone mass, body composition, and growth: a 7-year prospective study into young adulthood
    Gary M Leong
    Reproductive Biology and Medicine Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA
    J Bone Miner Res 22:110-8. 2007
    ..In 14 children followed for 3-7 years into young adulthood after cure of CS, whereas bone mass largely recovered, persisting increases in total body and visceral fat suggests an increase risk of the metabolic syndrome...
  39. ncbi Glucocorticoid action networks--an introduction to systems biology
    George P Chrousos
    J Clin Endocrinol Metab 89:563-4. 2004
  40. pmc Generalized glucocorticoid resistance: clinical aspects, molecular mechanisms, and implications of a rare genetic disorder
    Evangelia Charmandari
    Division of Endocrinology and Metabolism, Clinical Research Center, Biomedical Research Foundation of the Academy of Athens, 4 Soranou tou Efessiou Street, Athens, Greece
    J Clin Endocrinol Metab 93:1563-72. 2008
    ..We review the clinical aspects, molecular mechanisms, and implications of this disorder...