Skip to main content
SpringerLink
Log in
Book cover

Handbook of Gynecology pp 1–15Cite as

Congenital Adrenal Hyperplasia in the Adolescent

  • Living reference work entry
  • First Online:

  • 166 Accesses

Abstract

Congenital adrenal hyperplasia (CAH) is a primary adrenal insufficiency and an autosomal recessive disorder that ranges in clinical severity from a classical, severe salt-wasting form to a mild, nonclassical form. In all patients, CAH involves adrenal hormone deficiencies and androgen excess. Females with CAH can have hyperandrogenism symptoms. Classical CAH presents in the newborn period with ambiguous genitalia in females and can be detected early in males with newborn screening. Clitoroplasty and vaginoplasty are surgical procedures that can correct ambiguous genitalia in females with CAH. Nonclassical CAH has a broader spectrum of clinical presentations, including premature pubarche, menstrual irregularities, and infertility. Glucocorticoid replacement therapy remains the mainstay of treatment, with the use of mineralocorticoid replacement in some classical patients and antiandrogen therapies in select female patients. Medication dosages, hormone levels, growth, and puberty need to be carefully monitored during childhood and adolescence, and hypercortisolism should be avoided in order to optimize final adult height and reduce potential morbidity. Long-term considerations of cardiovascular disease risk, bone mineral density, and fertility in adults remain under investigation.

This is a preview of subscription content, log in via an institution.

References

  • Amr NH, Ahmed AY, Ibrahim YA. Carotid intima media thickness and other cardiovascular risk factors in children with congenital adrenal hyperplasia. J Endocrinol Investig. 2014;37(10):1001–8.

    Article  CAS  Google Scholar 

  • Arlt W, Willis DS, Wild SH, Krone N, Doherty EJ, Hahner S, et al. Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients. J Clin Endocrinol Metab. 2010;95(11):5110–21.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Auchus RJ, Arlt W. Approach to the patient: the adult with congenital adrenal hyperplasia. J Clin Endocrinol Metab. 2013;98(7):2645–55.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Auchus RJ, Witchel SF, Leight KR, Aisenberg J, Azziz R, Bachega TA, et al. Guidelines for the development of comprehensive care centers for congenital adrenal hyperplasia: guidance from the CARES Foundation initiative. Int J Pediatr Endocrinol. 2010;2010:275213.

    Article  PubMed  Google Scholar 

  • Bonfig W, Schwarz HP. Blood pressure, fludrocortisone dose and plasma renin activity in children with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency followed from birth to 4 years of age. Clin Endocrinol. 2014;81(6):871–5.

    Article  CAS  Google Scholar 

  • Bonfig W, Pozza SB, Schmidt H, Pagel P, Knorr D, Schwarz HP. Hydrocortisone dosing during puberty in patients with classical congenital adrenal hyperplasia: an evidence-based recommendation. J Clin Endocrinol Metab. 2009;94(10):3882–8.

    Article  CAS  PubMed  Google Scholar 

  • Casteras A, De Silva P, Rumsby G, Conway GS. Reassessing fecundity in women with classical congenital adrenal hyperplasia (CAH): normal pregnancy rate but reduced fertility rate. Clin Endocrinol. 2009;70(6):833–7.

    Article  Google Scholar 

  • Chakhtoura Z, Bachelot A, Samara-Boustani D, Ruiz JC, Donadille B, Dulon J, et al. Impact of total cumulative glucocorticoid dose on bone mineral density in patients with 21-hydroxylase deficiency. Eur J Endocrinol. 2008;158(6):879–87.

    Article  CAS  PubMed  Google Scholar 

  • Charmandari E, Eisenhofer G, Mehlinger SL, Carlson A, Wesley R, Keil MF, et al. Adrenomedullary function may predict phenotype and genotype in classic 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2002a;87(7):3031–7.

    Article  CAS  PubMed  Google Scholar 

  • Charmandari E, Weise M, Bornstein SR, Eisenhofer G, Keil MF, Chrousos GP, et al. Children with classic congenital adrenal hyperplasia have elevated serum leptin concentrations and insulin resistance: potential clinical implications. J Clin Endocrinol Metab. 2002b;87(5):2114–20.

