Cited by 134Sexual Differentiation and DisordersAdrenal Hormones and DisordersOvarian function and disorders

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Management of adolescents with congenital adrenal hyperplasia

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Merke, Deborah P, Poppas, Dix P·2013·The Lancet Diabetes & Endocrinology

DOI 10.1016/s2213-8587(13)70138-4PMC PMC4163910

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Abstract

The management of congenital adrenal hyperplasia involves suppression of adrenal androgen production, in addition to treatment of adrenal insufficiency. Management of adolescents with congenital adrenal hyperplasia is especially challenging because changes in the hormonal milieu during puberty can lead to inadequate suppression of adrenal androgens, psychosocial issues often affect adherence to medical therapy, and sexual function plays a major part in adolescence and young adulthood. For these reasons, treatment regimen reassessment is indicated during adolescence. Patients with non-classic congenital adrenal hyperplasia require reassessment regarding the need for glucocorticoid drug treatment. No clinical trials have compared various regimens for classic congenital adrenal hyperplasia in adults, thus therapy is individualised and based on the prevention of adverse outcomes. Extensive patient education is key during transition from paediatric care to adult care and should include education of females with classic congenital adrenal hyperplasia regarding their genital anatomy and surgical history. Common issues for these patients include urinary incontinence, vaginal stenosis, clitoral pain, and cosmetic concerns; for males with classic congenital adrenal hyperplasia, common issues include testicular adrenal rest tumours. Transition from paediatric to adult care is most successful when phased over many years. Education of health-care providers on how to successfully transition patients is greatly needed.

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Consensus Statement on Management of Intersex Disorders
Lee, P. A., Houk, C. P., Ahmed, S. F., Hughes, I. A., & in collaboration with the participants in the International Consensus Conference on Intersex organized by the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology (2006) · Pediatrics · doi:10.1542/peds.2006-0738 · Cited by 2,324
Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society Clinical Practice Guideline.
Speiser, P. W., Azziz, R., Baskin, L. S., Ghizzoni, L., Hensle, T. W., Merke, D. P., Meyer-Bahlburg, H. F. L., Miller, W. L., Montori, V. M., Oberfield, S. E., et al. (2010) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2009-2631 · Cited by 1,468
Self-citation
Congenital Adrenal Hyperplasia
Speiser, P., & White, P. C. (1987) · New England Journal of Medicine · doi:10.1056/nejm198711263172211 · Cited by 1,420
Consensus statement on management of intersex disorders
Hughes, I. A. (2005) · Archives of Disease in Childhood · doi:10.1136/adc.2006.098319 · Cited by 1,414
Congenital adrenal hyperplasia
Speiser, P. W., & White, P. C. (2003) · New England Journal of Medicine · doi:10.1056/nejmra021561 · Cited by 1,405
Menstruation in girls and adolescents: using the menstrual cycle as a vital sign
American Academy of Pediatrics, Committee on Adolescence, American College of Obstetricians and Gynecologists, & Committee on Adolescent Health Care (2006) · PEDIATRICS · doi:10.1542/peds.2006-2481 · Cited by 651
Improving the transition between paediatric and adult healthcare: a systematic review
Crowley, R., Wolfe, I., Lock, K., & McKee, M. (2011) · Archives of Disease in Childhood · doi:10.1136/adc.2010.202473 · Cited by 649
Diabetes care for emerging adults: recommendations for transition from pediatric to adult diabetes care systems
