Clinical practice guidelines "Congenital adrenal hyperplasia in children" with comments

The clinical practice guidelines for congenital adrenal hyperplasia (CAH) in children clearly and systematically present the key information on the epidemiology, etiology, pathogenesis, and diagnostic methods for all forms of the disease. Protocols for glucocorticoid and mineralocorticoid replacement therapy, as well as follow-up regimens for patients in different age groups, are provided. These clinical guidelines were developed and approved by the expert community of pediatric endocrinologists from the Russian Association of Endocrinologists and endorsed by the council of the Russian Federation Ministry of Health. The guidelines are based on systematic reviews, meta-analyses, original articles, and scientific studies on this pathology conducted in the Russian Federation and other countries. This article provides updated author comments and clarifications on the most critical aspects of the diagnosis and treatment of congenital adrenal hyperplasia in childhood.

1. Hannah-Shmouni F, Morissette R, Sinaii N, Elman M, Prezant TR, Chen V, Pulver A, Merke DP. Revisiting the prevalence of nonclassic congenital adrenal hyperplasia in US Ashkenazi Jews and Caucasians. Genetics in Medicine. 2017;19(11):1276–1279.

2. Melcescu E., Phillips J, Moll G, Subauste JS. 11Beta-hydroxylase deficiency and other syndromes of mineralocorticoid excess as a rare cause of endocrine hypertension. Hormone and Metabolic Research. 2012;44(12):867–878.

3. Concolino P, Costella A. Congenital Adrenal Hyperplasia (CAH) due to 21-Hydroxylase Deficiency: A Comprehensive Focus on 233 Pathogenic Variants of CYP21A2 Gene. Molecular Diagnosis & Therapy. 2018;22(3):261–280.

4. El-Maouche D, Arlt W, Merke DP. Congenital adrenal hyperplasia. Lancet. 2017;390(10108): 2194–2210.

5. Falhammar H, Nordenström A. Nonclassic congenital adrenal hyperplasia due to 21-hydroxylase deficiency: clinical presentation, diagnosis, treatment, and outcome. Endocrine. 2015;50(1):32–50.

6. Flück CE, Miller WL. P450 oxidoreductase deficiency: a new form of congenital adrenal hyperplasia. Current Opinion in Pediatrics. 2006;18(4):435–441.

7. Miller WL. The syndrome of 17,20 lyase deficiency. The Journal of Clinical Endocrinology and Metabolism. 2012;97(1):59–67.

8. Yanase T., Imai T, Simpson ER, Waterman MR. Molecular basis of 17α-hydroxylase/17,20-lyase deficiency. The Journal of Steroid Biochemistry and Molecular Biology. 1992;43(8):973–979.

9. Trapp CM., Oberfield SE. Recommendations for treatment of nonclassic congenital adrenal hyperplasia (NCCAH): an update. Steroids. 2012;77(4):342–346

10. White PC. Update on diagnosis and management of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Current Opinion in Endocrinology, Diabetes, and Obesity. 2018;25(3):178–184.

11. Pang SY, Wallace MA, Hofman L, Thuline HC, Dorche C, Lyon IC, Dobbins RH, Kling S, Fujieda K, Suwa S. Worldwide experience in newborn screening for classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Pediatrics. 1988;81(6):866–874.

12. Kareva M. A. Vrozhdennaya disfunkciya kory nadpochechnikov u detej: ehpidemiologiya, geneticheskaya osnova, personalizirovannyj podkhod k diagnostike i lecheniyu, monitoring somaticheskogo i reproduktivnogo zdorov'ya. [dissertation] Moscow; 2019.(in Russ.).

13. Ionova T.A., Tiul'pakov A.N., Kalinenkova S.G. The prevalence of the non-classical form of congenital adrenal hyperplasia as exemplified (by the population of the Moscow region). Problems of Endocrinology. 2013;59(4):18-22. (In Russ.) doi: https://doi.org/10.14341/probl201359418-22

14. Tebieva I.S., Calinchenko N.Y., Getoeva Z.K., Tiulpakov A.N. Genetical aspects of congenital adrenal hyperplasia in the Republic of North Ossetia Alania. Medical Genetics. 2019;18(6):11-20. (InRuss.) doi: https://doi.org/10.25557/2073-7998.2019.06.11-20

15. Armengaud J-B, Charkaluk M-L, Trivin C, Tardy V, Breart G, Brauner R, Chaumelau M. Precocious Pubarche: Distinguishing Late-Onset Congenital Adrenal Hyperplasia from Premature Adrenarche. The Journal of Clinical Endocrinology & Metabolism. 2009;94(8):2835–2840.