    Article  CAS  PubMed  Google Scholar 

  • Claahsen-van der Grinten HL, Otten BJ, Stikkelbroeck MM, Sweep FC, Hermus AR. Testicular adrenal rest tumours in congenital adrenal hyperplasia. Best Pract Res Clin Endocrinol Metab. 2009;23(2):209–20.

    Article  CAS  PubMed  Google Scholar 

  • Cooley WC, Sagerman PJ. Supporting the health care transition from adolescence to adulthood in the medical home. Pediatrics. 2011;128(1):182–200.

    Article  PubMed  Google Scholar 

  • Cornean RE, Hindmarsh PC, Brook CG. Obesity in 21-hydroxylase deficient patients. Arch Dis Child. 1998;78(3):261–3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Crocker MK, Barak S, Millo CM, Beall SA, Niyyati M, Chang R, et al. Use of PET/CT with cosyntropin stimulation to identify and localize adrenal rest tissue following adrenalectomy in a woman with congenital adrenal hyperplasia. J Clin Endocrinol Metab. 2012;97(11):E2084–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Escobar-Morreale HF, Sanchon R, San Millan JL. A prospective study of the prevalence of nonclassical congenital adrenal hyperplasia among women presenting with hyperandrogenic symptoms and signs. J Clin Endocrinol Metab. 2008;93(2):527–33.

    Article  CAS  PubMed  Google Scholar 

  • Falhammar H, Filipsson H, Holmdahl G, Janson PO, Nordenskjold A, Hagenfeldt K, et al. Fractures and bone mineral density in adult women with 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2007;92(12):4643–9.

    Article  CAS  PubMed  Google Scholar 

  • Falhammar H, Frisen L, Hirschberg AL, Norrby C, Almqvist C, Nordenskjold A, et al. Increased cardiovascular and metabolic morbidity in patients with 21-hydroxylase deficiency: a swedish population-based national cohort study. J Clin Endocrinol Metab. 2015;100(9):3520–8.

    Article  CAS  PubMed  Google Scholar 

  • Fanta M, Cibula D, Vrbikova J. Prevalence of nonclassic adrenal hyperplasia (NCAH) in hyperandrogenic women. Gynecol Endocrinol. 2008;24(3):154–7.

    Article  CAS  PubMed  Google Scholar 

  • Feldman S, Billaud L, Thalabard JC, Raux-Demay MC, Mowszowicz I, Kuttenn F, et al. Fertility in women with late-onset adrenal hyperplasia due to 21-hydroxylase deficiency. J Clin Endocrinol Metab. 1992;74(3):635–9.

    CAS  PubMed  Google Scholar 

  • Finkielstain GP, Kim MS, Sinaii N, Nishitani M, Van Ryzin C, Hill SC, et al. Clinical characteristics of a cohort of 244 patients with congenital adrenal hyperplasia. J Clin Endocrinol Metab. 2012;97(12):4429–38.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • German A, Suraiya S, Tenenbaum-Rakover Y, Koren I, Pillar G, Hochberg Z. Control of childhood congenital adrenal hyperplasia and sleep activity and quality with morning or evening glucocorticoid therapy. J Clin Endocrinol Metab. 2008;93(12):4707–10.

    Article  CAS  PubMed  Google Scholar 

  • Hagenfeldt K, Janson PO, Holmdahl G, Falhammar H, Filipsson H, Frisen L, et al. Fertility and pregnancy outcome in women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Hum Reprod. 2008;23(7):1607–13.

    Article  CAS  PubMed  Google Scholar 

  • Han TS, Conway GS, Willis DS, Krone N, Rees DA, Stimson RH, et al. Relationship between final height and health outcomes in adults with congenital adrenal hyperplasia: United Kingdom congenital adrenal hyperplasia adult study executive (CaHASE). J Clin Endocrinol Metab. 2014;99(8):E1547–55.

    Article  CAS  PubMed  Google Scholar 

  • Harrington J, Pena AS, Gent R, Hirte C, Couper J. Adolescents with congenital adrenal hyperplasia because of 21-hydroxylase deficiency have vascular dysfunction. Clin Endocrinol. 2012;76(6):837–42.