Peters, A., Laffel, L., & the American Diabetes Association Transitions Working Group (2011) · Diabetes Care · doi:10.2337/dc11-1723 · Cited by 581
Disease expression and molecular genotype in congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Speiser, P. W., Dupont, J., Zhu, D., Serrat, J., Buegeleisen, M., Tusie-Luna, M. T., Lesser, M., New, M. I., & White, P. C. (1992) · Journal of Clinical Investigation · doi:10.1172/jci115897 · Cited by 572
High frequency of nonclassical steroid 21-hydroxylase deficiency
SPEISER, P. W., DUPONT, B. O., RUBINSTEIN, P., PIAZZA, A., KASTELAN, A., & NEW, M. I. (1986) · Obstetrical & Gynecological Survey · doi:10.1097/00006254-198604000-00017 · Cited by 523
Evaluation and Treatment of Hirsutism in Premenopausal Women: An Endocrine Society Clinical Practice Guideline
Martin, K. A., Chang, R. J., Ehrmann, D. A., Ibáñez, L., Løbo, R. A., Rosenfield, R. L., Shapiro, J., Montori, V. M., & Swiglo, B. A. (2008) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2007-2437 · Cited by 511
Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients
Arlt, W., Willis, D. S., Wild, S. H., Krone, N., Doherty, E. J., Hahner, S., Han, T. S., Carroll, P. V., Conway, G. S., Rees, D. A., et al. (2010) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2010-0917 · Cited by 506
Consensus Statement on 21-Hydroxylase Deficiency from The European Society for Paediatric Endocrinology and The Lawson Wilkins Pediatric Endocrine Society
Vogeser, M., Zachoval, R., Felbinger, T. W., Jacob, K., Radetti, G., Bona, G., Corrias, A., Cappa, M., DeSanctis, V., Lauriola, S., et al. (2002) · Hormone Research in Paediatrics · doi:10.1159/000065490 · Cited by 452
Predicting phenotype in steroid 21-hydroxylase deficiency? Comprehensive genotyping in 155 unrelated, well defined patients from southern Germany
Krone, N., Braun, A., Roscher, A. A., Knorr, D., & Schwarz, H. P. (2000) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.85.3.6441 · Cited by 397
Genotype-phenotype correlation in 1,507 families with congenital adrenal hyperplasia owing to 21-hydroxylase deficiency
New, M. I., Abraham, M., Gonzalez, B., Dumic, M., Razzaghy-Azar, M., Chitayat, D., Sun, L., Zaidi, M., Wilson, R. C., & Yuen, T. (2013) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1300057110 · Cited by 365
Total urogenital mobilization–an easier way to repair cloacas
Peña, A. (1997) · Journal of Pediatric Surgery · doi:10.1016/s0022-3468(97)90191-3 · Cited by 343
Consensus statement on 21-hydroxylase deficiency from the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology
Joint LWPES/ESPE CAH Working Group (2002) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2002-020611 · Cited by 332
The effect of clitoral surgery on sexual outcome in individuals who have intersex conditions with ambiguous genitalia: a cross-sectional study
Minto, C. L., Liao, L., Woodhouse, C. R. J., Ransley, P. G., & Creighton, S. M. (2003) · The Lancet · doi:10.1016/s0140-6736(03)12980-7 · Cited by 319
Nonclassical 21-Hydroxylase Deficiency
New, M. I. (2006) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2006-1645 · Cited by 315
Clinical characteristics of a cohort of 244 patients with congenital adrenal hyperplasia
Finkielstain, G. P., Kim, M. S., Sinaii, N., Nishitani, M., Van Ryzin, C., Hill, S. C., Reynolds, J. C., Hanna, R. M., & Merke, D. P. (2012) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2012-2102 · Cited by 313
Self-citation
High prevalence of testicular adrenal rest tumors, impaired spermatogenesis, and Leydig cell failure in adolescent and adult males with congenital adrenal hyperplasia
Stikkelbroeck, N. M. M. L., Otten, B. J., Pasic, A., Jager, G. J., Sweep, C. G. J. F., Noordam, K., & Hermus, A. R. M. M. (2001) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.86.12.8090 · Cited by 305
High frequency of nonclassical steroid 21-hydroxylase deficiency.