16. Speiser PW, White PC. Congenital adrenal hyperplasia. The New England Journal of Medicine. 2003;349(8):776–788.

17. Walia R, Singla M, Vaiphei K, Kumar S, Bhansali A. Disorders of sex development: a study of 194 cases. Endocrine Connections. 2018;7(2):364–371.

18. Mieszczak J., Houk C.P., Lee P.A. Assignment of the sex of rearing in the neonate with a disorder of sex development. Current Opinion Pediatrics. 2009:21(4):541–547.

19. Raza J, Zaidi SZ, Warne GL. Management of disorders of sex development With a focus on development of the child and adolescent through the pubertal years. Best Practice&Research: Clinical Endocrinology&Metabolism. 2019;33(3):101297.

20. Speiser PW, Arlt W, Auchus RJ, Baskin LS et al. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. The Journal of Clinical Endocrinology and Metabolism. 2010;95(9):4133–4160.

21. Kurtoğlu S, Hatipoğlu N. Non-Classical Congenital Adrenal Hyperplasia in Childhood. Journal of Clinical Research in Pediatric Endocrinology. 2017;9(1):1–7.

22. Carmina E, Dewailly D, Escobar-Morreale HF, Kelestimur F, Moran C, Oberfield Sh, Witchel SF, Azziz R. Non-classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency revisited: an update with a special focus on adolescent and adult women. Human Reproduction Update. 2017;23(5):580–599.

23. Whitehead FJ, Couper RT, Moore L, Bourne AJ, Byard RW. Dehydration deaths in infants and young children. The American Journal of Forensic Medicine and Pathology. 1996;17(1):73–78.

24. Turcu AF, Auchus RJ. Adrenal steroidogenesis and congenital adrenal hyperplasia. Endocrinology and Metabolism Clinics of North America. 2015;44(2):275–296.

25. Gau M, Konishi K, Takasawa T, Nakagawa R, Tsuji-Hosokawa A. The progression of salt-wasting and the body weight change during the first 2 weeks of life in classical 21-hydroxylase deficiency patients. Clinical Endocrinology. 2021;94(2):229–236.

26. Thil’en A, Nordenström, Hagenfeldt L, von Döbeln U, Guthenberg C, Larsson A. Benefits of neonatal screening for congenital adrenal hyperplasia (21-hydroxylase deficiency) in Sweden. Pediatrics. 1998;101(4):E11.

27. Brosnan PG, Brosnan CA, Kemp SF, Domek DB, Jelley DH, Blackett PR, Riley WJ. Effect of newborn screening for congenital adrenal hyperplasia. Archives of Pediatrics&Adolescent Medicine. 1999;153(12):1272–1278.

28. Van der Kamp HJ, Noordam K, Elvers B, Van Baarle M, Otten BJ, Verkerk PH. Newborn screening for congenital adrenal hyperplasia in the Netherlands. Pediatrics. 2001;108(6):1320–1324.

29. Hird BE, Tetlow L, Toby S. No evidence of an increase in early infant mortality from congenital adrenal hyperplasia in the absence of screening. Archives of Disease in Childhood. 2014; 99(2):158–164.

30. Van der Kamp HJ, Oudshoorn CGM, Elvers BH, Van Baarle M, Otten BJ, Wit JM, Verkerk PH. Cutoff levels of 17-alpha-hydroxyprogesterone in neonatal screening for congenital adrenal hyperplasia should be based on gestational age rather than on birth weight. Journal of Clinical Endocrinology and Metabolism. 2005;90(7):3904–3907.

31. Dedov II, Peterkova VA. Detskaya endokrinologiya. Moscow: UP-Print; 2006. (in Russ.)

32. Rauh M, Gröschl M, Rascher W, Dörr HG. Automated, fast and sensitive quantification of 17 alpha-hydroxy-progesterone, androstenedione and testosterone by tandem mass spectrometry with on-line extraction. Steroids. 2006;71(6):450–458.

33. Lacey JM, Minutti CZ, Magera MJ, Tauscher AL, Casetta B, McCann M, Lymp L, Hahn SH, Rinaldo P, Matern D. Improved specificity of newborn screening for congenital adrenal hyperplasia by second-tier steroid profiling using tandem mass spectrometry. Clinical Chemistry. 2004;50(3):621–625.

