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** [[Dexamethasone]] crosses the [[placenta]] into the [[fetal circulation]] and prevents [[ambiguous genitalia]] in female [[fetus]]   
** [[Dexamethasone]] crosses the [[placenta]] into the [[fetal circulation]] and prevents [[ambiguous genitalia]] in female [[fetus]]   
** This treatment should be started before 9 weeks of [[pregnancy]] age; if treatment cannot be started by 9 weeks, it should not be given at all
** This treatment should be started before 9 weeks of [[pregnancy]] age; if treatment cannot be started by 9 weeks, it should not be given at all
** If in [[Cell-free system|cell-free]] [[fetal]] [[DNA testing]] male [[fetus]] is detected, treatment should be discontinued
** If [[Cell-free system|cell-free]] [[fetal]] [[DNA testing]] reveals the gender to be male, treatment should be discontinued
** Approximately 85% of managed cases appear quite normal after [[delivery]]
** Approximately 85% of managed cases appear quite normal after [[delivery]]
** [[Side effects]] of [[prenatal]] [[dexamethasone]] are:<ref name="pmid208234662">{{cite journal| author=Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP et al.| title=Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. | journal=J Clin Endocrinol Metab | year= 2010 | volume= 95 | issue= 9 | pages= 4133-60 | pmid=20823466 | doi=10.1210/jc.2009-2631 | pmc=2936060 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20823466  }}</ref><ref name="pmid9814461">{{cite journal| author=Lajic S, Wedell A, Bui TH, Ritzén EM, Holst M| title=Long-term somatic follow-up of prenatally treated children with congenital adrenal hyperplasia. | journal=J Clin Endocrinol Metab | year= 1998 | volume= 83 | issue= 11 | pages= 3872-80 | pmid=9814461 | doi=10.1210/jcem.83.11.5233 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9814461  }}</ref><ref name="pmid18060943">{{cite journal| author=Carmichael SL, Shaw GM, Ma C, Werler MM, Rasmussen SA, Lammer EJ et al.| title=Maternal corticosteroid use and orofacial clefts. | journal=Am J Obstet Gynecol | year= 2007 | volume= 197 | issue= 6 | pages= 585.e1-7; discussion 683-4, e1-7 | pmid=18060943 | doi=10.1016/j.ajog.2007.05.046 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18060943  }}</ref><ref name="pmid27482827">{{cite journal| author=Wallensteen L, Zimmermann M, Thomsen Sandberg M, Gezelius A, Nordenström A, Hirvikoski T et al.| title=Sex-Dimorphic Effects of Prenatal Treatment With Dexamethasone. | journal=J Clin Endocrinol Metab | year= 2016 | volume= 101 | issue= 10 | pages= 3838-3846 | pmid=27482827 | doi=10.1210/jc.2016-1543 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27482827  }}</ref><ref name="pmid24278432">{{cite journal| author=Khalife N, Glover V, Taanila A, Ebeling H, Järvelin MR, Rodriguez A| title=Prenatal glucocorticoid treatment and later mental health in children and adolescents. | journal=PLoS One | year= 2013 | volume= 8 | issue= 11 | pages= e81394 | pmid=24278432 | doi=10.1371/journal.pone.0081394 | pmc=3838350 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24278432  }}</ref>   
** [[Side effects]] of [[prenatal]] [[dexamethasone]] are:<ref name="pmid208234662">{{cite journal| author=Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP et al.| title=Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. | journal=J Clin Endocrinol Metab | year= 2010 | volume= 95 | issue= 9 | pages= 4133-60 | pmid=20823466 | doi=10.1210/jc.2009-2631 | pmc=2936060 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20823466  }}</ref><ref name="pmid9814461">{{cite journal| author=Lajic S, Wedell A, Bui TH, Ritzén EM, Holst M| title=Long-term somatic follow-up of prenatally treated children with congenital adrenal hyperplasia. | journal=J Clin Endocrinol Metab | year= 1998 | volume= 83 | issue= 11 | pages= 3872-80 | pmid=9814461 | doi=10.1210/jcem.83.11.5233 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9814461  }}</ref><ref name="pmid18060943">{{cite journal| author=Carmichael SL, Shaw GM, Ma C, Werler MM, Rasmussen SA, Lammer EJ et al.| title=Maternal corticosteroid use and orofacial clefts. | journal=Am J Obstet Gynecol | year= 2007 | volume= 197 | issue= 6 | pages= 585.e1-7; discussion 683-4, e1-7 | pmid=18060943 | doi=10.1016/j.ajog.2007.05.046 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18060943  }}</ref><ref name="pmid27482827">{{cite journal| author=Wallensteen L, Zimmermann M, Thomsen Sandberg M, Gezelius A, Nordenström A, Hirvikoski T et al.