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Hydrocortisone Infusion Pump Study

Sheila Gunn, M.D.

Assistant Professor of Pediatrics, Baylor College of Medicine

Congenital Adrenal Hyperplasia (CAH) is caused by a metabolic defect in the production of cortisol and aldosterone in the adrenal gland. Adrenocorticotropin hormone (ACTH) is released by the pituitary gland and stimulates the adrenal gland to produce cortisol. Most of the release of ACTH occurs during the night in pulses that rise in the early morning between 4-6 am. In the child with CAH, cortisol is not produced causing a feedback rise in the amount of ACTH. There is also a rise in other hormones including 17-hydroxyprogesterone (17-OHP) and the male hormone androstenedione. Management of CAH requires the administration of hydrocortisone (cortisol) and fludrocortisone several times daily in amounts that will suppress the 17-OHP, androstenedione and ACTH to normal levels. One of the problems is that the amount of hydrocortisone that is needed to suppress the hormone levels may cause poor growth and weight gain.

The purpose of this study is to determine if administration of hydrocortisone by an infusion pump during the night will suppress ACTH, 17-OHP and androstenedione levels so that a lower dose of hydrocortisone can be used. In this study, a group of girls with CAH age 9-15 and a group of normal girls age 9-15 will have two studies to determine their hormone levels. Each girl will have a screening history, physical exam and test for anemia. In the first study, the girls will be admitted to the GCRC and have an IV placed using a numbing medication and they will continue to take their home medication. They will have blood drawn several times through the IV to determine the levels and pulses of the ACTH, 17-OHP and androstenedione for 24 hr. In the second study, the girls with CAH will return at a later date to have the study repeated while off their hydrocortisone. They will receive several pulses of hydrocortisone through an infusion pump during the night. The hormone levels will be analyzed to determine if the infusions of hydrocortisone suppressed the ACTH levels and lowered the 17-OHP and androstenedione levels.

Interested persons can call the endocrine research nurse Sue McGirk, R.N at 832-822-3063 or e-mail at tsmcgirk@TexasChildrensHospital.org.

Sheila Gunn is the principle investigator for this study. She is an assistant professor of pediatrics at Baylor College of Medicine in the Pediatric Endocrine Section. This research is being done at Texas Children’s Hospital at the General Clinical Research Center (GCRC).

     

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