Abrupt Onset of Adrenal Crisis during Routine Preoperative Examination in a Patient with Unknown Addison's Disease 

1999 ◽  
Vol 90 (1) ◽  
pp. 313-314 ◽  
Author(s):  
Jun Aono ◽  
Kikyo Mamiya ◽  
Wasa Ueda
2008 ◽  
Vol 158 (5) ◽  
pp. 705-709 ◽  
Author(s):  
Ng'weina F. Magitta ◽  
Mikuláš Pura ◽  
Anette S Bøe Wolff ◽  
Peter Vanuga ◽  
Anthony Meager ◽  
...  

BackgroundAutoimmune polyendocrine syndrome type I (APS I) is a monogenic disease affecting endocrine glands and other organs due to mutations of the autoimmune regulator (AIRE) gene. There is a wide variability in clinical phenotypes in patients with APS I, which makes the diagnosis a challenge.ObjectiveTo screen for APS I among Slovakian patients with sporadic Addison's disease and clinical features that raised the suspicion of APS I.MethodsAll 14 exons and exon–intron boundaries of the AIRE gene were sequenced. In addition, autoantibodies specific for Addison's disease and polyendocrine syndromes were assayed.ResultsUsing clinical criteria we identified four patients with APS I in three families. Two patients had a novel missense mutation in exon 2 (c.274C>T, p.R92W) and either the Finnish major mutation (c.769C>T) or the common 13 bp deletion (c.967–979del13bp). APS I was diagnosed in a brother of the latter after his death due to an adrenal crisis. A fourth patient had primary adrenal failure and hypoparathyroidism without AIRE mutations or APS-I specific autoantibodies.ConclusionsFour patients with APS I were found in a Slovakian cohort of Addison patients, although the lack of detectable AIRE mutations and APS I-specific autoantibodies raises uncertainty regarding the pathogenesis in one of the patients. This study demonstrates the merits of screening patients with phenotypic features or autoantibody findings that could indicate APS I, even in adult patients. It is necessary to identify APS I patients in order to provide appropriate treatment and follow-up of the various components of APS I.


2017 ◽  
Vol 45 (4) ◽  
pp. 419-426 ◽  
Author(s):  
Jo Daniels ◽  
Elizabeth Sheils

Background: Addison's disease (AD) is a rare chronic illness caused by adrenocortical insufficiency. Due to the pivotal role of the regulating hormone cortisol in AD, there is a common symptom overlap between the presentation of anxiety and adrenal crisis. Previous literature has identified the prevalence of anxiety in endocrinological disorders, however there is a paucity of research examining the complex interplay between AD and anxiety. Aims: This paper describes a single case study of a patient with severe health anxiety and co-morbid AD. The aims of the study were to establish if standard cognitive behavioural therapy for health anxiety in AD can lead to a reduction in psychological distress, and whether this approach is an effective intervention for the reduction of Emergency Department admissions. Method: A single case design was used, with pre- and post-measures of health anxiety, general anxiety and depression. Data on Emergency Department admissions prior to and following treatment were used to assess change in this domain. Results: Reliable and clinically significant reductions were seen across all measures, from severe to sub-clinical levels. There was a complete amelioration of Emergency Department admissions in the 12 months following completion of treatment. Conclusions: This preliminary study provides a sound rationale for further research into AD complicated by anxiety. Findings support the clinical utility of the cognitive behavioural therapy model for complex presentations of AD, offering a potential treatment option where anxiety is elevated and interfering with self-management and leading to high levels of health service use.


Author(s):  
Georges Aoun

Adrenal crisis (AC) is an unexpected and possibly lethal situation of stressful interventions in patients with Addison’s disease (AD). Despite being rare in dentistry, it is to be noted that evidence indicates that 5-8% of patients with AD necessitate emergency glucocorticoid administration to treat AC annually. For that, dentists must be aware of this condition and be prepared when the clinical signs and symptoms occur.


2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Kundan Jana ◽  
Kalyana Janga ◽  
Sheldon Greenberg ◽  
Amit Gulati

Hyperkalemic paralysis in the setting of acute renal failure can lead to a missed or delayed diagnosis of adrenal insufficiency as the raised potassium can be attributed to the renal failure. Acute kidney injury as the presenting manifestation in an adrenal crisis due to Addison’s disease has been rarely reported in the literature. Here, we present the case of a young 37-year-old male who came with hyperkalemic paralysis and acute renal failure needing emergent hemodialysis. He had no past medical history and no medication history. His hyponatremia, hypotension, and hyperkalemia pointed to a picture of adrenal insufficiency confirmed by undetectable serum cortisol, elevated ACTH, renin, and low aldosterone levels and imaging. Replacement steroid therapy was given, and the patient made a steady recovery. He was advised on the importance of compliance to treatment at discharge to prevent another crisis event. Acute renal failure with hyperkalemia as a presenting manifestation of Addison’s disease can be very misleading. It is especially important to be vigilant of adrenal insufficiency in such patients as the hyperkalemia is resistant to standard therapy of insulin dextrose and can precipitate fatal arrhythmia if treatment is delayed.