Speiser, P., Dupont, B., Rubinstein, P., Piazza, A., Kaštelan, A., & New, M. I. (1985) · PubMed · Cited by 265
Summary of Consensus Statement on Intersex Disorders and Their Management
Houk, C. P., Hughes, I. A., Ahmed, S. F., & Lee, P. A. (2006) · PEDIATRICS · doi:10.1542/peds.2006-0737 · Cited by 254
ANATOMICAL STUDIES OF THE HUMAN CLITORIS
Baskin, L. S., Erol, A., LI, Y. W., LIU, W. H., Kurzrock, E. A., & Cunha, G. R. (1999) · The Journal of Urology · doi:10.1016/s0022-5347(01)68052-2 · Cited by 240
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
Bidet, M., Bellanné-Chantelot, C., Galand-Portier, M.-B., Tardy, V., Billaud, L., Laborde, K., Coussieu, C., Morel, Y., Vaury, C., Golmard, J.-L., et al. (2009) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2008-1582 · Cited by 213
Cystic Fibrosis and Transition to Adult Medical Care
Tuchman, L. K., Schwartz, L. A., Sawicki, G. S., & Britto, M. T. (2010) · PEDIATRICS · doi:10.1542/peds.2009-2791 · Cited by 213
Fertility in women with nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Bidet, M., Bellanné-Chantelot, C., Galand-Portier, M.-B., Golmard, J.-L., Tardy, V., Morel, Y., Clauin, S., Coussieu, C., Boudou, P., Mowzowicz, I., et al. (2010) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2009-1383 · Cited by 204
Comprehensive genetic analysis of 182 unrelated families with congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Finkielstain, G. P., Chen, W., Mehta, S. P., Fujimura, F. K., Hanna, R. M., Van Ryzin, C., McDonnell, N. B., & Merke, D. P. (2011) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2010-0319 · Cited by 192
Self-citation
Increased activation of the alternative “backdoor” pathway in patients with 21-hydroxylase deficiency: evidence from urinary steroid hormone analysis
Kamrath, C., Hochberg, Z., Hartmann, M. F., Remer, T., & Wudy, S. A. (2012) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2011-1997 · Cited by 191
Impaired Sexual and Reproductive Outcomes in Women with Classical Forms of Congenital Adrenal Hyperplasia
Gastaud, F., Bouvattier, C. M., Duranteau, L., Brauner, R., Thibaud, E., Kutten, F., & Bougneres, P. F. (2007) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2006-1757 · Cited by 187
Long‐term follow‐up of female patients with congenital adrenal hyperplasia from 21‐hydroxylase deficiency, with special emphasis on the results of vaginoplasty
Krege, S., Walz, K., Hauffa, B. P., KÖRNER, I., & Rubben, H. (2000) · British Journal of Urology · doi:10.1046/j.1464-410x.2000.00789.x · Cited by 176
Consequences of the ESPE/LWPES guidelines for diagnosis and treatment of disorders of sex development
Hughes, I. A., Nihoul‐Feketé, C., Thomas, B., & Cohen‐Kettenis, P. T. (2007) · Best Practice & Research Clinical Endocrinology & Metabolism · doi:10.1016/j.beem.2007.06.003 · Cited by 173
CYP21 gene mutation analysis in 198 patients with 21-hydroxylase deficiency in The Netherlands: six novel mutations and a specific cluster of four mutations
Stikkelbroeck, N. M. M. L., Hoefsloot, L. H., de Wijs, I. J., Otten, B. J., Hermus, A. R. M. M., & Sistermans, E. A. (2003) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2002-021681 · Cited by 171
High prevalence of reduced fecundity in men with congenital adrenal hyperplasia
Reisch, N., Flade, L., Scherr, M., Rottenkolber, M., Pedrosa Gil, F., Bidlingmaier, M., Wolff, H., Schwarz, H.-P., Quinkler, M., Beuschlein, F., et al. (2009) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2008-1414 · Cited by 170
Metabolic profile and body composition in adult women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Falhammar, H., Filipsson, H., Holmdahl, G., Janson, P.-O., Nordenskjöld, A., Hagenfeldt, K., & Thorén, M. (2007) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2006-1350 · Cited by 167
Reduced Final Height Outcome in Congenital Adrenal Hyperplasia under Prednisone Treatment: Deceleration of Growth Velocity during Puberty
Bonfig, W., Bechtold, S., Schmidt, H., Knorr, D., & Schwarz, H. P. (2007) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2006-2109 · Cited by 167
Children with classic congenital adrenal hyperplasia have elevated serum leptin concentrations and insulin resistance: potential clinical implications.