    Article  CAS  Google Scholar 

  • Helleday J, Siwers B, Ritzen EM, Carlstrom K. Subnormal androgen and elevated progesterone levels in women treated for congenital virilizing 21-hydroxylase deficiency. J Clin Endocrinol Metab. 1993;76(4):933–6.

    CAS  PubMed  Google Scholar 

  • Hero M, Janne OA, Nanto-Salonen K, Dunkel L, Raivio T. Circulating antiandrogenic activity in children with congenital adrenal hyperplasia during peroral flutamide treatment. J Clin Endocrinol Metab. 2005;90(9):5141–5.

    Article  CAS  PubMed  Google Scholar 

  • Hofbauer LC, Gori F, Riggs BL, Lacey DL, Dunstan CR, Spelsberg TC, et al. Stimulation of osteoprotegerin ligand and inhibition of osteoprotegerin production by glucocorticoids in human osteoblastic lineage cells: potential paracrine mechanisms of glucocorticoid-induced osteoporosis. Endocrinology. 1999;140(10):4382–9.

    CAS  PubMed  Google Scholar 

  • Hughes IA, Houk C, Ahmed SF, Lee PA. Consensus statement on management of intersex disorders. J Pediatr Urol. 2006;2(3):148–62.

    Article  CAS  PubMed  Google Scholar 

  • Isguven P, Arslanoglu I, Mesutoglu N, Yildiz M, Erguven M. Bioelectrical impedance analysis of body fatness in childhood congenital adrenal hyperplasia and its metabolic correlates. Eur J Pediatr. 2008;167(11):1263–8.

    Article  CAS  PubMed  Google Scholar 

  • Kashimada K, Ono M, Onishi T, Koyama S, Toyoura T, Imai K, et al. Clinical course of patients with nonclassical 21-hydroxylase deficiency (21-OHD) diagnosed in infancy and childhood. Endocr J. 2008;55(2):397–404.

    Article  CAS  PubMed  Google Scholar 

  • Kim MS, Donohoue PA. Adrenal disorders. In: Kappy MS, Allen DB, Geffner ME, editors. Pediatric practice endocrinology. 2nd ed. New York, Chicago, San Francisco: McGraw-Hill Education; 2014.

    Google Scholar 

  • Kim MS, Merke DP. Cardiovascular disease risk in adult women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Semin Reprod Med. 2009;27(4):316–21.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Kim MS, Ryabets-Lienhard A, Geffner ME. Management of congenital adrenal hyperplasia in childhood. Curr Opin Endocrinol Diabetes Obes. 2012;19(6):483–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Kim MS, Ryabets-Lienhard A, Bali B, Lane CJ, Park AH, Hall S, et al. Decreased adrenomedullary function in infants with classical congenital adrenal hyperplasia. J Clin Endocrinol Metab. 2014;99(8):E1597–601.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Kim MS, Ryabets-Lienhard A, Dao-Tran A, Mittelman SD, Gilsanz V, Schrager SM, et al. Increased abdominal adiposity in adolescents and young adults with classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2015;100(8):E1153–9.

    Article  PubMed  PubMed Central  Google Scholar 

  • Kim MS, Dao-Tran A, Davidowitz E, Tseng T, Gilsanz V, Ryabets-Lienhard A, et al. Carotid intima-media thickness is associated with increased androgens in adolescents and young adults with classical congenital adrenal hyperplasia. Horm Res Paediatr 2016;85:221–31.

    Google Scholar 

  • King P, Paul A, Laufer E. Shh signaling regulates adrenocortical development and identifies progenitors of steroidogenic lineages. Proc Natl Acad Sci USA. 2009;106(50):21185–90.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Koetz KR, Ventz M, Diederich S, Quinkler M. Bone mineral density is not significantly reduced in adult patients on low-dose glucocorticoid replacement therapy. J Clin Endocrinol Metab. 2012;97(1):85–92.

    Article  CAS  PubMed  Google Scholar 

  • Kuiri-Hanninen T, Sankilampi U, Dunkel L. Activation of the hypothalamic-pituitary-gonadal axis in infancy: minipuberty. Horm Res Paediatr. 2014;82(2):73–80.

    Article  PubMed  Google Scholar 

  • Lee PA, Witchel SF. Genital surgery among females with congenital adrenal hyperplasia: changes over the past five decades. J Pediatr Endocrinol Metab. 2002;15(9):1473–7.

