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Basal 17‐hydroxyprogesterone cannot accurately predict nonclassical congenital adrenal hyperplasia in children and adolescents
Author(s) -
Bello Rachel,
Lebenthal Yael,
Lazar Liora,
Shalitin Shlomit,
Tenenbaum Ariel,
Phillip Moshe,
Vries Liat
Publication year - 2017
Publication title -
acta paediatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/apa.13630
Subject(s) - congenital adrenal hyperplasia , medicine , hydroxyprogesterone , basal (medicine) , endocrinology , adrenocorticotropic hormone , hyperplasia , bone age , hydrocortisone , glucocorticoid , hormone , steroid , insulin
Aim This study explored whether using the suggested diagnostic serum basal level of 17‐hydroxyprogesterone (6.0 nmol/L) would lead to underdiagnosis of nonclassical congenital adrenal hyperplasia. Methods We retrospectively studied 123 patients with nonclassical congenital adrenal hyperplasia, defined as an adrenocorticotropic hormone‐stimulated 17‐hydroxyprogesterone level of more than 45 nmol/L. Of these 13 had basal 17‐hydroxyprogesterone levels of less than 6.0 nmol/L and 110 exceeded that level. The 42 controls had idiopathic premature pubarche. Clinical and laboratory data were reviewed and compared. Results There were no differences between patients with 17‐hydroxyprogesterone levels of <6.0 nmol/L or ≥6.0 nmol/L based on age at presentation, gender, anthropometric measurements, bone age advancement, age at glucocorticoid initiation and hydrocortisone dosage. Patients with basal 17‐hydroxyprogesterone <6.0 nmol/L had significantly lower stimulated 17‐hydroxyprogesterone levels (p = 0.02) and higher stimulated serum cortisol levels (p < 0.008). Children with nonclassical congenital adrenal hyperplasia and premature pubarche were clinically indistinguishable from controls with idiopathic premature pubarche. Androgen levels were significantly higher in the nonclassical congenital adrenal hyperplasia group. Conclusion A basal 17‐hydroxyprogesterone threshold of 6.0 nmol/L was not a sensitive predictive marker for diagnosing nonclassical congenital adrenal hyperplasia. Children whose clinical presentation suggests nonclassical congenital adrenal hyperplasia should undergo diagnostic adrenocorticotropic hormone stimulation testing.