Background: Adrenal insufficiency is a well-recognised feature of congenital adrenal hyperplasia (CAH).
CAH is commonly treated with oral steroid replacement, taken 23 times a day, at doses that aim to reproduce normal diurnal variation. Though acceptable for most patients, this does not control others, resulting in high levels of 17-hydroxyprogesterone-acetate (17OHP), ACTH, and the need for increased doses of steroid replacement, with associated co-morbidities.
Insulin, too, has a diurnal rythmn, which can be difficult to replace physiologically. In some diabetic patients, continuous s.c. insulin infusion (CSII) has been used to good effect. Here we discuss a patient with CAH who was successfully treated with continuous subcutaneous steroid replacement therapy delivered through a CSII pump.
Methods: We present a 39-year-old lady with classical CAH poorly controlled on oral steroid replacement (hydrocortisone 10 mg mane and prednisolone 4 mg nocte). Body mass index 35.13, elevated 17-hydroxyprogesterone levels and long standing fertility problems.
Oral steroids were replaced by a hydrocortisone solution delivered via a CSII pump at a rate of 1.5 mg/h (00000800 h), 0.8 mg/h (08001600 h), and 0.4 mg/h (16002400 h). Total daily dose remained the same.
Education re. escape therapy and care of pump and lines was taught, and the patient had open access to specialist nurses.
Biochemical assessment with plasma and urinary steroid day-profiles was undertaken prior to, and regularly after, commencement of therapy.
Results: CSII pump delivery was associated with an improved physiological steroid day-profile, suppression of 17OHP and ACTH levels, and a one-third reduction in daily steroid dose.
Conclusion: CSII devices can be successfully used to deliver hormones other than insulin. This provides an alternative option for specific adrenal insufficiency patients.