    Article  PubMed  Google Scholar 

  • Lin-Su K, New MI. Effects of adrenal steroids on the bone metabolism of children with congenital adrenal hyperplasia. Ann N Y Acad Sci. 2007;1117:345–51.

    Article  CAS  PubMed  Google Scholar 

  • Lin-Su K, Harbison MD, Lekarev O, Vogiatzi MG, New MI. Final adult height in children with congenital adrenal hyperplasia treated with growth hormone. J Clin Endocrinol Metab. 2011;96(6):1710–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Looker AC, Johnson CL, Lacher DA, Pfeiffer CM, Schleicher RL, Sempos CT. Vitamin D status: United States, 2001–2006. NCHS Data Brief. 2011;59:1–8.

    Google Scholar 

  • Matthews D, Cheetham T. What is the best approach to the teenage patient presenting with nonclassical congenital adrenal hyperplasia: should we always treat with glucocorticoids? Clin Endocrinol. 2013;78(3):338–41.

    Article  CAS  Google Scholar 

  • Mendis S, Puska P, Norrving B. Global Atlas on cardiovascular disease prevention and control. Geneva: World Health Organization; 2011.

    Google Scholar 

  • Merke DP, Bornstein SR. Congenital adrenal hyperplasia. Lancet. 2005;365(9477):2125–36.

    Article  PubMed  Google Scholar 

  • Merke DP, Poppas DP. Management of adolescents with congenital adrenal hyperplasia. Lancet Diabet Endocrinol. 2013;1(4):341–52.

    Article  Google Scholar 

  • Merke DP, Keil MF, Jones JV, Fields J, Hill S, Cutler Jr GB. Flutamide, testolactone, and reduced hydrocortisone dose maintain normal growth velocity and bone maturation despite elevated androgen levels in children with congenital adrenal hyperplasia. J Clin Endocrinol Metab. 2000;85(3):1114–20.

    Article  CAS  PubMed  Google Scholar 

  • Meyer ML, Malek AM, Wild RA, Korytkowski MT, Talbott EO. Carotid artery intima-media thickness in polycystic ovary syndrome: a systematic review and meta-analysis. Hum Reprod Update. 2012;18(2):112–26.

    Article  PubMed  Google Scholar 

  • Mooij CF, Kroese JM, Sweep FC, Hermus AR, Tack CJ. Adult patients with congenital adrenal hyperplasia have elevated blood pressure but otherwise a normal cardiovascular risk profile. PLoS One. 2011;6(9):e24204.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Mouritsen A, Jorgensen N, Main KM, Schwartz M, Juul A. Testicular adrenal rest tumours in boys, adolescents and adult men with congenital adrenal hyperplasia may be associated with the CYP21A2 mutation. Int J Androl. 2010;33(3):521–7.

    Article  CAS  PubMed  Google Scholar 

  • Mullis PE, Hindmarsh PC, Brook CG. Sodium chloride supplement at diagnosis and during infancy in children with salt-losing 21-hydroxylase deficiency. Eur J Pediatr. 1990;150(1):22–5.

    Article  CAS  PubMed  Google Scholar 

  • Nebesio TD, Eugster EA. Observation of hypertension in children with 21-hydroxylase deficiency: a preliminary report. Endocrine. 2006;30(3):279–82.

    Article  CAS  PubMed  Google Scholar 

  • New MI. Extensive clinical experience: nonclassical 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2006;91(11):4205–14.

    Article  CAS  PubMed  Google Scholar 

  • New M, Lekarev O, Lin-Su K, Parsa A, Khattab A, Pina C, et al. Congenital adrenal hyperplasia. In: De Groot LJ, Beck-Peccoz P, Chrousos G, Dungan K, Grossman A, Hershman JM, et al., editors. Endotext. South Dartmouth, MDText.com, Inc. 2000.

    Google Scholar 

  • New MI, Abraham M, Gonzalez B, Dumic M, Razzaghy-Azar M, Chitayat D, et al. Genotype-phenotype correlation in 1,507 families with congenital adrenal hyperplasia owing to 21-hydroxylase deficiency. Proc Natl Acad Sci USA. 2013;110(7):2611–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Pall M, Azziz R, Beires J, Pignatelli D. The phenotype of hirsute women: a comparison of polycystic ovary syndrome and 21-hydroxylase-deficient nonclassic adrenal hyperplasia. Fertil Steril. 2010;94(2):684–9.