| title=Sex-Dimorphic Effects of Prenatal Treatment With Dexamethasone. | journal=J Clin Endocrinol Metab | year= 2016 | volume= 101 | issue= 10 | pages= 3838-3846 | pmid=27482827 | doi=10.1210/jc.2016-1543 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27482827  }}</ref><ref name="pmid24278432">{{cite journal| author=Khalife N, Glover V, Taanila A, Ebeling H, Järvelin MR, Rodriguez A| title=Prenatal glucocorticoid treatment and later mental health in children and adolescents. | journal=PLoS One | year= 2013 | volume= 8 | issue= 11 | pages= e81394 | pmid=24278432 | doi=10.1371/journal.pone.0081394 | pmc=3838350 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24278432  }}</ref>   
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==== Neonatal treatment ====
==== Neonatal treatment ====
Medical therapy for 21-hydroxylase deficiency in the [[neonates]] is as follows:<ref name="pmid20823466">{{cite journal| author=Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP et al.| title=Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. | journal=J Clin Endocrinol Metab | year= 2010 | volume= 95 | issue= 9 | pages= 4133-60 | pmid=20823466 | doi=10.1210/jc.2009-2631 | pmc=2936060 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20823466  }}</ref>
Medical therapy for 21-hydroxylase deficiency in the [[neonates]] is as follows:<ref name="pmid20823466">{{cite journal| author=Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP et al.| title=Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. | journal=J Clin Endocrinol Metab | year= 2010 | volume= 95 | issue= 9 | pages= 4133-60 | pmid=20823466 | doi=10.1210/jc.2009-2631 | pmc=2936060 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20823466  }}</ref>
* Preferred regimen: [[Hydrocortisone]] 20 to 30 mg/m<sup>2</sup>/day divided in three doses PO '''<u>AND</u>''' [[Fludrocortisone]] 100 mcg twice daily PO '''<u>AND</u>''' [[sodium chloride]] one gram or 4 mEq/kg/day divided in several doses [[Orally ingested|PO]]
* Preferred regimen: [[Hydrocortisone]] 20 to 30 mg/m<sup>2</sup>/day divided in three doses [[Orally ingested|PO]] '''<u>AND</u>''' [[Fludrocortisone]] 100 mcg twice daily [[Orally ingested|PO]] '''<u>AND</u>''' [[sodium chloride]] one gram or 4 mEq/kg/day divided in several doses [[Orally ingested|PO]]
** The minimization of [[steroid]] doses should be considered to avoid [[steroid]] [[complications]] in [[infants]] 
** The minimization of [[steroid]] doses should be considered to avoid [[steroid]] [[complications]] in [[infants]] 
** Growth suppression and shorter height in adulthood are the [[complications]] of using high dose [[steroids]] which occurs in [[neonates]]
** Growth suppression and shorter height in adulthood are the [[complications]] of using high dose [[steroids]] which occurs in [[neonates]]
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==== '''Ambiguous genitalia''' ====
==== '''Ambiguous genitalia''' ====
* [[Ambiguous genitalia]] should be managed immediately   
* [[Ambiguous genitalia]] should be managed immediately   
* Infants with [[ambiguous genitalia]] and non palpable [[gonads]] should be considered to have [[congenital adrenal hyperplasia]] and [[empirical treatment]] should be start early after obtaining [[blood]] sample for [[17-hydroxyprogesterone]]  
* [[Infants]] with [[ambiguous genitalia]] and non palpable [[gonads]] should be considered to have [[congenital adrenal hyperplasia]] and [[empirical treatment]] should be start early after obtaining [[blood]] sample for [[17-hydroxyprogesterone]]  
* Initial [[empiric therapy]] should contains doses of [[glucocorticoid]] and [[mineralocorticoid]] and [[sodium chloride]] supplementation
* Initial [[empiric therapy]] should contains doses of [[glucocorticoid]] and [[mineralocorticoid]] and [[sodium chloride]] supplementation
** Preferred regimen: [[Hydrocortisone]] is 20 to 30 mg/m<sup>2</sup>/day divided in three doses PO '''<u>AND</u>''' [[Fludrocortisone]] 100 mcg twice daily PO '''<u>AND</u>''' [[sodium chloride]] one gram or 4 mEq/kg/day divided in several doses [[Route of administration|PO]]  
** Preferred regimen: [[Hydrocortisone]] is 20 to 30 mg/m<sup>2</sup>/day divided in three doses [[Orally ingested|PO]] '''<u>AND</u>''' [[Fludrocortisone]] 100 mcg twice daily [[Orally ingested|PO]] '''<u>AND</u>''' [[sodium chloride]] one gram or 4 mEq/kg/day divided in several doses [[Route of administration|PO]]  


