Clinical Characteristics, Management and Outcomes of Hospitalized Patients with Severe Hypercortisolism

Presentation Number: SAT 408
Date of Presentation: April 1st, 2017

Andrea Paissan*1, Valeria de Miguel1, Mariela Glerean1, Andrea Kozak1, Maria Ines Ortiz1, Ester Gabriela Scheinfeld1, Soledad Lovazzano1, Patricia Fainstein Day1, Demetrio Cavadas2 and Patricio Garcia Marchiñena1
1Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, 2Hospital Italiano de Buenos Aires, Buenos Aires

Abstract

Severe Cushing's syndrome (SCS) is considered an acute medical emergency. It is defined by massive cortisol excess with serum levels higher than 36 mcg/dL or a 24-h urinary free cortisol (UFC) more than fourfold the upper limit of normal and/or severe hypokalemia (<3.0 mmol/L) along with the recent onset of one or more of the following: sepsis, opportunistic infection, uncontrolled hypertension, heart failure, gastrointestinal hemorrhage, glucocorticoid-induced acute psychosis, progressive debilitating myopathy, thromboembolism or uncontrolled hyperglycemia. Our aim was to review the clinical characteristics, management and outcomes of hospitalized patients with SCS. This is a retrospective case record study. Thirteen patients, 9 females and 4 males with a median age of 49 years (range 24-85), 77% had ectopic ACTH secretion, with a median time from the onset of hypercortisolism of two months (range 1-36). The median follow up was 13.5 months (range 2.5 – 55). The most prevalent clinical features were hypertension (84.6%), diabetes mellitus (84.6%), proximal weakness (69.2%) and CS classic stigmata (61.5%). The more frequents complications were hypokalemia (92%), proximal myopathy (61.5%), bacterial infection (46%), arrhythmia (31%), thromboembolism (15%) and septic shock (15%). The median of UFC was 1750 mcg range 431 – 21040 (normal < 100), serum cortisol was 106 mcg/dL range 22.6 – 482 (normal 5-25) and ACTH was 184 pg/ml range 5-1000 (normal <37). Medical treatment in order to control hypercortisolism was prescribed: ketoconazole (75%), octreotide (58%) and intravenous fluconazole (25%). Bilateral laparoscopic adrenalectomy (BLA) was performed in five patients and unilateral open adrenalectomy in one patient with adrenal carcinoma. Morbidity (66%) was related to pulmonary embolism, hematoma in surgical bed and hospital acquired infections. The overall mortality was 30.8% and it was related to progression of the underlying oncological disease in three patients and due to consequences of SCS in one. In conclusion this group of patients presented high risk of complications and mortality. Acute stabilization management of metabolic and infectious disorders and simultaneous adrenostatic therapy in order to control hypercortisolism is essential. BLA is a safe and effective treatment and has to be performed in the shortest possible time span in high risk patients.

 

Nothing to Disclose: AP, VD, MG, AK, MIO, EGS, SL, PF, DC, PG