    Article  CAS  PubMed  Google Scholar 

  • Pang S, Murphey W, Levine LS, Spence DA, Leon A, LaFranchi S, et al. A pilot newborn screening for congenital adrenal hyperplasia in Alaska. J Clin Endocrinol Metab. 1982;55(3):413–20.

    Article  CAS  PubMed  Google Scholar 

  • Poppas DP, Hochsztein AA, Baergen RN, Loyd E, Chen J, Felsen D. Nerve sparing ventral clitoroplasty preserves dorsal nerves in congenital adrenal hyperplasia. J Urol. 2007;178(4 Pt 2):1802–6.. discussion 6

    Article  PubMed  Google Scholar 

  • Prader A, Gurtner HP. The syndrome of male pseudohermaphrodism in congenital adrenocortical hyperplasia without overproduction of androgens (adrenal male pseudohermaphrodism). Helv Paediatr Acta. 1955;10(4):397–412.

    CAS  PubMed  Google Scholar 

  • Premawardhana LD, Hughes IA, Read GF, Scanlon MF. Longer term outcome in females with congenital adrenal hyperplasia (CAH): the Cardiff experience. Clin Endocrinol. 1997;46(3):327–32.

    Article  CAS  Google Scholar 

  • Quintos JB, Vogiatzi MG, Harbison MD, New MI. Growth hormone therapy alone or in combination with gonadotropin-releasing hormone analog therapy to improve the height deficit in children with congenital adrenal hyperplasia. J Clin Endocrinol Metab. 2001;86(4):1511–7.

    CAS  PubMed  Google Scholar 

  • Richards GE, Grumbach MM, Kaplan SL, Conte FA. The effect of long acting glucocorticoids on menstrual abnormalities in patients with virilizing congenital adrenal hyperplasia. J Clin Endocrinol Metab. 1978;47(6):1208–15.

    Article  CAS  PubMed  Google Scholar 

  • Rink RC, Metcalfe PD, Kaefer MA, Casale AJ, Meldrum KK, Cain MP. Partial urogenital mobilization: a limited proximal dissection. J Pediatr Urol. 2006;2(4):351–6.

    Article  CAS  PubMed  Google Scholar 

  • Rosenfield RL, Barnes RB, Ehrmann DA. Studies of the nature of 17-hydroxyprogesterone hyperresponsiveness to gonadotropin-releasing hormone agonist challenge in functional ovarian hyperandrogenism. J Clin Endocrinol Metab. 1994;79(6):1686–92.

    CAS  PubMed  Google Scholar 

  • Rosenfield RL, Ehrmann DA, Littlejohn EE. Adolescent polycystic ovary syndrome due to functional ovarian hyperandrogenism persists into adulthood. J Clin Endocrinol Metab. 2015;100(4):1537–43.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Sartorato P, Zulian E, Benedini S, Mariniello B, Schiavi F, Bilora F, et al. Cardiovascular risk factors and ultrasound evaluation of intima-media thickness at common carotids, carotid bulbs, and femoral and abdominal aorta arteries in patients with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2007;92(3):1015–8.

    Article  CAS  PubMed  Google Scholar 

  • Schaeffer TL, Tryggestad JB, Mallappa A, Hanna AE, Krishnan S, Chernausek SD, et al. An evidence-based model of multidisciplinary care for patients and families affected by classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Int J Pediatr Endocrinol. 2010;2010:692439.

    Article  PubMed  PubMed Central  Google Scholar 

  • Sciannamblo M, Russo G, Cuccato D, Chiumello G, Mora S. Reduced bone mineral density and increased bone metabolism rate in young adult patients with 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2006;91(11):4453–8.

    Article  CAS  PubMed  Google Scholar 

  • Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP, et al. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2010;95(9):4133–60.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Stikkelbroeck NM, Oyen WJ, van der Wilt GJ, Hermus AR, Otten BJ. Normal bone mineral density and lean body mass, but increased fat mass, in young adult patients with congenital adrenal hyperplasia. J Clin Endocrinol Metab. 2003;88(3):1036–42.