==== Adrenal crisis ====
==== Adrenal crisis ====
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===Management in children===
===Management in children===
* Preferred regimen: [[Hydrocortisone]] ([[cortisol]]) in a dose of 10 to 15 mg/m<sup>2</sup> [[body surface area]]/day orally '''<u>AND</u>''' [[fludrocortisone]] in a dose of 50 to 200 mcg per day (0.05 to 0.20 mg/day) [[Orally ingested|orally]]
* Preferred regimen: [[Hydrocortisone]] ([[cortisol]]) in a dose of 10 to 15 mg/m<sup>2</sup> [[body surface area]]/day [[Orally ingested|PO]] '''<u>AND</u>''' [[fludrocortisone]] in a dose of 50 to 200 mcg per day (0.05 to 0.20 mg/day) [[Orally ingested|PO]]
** [[Mineralocorticoid]] replacement should be started in all 21-hydroxylase deficient patients, and often may be tapered after six months of age  
** [[Mineralocorticoid]] replacement should be started in all 21-hydroxylase deficient patients, and often may be tapered after six months of age  


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* Provide proper dosing of [[glucocorticoid]] and [[mineralocorticoid]]
* Provide proper dosing of [[glucocorticoid]] and [[mineralocorticoid]]
* Decrease secretion of [[cosyntropin]]; therefore decrease [[adrenal]] overstimulation and [[androgen]] production
* Decrease secretion of [[cosyntropin]]; therefore decrease [[adrenal]] overstimulation and [[androgen]] production
====='''Glucocorticoids and mineralocorticoid replacement:''' =====
====='''Glucocorticoids and mineralocorticoid replacement''' =====
* Preferred regimen: [[Hydrocortisone]] 15-30 mg per day divided into three doses [[Orally ingested|PO]] '''<u>AND</u>''' [[Fludrocortisone Acetate|9-alpha-fludrocortisone acetate]] 0.1 to 0.2 mg per day [[Orally ingested|PO]]
* Preferred regimen: [[Hydrocortisone]] 15-30 mg per day divided into three doses [[Orally ingested|PO]] '''<u>AND</u>''' [[Fludrocortisone Acetate|9-alpha-fludrocortisone acetate]] 0.1 to 0.2 mg per day [[Orally ingested|PO]]
* Alternative regimen (1): [[Dexamethasone]] 0.75 mg per day [[Orally ingested|PO]] '''<u>AND</u>''' [[Fludrocortisone Acetate|9-alpha-fludrocortisone acetate]] 0.1 to 0.2 mg per day [[Route of administration|PO]]
* Alternative regimen (1): [[Dexamethasone]] 0.75 mg per day [[Orally ingested|PO]] '''<u>AND</u>''' [[Fludrocortisone Acetate|9-alpha-fludrocortisone acetate]] 0.1 to 0.2 mg per day [[Route of administration|PO]]
* Alternative regimen (2): [[Prednisone]] 5mg per day [[Orally ingested|PO]] '''<u>AND</u>''' [[Fludrocortisone Acetate|9-alpha-fludrocortisone acetate]] 0.1 to 0.2 mg per day [[Orally ingested|PO]]
* Alternative regimen (2): [[Prednisone]] 5mg per day [[Orally ingested|PO]] '''<u>AND</u>''' [[Fludrocortisone Acetate|9-alpha-fludrocortisone acetate]] 0.1 to 0.2 mg per day [[Orally ingested|PO]]


