Measurements of levels of 17α-OHP are useful in the evaluation of patients with suspected congenital adrenal hyperplasia as the typical enzymes that are defective, namely 21-hydroxylase and 11β-hydroxylase , lead to a build-up of 17α-OHP. In contrast, the rare patient with 17α-hydroxylase deficiency will have very low or undetectable levels of 17α-OHP. 17α-OHP levels can also be used to measure contribution of progestational activity of the corpus luteum during pregnancy as progesterone but not 17α-OHP is also contributed by the placenta .
There are no specific recommendations about when to initiate corticosteroid treatment. Data suggest that oral steroids should be used in patients with stages II and III disease, with moderate to severe or progressive symptoms and chest radiograph changes. It is unclear if asymptomatic patients will ever need therapy, even if they have diffuse lung infiltration. Because there is no consensus on the optimal dosage or duration of therapy, the course is individualized for each patient. A recent joint statement of the American Thoracic Society, the European Respiratory Society, and the World Association of Sarcoidosis and Other Granulomatous Disorders 1 included the following guidelines: an initiation dose of prednisone of 20 to 40 mg per day or its equivalent is recommended. Every-otherday dosing may be considered. Patients should be evaluated after one to three months for response. Patients who fail treatment after three months usually will not respond to a more protracted course of treatment. In responders, the prednisone dosage should be tapered to 5 to 10 mg per day or to an every-otherday regimen, and therapy should continue for a minimum of 12 months. 1 There is no consensus guidance on treatment beyond two years. 2 Patients must be monitored after cessation of treatment for possible relapse; some patients will require long-term low-dose therapy to prevent recurrent disease. 1