Charmandari, E., Weise, M., Bornstein, S. R., Eisenhofer, G., Keil, M. F., Chrousos, G. P., & Merke, D. P. (2002) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.87.5.8456 · Cited by 164
Self-citation
Genital sensation after feminizing genitoplasty for congenital adrenal hyperplasia: a pilot study
Crouch, N. S., Minto, C. L., Laio, L. ‐., Woodhouse, C. R. J., & Creighton, S. M. (2003) · British Journal of Urology · doi:10.1111/j.1464-410x.2004.04572.x · Cited by 160
Twenty-Four-Hour Profiles of Luteinizing Hormone, Follicle-Stimulating Hormone, Testosterone, and Estradiol Levels: A Semilongitudinal Study throughout Puberty in Healthy Boys
Albertsson-Wikland, K. G., Rosberg, S., Lannering, B., Dunkel, L., Selstam, G., & Norjavaara, E. (1997) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.82.2.3778 · Cited by 158
Fractures and bone mineral density in adult women with 21-hydroxylase deficiency
Falhammar, H., Filipsson, H., Holmdahl, G., Janson, P.-O., Nordenskjöld, A., Hagenfeldt, K., & Thorén, M. (2007) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2007-0744 · Cited by 151
Cyproterone Acetate Versus Hydrocortisone Treatment in Late-Onset Adrenal Hyperplasia
SPRITZER, P., BILLAUD, L., THALABARD, J.-C., BIRMAN, P., MOWSZOWICZ, I., RAUX-DEMAY, M.-C., CLAIR, F., KUTTENN, F., & MAUVAIS-JARVIS, P. (1990) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem-70-3-642 · Cited by 146
Insulin insensitivity in adrenal hyperplasia due to nonclassical steroid 21-hydroxylase deficiency.
Speiser, P. W., Serrat, J., New, M. I., & Gertner, J. M. (1992) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.75.6.1464643 · Cited by 143
Prevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency
der Grinten, H. L. C., Sweep, F. C. G. J., Blickman, J. G., Hermus, A. R., & Otten, B. J. (2007) · European Journal of Endocrinology · doi:10.1530/eje-07-0201 · Cited by 142
Testicular adrenal rest tumors in patients with congenital adrenal hyperplasia can cause severe testicular damage
der Grinten, H. L. C., Otten, B. J., Hermus, A. R., Sweep, F. C. G. J., & de Kaa, C. A. H. (2007) · Fertility and Sterility · doi:10.1016/j.fertnstert.2007.03.051 · Cited by 139
Hydrocortisone dosing during puberty in patients with classical congenital adrenal hyperplasia: an evidence-based recommendation
Bonfig, W., Dalla Pozza, S. B., Schmidt, H., Pagel, P., Knorr, D., & Schwarz, H. P. (2009) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2009-0942 · Cited by 136
Use of autologous buccal mucosa for vaginoplasty: a study of eight cases
Lin, W.-C. (2003) · Human Reproduction · doi:10.1093/humrep/deg095 · Cited by 131
Dexamethasone treatment of virilizing congenital adrenal hyperplasia: the ability to achieve normal growth
Rivkees, S. A., & Crawford, J. D. (2000) · PEDIATRICS · doi:10.1542/peds.106.4.767 · Cited by 130
Neonatal screening for congenital adrenal hyperplasia
van der Kamp, H., & Wit, J. (2004) · European Journal of Endocrinology · doi:10.1530/eje.0.151u071 · Cited by 128
Approach to the Adult with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency
Merke, D. P. (2008) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2007-2417 · Cited by 128
Self-citation
Holistic management of DSD