2020 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Tahereh Sabaghian ◽  
Minoo Heidari Almasi

Introduction: Chronic kidney disease (CKD) rarely occurs at the same time as endocrine diseases such as adrenal failure. There are some reports of cases with acute kidney failure accompanied by Addison’s disease and adrenal crisis. The studied case was a patient with Addison’s disease referring with manifestations of AKI on CKD and hyperkalemia without hypotension. Case Presentation: This report describes a 34-year-old man with the primary diagnosis of CKD and the subsequent diagnosis of Addison’s disease. Conclusions: Since renal failure is accompanied by hyperkalemia, the diagnosis of adrenal failure will be difficult in the case of no obvious hyponatremia and hypotension. Thus, it is necessary to carefully check the clinical and laboratory symptoms and high clinical suspicions in CKD patients.


Author(s):  
Diana Oliveira ◽  
Adriana Lages ◽  
Sandra Paiva ◽  
Francisco Carrilho

Summary Addison’s disease, or primary adrenocortical insufficiency, is a long-term, potentially severe, rare endocrine disorder. In pregnancy, it is even rarer. We report the case of a 30-year-old pregnant patient with Addison’s disease, referred to Obstetrics-Endocrinology specialty consult at 14 weeks gestation. She had been to the emergency department of her local hospital various times during the first trimester presenting with a clinical scenario suggestive of glucocorticoid under-replacement (nausea, persistent vomiting and hypotension), but this was interpreted as normal pregnancy symptoms. Hydrocortisone dose was adjusted, and the patient maintained regular follow-up. No complications were reported for the remainder of gestation and delivery. Pregnant patients with Addison’s disease should be monitored during gestation and in the peripartum period by multidisciplinary teams. Adjustments in glucocorticoid and mineralocorticoid replacement therapy are often necessary, and monitoring should be based mainly on clinical findings, which becomes increasingly difficult during pregnancy. Patient education and specialized monitoring are key to avoiding complications from under- or over-replacement therapy in this period. Learning points: An increase in glucocorticoid replacement dose is expected to be necessary during pregnancy in a woman with Addison’s disease. Patient education regarding steroid cover and symptoms of acute adrenal crisis are fundamental. Monitoring in this period is challenging and remains mainly clinical. The increase in hydrocortisone dose often obviates the need to increase fludrocortisone dose.


2018 ◽  
Vol 127 (02/03) ◽  
pp. 165-175 ◽  
Author(s):  
Andreas Barthel ◽  
Georg Benker ◽  
Kai Berens ◽  
Sven Diederich ◽  
Burkhard Manfras ◽  
...  

AbstractAddison’s disease – the traditional term for primary adrenal insufficiency (PAI) – is defined as the clinical manifestation of chronic glucocorticoid- and/or mineralocorticoid deficiency due to failure of the adrenal cortex which may result in an adrenal crisis with potentially life-threatening consequences. Even though efficient and safe pharmaceutical preparations for the substitution of endogenous gluco- and mineralocorticoids are established in therapy, the mortality in patients with PAI is still increased and the health-related quality of life (HRQoL) is often reduced.PAI is a rare disease but recent data report an increasing prevalence. In addition to the common “classical” causes of PAI like autoimmune, infectious, neoplastic and genetic disorders, other iatrogenic conditions – mostly pharmacological side effects (e. g., adrenal haemorrhage associated with anticoagulants, drugs affecting glucocorticoid synthesis, action or metabolism and some of the novel anti-cancer checkpoint inhibitors) are contributing factors to this phenomenon.Due to the rarity of the disease and often non-specific symptoms at least in the early stages, PAI is frequently not considered resulting in a delayed diagnosis. Successful therapy is mainly based on adequate patient education as a cornerstone in the prevention and management of adrenal crisis. A focus of current research is in the development of pharmacokinetically optimized glucocorticoid preparations as well as regenerative therapies.


Sign in / Sign up

Export Citation Format

Share Document