===== Considerations: =====
===== Considerations =====
*[[Glucocorticoids]] reduce the excess production of [[adrenal]] [[androgens]] and reduce the excessive secretion of both [[corticotropin-releasing hormone]] and [[ACTH]]
*[[Glucocorticoids]] reduce the excess production of [[adrenal]] [[androgens]] and reduce the excessive secretion of both [[corticotropin-releasing hormone]] and [[ACTH]]
* Stress dosing: In patients with 21-hydroxylase deficiency and serious [[illness]], [[glucocorticoids]] stress dosing is necessary
* Stress dosing: In patients with 21-hydroxylase deficiency and serious [[illness]], [[glucocorticoids]] stress dosing is necessary
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== Medical Therapy for non-classic type of 21 hydroxylase deficiency ==
== Medical Therapy for non-classic type of 21 hydroxylase deficiency ==
Medical Therapy for non-classic type of 21 hydroxylase deficiency is as following:<ref name="pmid2137832">{{cite journal |vauthors=Spritzer P, Billaud L, Thalabard JC, Birman P, Mowszowicz I, Raux-Demay MC, Clair F, Kuttenn F, Mauvais-Jarvis P |title=Cyproterone acetate versus hydrocortisone treatment in late-onset adrenal hyperplasia |journal=J. Clin. Endocrinol. Metab. |volume=70 |issue=3 |pages=642–6 |year=1990 |pmid=2137832 |doi=10.1210/jcem-70-3-642 |url=}}</ref><ref name="pmid20823466">{{cite journal |vauthors=Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP, Meyer-Bahlburg HF, Miller WL, Montori VM, Oberfield SE, Ritzen M, White PC |title=Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline |journal=J. Clin. Endocrinol. Metab. |volume=95 |issue=9 |pages=4133–60 |year=2010 |pmid=20823466 |pmc=2936060 |doi=10.1210/jc.2009-2631 |url=}}</ref><ref name="pmid2142968">{{cite journal |vauthors=Frank-Raue K, Junga G, Raue F, Vecsei P, Ziegler R |title=[Therapy of hirsutism in females with adrenal enzyme defects of steroid hormone biosynthesis: comparison of dexamethasone with cyproterone acetate] |language=German |journal=Klin. Wochenschr. |volume=68 |issue=12 |pages=597–601 |year=1990 |pmid=2142968 |doi= |url=}}</ref><ref name="pmid24622419">{{cite journal |vauthors=Merke DP, Poppas DP |title=Management of adolescents with congenital adrenal hyperplasia |journal=Lancet Diabetes Endocrinol |volume=1 |issue=4 |pages=341–52 |year=2013 |pmid=24622419 |pmc=4163910 |doi=10.1016/S2213-8587(13)70138-4 |url=}}</ref>
Medical therapy for non-classic type of 21 hydroxylase deficiency is as following:<ref name="pmid2137832">{{cite journal |vauthors=Spritzer P, Billaud L, Thalabard JC, Birman P, Mowszowicz I, Raux-Demay MC, Clair F, Kuttenn F, Mauvais-Jarvis P |title=Cyproterone acetate versus hydrocortisone treatment in late-onset adrenal hyperplasia |journal=J. Clin. Endocrinol. Metab. |volume=70 |issue=3 |pages=642–6 |year=1990 |pmid=2137832 |doi=10.1210/jcem-70-3-642 |url=}}</ref><ref name="pmid20823466">{{cite journal |vauthors=Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP, Meyer-Bahlburg HF, Miller WL, Montori VM, Oberfield SE, Ritzen M, White PC |title=Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline |journal=J. Clin. Endocrinol. Metab. |volume=95 |issue=9 |pages=4133–60 |year=2010 |pmid=20823466 |pmc=2936060 |doi=10.1210/jc.2009-2631 |url=}}</ref><ref name="pmid2142968">{{cite journal |vauthors=Frank-Raue K, Junga G, Raue F, Vecsei P, Ziegler R |title=[Therapy of hirsutism in females with adrenal enzyme defects of steroid hormone biosynthesis: comparison of dexamethasone with cyproterone acetate] |language=German |journal=Klin. Wochenschr. |volume=68 |issue=12 |pages=597–601 |year=1990 |pmid=2142968 |doi= |url=}}</ref><ref name="pmid24622419">{{cite journal |vauthors=Merke DP, Poppas DP |title=Management of adolescents with congenital adrenal hyperplasia |journal=Lancet Diabetes Endocrinol |volume=1 |issue=4 |pages=341–52 |year=2013 |pmid=24622419 |pmc=4163910 |doi=10.1016/S2213-8587(13)70138-4 |url=}}</ref>
=== Children ===
=== Children ===
* Preferred regimen: [[Hydrocortisone]] 10 to 15 mg/m<sup>2</sup> divided into three doses per day.
* Preferred regimen: [[Hydrocortisone]] 10 to 15 mg/m<sup>2</sup> divided into three doses per day.