    Article  CAS  PubMed  Google Scholar 

  • Subbarayan A, Dattani MT, Peters CJ, Hindmarsh PC. Cardiovascular risk factors in children and adolescents with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Clin Endocrinol. 2014;80(4):471–7.

    Article  CAS  Google Scholar 

  • Thomas TT, Ruscher KR, Mandavilli S, Balarezo F, Finck CM. Ovarian steroid cell tumor, not otherwise specified, associated with congenital adrenal hyperplasia: rare tumors of an endocrine disease. J Pediatr Surg. 2013;48(6):E23–7.

    Article  PubMed  Google Scholar 

  • Tiosano D, Vlodavsky E, Filmar S, Weiner Z, Goldsher D, Bar-Shalom R. Ovarian adrenal rest tumor in a congenital adrenal hyperplasia patient with adrenocorticotropin hypersecretion following adrenalectomy. Horm Res Paediatr. 2010;74(3):223–8.

    Article  CAS  PubMed  Google Scholar 

  • Volkl TM, Simm D, Beier C, Dorr HG. Obesity among children and adolescents with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Pediatrics. 2006;117(1):e98–105.

    Article  PubMed  Google Scholar 

  • Weldon VV, Kowarski A, Migeon CJ. Aldosterone secretion rates in normal subjects from infancy to adulthood. Pediatrics. 1967;39(5):713–23.

    CAS  PubMed  Google Scholar 

  • Williams RM, Deeb A, Ong KK, Bich W, Murgatroyd PR, Hughes IA, et al. Insulin sensitivity and body composition in children with classical and nonclassical congenital adrenal hyperplasia. Clin Endocrinol. 2010;72(2):155–60.

    Article  CAS  Google Scholar 

  • Witchel SF. Non-classic congenital adrenal hyperplasia. Steroids. 2013;78(8):747–50.

    Article  CAS  PubMed  Google Scholar 

  • Witchel SF, Azziz R. Nonclassic congenital adrenal hyperplasia. Int J Pediatr Endocrinol. 2010;2010:625105.

    Article  PubMed  PubMed Central  Google Scholar 

  • Woelfle J, Hoepffner W, Sippell WG, Bramswig JH, Heidemann P, Deiss D, et al. Complete virilization in congenital adrenal hyperplasia: clinical course, medical management and disease-related complications. Clin Endocrinol. 2002;56(2):231–8.

    Article  CAS  Google Scholar 

  • Yang J, Felsen D, Poppas DP. Nerve sparing ventral clitoroplasty: analysis of clitoral sensitivity and viability. J Urol. 2007;178(4 Pt 2):1598–601.

    Article  PubMed  Google Scholar 

  • Zaarour MG, Atallah DM, Trak-Smayra VE, Halaby GH. Bilateral ovary adrenal rest tumor in a congenital adrenal hyperplasia following adrenalectomy. Endocr Pract. 2014;20:e69–74.

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

We thank Paul Kokorowski, MD, MPH, for his review of the Surgical Considerations section.

Author information

Authors and Affiliations

  1. Center for Endocrinology, Diabetes and Metabolism, Children’s Hospital Los Angeles, University of Southern California, Los Angeles, CA, USA

    Mimi S. Kim, Teresa Tseng, Christina M. Koppin & Mitchell E. Geffner

Authors
  1. Mimi S. Kim
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Teresa Tseng
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Christina M. Koppin
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Mitchell E. Geffner
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mimi S. Kim .

Editor information

Editors and Affiliations

  1. Department of Obstetrics and Gynecology and Family Medicine, University of Southern California Keck School of Medicine, Los Angeles, California, USA

    Donna Shoupe

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer International Publishing AG

About this entry

Cite this entry

Kim, M.S., Tseng, T., Koppin, C.M., Geffner, M.E. (2016). Congenital Adrenal Hyperplasia in the Adolescent. In: Shoupe, D. (eds) Handbook of Gynecology. Springer, Cham. https://doi.org/10.1007/978-3-319-17002-2_59-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-17002-2_59-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Cham

  • Online ISBN: 978-3-319-17002-2

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation