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). Currently, complete tumor resection is the only curative alternative for ACC, although retrospective studies ( 2 , 3 , 4 ) have revealed the potential of adjuvant therapy in improving the prognosis, such as mitotane. The 5-year and median overall survival
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.002 Has the flu vaccine every year, n (%) b 60 (73.2) 42 (52.5) 0.009 Is aware of the sick day rules, n (%) b 82 (100) 75 (93.8) 0.028 Has a steroid emergency card, n (%) 75 (91.5) 70 (87.5) 0.452 Always carries
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) prednisolone ( 4 ). Over-replacement with glucocorticoids avoids adrenal crisis at the expense of an increased risk of developing multiple comorbidities, including obesity, diabetes, cardiovascular disease, and osteoporosis ( 5 ). It is crucial to achieve an
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Introduction Endogenous hypercortisolism (Cushing syndrome, CS) is a rare disorder with an estimated incidence of 0.2–5.0 cases per million inhabitants per year in various populations, whereas its prevalence is close to 39–79 cases per million
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, pheochromocytoma, aldosteronism, or overt cortisol excess due to hyperfunction may be observed in patients with adrenal incidentaloma. It has been shown that cortical adenoma is the most frequent tumour type among adrenal incidentalomas ( 5 ), whereas adrenal
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(CSS) can reduce the risk of AI and has a low risk for recurrence in hereditary PHEOs ( 3 , 8 , 12 ). CSS is mostly recommended in patients with (i) bilateral PHEOs, (ii) tumor size <5 cm, or (iii) in case of a mutation that leads to high risk of
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). Increased mortality is due to factors either directly related to the disease such as fatal adrenal crisis (AC), indirectly related such as the increased risk of cardiovascular disease or cancers, or due to complications of glucocorticoid treatment ( 5
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-only (OR 7.7 (2.64–22.32)), and patients with ACS–PA had a five-fold higher risk of having experienced a cardiovascular event than those with ACS-only (OR 5.0 (2.29–11.07)). However, patients with isolated ACS had higher HbA1c levels than patients with ACS
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Faculty of Life Sciences and Medicine, King’s College London, London, UK
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case and other affected family members, considering high-risk features for aggressive disease outlined in the literature including metastatic disease, multifocality, primary tumour size (>5 cm for PCC and >3 cm for PGL), pathogenic variant, extra
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activation of multiple targets of HIF1α. SDHx germline pathological variants are a major cause of familial disease, and SDHB accounts for about 10% of familial cases and has a relatively high risk of metastasis. Genetic testing regimes are in place