Brain, C. E., Creighton, S. M., Mushtaq, I., Carmichael, P. A., Barnicoat, A., Honour, J. W., Larcher, V., & Achermann, J. C. (2010) · Best Practice & Research Clinical Endocrinology & Metabolism · doi:10.1016/j.beem.2010.01.006 · Cited by 126
Testicular Tumors in Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency Show Functional Features of Adrenocortical Tissue
der Grinten, H. L. C., Otten, B. J., Sweep, F. C. G. J., Span, P. N., Ross, H. A., MEULEMAN, E. J. H., & Hermus, A. R. (2007) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2007-0337 · Cited by 123
Approach to the patient: the adult with congenital adrenal hyperplasia
Auchus, R. J., & Arlt, W. (2013) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2013-1440 · Cited by 121
Genotype-phenotype correlation in 153 adult patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency: analysis of the United Kingdom Congenital adrenal Hyperplasia Adult Study Executive (CaHASE) cohort
Krone, N., Rose, I. T., Willis, D. S., Hodson, J., Wild, S. H., Doherty, E. J., Hahner, S., Parajes, S., Stimson, R. H., Han, T. S., et al. (2013) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2012-3343 · Cited by 119
VAGINOPLASTY TECHNIQUE FOR FEMALE PSEUDOHERMAPHRODITES.
Fortunoff, S., Jk, L., & Edson, M. (1964) · PubMed · Cited by 119
Testicular adrenal rest tissue in congenital adrenal hyperplasia: comparison of MR imaging and sonographic findings.
Avila, N. A., Premkumar, A., & Merke, D. P. (1999) · American Journal of Roentgenology · doi:10.2214/ajr.172.4.10587136 · Cited by 109
Self-citation
Long-Term Outcome of Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency
Bachelot, A., Plu-Bureau, G., Thibaud, E., Laborde, K., Pinto, G., Samara, D., Nihoul-Fékété, C., Kuttenn, F., Polak, M., & Touraine, P. (2007) · Hormone Research in Paediatrics · doi:10.1159/000098017 · Cited by 106
Management of the child with congenital adrenal hyperplasia
Hindmarsh, P. C. (2009) · Best Practice & Research Clinical Endocrinology & Metabolism · doi:10.1016/j.beem.2008.10.010 · Cited by 106
The challenge of delivering endocrine care and successful transition to adult services in adolescents with congenital adrenal hyperplasia: experience in a single centre over 18 years
Gleeson, H., Davis, J., Jones, J., O'Shea, E., & Clayton, P. E. (2013) · Clinical Endocrinology · doi:10.1111/cen.12053 · Cited by 103
High frequency of adrenal myelolipomas and testicular adrenal rest tumours in adult Norwegian patients with classical congenital adrenal hyperplasia because of 21-hydroxylase deficiency: Adrenal tumours and TART in 21-OHD
Nermoen, I., Rørvik, J., Holmedal, S. H., Hykkerud, D. L., Fougner, K. J., Svartberg, J., Husebye, E. S., & Løvås, K. (2011) · Clinical Endocrinology · doi:10.1111/j.1365-2265.2011.04151.x · Cited by 103
Long-Term Corticosteroid Replacement and Bone Mineral Density in Adult Women with Classical Congenital Adrenal Hyperplasia
King, J. A., Wisniewski, A. B., Bankowski, B. J., Carson, K. A., Zacur, H. A., & Migeon, C. J. (2006) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2005-0745 · Cited by 101
Early growth, pubertal development, body mass index and final height of patients with congenital adrenal hyperplasia: factors influencing the outcome
