SFEBES2018 ePoster Presentations Adrenal and steroids (19 abstracts)
Cushing’s syndrome due to primary bilateral macronodular adrenal hyperplasia (PBMAH) - clinical and hormonal characterisation
Andreea Vladan 1 , Serban Radian 1, , Iuliana Baranga 1 , Catalina Moraru 1 , Diana Deciu 1 , Anda Dumitrascu 1 , Dan Hortopan 1 , Radu Iorgulescu 3 & Catalina Poiana 1,
1C.I Parhon National Institute of Endocrinology, Bucharest, Romania; 2C. Davila University of Medicine and Pharmacy, Bucharest, Romania; 3Sf Ioan Emergency Hospital, Bucharest, Romania.
Background: PBMAH is a rare cause of adrenal Cushing’s syndrome, frequently due to aberrant adrenal expression of hormonal receptors.
Aim: To describe 6 patients with PBMAH.
Methods: Clinical, hormonal and imagistic evaluation.
Results: Age at diagnosis of patients (4M/2F) was 50–79 years. One asymptomatic patient was incidentally diagnosed on abdominal CT, two patients had overt Cushing’s (central obesity, severe diabetes mellitus, arterial hypertension/renal insufficiency), three patients with metabolic syndrome (central obesity, diabetes mellitus/impaired fasting glucose, arterial hypertension, hyperlipidemia) were positive on biochemical CS screening. All patients had unsupressed cortisolemia after overnight and low-dose dexamethasone (2×2 mg) testing and 8AM ACTH was supressed. Urinary free cortisol (UFC) levels were elevated in one patient, while two patients with chronic kidney disease had UFC within the reference range. The adrenal CT imaging phenotype was variable in terms of number of nodules and size, with nodule diameters on between 1.5-5 cm. Two patients had secondary osteoporosis with prevalent vertebral fractures. Four patients were tested biochemically for the presence of aberrantly expressed receptors: cortisolemia responded to a mixed meal (GIP receptors) in two patients, one patient responded to triptorelin (gonadotropin receptors) and one patient responded to posture (adrenergic receptors), mixed meal and triptorelin. The two patients with overt Cushing’s received Metyrapone, with one undergoing bilateral adrenalectomy; she developed a fatal septic shock after the second adrenalectomy, while the other patient awaits surgery. One patient refused adrenalectomy, the asymptomatic patient is being monitored, one patient received intramuscular triptorelin and one patient has been lost to follow-up.
Conclusions: PBMAH is frequently oligo-symptomatic, warranting biochemical screening in patients with metabolic syndrome and atypical Cushing’s. Bilateral adrenalectomy is curative. Management depends on the severity of Cushing’s and availability of medical therapy (requiring biochemical aberrant adrenal receptor profiling). Patients subjected to conservative management adrenalectomy need longterm follow-up.
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Endocrine Abstracts
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