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THE JHU CHILDRENS CENTER

V. SPECIFICS OF ENDOCRINE TREATMENT

The following section is meant to inform patients and parents about endocrine guidelines for treatment of 21-hydroxylase deficiency. While it is important for patients to be informed about the type of treatment they receive, this information is not meant to take the place of regular visits to a Pediatric or Adult Endocrinologist. Every patient is different, and as a result treatment guidelines often need to be "custom-tailored" to fit individual needs.

A. Glucocorticoids

As already noted, the goal is to administer a dose of hormone equal to normal cortisol production. Cortisol production varies greatly among individuals, from 8 to 14 mg per square meter of body surface area every 24 hours.

1. Cortisol treatment

The oral preparations available include Cortef tablets (5,10 or 20 mg.) and Cortef suspension (2 mg. of cortisol per 1 ml). Because gastric acid destroys some of the oral cortisol, the treatment dose must be about twice that of normal production (15 to 30 mg per square meter of body surface area every 24 hours).

Due to rapid disappearance of cortisol from blood, it is necessary to administer 1/3 of a daily dose three times a day (5 to 10 mg per square meter of body surface area, every 8 hours, by mouth).

2. Prednisolone treatment

Prednisolone is about 5 times more active than cortisol (Pediapred syrup contains 1 mg prednisolone per 1 ml). Hence, the daily dose is 3 to 6 mg per square meter of body surface area every 24 hours orally. Because prednisolone has a longer half-life in blood than cortisol, 1/2 the daily dose can be administered twice per 24 hour period (1.5 to 3 mg per square meter of body surface area, twice daily).

3. Glucocorticoids during stress

If a person with CAH is sick with a fever of 101°F or higher, or has a serious injury (such as a broken bone), he or she may need additional glucocorticoid treatment. If surgery is planned, glucocorticoid treatment will probably be increased before, during and after the surgery.

With a temperature of 101°F, a triple baseline dose is recommended for the period of stress. It is important to limit the stress dose to the period of stress in order to avoid symptoms of over-treatment. You should discuss with your doctor or nurse the appropriate guidelines for increasing glucocorticoid treatment.

4. Glucocorticoids in conditions of repeated vomiting

If a person with CAH vomits their oral medicine, an increased oral dose should be administered 30 minutes after vomiting. If this in turn is vomited, a cortisol injection is needed. Other circumstances in which affected individuals may need to receive their treatment via injection is if they are unconscious, have severe diarrhea, or are unable to take anything by mouth prior to surgery.

Injectable hydrocortisone is sold as Solu-Cortef (Acti-Vial) and should be kept in the home for emergency use. Your nurse or doctor will show you how to mix the vial of Solu-Cortef and give it as a shot. Give 25 to 100 mg Solu-Cortef via intramuscular injection. This provides a window of approximately 6 hours to get to an Emergency Room.

People with salt-losing CAH lose salt rapidly with vomiting and diarrhea. Florinef cannot be administered as an injection, but injected cortisol (Solu-Cortef) has some salt-retaining action. A triple baseline dose of cortisol produces adequate salt retention. Additionally, a salt-water solution can be administered intravenously, if needed, in an emergency room.

5. Medic-Alert

Because a person may not alwys be able to administer their own treatment (i.e. they are too young, they are unconscious), people with CAH are stongly advised to wear a medical identification bracelet or necklace (Medic-Alert) stating that he or she takes glucocorticoids and Florinef. This notifies medical personnel to administer stress doses of treatment if needed. For more information about Medic-Alert jewelry, refer to the following address:

MedicAlert Foundation

2323 Colorado Avenue

Turlock, CA 95382-2018

http://www.medicalert.org/

phone: 1-800-IDALERT

fax: 209-669-2495

B. Salt-retaining hormone

The only available preparation for salt-retaining hormone treatment is Florinef (9a-fluoro-hydrocortisone), 0.1 mg tablets. The daily dose varies from 0.05 to 0.15 mg daily, taken once a day orally. In contrast to the cortisol dose, the Florinef dose does not vary according to age or body size of the patient.

C. Added Salt

Typically, normal infant food is low in salt (about 10-12 mEq of sodium). Infants with salt-losing CAH may profit from added salt to their diet until they begin to eat table food which has a higher salt content.


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