Sudden death due to auto-immune Addison's disease in a 12-year-old girl

Sabri, AN; Smith, N.; Busuttil, A.

doi:10.1007/s004140050085

Cited by 22 publications

(20 citation statements)

References 22 publications

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“…At the time of diagnosis, and before the start of treatment for Addison's disease, the patients can be critically ill with increased risk of death (15)(16)(17)(18). The compensating mechanisms of young people are more prone to keep them circulatory stable until they eventually collapse with a crisis; older patients may call for medical assistance at an earlier stage.…”

Section: Discussionmentioning

confidence: 99%

Normal overall mortality rate in Addison's disease, but young patients are at risk of premature death

Erichsen

Løvås

Fougner

et al. 2009

European Journal of Endocrinology

213

171

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Context: Primary adrenal insufficiency (Addison's disease) is a rare autoimmune disease. Until recently, life expectancy in Addison's disease patients was considered normal. Objective: To determine the mortality rate in Addison's disease patients. Design and methods: i) Patients registered with Addison's disease in Norway during 1943-2005 were identified through search in hospital diagnosis registries. Scrutiny of the medical records provided diagnostic accuracy and age at diagnosis. ii) The patients who had died were identified from the National Directory of Residents. iii) Background mortality data were obtained from Statistics Norway, and standard mortality rate (SMR) calculated. iv) Death diagnoses were obtained from the Norwegian Death Cause Registry. Results: Totally 811 patients with Addison's disease were identified, of whom 147 were deceased. Overall SMR was 1.15 (95% confidence intervals (CI) 0.96-1.35), similar in females (1.18 (0.92-1.44)) and males (1.10 (0.80-1.39)). Patients diagnosed before the age of 40 had significantly elevated SMR at 1.50 (95% CI 1.09-2.01), most pronounced in males (2.03 (1.19-2.86)). Acute adrenal failure was a major cause of death; infection and sudden death were more common than in the general population. The mean ages at death for females (75.7 years) and males (64.8 years) were 3.2 and 11.2 years less than the estimated life expectancy. Conclusion: Addison's disease is still a potentially lethal condition, with excess mortality in acute adrenal failure, infection, and sudden death in patients diagnosed at young age. Otherwise, the prognosis is excellent for patients with Addison's disease.

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Section: Discussionmentioning

confidence: 99%

Normal overall mortality rate in Addison's disease, but young patients are at risk of premature death

Erichsen

Løvås

Fougner

et al. 2009

European Journal of Endocrinology

213

171

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“…Addison's disease is defined as any pathology leading to the destruction or atrophy of the adrenal cortex. This results in a failure to synthesize and secrete glucocorticoids (primarily cortisol), mineralocorticoids (primarily aldosterone), and androgens [2,3]. A variety of pathological processes may cause Addison's disease.…”

Section: Discussionmentioning

confidence: 99%

“…The symptoms of chronic primary adrenal insufficiency are varied and nonspecific, including weakness, fatigue, dysphagia, hypotension, nausea, and weight loss [1][2][3][4][5][6][7]. One of the most characteristic signs of Addison's disease is hyperpigmentation of the skin.…”

Section: Discussionmentioning

confidence: 99%

Sudden death due to acute adrenal crisis

Govi

Fersini

Tsokos

2015

Forensic Sci Med Pathol

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“…In one case report, a 12-year-old girl hospitalized following a 2-day history of recurrent vomiting was given the misdiagnosis of diabetic ketoacidosis [5]. She died shortly after hospitalization.…”

Section: Clinical Management Of Adrenal Tumorsmentioning

confidence: 99%

Sudden Death due to Diseases of the Adrenal Glands and Paraganglia

Visonà¹,

Tajana²,

Osculati³

2017

Clinical Management of Adrenal Tumors

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Coroners and pathologists commonly evaluate unexpected deaths due to diseases of the adrenal glands and paraganglia, which are, unfortunately, not rare in their totality. Although cardiac causes are the main cause of sudden death, endocrine conditions can produce sudden, unexpected deaths that need further investigation, especially in younger patients. This chapter focuses on the issue of sudden death due to diseases involving adrenal glands and paraganglia. The main causes of sudden death due to adrenal gland pathology will be examined, paying particular attention to the pathophysiology of sudden death, macroscopic and microscopic characteristics and their correlation with clinical features. These issues are of great interest, especially considering the clinical impact of sudden death and its rarity among patients with adrenal gland diseases. The forensic pathologist's examination is extremely important in determining the cause of death and findings not clinically observable and can contribute to the improvement of the clinical and surgical approach in treating such patients.

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Sudden death due to auto-immune Addison's disease in a 12-year-old girl

Cited by 22 publications

References 22 publications

Normal overall mortality rate in Addison's disease, but young patients are at risk of premature death

Normal overall mortality rate in Addison's disease, but young patients are at risk of premature death

Sudden death due to acute adrenal crisis

Sudden Death due to Diseases of the Adrenal Glands and Paraganglia

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