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Addison Disease: HELP
Articles from Wales
Based on 4 articles published since 2008

These are the 4 published articles about Addison Disease that originated from Wales during 2008-2019.
+ Citations + Abstracts
1 Review Measuring cortisol in serum, urine and saliva - are our assays good enough? 2017

El-Farhan, Nadia / Rees, D Aled / Evans, Carol. ·1 Biochemistry Department, Royal Gwent Hospital, Newport, UK. · 2 Neuroscience and Mental Health Research Institute, Cardiff University, Cardiff, UK. · 3 Department of Medical Biochemistry and Immunology, University Hospital of Wales, Cardiff, UK. ·Ann Clin Biochem · Pubmed #28068807.

ABSTRACT: Cortisol is a steroid hormone produced in response to stress. It is essential for maintaining health and wellbeing and leads to significant morbidity when deficient or present in excess. It is lipophilic and is transported bound to cortisol-binding globulin (CBG) and albumin; a small fraction (∼10%) of total serum cortisol is unbound and biologically active. Serum cortisol assays measure total cortisol and their results can be misleading in patients with altered serum protein concentrations. Automated immunoassays are used to measure cortisol but lack specificity and show significant inter-assay differences. Liquid chromatography - tandem mass spectrometry (LC-MS/MS) offers improved specificity and sensitivity; however, cortisol cut-offs used in the short Synacthen and Dexamethasone suppression tests are yet to be validated for these assays. Urine free cortisol is used to screen for Cushing's syndrome. Unbound cortisol is excreted unchanged in the urine and 24-h urine free cortisol correlates well with mean serum-free cortisol in conditions of cortisol excess. Urine free cortisol is measured predominantly by immunoassay or LC-MS/MS. Salivary cortisol also reflects changes in unbound serum cortisol and offers a reliable alternative to measuring free cortisol in serum. LC-MS/MS is the method of choice for measuring salivary cortisol; however, its use is limited by the lack of a single, validated reference range and poorly standardized assays. This review examines the methods available for measuring cortisol in serum, urine and saliva, explores cortisol in disease and considers the difficulties of measuring cortisol in acutely unwell patients and in neonates.

2 Article Polyglandular endocrine emergency: lessons from a patient, which a book cannot teach. 2018

Ahmad, Sajjad / Giannopoulou, Angeliki / Owen, Penelope / Kalhan, Atul. ·Diabetes and Endocrinology, Cardiff University, Cardiff, UK. · Department of Diabetes and Endocrinology, Royal Glamorgan Hospital, Llantrisant, UK. ·BMJ Case Rep · Pubmed #30413457.

ABSTRACT: A 30-year-old woman with polyglandular autoimmune type 2 syndrome was found collapsed at home with a cardiac arrest, which required direct current cardioversion. On admission, she was hypothermic, hypotensive and bradycardic. Initial biochemical investigations were consistent with a pre-renal acute kidney injury, metabolic acidosis and a possible sepsis. She had significantly elevated thyroid-stimulating hormone levels on admission with the clinical profile consistent with dual Addisonian and myxoedema crisis. She received intravenous liothyronine and hydrocortisone along with supportive therapy. Echo showed severe left ventricular impairment with apical ballooning although coronary angiogram disclosed nothing abnormal. She made a gradual recovery and was discharged home after 2 weeks. She was diagnosed to have primary autoimmune hypothyroidism, Addison's diseaseand type 1 diabetes and coeliac disease in October 2006, July 2007, May 2010 and September 2016, respectively. Her inability to stick to gluten-free diet at her workplace was considered a significant contributory factor for out-of-hospital cardiac arrest.

3 Article A new ELISA for autoantibodies to steroid 21-hydroxylase. 2018

Del Pilar Larosa, Maria / Chen, Shu / Steinmaus, Nora / Macrae, Hannah / Guo, Liang / Masiero, Stefano / Garelli, Silvia / Costa, Miriam Dalla / Bossowski, Artur / Furmaniak, Jadwiga / Betterle, Corrado / Smith, Bernard Rees. ·FIRS Laboratories, RSR Ltd., Parc Ty Glas, Llanishen, Cardiff, UK. · Unit of Endocrinology, Department of Medical and Surgical Sciences, University of Padua, Padua, Italy. · Department of Pediatrics, Endocrinology and Diabetes with a Cardiology Unit, Medical University in Bialystok, Bialystok, Poland. ·Clin Chem Lab Med · Pubmed #29267164.

