Abstract
Background: We reviewed the spectrum of clinical presentation of Addison's disease in order to raise awareness of atypical presentations and chronic features.
Methods: All patients with a diagnosis of Addison's disease attending our hospital over a fifteen-year period were identified. A retrospective chart review was carried out. Details of acute and chronic presenting features and initial investigations were recorded.
Results: 12 patients were identified and 11 charts located. There were 7 males and 4 females with a median age at diagnosis of 8.3 years. 7 patients presented with an adrenal crisis following an acute illness. These ranged in severity from a short episode of vomiting to a VF arrest. All of these patients had hyponatraemia (106 to 127 mmol/l); however only 5 had a hyperkalaemia (5.6 to7.6 mmol/l) and only 1 had documented hypoglycaemia. 5 had identifiable chronic features including weight loss, lethargy, postural hypotension, poor school performance and hyperpigmentation. 2 had previously been reviewed at an out-patient department with regard to these problems. 4 of the 11 patients received a diagnosis of Addison's disease without suffering an adrenal crisis. Their presenting features included pigmentation of a scar, unexplained chronic hyponatraemia, family history and associated endocrinopathies. 2 of the male patients had elevated VLCFAs leading to a subsequent diagnosis of adrenoleucodystrophy.
Discussion: Most acute clinicians are aware of the classical presentation of an adrenal crisis. Knowledge of atypical presentations and the chronic features of adrenal insufficiency could facilitate earlier diagnosis and potentially avoid life threatening adrenal crises.
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Bourke, T., Carson, D. 1107 Addison's Disease, a Spectrum of Presentation. Pediatr Res 68 (Suppl 1), 549 (2010). https://doi.org/10.1203/00006450-201011001-01107
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DOI: https://doi.org/10.1203/00006450-201011001-01107