34. Matern D, Tortorelli S, Oglesbee D, Gavrilov D, Rinaldo P. Reduction of the false-positive rate in newborn screening by implementation of MS/MS-based second-tier tests: the Mayo Clinic experience (2004-2007). Journal of Inherited Metabolic Disease. 2007;30(4):585–592.

35. Minutti CZ, Lasey JM, Magera MJ, Hahn SH, McCann M, Schulze A, Cheillan D, Dorche C, Chace DH, Lymp JF, Zimmerman D, Rinaldo P, Matern D. Steroid profiling by tandem mass spectrometry improves the positive predictive value of newborn screening for congenital adrenal hyperplasia. Journal of Clinical Endocrinology and Metabolism. 2004;89(8):3687–3693.

36. Janzen N, Peter M, Sander S, Steuerwald U, Terhardt M, Holtkamp U, Sander J. Newborn screening for congenital adrenal hyperplasia: additional steroid profile using liquid chromatography-tandem mass spectrometry. Journal of Clinical Endocrinology and Metabolism. 2007;92(7):2581–2589.

37. Janzen N, Riepe FG, Peter M, Sander S, Steuerwald U, Korsch E, Krull F, Muller HL, Heger S, Brack Ch, Sander J. Neonatal screening: identification of children with 11β-hydroxylase deficiency by secondtier testing. Hormone Research in Paediatrics. 2012;77(3):195–199.

38. Svinarev MYu, Andreeva LP, Arakovich VV, et al. Neonatal’nyi skrining na VDKN v Saratovskoi oblasti // Sbornik tezisov Vseros. konf. pediatrov endokrinologov «Dostizheniya endokrinologii — zdorov’yu detei». Moscow; 2011:19-20(In Russ.)

39. Kareva MA, Peterkova VA. Aktual'nye voprosy neonatal'nogo skrininga na vrozhdennuyu disfunkciyu kory nadpochechnikov // Sbornik tezisov Vseros. konf. pediatrov ehndokrinologov «Dostizheniya ehndokrinologii -zdorov'yu detej». Moscow; 2011:4-6 (In Russ.)

40. Therrell BL, Berenbaum SA, Manter-Kapanke V, Simmank J, Korman K, Prentice L, Gonzalez J, Gunn S. Results of screening 1.9 million Texas newborns for 21-hydroxylase-deficient congenital adrenal hyperplasia. Pediatrics. 1998;101(4):583–590.

41. Khramova EB. Epidemiologiya, skrining, diagnostika vrozhdennoi disfunktsii kory nadpochechnikov v Zapadno-Sibirskom regione [dissertation]. Tyumen’; 2013. (In Russ.)

42. Frisch H, Battelino T., Schober E, Baumgartner-Parzer S, Nowotny P, Vierhapper H. Saltwasting in simple virilizing congenital adrenal hyperplasia. Journal of Pediatric Endocrinology&Metabolism. 2001;14(9):1649–1655.

43. Dedov II, Kalinchenko NY, Semicheva TV, Kuznetsova ES, Bakanova TD, Sverdlova PS, et al. Molekulyarnyy analiz gena CYP21 u patsientov s vrozhdennoy disfunktsiey kory nadpochechnikov, obuslovlennoy defi tsitom 21-gidroksilazy. Problemy endokrinologii 2004;50(2):3-7. (In Russ.].

44. Hannah-Shmouni F, Chen W, Merke DP. Genetics of Congenital Adrenal Hyperplasia. Best Practice and Research in Clinical Endocrinology and Metabolism. 2009;23(2):181–192.

45. Bidet M, Bellane-Chantelot C, Galand-Portier B, Tardy V, Billaud L, Laborde K, Coussieu C, Morel Y et. al. 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. The Journal of Clinical Endocrinology and Metabolism. 2009;94(5):1570–1578.

46. Bulsari K, Falhammar H. Clinical perspectives in congenital adrenal hyperplasia due to 11β-hydroxylase deficiency. Endocrine. 2017;55(1):19–36.

47. Ray JA, Kushnir MM, Yost RA, Rockwood AL, Meikle AW. Performance enhancement in the measurement of 5 endogenous steroids by LC-MS/MS combined with differential ion mobility spectrometry. Clinica Chimica Acta. 2015;438:330–336.

48. Nimkarn S, Lin-Su K, Berglind N, Wilson RC, New MI. Aldosteroneto-renin ratio as a marker for disease severity in 21-hydroxylase deficiency congenital adrenal hyperplasia. The Journal of Clinical Endocrinology and Metabolism. 2007;92(1):137–142.

49. Bonfig W, Roehl F, Riedl S, Brämswig J, Richter-Unruh A, Fricke-Otto S, Hübner A, Bettendorf M et al. Sodium Chloride Supplementation Is Not Routinely Performed in the Majority of German and Austrian Infants with Classic Salt-Wasting Congenital Adrenal Hyperplasia and Has No Effect on Linear Growth and Hydrocortisone or Fludrocortisone Dose. Hormone Researsh in Paediatrics. 2018;89(1):7–12.

50. Balsamo A, Baronio F, Ortolano R, Menabo S, Baldazzi L, Natale VD, Vissani S, Cassio A. Congenital Adrenal Hyperplasias Presenting in the Newborn and Young Infant. Frontiers in Pediatrics. 2020;8:593315.

51. Kalinchenko N. YU. Klinicheskaya, gormonal'naya i molekulyarnaya kharakteristika razlichnykh form vrozhdennoj disfunkcii kory nadpochechnikov. [dissertation] Moscow; 2005. (In Russ.).

52. Zhukovskij MA, Buraya TI, Kuznecova ES. Vrozhdennye disfunkcii kory nadpochechnikov u detej. Moscow: Medicina; 1977.(In Russ.)

53. Pinto E.M., Zambetti G.P., Rodriguez-Galindo C. Pediatric adrenocortical tumours. Best Practice&Research. Clinical Endocrinology & Metabolism. 2020;34(3)101448.

54. Sangüesa C, Veiga D, Llavador M, Serrano A. Testicular tumours in children: an approach to diagnosis and management with pathologic correlation. Insights into Imaging. 2020;11(1):74.

55. Shah RU, Lawrence C, Fickenscher KA, Shao L, Lowe LH. Imaging of Pediatric Pelvic Neoplasms. Radiologic Clinics of North America. 2011;49(4):729–748.

56. Kopylova I. V. Sostoyanie ehstrogenchuvstvitel'nykh tkanej u devochek s klassicheskimi formami vrozhdennoj disfunkcii kory nadpochechnikov. [dissertation] Moscow; 2015. (In Russ.).

57. Abdelhamed MH, Al-Ghamdi WM, Al-Agha AE. Polycystic Ovary Syndrome Among Female Adolescents With Congenital Adrenal Hyperplasia. Cureus. 2021;13(12):e20698. doi: 10.7759/cureus.20698

58. Okulov A.B., Negmadzhanov B.B. Khirurgicheskie bolezni reproduktivnoj sistemy i sekstransformacionnye operacii: Rukovodstvo dlya vrachej. Moscow: Medicine; 2000. (in Russ.)

59. Yankovic F, Cherian A, Steven L, Mathur A, Cuckow P. Current practice in feminizing surgery for congenital adrenal hyperplasia; a specialist survey. Journal of Pediatric Urology. 2013;9(6):1103–1107.

60. Houben CH, Tsui SY, Mou JW, Chan KW, Tam YH, Lee KH. Reconstructive surgery for females with congenital adrenal hyperplasia due to 21-hydroxylase deficiency: a review from the Prince of Wales Hospital. Hong Kong Medical Journal. 2014;20(6):481–485.

61. Sturm RM, Durbin-Johnson B, Kurzrock EA. Congenital adrenal hyperplasia: current surgical management at academic medical centers in the United States. The Journal of Urology. 2015:193(5):1796–1801.

62. Bonfig W, Dalla Pozza SB, Schmidt H, Pagel Ph, Knorr D, Schwarz HP. Hydrocortisone dosing during puberty in patients with classical congenital adrenal hyperplasia: an evidence-based recommendation. The Journal of Clinical Endocrinology and Metabolism. 2009;94(10):3882–3888.

63. Van der Kamp HJ, Otten BJ, Buitenweg N, De Muinck Keizer-Schrama SMPF, Oostdijk W, Jansen M et al. Longitudinal analysis of growth and puberty in 21-hydroxylase deficiency patients. Archives of Disease in Childhood. 2002;87(2):139–144.

64. Finkielstain GP, Kim MS, Sinaii N, Nishitani M, Van Ryzin C, Hill SC, Reynolds JC, Hanna RM, Merke DP. Clinical Characteristics of a Cohort of 244 Patients with Congenital Adrenal Hyperplasia. The Journal of Clinical Endocrinology and Metabolism. 2012;97(12):4429-4438.

65. Bonfig W, Bechtold S, Schmidt H, Knorr D, Schwarz HP. Reduced final height outcome in congenital adrenal hyperplasia under prednisone treatment: deceleration of growth velocity during puberty. The Journal of Clinical Endocrinoly and Metabolism. 2007;92(5):1635–1639.

66. Melin J, Parra-Guillen ZP, Michelet R, Truong T, Huisinga W, Hartung N, Hindmarsh H, Kloft Ch. Pharmacokinetic/Pharmacodynamic Evaluation of Hydrocortisone Therapy in Pediatric Patients with Congenital Adrenal Hyperplasia. The Journal of Clinical Endocrinology & Metabolism. [Internet]. 2020;105(3):dgaa071. Available from: https:// academic.oup.com/jcem/article/105/4/e1729/5735214.

67. Hindmarsh PC, Charmandari E. Variation in absorption and half-life of hydrocortisone influence plasma cortisol concentrations. Clin Endocrinol (Oxf ). 2015;82(4):557–561.

68. Claahsen van der Grinten H. L. et al. Congenital Adrenal Hyperplasia—Current Insights in Pathophysiology, Diagnostics, and Management. Endocr Rev. 2021;43(1):91–159.

69. Arlt W, Willis DS, Wild SH, Krone N, Doherty EJ, Hahner S, Han TS et al. Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients. The Journal of Clinical Endocrinoly and Metabolism. 2010;95(11):5110–5121.

70. Charmandari E, Hindmarsh PC, Johnston A, Brook CG. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: alterations in cortisol pharmacokinetics at puberty. The Journal of Clinical Endocrinoly and Metabolism. 2001;86(6):2701–2708.

71. Mullis PE, Hindmarsh PC, Brook CG. Sodium chloride supplement at diagnosis and during infancy in children with salt-losing 21-hydroxylase deficiency. European Journal of Pediatrics. 1990;150(1):22–25.

72. Fomina MA, Bizhanova DA, Arzamasceva LV, Protasov AA, Karmanov ME, Dyadik TG. Evaluation the results of feminizing plasty in female adolescents with congenital adrenal cortex dysfunction in the remote postoperative period. Problemy endocrinologii. 2010;2:35-39. (In Russ.)

73. Fajzulin AK, Batygin MP, Glybina TM, Shkityr' ZV. Sovremennye metody khirurgicheskoj korrekcii porokov naruzhnykh genitalij u devochek s vrozhdennoj disfunkciej kory nadpochechnikov. Andrologiya i genital'naya khirurgiya. 2011;3:69–73.

74. Dacou-Voutetakis C, Karidis N. Congenital adrenal hyperplasia complicated by central precocious puberty: treatment with LHRH-agonist analogue. Annal of the New York Academi of Sciences. 1993;687:250–254.

75. Muthusamy K, Elamin MB, Smushkin G, Murad MH, Lampropulos JF, Elamin KB. et al. Clinical review: Adult height in patients with congenital adrenal hyperplasia: a systematic review and metaanalysis. The Journal of Clinical Endocrinoly and Metabolism. 2010;95(9):4161–4172.

76. Quintos JBQ, 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. The Journal of Clinical Endocrinology & Metabolism. 2001;86(4):1511–1517.

77. Merke DP, Keil MF, Jones JV, Fields J, Hill S, Cutler GB. Flutamide, testolactone, and reduced hydrocortisone dose maintain normal growth velocity and bone maturation despite elevated androgen levels in children with congenital adrenal hyperplasia. The Journal of Clinical Endocrinoly and Metabolism. 2000;85(3):1114–1120.

78. Klinicheskie rekomendacii «Prezhdevremennoe polovoe razvitiе» (in Russ.). Доступен по: https://cr.minzdrav.gov.ru/view-cr/648_2

79. Mercado Santis E, Campos A, Fernandez P, Oriola J, Yeste D, Perez V, Clemente M. Congenital adrenal hyperplasia due to 11-betahydroxylase deficiency: clinical, biochemical and molecular characteristics and long-term outcomes. Anales de Pediatria. 2025;102(2): 503747

80. Kennedy EC, Stack M, Carolan E, Durkan M, Joyce CM, Hawkes CP. Mineralocorticoid receptor antagonist monotherapy in pediatric non-classical 11β-hydroxylase deficiency. Journal of Pediatric Endocrinology&Metabolism. 2024;37(12):1100–1103.

