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hrp0098p2-5 | Adrenals and HPA Axis | ESPE2024

Pharmacological therapy of adrenal hypercortisolism in a boy with suspected isolated micronodular adrenal hyperplasia (iMAD) due to a mutation in the PDE11A gene

Marx Michaela , F. Gaßmann Katja , Tzschoppe Anja , Nurcan Cebeci Ayse , Woelfle Joachim

Background: iMAD is a very rare cause of ACTH-independent Cushing´s syndrome in young children. Bilateral adrenalectomy is a definitive treatment, but poses young children at a high risk due to life-long adrenal insufficiency. Therefore, pharmacological treatment with metyrapone seems an alternative bridging option for a limited period of time.Case report: A 3 6/12 year old boy presented with a history of rapid wei...

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Pharmacological therapy of adrenal hypercortisolism in a boy with suspected isolated micronodular adrenal hyperplasia (iMAD) due to a mutation in the PDE11A gene

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