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Addison Disease HELP
Based on 652 articles published since 2009

These are the 652 published articles about Addison Disease that originated from Worldwide during 2009-2019.
+ Citations + Abstracts
Pages: 1 · 2 · 3 · 4 · 5 · 6 · 7 · 8 · 9 · 10 · 11 · 12 · 13 · 14 · 15 · 16 · 17 · 18 · 19 · 20
1 Guideline Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. 2014

Husebye, E S / Allolio, B / Arlt, W / Badenhoop, K / Bensing, S / Betterle, C / Falorni, A / Gan, E H / Hulting, A-L / Kasperlik-Zaluska, A / Kämpe, O / Løvås, K / Meyer, G / Pearce, S H. ·Department of Clinical Science, University of Bergen, Bergen, Norway; Department of Medicine, Haukeland University Hospital, Bergen, Norway. ·J Intern Med · Pubmed #24330030.

ABSTRACT: Primary adrenal insufficiency (PAI), or Addison's disease, is a rare, potentially deadly, but treatable disease. Most cases of PAI are caused by autoimmune destruction of the adrenal cortex. Consequently, patients with PAI are at higher risk of developing other autoimmune diseases. The diagnosis of PAI is often delayed by many months, and most patients present with symptoms of acute adrenal insufficiency. Because PAI is rare, even medical specialists in this therapeutic area rarely manage more than a few patients. Currently, the procedures for diagnosis, treatment and follow-up of this rare disease vary greatly within Europe. The common autoimmune form of PAI is characterized by the presence of 21-hydroxylase autoantibodies; other causes should be sought if no autoantibodies are detected. Acute adrenal crisis is a life-threatening condition that requires immediate treatment. Standard replacement therapy consists of multiple daily doses of hydrocortisone or cortisone acetate combined with fludrocortisone. Annual follow-up by an endocrinologist is recommended with the focus on optimization of replacement therapy and detection of new autoimmune diseases. Patient education to enable self-adjustment of dosages of replacement therapy and crisis prevention is particularly important in this disease. The authors of this document have collaborated within an EU project (Euadrenal) to study the pathogenesis, describe the natural course and improve the treatment for Addison's disease. Based on a synthesis of this research, the available literature, and the views and experiences of the consortium's investigators and key experts, we now attempt to provide a European Expert Consensus Statement for diagnosis, treatment and follow-up.

2 Editorial How best to treat Addison's disease in dogs? 2016

Carr, Anthony P. ·Department of Small Animal Clinical Sciences, Western College of Veterinary Medicine, University of Saskatchewan, Saskatoon S7N 5B4, Canada, e-mail: tony.carr@usask.ca. ·Vet Rec · Pubmed #27450847.

ABSTRACT: -- No abstract --

3 Editorial Adrenal insufficiency with special reference to tuberculosis. 2014

Parameswaran, V. · ·Indian J Tuberc · Pubmed #25509930.

ABSTRACT: -- No abstract --

4 Editorial Clinicians sometimes miss cases of latent primary adrenal insufficiency involving stress-related health changes. 2014

Nishikawa, Tetsuo / Omura, Masao / Saito, Jun / Matsuzawa, Yoko. ·Endocrinology & Diabetes Center, Yokohama Rosai Hospital, Japan. ·Intern Med · Pubmed #24492682.

ABSTRACT: -- No abstract --

5 Editorial How to avoid precipitating an acute adrenal crisis. 2012

Wass, John A H / Arlt, Wiebke. · ·BMJ · Pubmed #23048013.

ABSTRACT: -- No abstract --

6 Editorial Compounding risk for hypoglycemia: type 1 diabetes and Addison's disease. 2012

Barker, Jennifer M. · ·Diabetes Technol Ther · Pubmed #22506859.

ABSTRACT: -- No abstract --

7 Editorial [Full-blown diseases]. 2009

Scriba, P C. · ·Dtsch Med Wochenschr · Pubmed #19746327.

ABSTRACT: -- No abstract --

8 Review An Update on Addison's Disease. 2019

Barthel, Andreas / Benker, Georg / Berens, Kai / Diederich, Sven / Manfras, Burkhard / Gruber, Matthias / Kanczkowski, Waldemar / Kline, Greg / Kamvissi-Lorenz, Virginia / Hahner, Stefanie / Beuschlein, Felix / Brennand, Ana / Boehm, Bernhard O / Torpy, David J / Bornstein, Stefan R. ·Medicover, Bochum, Germany. · Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany. · Medicover, Berlin-Mitte, Germany. · Medicover, Ulm and Neu-Ulm, Germany. · University of Calgary, Calgary, AB,Canada. · Division of Diabetes & Nutritional Sciences, Faculty of Life Sciences & Medicine, King's College London, London, United Kingdom. · Department of Medicine I, Würzburg University Hospital, Würzburg, Germany. · Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, University Hospital, Zürich, Switzerland. · Lee Kong Chian School of Medicine, NTU Nanyang Technological University, Singapore, Singapore. · Endocrine and Metabolic Unit, Royal Adelaide Hospital, University of Adelaide, Adelaide SA, Australia. ·Exp Clin Endocrinol Diabetes · Pubmed #30562824.

ABSTRACT: 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.

9 Review Endocrine Toxicity of Cancer Immunotherapy Targeting Immune Checkpoints. 2019

Chang, Lee-Shing / Barroso-Sousa, Romualdo / Tolaney, Sara M / Hodi, F Stephen / Kaiser, Ursula B / Min, Le. ·Division of Endocrinology, Diabetes, and Hypertension, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. · Department of Medical Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts. ·Endocr Rev · Pubmed #30184160.