Manoli, I., Kanaka-Gantenbein, C., Voutetakis, A., Maniati-Christidi, M., & Dacou‐Voutetakis, C. (2002) · Clinical Endocrinology · doi:10.1046/j.1365-2265.2002.01645.x · Cited by 100
Prevalence and long-term follow-up outcomes of testicular adrenal rest tumours in children and adolescent males with congenital adrenal hyperplasia
Aycan, Z., Bas, V. N., Cetinkaya, S., Yilmaz Agladioglu, S., & Tiryaki, T. (2013) · Clinical Endocrinology · doi:10.1111/cen.12033 · Cited by 98
Repeated successful induction of fertility after replacing hydrocortisone with dexamethasone in a patient with congenital adrenal hyperplasia and testicular adrenal rest tumors
der Grinten, H. L. C., Otten, B. J., Sweep, F. C. G. J., & Hermus, A. R. (2007) · Fertility and Sterility · doi:10.1016/j.fertnstert.2006.11.148 · Cited by 97
Testicular Adrenal Rest Tumours in Congenital Adrenal Hyperplasia
der Grinten, H. L. C., Hermus, A. R. M. M., & Otten, B. J. (2009) · International Journal of Pediatric Endocrinology · doi:10.1155/2009/624823 · Cited by 96
Mutational spectrum of steroid 21-hydroxylase and the genotype-phenotype association in Middle European patients with congenital adrenal hyperplasia
Dolž;an, V, Sólyom, J., Fekete, G., Kovács, J., Rakosnikova, V, Votava, F., Lebl, J., Pribilincova, Z., Baumgartner-Parzer, S., Riedl, S., et al. (2005) · European Journal of Endocrinology · doi:10.1530/eje.1.01944 · Cited by 96
Changes of diurnal rhythm and levels of total and free testosterone secretion from pre to late puberty in boys: testis size of 3 ml is a transition stage to puberty
Ankarberg‐Lindgren, C., & Norjavaara, E. (2004) · European Journal of Endocrinology · doi:10.1530/eje.0.1510747 · Cited by 95
Male infertility due to congenital adrenal hyperplasia: testicular biopsy findings, hormonal evaluation, and therapeutic results in three patients
Bonaccorsi, A. C., Adler, I., & Figueiredo, J. G. (1987) · Fertility and Sterility · doi:10.1016/s0015-0282(16)59119-5 · Cited by 93
Bone mineral density in relation to glucocorticoid substitution therapy in adult patients with 21‐hydroxylase deficiency
Jääskeläinen, J., & Voutilainen, R. (1996) · Clinical Endocrinology · doi:10.1046/j.1365-2265.1996.8620871.x · Cited by 92
Total Adrenal Volume But Not Testicular Adrenal Rest Tumor Volume Is Associated with Hormonal Control in Patients with 21-Hydroxylase Deficiency
Reisch, N., Scherr, M., Flade, L., Bidlingmaier, M., Schwarz, H.-P., Müller-Lisse, U., Reincke, M., Quinkler, M., & Beuschlein, F. (2010) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2009-1929 · Cited by 92
Extraadrenal 21-Hydroxylation by CYP2C19 and CYP3A4: Effect on 21-Hydroxylase Deficiency
Gomes, L. G., Huang, N., Agrawal, V., Mendonça, B. B., Bachega, T. A. S. S., & Miller, W. L. (2009) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2008-1174 · Cited by 91
Psychiatric characterization of children with genetic causes of hyperandrogenism
Mueller, S. C., Ng, P., Sinaii, N., Leschek, E. W., Green-Golan, L., VanRyzin, C., Ernst, M., & Merke, D. P. (2010) · European Journal of Endocrinology · doi:10.1530/eje-10-0693 · Cited by 88
Self-citation
Biopsychological and Cognitive Differences in Children With Premature vs On-Time Adrenarche
Dorn, L. D., Hitt, S., & Rotenstein, D. (1999) · Archives of Pediatrics and Adolescent Medicine · doi:10.1001/archpedi.153.2.137 · Cited by 86
