Treatment of Nonclassic CAH in Children and Adolescents with Early Pubarche, Rapid Bone Age Progression, or Overt Virilization
This protocol covers nonclassic congenital adrenal hyperplasia (21-hydroxylase deficiency) specifically in children and adolescents presenting with inappropriately early onset and rapid progression of pubarche or bone age, or adolescents with overt virilization — a clinical sub-population in which active treatment is indicated.
The relevant presentation includes children in whom pubarche appears earlier than expected and is advancing at an abnormally rapid pace, those with a significantly advanced bone age relative to chronological age, and adolescents exhibiting overt signs of virilization. These features point to a degree of androgen excess that warrants evaluation and management within the nonclassic CAH framework.
Glucocorticoid treatment is the basis of management in this population. Some patients with intermediate phenotypes may benefit from a chronic low-dose approach — the specific regimen, patient selection criteria, and monitoring details are contained in the full structured protocol.
DOI: 10.1210/jc.2018-01865
In children and adolescents with inappropriately early onset and rapid progression of pubarche or bone age and in adolescent patients with overt virilization we suggest GC treatment of NCCAH.
Some of these patients benefit from chronic low-dose HC therapy.
In adolescents with irregular menses and acne, symptoms are usually reversed within 3 months of GC treatment, whereas hirsutism remission is more difficult with GC monotherapy.
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