81. Lundberg T, Lindström A, Roen K, Hegarty P. From Knowing Nothing to Knowing What, How and Now: Parents’ Experiences of Caring for their Children With Congenital Adrenal Hyperplasia. Journal of Pediatric Psychology. 2017;42(5):520–529.

82. Nordenström A, Thyen U. Improving the communication of healthcare professionals with affected children and adolescents. Endocrine Development. 2014;27:113–127.

83. Sanches SA, Wiegers TA, Otten BJ, Claahsen-van der Griten HL. Physical, social and societal functioning of children with congenital adrenal hyperplasia (CAH) and their parents, in a Dutch population. International Journal of Pediatric Endocrinology. 2012;1:2

84. El-Maouche D, Hargreaves CJ, Sinaii N, Mallappa A, Veeraraghavan P, Merke DP. Longitudinal Assessment of Illnesses, Stress Dosing, and Illness Sequelae in Patients With Congenital Adrenal Hyperplasia. The Journal of Clinical Endocrinoly and Metabolism 2018;103(6):2336–2345.

85. Speiser PW. Emerging medical therapies for congenital adrenal hyperplasia. F1000Research. 2019;8:363.

86. Weise M, Drinkard B, Mehlinger SL, Holzer SM, Eisenhofer G, Charmandari E, Chrousos GP. Stress dose of hydrocortisone is not beneficial in patients with classic congenital adrenal hyperplasia undergoing short-term, high-intensity exercise. The Journal of Clinical Endocrinoly and Metabolism. 2004;89(8):3679–3684.

87. Nisticò D, Bossini B, Benvenuto S, Pellegrin MC, Tornese G. Pediatric Adrenal Insufficiency: Challenges and Solutions. Theraupetic and Clinical Risk Management. 2022;18:47–60.

88. Nowotny H, Ahmed SF, Bensig S, Beun JG, Brösamle M, Chifu I et al. Therapy options for adrenal insufficiency and recommendations for the management of adrenal crisis. Endocrine. 2021;71(3):586–594.

89. Rushworth RL, Torpy DJ, Falhammar H. Adrenal Crisis. The New England Journal of Medicine. 2019;381(9):852–861.

90. Mushtaq T, Ali SR, Boulos N, Boyle R, Cheetham T, Davies JH, Elder CJ et al. Emergency and perioperative management of adrenal insufficiency in children and young people: British Society for Paediatric Endocrinology and Diabetes consensus guidance. Archives of disease in childhood. 2023;108(11):871-878.

91. Shulman DI, Palmert MR, Kemp SF. Adrenal insufficiency: still a cause of morbidity and death in childhood. Pediatrics. 2007;119(2):484-494.

92. Woodcock T, Barker P, Daniel S, Fletcher S, Wass JAH, Tomlinson JW, Misra U, Dattani M. Guidelines for the management of glucocorticoids during the peri-operative period for patients with adrenal insufficiency: Guidelines from the Association of Anaesthetists, the Royal College of Physicians and the Society for Endocrinology UK. Anaesthesia. 2020;75(5):654–663.

93. Bonfig W, Roehl F-W, Riedl S, Dörr HG, Bettendorf M, Brämswig J, Schönau E.Blood Pressure in a Large Cohort of Children and Adolescents With Classic Adrenal Hyperplasia (CAH) Due to 21-Hydroxylase Deficiency. American Journal of Hypertension. 2016;29(2):266–272.

94. 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. Clinical Endocrinology (Oxf). 2014;81(6):871–875.

95. New MI, Carlson A, Obeid J, Marshall I, Cabrera MS, Goseco A, Lin-Su K, Putnam AS et al. Prenatal diagnosis for congenital adrenal hyperplasia in 532 pregnancies. The Journal of Clinical Endocrinoly and Metabolism. 2001;86(12):5651–5657.

96. Meyer-Bahlburg HFL, Dolezal C, Baker SW, Carlson AD, Obeid JS, New MI. Cognitive and motor development of children with and without congenital adrenal hyperplasia after early-prenatal dexamethasone. The Journal of Clinical Endocrinoly and Metabolism. 2004;89(2):610–614.

97. Mercè Fernández-Balsells M, Muthusamy K, Smushkin G, Lampropulos JF, Elamin MB, Elnour NOA, Elamin KB et al. Prenatal dexamethasone use for the prevention of virilization in pregnancies at risk for classical congenital adrenal hyperplasia because of 21-hydroxylase (CYP21A2) deficiency: a systematic review and metaanalyses. Clinical Endocrinology (Oxf ). 2010;73(4):436–444.