Junction Site Analysis of Chimeric CYP21A1P/CYP21A2 Genes in 21-Hydroxylase Deficiency
Chen, W., Xu, Z., Sullivan, A., Finkielstain, G. P., Van Ryzin, C., Merke, D. P., & McDonnell, N. B. (2012) · Clinical Chemistry · doi:10.1373/clinchem.2011.174037 · Cited by 86
Self-citation
Systematic Review of Transition From Adolescent to Adult Care in Patients With Sickle Cell Disease
Jordan, L. B., Swerdlow, P. S., & Coates, T. D. (2013) · Journal of Pediatric Hematology/Oncology · doi:10.1097/mph.0b013e3182847483 · Cited by 85
Tenascin-X Haploinsufficiency Associated with Ehlers-Danlos Syndrome in Patients with Congenital Adrenal Hyperplasia
Merke, D. P., Chen, W., Morissette, R., Xu, Z., Van Ryzin, C., Sachdev, V., Hannoush, H., Shanbhag, S. M., Acevedo, A. T., Nishitani, M., et al. (2013) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2012-3148 · Cited by 82
Self-citation
Phenotypic profiling of parents with cryptic nonclassic congenital adrenal hyperplasia: findings in 145 unrelated families
Nandagopal, R., Sinaii, N., Avila, N. A., Van Ryzin, C., Chen, W., Finkielstain, G. P., Mehta, S. P., McDonnell, N. B., & Merke, D. P. (2011) · European Journal of Endocrinology · doi:10.1530/eje-11-0019 · Cited by 81
Self-citation
Vaginoplasty technique for female pseudohermaphrodites
&NA; (1964) · Plastic & Reconstructive Surgery · doi:10.1097/00006534-196409000-00031 · Cited by 76
Classic congenital adrenal hyperplasia and puberty
Charmandari, E., Brook, C. G. D., & Hindmarsh, P. C. (2004) · European Journal of Endocrinology · doi:10.1530/eje.0.151u077 · Cited by 75
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Authors: Merke, D. P., & Poppas, D. P.
Affiliations: National Institutes of Health (US)
NewYork–Presbyterian Hospital (US)
Corresponding author: Merke, Deborah P
ORCID: Merke, Deborah P
Venue: The Lancet Diabetes & Endocrinology
Publisher: Elsevier BV
Published: 2013-12
Volume: 1
Issue: 4
Pages: 341-352
ISSN: 2213-8587 (ISSN-L)
DOI: 10.1016/s2213-8587(13)70138-4
PMC: PMC4163910
OpenAlex: W2149437278
Cited by: 134
References: 108
Citations by year: 2026: 6 · 2025: 6 · 2024: 12 · 2023: 11 · 2022: 30 · 2021: 13 · 2020: 6 · 2019: 11 · 2018: 12 · 2017: 11 · 2016: 8 · 2015: 6
FWCI: 0.44
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Locations: The Lancet Diabetes & Endocrinology (publishedVersion)
PubMed (publishedVersion)
Europe PMC (PubMed Central) (acceptedVersion)
PubMed Central (acceptedVersion)
Funders: National Institutes of Health (Z99CL999999)
Topics: Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Sexual Differentiation and Disorders
Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
Health Sciences › Medicine › Reproductive Medicine › Ovarian function and disorders
Keywords: congenital adrenal hyperplasia, adolescents, puberty, androgen deprivation therapy, glucocorticoid therapy, non-classical congenital adrenal hyperplasia, adrenal insufficiency, medication adherence, sexual function, transition to adult care, patient education, urinary incontinence, vaginal stenosis, clitoral pain, personalized treatment
MeSH terms: Adolescent, Adrenal Hyperplasia, Congenital, Glucocorticoids, Humans, Disease Management

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