Department of Medicine, Mater Dei Hospital, Msida, Malta
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Department of Medicine, Mater Dei Hospital, Msida, Malta
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(presentation at < 20 years of age) and adult-onset (presentation at ≥ 20 years of age). Data on the population at risk for each calendar year was obtained from the National Statistics Office. For prevalence estimates, patients who were alive and fulfilled the
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NH Shaw JE Karuranga S Huang YD da Rocha Fernandes JD Ohlrogge AW & Malanda B 2018 IDF Diabetes Atlas: global estimates of diabetes prevalence for 2017 and projections for 2045 . Diabetes Research and Clinical Practice 138 271 – 281
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Summary
Short synacthen tests (SST) are frequently used for assessing adrenocorticotropin hormone (ACTH) deficiency. In this study, we present the case of a 53-year-old man receiving immunotherapy for metastatic melanoma, who subsequently developed immune checkpoint inhibitor (ICI)-induced hypothyroidism and was investigated for the presence of ICI-induced hypocortisolaemia on different occasions. Despite two reassuring SSTs, he subsequently developed clinical and biochemical evidence of ACTH deficiency. The ACTH on local measurement was not conclusive in keeping with ICI-related ACTH deficiency but when repeated using an alternative assay confirmed the diagnosis. The case illustrates the evolution of ACTH deficiency and exposes the potential pitfalls of screening strategies. Two important lessons may be gleaned from this case: (i) SSTs can be normal in early cases of secondary adrenal insufficiency, for example, hypophysitis due to adrenal reserve and (ii) when there is mismatch between the clinical and biochemical presentation, the ACTH should be repeated using a different assay.
Learning points
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Short synacthen tests, useful for ruling out adrenalitis and primary adrenal failure, may be normal in early adrenocorticotrophic hormone deficiency and secondary adrenal failure due to residual adrenal reserve.
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If clinical suspicion of adrenal insufficiency persists despite an initial satisfactory SST, it is important to recognise the need for re-assessment of cortisol levels.
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Insulin tolerance test remains a useful tool in the investigation of secondary adrenal insufficiency.
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Levothyroxine replacement may accelerate the metabolism of cortisol in cases of concurrent hypothyroidism and hypoadrenalism, and therefore, glucocorticoid replacement must precede levothyroxine treatment, to avoid adrenal crisis.
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The prevalence of immunotherapy-related endocrinopathies is likely to increase with increasing use of ICI and it is crucial that clinicians are alert to their subtle symptoms.
Garvan Institute of Medical Research, Sydney, NSW, Australia
St Vincent’s Clinical School, University of New South Wales, Sydney, NSW, Australia
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Faculty of Medicine, the University of Queensland, Brisbane, QLD, Australia
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Garvan Institute of Medical Research, Sydney, NSW, Australia
St Vincent’s Clinical School, University of New South Wales, Sydney, NSW, Australia
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a prevalence of approximately 1 in 1000 people, presenting with hormonal hyper or hypofunction, and/or mass effect symptoms ( Daly et al. 2006 , Fernandez et al. 2010 ). In addition, 10–15% of tumours demonstrate ‘aggressive behaviour
University of Adelaide, Adelaide, SA, Australia
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Department of Cardiology, Royal Adelaide Hospital, Adelaide, SA, Australia
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Department of Cardiology, Royal Adelaide Hospital, Adelaide, SA, Australia
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Endocrine and Metabolic Unit, Royal Adelaide Hospital, Adelaide, SA, Australia
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Endocrine and Metabolic Unit, Royal Adelaide Hospital, Adelaide, SA, Australia
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of the pituitary. The prevalence of clinically apparent prolactinoma ranges from 10 to 50 per 100,000 ( Melmed et al. 2011 ). Dopamine agonist (DA) therapy is currently recommended as first-line therapy in prolactinoma. Cabergoline, an ergot
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Division of Biomedical Informatics and Personalized Medicine, University of Colorado School of Medicine, Aurora, Colorado, USA
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epinephrine ( Eisenhofer et al. 2017 ). Given the high prevalence and early-onset of PCC/PGL for those with VHL, guidelines recommend screening for PCC/PGL starting at age 5 with annual lifelong biochemical testing ( VHL Alliance 2015 , Rednam et al. 2017
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studies have shown that the prevalence of clinically diagnosed pituitary tumors ranges from approximately 78–116 cases per 100,000 people ( Daly et al. 2006 , Fontana & Gaillard 2009 , Fernandez et al. 2010 , Agustsson et al. 2015 ). The annual
South Australian Adult Genetics Unit, Royal Adelaide Hospital, Adelaide, Australia
Adelaide Medical School, University of Adelaide, Adelaide, Australia
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-selective agents and DA doses that are 5–10 times lower compared to the Parkinson’s disease setting ( Bancos et al. 2014 ). Recent studies of select hyperprolactinaemic patient samples have since shown ICD prevalence to be 8–25% in DA-treated patients
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. The prevalence of MS in autopsy cases with cerebral infarction and advanced malignancy was significantly higher than in patients without malignant ( Shichijo et al . 2021 ). In a recent study, Mönckeberg MS was associated with the presence of tumor
Clinic for Endocrinology, Diabetes and Metabolic Diseases, University Clinical Center Belgrade, Belgrade, Serbia
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Clinic for Endocrinology, Diabetes and Metabolic Diseases, University Clinical Center Belgrade, Belgrade, Serbia
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pituitary disease had previously undiagnosed pituitary tumors, incidentalomas and clinically relevant pituitary tumors ( Hall et al. 1994 , Burman & Saeger 2006 ). Epidemiological studies show an increased incidence and prevalence of pituitary tumors in