ABSTRACT: BACKGROUND: A new ELISA for autoantibodies to steroid 21-hydroxylase (21-OH Ab) is described. METHODS: In the assay test sample autoantibodies form a bridge between 21-OH coated onto the plate well and liquid phase 21-OH-biotin. Bound 21-OH-biotin is detected by the addition of streptavidin peroxidase and colorogenic peroxidase substrate. RESULTS: Of 100 samples from patients with autoimmune Addison's disease, 86 (86%) were positive for 21-OH Ab ELISA whereas 84 (84%) were positive in an immunoprecipitation assay based on 125I-labeled 21-OH. Six (0.6%) of 928 healthy adult blood donors and 1 (2.0%) of 49 adult patients with type 1 diabetes mellitus (T1DM) were positive by ELISA. No samples from adult patients with Graves' disease (GD; n=50), celiac disease (n=29), systemic lupus erythematosis (n=9) or rheumatoid arthritis (n=20) were positive by ELISA. However, 2/51 (3.9%) children with GD, 3/69 (4.3%) children with Hashimoto's thyroiditis (HT) and 3/119 (2.5%) children with T1DM alone or associated with autoimmune thyroid disorders were ELISA positive. CONCLUSIONS: The new assay should be useful for screening patients known to be at increased risk of developing clinical autoimmune Addison's disease, in particular children with HT, GD and/or T1DM.

4 Article Residual adrenal function in autoimmune Addison's disease: improvement after tetracosactide (ACTH1-24) treatment. 2014

Gan, Earn H / MacArthur, Katie / Mitchell, Anna L / Hughes, Beverly A / Perros, Petros / Ball, Stephen G / James, R Andrew / Quinton, Richard / Chen, Shu / Furmaniak, Jadwiga / Arlt, Wiebke / Pearce, Simon H S. ·Institute of Genetic Medicine (E.H.G., K.M., A.L.M., P.P., R.Q., S.H.S.P.), Newcastle University, Newcastle upon Tyne NE1 3BZ, United Kingdom · the Endocrine Unit (E.H.G., A.L.M., P.P., S.G.B., R.A.J., R.Q., S.H.S.P.), Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, United Kingdom · the Centre for Endocrinology, Diabetes, and Metabolism (B.A.H., W.A.), University of Birmingham, Birmingham B15 2TT, United Kingdom · and RSR Ltd (S.C., J.F.), FIRS Laboratories, Cardiff CF4 5DU, United Kingdom. ·J Clin Endocrinol Metab · Pubmed #24170102.

ABSTRACT: CONTEXT: Despite lifelong steroid hormone replacement, there is excess morbidity and mortality associated with autoimmune Addison's disease. In health, adrenocortical cells undergo continuous self-renewal from a population of subcapsular progenitor cells, under the influence of ACTH, suggesting a therapeutic possibility. OBJECTIVE: We aimed to determine whether tetracosactide (synthetic ACTH1-24) could revive adrenal steroidogenic function in autoimmune Addison's disease. DESIGN, SETTING, AND PATIENTS: Thirteen patients (aged 16-65 y) with established autoimmune Addison's disease for more than 1 year were recruited at the Newcastle University Clinical Research Facility. INTERVENTION: The intervention included a 20-week study of regular sc tetracosactide (ACTH1-24) therapy. MAIN OUTCOME MEASURES: Serum and urine corticosteroids were measured during medication withdrawal at baseline and every 5 weeks during the study. RESULTS: Serum cortisol levels remained less than 100 nmol/L in 11 of 13 participants throughout the study. However, two women achieved peak serum cortisol concentrations greater than 400 nmol/L after 10 and 29 weeks of tetracosactide therapy, respectively, allowing withdrawal of corticosteroid replacement. Concurrently, urine glucocorticoid and mineralocorticoid metabolite excretion increased from subnormal to above the median of healthy controls. One of these responders remains well with improving peak serum cortisol (672 nmol/L) 28 months after stopping all treatments. The other responder showed a gradual reduction in serum cortisol and aldosterone over time, and steroid therapy was recommenced after a 28-week period without glucocorticoid replacement. CONCLUSION: This is the first study to demonstrate that established autoimmune Addison's disease is amenable to a regenerative medicine therapy approach.