Familial Glucocorticoid Deficiency Follow-up
- Author: Andrea Haqq, MD; Chief Editor: Stephen Kemp, MD, PhD more...
Further Outpatient Care
See the list below:
- Glucocorticoid replacement: Clinically judge the adequacy of glucocorticoid treatment by documenting reduced hyperpigmentation, absence of hypoglycemia and weakness, and normal growth at frequent follow-up visits. Administer the lowest dosage of glucocorticoid sufficient to control symptoms of adrenal insufficiency to permit normal growth in these patients.
Further Inpatient Care
See the list below:
- Glucocorticoid replacement: Focus inpatient care of familial glucocorticoid deficiency on providing adequate replacement of glucocorticoids. Administer an initial maintenance dose of 12-16 mg/m2/d of oral hydrocortisone divided in 3 doses.
Inpatient & Outpatient Medications
Glucocorticoids are previously discussed.
FGD, if left untreated or inadequately treated, may lead to death from adrenal crisis or to severe mental disability as a result of recurrent hypoglycemia. Alternatively, overtreatment with glucocorticoids may result in growth failure and features of Cushing syndrome, which include weight gain, hypertension, obesity, skin changes, osteoporosis, glucose intolerance, and muscle weakness.
Patients with FGD have a lifelong loss of adrenal function. They remain at risk of adrenal insufficiency during periods of stress when the adrenal gland normally secretes more hormones. If patients receive adequate glucocorticoid replacement and are properly educated regarding readjustment of medication during times of illness and stress, they should have a normal lifespan and be able to have children of their own.
See the list below:
- Glucocorticoid therapy
- Educate patients regarding readjustment of glucocorticoid dosage during intercurrent illness or stress and during minor stress. Regarding fever or upper respiratory tract infections, double or triple the dosage of glucocorticoid until the illness is resolved.
- Advise parents and/or caregivers to contact their pediatric endocrinologist if vomiting or diarrhea is present and the child cannot tolerate oral fluids and medication. They may be instructed to administer a prefilled Solu-Cortef syringe intramuscularly in a dose appropriate for the size of the patient. Alternatively, hospitalization may be required in this situation.
- In cases of major stress, such as surgery or serious illness, advise parents to contact their pediatric endocrinologist. As a life-saving measure, parents may need to administer a prefilled Solu-Cortef syringe intramuscularly if medical assistance is not immediately available. Following this, advise parents to arrange transfer of their child to the hospital.
- In serious illness, the daily requirement of parenteral hydrocortisone is 40-100 mg/m2 (approximately 4-10 times the maintenance dose) in 3 or 4 divided doses. Advise all patients with FGD to wear a MedicAlert bracelet outlining their condition and medical treatment.
- Because of the rarity of this condition, provide families with a physician letter outlining FGD and its potential complications and treatments to present to an emergency care facility should a visit to the emergency department be necessary. Counsel patients, families, and/or caregivers regarding the importance of compliance in taking this life-sustaining medication; this medication should never be stopped in any circumstance.
- Counsel patients with FGD and their families regarding the autosomal recessive inheritance pattern of FGD.
- Monitor siblings and close relatives for potential symptoms of FGD; obtain appropriate laboratory screening to rule out this condition, which is potentially fatal if left untreated.
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