Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline

Врожденная надпочечниковая гиперплазия вследствие дефицита стероидной 21-гидроксилазы: клиническое руководство Endocrine Society
M. Hassan Murad, Wiebke Arlt, Richard J. Auchus, Heino F. L. Meyer‐Bahlburg, Gerard S. Conway, Sharon E. Oberfield, Laurence S. Baskin, Phyllis Speiser, Perrin C. White, Deborah P. Merke, Walter L. Miller
2018-09-27

17-hydroxyprogesterone assays21-hydroxylase deficiencycongenital adrenal hyperplasialiquid chromatography-tandem mass spectrometrynewborn screening
Objective: To update the congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency clinical practice guideline
1
All newborn screening programs should include screening for congenital adrenal hyperplasia (21-hydroxylase deficiency).
2
Clinicians should be aware that immunoassays remain in use and can produce false-positive results; specificity may improve with organic extraction to remove cross-reacting substances.
3
First-tier screening should use 17-hydroxyprogesterone assays standardized to a common technology with norms stratified by gestational age.
4
Screening laboratories should employ a second-tier screen using liquid chromatography–tandem mass spectrometry (LC-MS/MS) in preference to other methods.

Newborn screening programs for congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Recommendations for screening methods and protocols including use of 17-hydroxyprogesterone assays standardized by gestational-age norms and second-tier LC-MS/MS to improve specificity and cost-effectiveness

Publication Details
Publication Date
2018-09-27
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Authors
M. Hassan Murad
Wiebke Arlt
Richard J. Auchus
Heino F. L. Meyer‐Bahlburg
Gerard S. Conway
Sharon E. Oberfield
Laurence S. Baskin
Phyllis Speiser
Perrin C. White
Deborah P. Merke
Walter L. Miller
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