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Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline.
J Clin Endocrinol Metab. 2010 Sep; 95(9):4133-60.JC

Abstract

OBJECTIVE

We developed clinical practice guidelines for congenital adrenal hyperplasia (CAH).

PARTICIPANTS

The Task Force included a chair, selected by The Endocrine Society Clinical Guidelines Subcommittee (CGS), ten additional clinicians experienced in treating CAH, a methodologist, and a medical writer. Additional experts were also consulted. The authors received no corporate funding or remuneration.

CONSENSUS PROCESS

Consensus was guided by systematic reviews of evidence and discussions. The guidelines were reviewed and approved sequentially by The Endocrine Society's CGS and Clinical Affairs Core Committee, members responding to a web posting, and The Endocrine Society Council. At each stage, the Task Force incorporated changes in response to written comments.

CONCLUSIONS

We recommend universal newborn screening for severe steroid 21-hydroxylase deficiency followed by confirmatory tests. We recommend that prenatal treatment of CAH continue to be regarded as experimental. The diagnosis rests on clinical and hormonal data; genotyping is reserved for equivocal cases and genetic counseling. Glucocorticoid dosage should be minimized to avoid iatrogenic Cushing's syndrome. Mineralocorticoids and, in infants, supplemental sodium are recommended in classic CAH patients. We recommend against the routine use of experimental therapies to promote growth and delay puberty; we suggest patients avoid adrenalectomy. Surgical guidelines emphasize early single-stage genital repair for severely virilized girls, performed by experienced surgeons. Clinicians should consider patients' quality of life, consulting mental health professionals as appropriate. At the transition to adulthood, we recommend monitoring for potential complications of CAH. Finally, we recommend judicious use of medication during pregnancy and in symptomatic patients with nonclassic CAH.

Authors+Show Affiliations

Cohen Children's Medical Center of New York and Hofstra University School of Medicine, New Hyde Park, New York 11040, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Practice Guideline
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

20823466

Citation

Speiser, Phyllis W., et al. "Congenital Adrenal Hyperplasia Due to Steroid 21-hydroxylase Deficiency: an Endocrine Society Clinical Practice Guideline." The Journal of Clinical Endocrinology and Metabolism, vol. 95, no. 9, 2010, pp. 4133-60.
Speiser PW, Azziz R, Baskin LS, 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.
Speiser, P. W., Azziz, R., Baskin, L. S., Ghizzoni, L., Hensle, T. W., Merke, D. P., Meyer-Bahlburg, H. F., Miller, W. L., Montori, V. M., Oberfield, S. E., Ritzen, M., & White, P. C. (2010). Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. The Journal of Clinical Endocrinology and Metabolism, 95(9), 4133-60. https://doi.org/10.1210/jc.2009-2631
Speiser PW, 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. PubMed PMID: 20823466.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. AU - Speiser,Phyllis W, AU - Azziz,Ricardo, AU - Baskin,Laurence S, AU - Ghizzoni,Lucia, AU - Hensle,Terry W, AU - Merke,Deborah P, AU - Meyer-Bahlburg,Heino F L, AU - Miller,Walter L, AU - Montori,Victor M, AU - Oberfield,Sharon E, AU - Ritzen,Martin, AU - White,Perrin C, AU - ,, PY - 2010/9/9/entrez PY - 2010/9/9/pubmed PY - 2010/9/30/medline SP - 4133 EP - 60 JF - The Journal of clinical endocrinology and metabolism JO - J Clin Endocrinol Metab VL - 95 IS - 9 N2 - OBJECTIVE: We developed clinical practice guidelines for congenital adrenal hyperplasia (CAH). PARTICIPANTS: The Task Force included a chair, selected by The Endocrine Society Clinical Guidelines Subcommittee (CGS), ten additional clinicians experienced in treating CAH, a methodologist, and a medical writer. Additional experts were also consulted. The authors received no corporate funding or remuneration. CONSENSUS PROCESS: Consensus was guided by systematic reviews of evidence and discussions. The guidelines were reviewed and approved sequentially by The Endocrine Society's CGS and Clinical Affairs Core Committee, members responding to a web posting, and The Endocrine Society Council. At each stage, the Task Force incorporated changes in response to written comments. CONCLUSIONS: We recommend universal newborn screening for severe steroid 21-hydroxylase deficiency followed by confirmatory tests. We recommend that prenatal treatment of CAH continue to be regarded as experimental. The diagnosis rests on clinical and hormonal data; genotyping is reserved for equivocal cases and genetic counseling. Glucocorticoid dosage should be minimized to avoid iatrogenic Cushing's syndrome. Mineralocorticoids and, in infants, supplemental sodium are recommended in classic CAH patients. We recommend against the routine use of experimental therapies to promote growth and delay puberty; we suggest patients avoid adrenalectomy. Surgical guidelines emphasize early single-stage genital repair for severely virilized girls, performed by experienced surgeons. Clinicians should consider patients' quality of life, consulting mental health professionals as appropriate. At the transition to adulthood, we recommend monitoring for potential complications of CAH. Finally, we recommend judicious use of medication during pregnancy and in symptomatic patients with nonclassic CAH. SN - 1945-7197 UR - https://www.unboundmedicine.com/medline/citation/20823466/Congenital_adrenal_hyperplasia_due_to_steroid_21_hydroxylase_deficiency:_an_Endocrine_Society_clinical_practice_guideline_ L2 - https://academic.oup.com/jcem/article-lookup/doi/10.1210/jc.2009-2631 DB - PRIME DP - Unbound Medicine ER -