Revision as of 16:27, 5 September 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]

Overview

Medical therapy for classic type of 21-hydroxylase deficiency includes maternal administration of dexamethasone for genetically recognized patients. Hydrocortisone and fludrocortisone is given in children and adults. Treatment for non-classic type of 21 hydroxylase deficiency in children includes hydrocortisone until puberty and in women oral contraceptive pills are given for regulating menstrual cycle. Men with non-classic type of 21 hydroxylase deficiency are asymptomatic and they do not need treatment.

Medical Therapy for classic type of 21 hydroxylase deficiency

Management in neonates

Medical therapy for 21-hydroxylase deficiency in prenatal period, neonates, children and adults, is as below:[1][2][3][4][5]

Prenatal treatment

In the prenatal period virilization of female fetus begins early; therefore, early diagnosis and treatment are required as follows:

Neonatal treatment

Medical therapy for 21-hydroxylase deficiency in the neonates is as follows:[4]

Ambiguous genitalia 

Adrenal crisis

Management in children

Response to therapy can be monitored by checking the following parameters:

Management in adults

21 hydroxylase deficiency should be managed as follows:[4][11][12][13][3][14][15]

Treatment goals

Glucocorticoids and mineralocorticoid replacement 
Considerations
Therapy consideration in women

Medical Therapy for non-classic type of 21 hydroxylase deficiency

Medical therapy for non-classic type of 21 hydroxylase deficiency is as following:[16][4][17][18]

Children

  • Preferred regimen: Hydrocortisone 10 to 15 mg/m2 divided into three doses per day.
    • Treatment should be continued until puberty.

Adults

  • Male patient with non-classic 21-hydroxylase deficiency are asymptomatic and they do not need treatment.

