An Update on Addison’s Disease

Autor: Greg Kline, Bernhard O. Boehm, Georg Benker, Matthias Gruber, Virginia Kamvissi-Lorenz, Felix Beuschlein, Kai Berens, Sven Diederich, Ana Brennand, David J. Torpy, Stefanie Hahner, Andreas Barthel, Burkhard J. Manfras, Waldemar Kanczkowski, Stefan R. Bornstein
Rok vydání: 2018
Předmět:
Zdroj: Experimental and Clinical Endocrinology & Diabetes. 127:165-175
ISSN: 1439-3646
0947-7349
Popis: Addison’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.
Databáze: OpenAIRE
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