Hydrocortisone
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Hydrocortisone Suppression of hypothalamic-pituitary-adrenal axis due to medication error eventually leading to Cushing’s syndrome: case report
An infant girl developed suppression of hypothalamic-pituitary-adrenal axis due to drug administration error eventually leading to Cushing’s syndrome during treatment with hydrocortisone for salt-wasting classic congenital adrenal hyperplasia (SW-CAH). The girl presented at the age of 6 years and 11 months for endocrine management. She had been diagnosed with SW-CAH at birth. On day of life 6, she had started receiving hydrocortisone 1.3mg three times daily (18.7 mg/m2/day), fludrocortisone and sodium chloride. Initially, hydrocortisone was administered using a compounded 2 mg/mL suspension. At the age of 4 months, hydrocortisone dose was reduced to 1.2mg three times a day (12.6 mg/m2/day) due to undetectable androgen levels. At the age of 5 months, she was noted to have hypertension. She was then discharged with amlodipine and captopril. Suspecting the suspension might have produced inaccurate dosing, the hydrocortisone formulation was changed to tablets, which were initially quartered and given three times daily. Also, fludrocortisone was decreased and salt was reduced. In the following months, hydrocortisone doses were further reduced, and her parents were advised to crush the tablets and make a solution in water, then draw the prescribed amount. At the age of 12 months, as she was showing poor linear growth compared to her parental target height and persistently undetectable adrenal androgens, her parents sought a second opinion. At this point, hydrocortisone dose was 6.6 mg/m2/day. They were advised to target 17-hydroxyprogesterone levels between 400 and 1200 ng/dL; thus, her doses were titrated accordingly. Till the age of 4 years, her daily hydrocortisone doses were between 4.35 and 5.8 mg/m2/day divided three times daily. At the age of 3.5 years, she was noted to have "mild global increased lanugo, and slightly rounded cheeks (cushingoid facies)." Cushingoid features continued to persist, with increased downy hair growth on back, face and upper arms. At the age of 4 years, her linear growth dropped to the fourth percentile. At that time, fludrocortisone was discontinued without tapering. Two days later, she developed fatigue, vomiting, hypotension, polyuria and natriuresis. Therefore, fludrocortisone was restarted. Consequently, her symptoms resolved. The hydrocortisone dose was split into four doses a day every 6 hours starting at 6am (5.8 mg/m2/day). Captopril was stopped for better monitoring of her fludrocortisone dose. At the age of 4.4 years, her family sought a third opinion. CYP21A2 molecular testing confirmed the diagnosis of SW-CAH. The consultant endocrinologist was concerned about the accuracy of the hydrocortisone formulation because her parents dissolved 5mg tablets in 5mL of water, either after crushing or by letting the tablet dissolve over time in warm water at room temperature. And then used that solution for the rest of the day. The endocrin
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