References

  1. Merke DP, Bornstein SR (2005). "Congenital adrenal hyperplasia". Lancet. 365 (9477): 2125–36. doi:10.1016/S0140-6736(05)66736-0. PMID 15964450.
  2. "Consensus statement on 21-hydroxylase deficiency from the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology". J. Clin. Endocrinol. Metab. 87 (9): 4048–53. 2002. doi:10.1210/jc.2002-020611. PMID 12213842.
  3. 3.0 3.1 Speiser PW (2001). "Congenital adrenal hyperplasia owing to 21-hydroxylase deficiency". Endocrinol. Metab. Clin. North Am. 30 (1): 31–59, vi. PMID 11344938.
  4. 4.0 4.1 4.2 4.3 Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP; et al. (2010). "Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline". J Clin Endocrinol Metab. 95 (9): 4133–60. doi:10.1210/jc.2009-2631. PMC 2936060. PMID 20823466.
  5. Bose KS, Sarma RH (1975). "Delineation of the intimate details of the backbone conformation of pyridine nucleotide coenzymes in aqueous solution". Biochem Biophys Res Commun. 66 (4): 1173–9. PMID 22237438 2 22237438 Check |pmid= value (help).
  6. Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP; et al. (2010). "Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline". J Clin Endocrinol Metab. 95 (9): 4133–60. doi:10.1210/jc.2009-2631. PMC 2936060. PMID 20823466.
  7. Lajic S, Wedell A, Bui TH, Ritzén EM, Holst M (1998). "Long-term somatic follow-up of prenatally treated children with congenital adrenal hyperplasia". J Clin Endocrinol Metab. 83 (11): 3872–80. doi:10.1210/jcem.83.11.5233. PMID 9814461.
  8. Carmichael SL, Shaw GM, Ma C, Werler MM, Rasmussen SA, Lammer EJ; et al. (2007). "Maternal corticosteroid use and orofacial clefts". Am J Obstet Gynecol. 197 (6): 585.e1–7, discussion 683-4, e1–7. doi:10.1016/j.ajog.2007.05.046. PMID 18060943.
  9. Wallensteen L, Zimmermann M, Thomsen Sandberg M, Gezelius A, Nordenström A, Hirvikoski T; et al. (2016). "Sex-Dimorphic Effects of Prenatal Treatment With Dexamethasone". J Clin Endocrinol Metab. 101 (10): 3838–3846. doi:10.1210/jc.2016-1543. PMID 27482827.
  10. Khalife N, Glover V, Taanila A, Ebeling H, Järvelin MR, Rodriguez A (2013). "Prenatal glucocorticoid treatment and later mental health in children and adolescents". PLoS One. 8 (11): e81394. doi:10.1371/journal.pone.0081394. PMC 3838350. PMID 24278432.
  11. Horrocks PM, London DR (1987). "Effects of long term dexamethasone treatment in adult patients with congenital adrenal hyperplasia". Clin Endocrinol (Oxf). 27 (6): 635–42. PMID 2843311.
  12. Stewart PM, Biller BM, Marelli C, Gunnarsson C, Ryan MP, Johannsson G (2016). "Exploring Inpatient Hospitalizations and Morbidity in Patients With Adrenal Insufficiency". J Clin Endocrinol Metab. 101 (12): 4843–4850. doi:10.1210/jc.2016-2221. PMID 27623069.
  13. Hughes IA (1988). "Management of congenital adrenal hyperplasia". Arch Dis Child. 63 (11): 1399–404. PMC 1779155. PMID 3060026.
  14. Lopes LA, Dubuis JM, Vallotton MB, Sizonenko PC (1998). "Should we monitor more closely the dosage of 9 alpha-fluorohydrocortisone in salt-losing congenital adrenal hyperplasia?". J. Pediatr. Endocrinol. Metab. 11 (6): 733–7. PMID 9829228.
  15. Jansen M, Wit JM, van den Brande JL (1981). "Reinstitution of mineralocorticoid therapy in congenital adrenal hyperplasia. Effects on control and growth". Acta Paediatr Scand. 70 (2): 229–33. PMID 7015786.
  16. Spritzer P, Billaud L, Thalabard JC, Birman P, Mowszowicz I, Raux-Demay MC, Clair F, Kuttenn F, Mauvais-Jarvis P (1990). "Cyproterone acetate versus hydrocortisone treatment in late-onset adrenal hyperplasia". J. Clin. Endocrinol. Metab. 70 (3): 642–6. doi:10.1210/jcem-70-3-642. PMID 2137832.
  17. Frank-Raue K, Junga G, Raue F, Vecsei P, Ziegler R (1990). "[Therapy of hirsutism in females with adrenal enzyme defects of steroid hormone biosynthesis: comparison of dexamethasone with cyproterone acetate]". Klin. Wochenschr. (in German). 68 (12): 597–601. PMID 2142968.
  18. Merke DP, Poppas DP (2013). "Management of adolescents with congenital adrenal hyperplasia". Lancet Diabetes Endocrinol. 1 (4): 341–52. doi:10.1016/S2213-8587(13)70138-4. PMC 4163910. PMID 24622419.