ABSTRACT: Immune checkpoints are small molecules expressed by immune cells that play critical roles in maintaining immune homeostasis. Targeting the immune checkpoints cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) and programmed death 1 (PD-1) with inhibitory antibodies has demonstrated effective and durable antitumor activity in subgroups of patients with cancer. The US Food and Drug Administration has approved several immune checkpoint inhibitors (ICPis) for the treatment of a broad spectrum of malignancies. Endocrinopathies have emerged as one of the most common immune-related adverse events (irAEs) of ICPi therapy. Hypophysitis, thyroid dysfunction, insulin-deficient diabetes mellitus, and primary adrenal insufficiency have been reported as irAEs due to ICPi therapy. Hypophysitis is particularly associated with anti-CTLA-4 therapy, whereas thyroid dysfunction is particularly associated with anti-PD-1 therapy. Diabetes mellitus and primary adrenal insufficiency are rare endocrine toxicities associated with ICPi therapy but can be life-threatening if not promptly recognized and treated. Notably, combination anti-CTLA-4 and anti-PD-1 therapy is associated with the highest incidence of ICPi-related endocrinopathies. The precise mechanisms underlying these endocrine irAEs remain to be elucidated. Most ICPi-related endocrinopathies occur within 12 weeks after the initiation of ICPi therapy, but several have been reported to develop several months to years after ICPi initiation. Some ICPi-related endocrinopathies may resolve spontaneously, but others, such as central adrenal insufficiency and primary hypothyroidism, appear to be persistent in most cases. The mainstay of management of ICPi-related endocrinopathies is hormone replacement and symptom control. Further studies are needed to determine (i) whether high-dose corticosteroids in the treatment of ICPi-related endocrinopathies preserves endocrine function (especially in hypophysitis), and (ii) whether the development of ICPi-related endocrinopathies correlates with tumor response to ICPi therapy.

10 Review Latent Adrenal Insufficiency: Concept, Clues to Detection, and Diagnosis. 2018

Yamamoto, Toshihide. · ·Endocr Pract · Pubmed #30084678.

ABSTRACT: In 1855, Thomas Addison described an illness now known as Addison disease (AD) caused by damage to the adrenal cortex and manifesting in weakness, weight loss, hypotension, gastrointestinal disturbances, and brownish pigmentation of the skin and mucous membranes. Corticosteroid supplementation, corticotropin (adrenocorticotropic hormone [ACTH] of medicinal use) test, and anti-adrenal auto-antibodies (AA) have come into use in the 100 years since Addison's death. Following the methodological innovations, 4 disorders which share impaired response to corticotropin in common have been discovered (i.e., partial AD, apigmented adrenal insufficiency [AI], subclinical AI, and the AA-positive state exclusively in subjects proven to have an impaired response to corticotropin). As they are hidden, potentially serious conditions, these disorders are bound together as latent AI (LAI). Diagnosis of AD is often delayed, which may lead to adrenal crisis. If LAI were widely recognized, such delays would not exist and crises would be averted. The 3 existing guidelines do not refer much to LAI patients outside those in acute situations. To address this, information relevant to clinical manifestations and diagnostic tests of LAI was sought in the literature. Signs and symptoms that are useful clues to begin a diagnostic workup are presented for endocrinologists to identify patients with suspected LAI. The utility of 2 corticotropin test protocols is reviewed. To endorse LAI shown by the corticotropin test, monitoring items following corticosteroid supplementation are cited from the guidelines and supplemented with the author's observations. ABBREVIATIONS: AA = anti-adrenal auto-antibodies; Ab = antibodies; ACA = AA detected by immunofluorescence; ACTH = adrenocorticotropic hormone; AD = Addison disease; AI = adrenal insufficiency; DHEA = dehydroepiandrosterone; GC = glucocorticoid; IFA = immunofluorescence assay; LAI = latent AI; LDT = low-dose test; MC = mineralocorticoid; 21OHAb = anti-21-hydroxylase Ab; ST = standard test; URI = upper respiratory infection.

11 Review Salivary cortisol testing: preanalytic and analytic aspects. 2018

Bastin, Pierre / Maiter, Dominique / Gruson, Damien. ·Département des laboratoires, Cliniques universitaires Saint-Luc et Université catholique de Louvain, Bruxelles, Belgique. · Pôle de recherche en endocrinologie, diabète et nutrition, Institut de recherche expérimentale et clinique, Cliniques universitaires Saint-Luc et Université catholique de Louvain, Bruxelles, Belgique. · Département des laboratoires, Cliniques universitaires Saint-Luc et Université catholique de Louvain, Bruxelles, Belgique, Pôle de recherche en endocrinologie, diabète et nutrition, Institut de recherche expérimentale et clinique, Cliniques universitaires Saint-Luc et Université catholique de Louvain, Bruxelles, Belgique. ·Ann Biol Clin (Paris) · Pubmed #29952304.

ABSTRACT: Salivary cortisol assay, described for the first time almost forty years ago, has not been expanding until the last decade. Its simplicity, non-invasiveness and the easy repetition of sampling make it an analytical matrix of interest. Since the publication of the recommendations of the American endocrinology society in 2008, salivary cortisol is recognized as one of the three main tests to screen for Cushing's syndrome. In addition, salivary cortisone, the major metabolite of salivary cortisol, still represents a severe potential interferent but could also be a complementary analyte for indications where evaluation of cortisol secretion is sought. Moreover, in the current context of practices and methods harmonization, the problem of lack of standardization presents also for salivary cortisol. This review briefly develops the three main tests of Cushing's syndrome screening to explain the reasons for integrating the saliva test into this screening. Then we will develop the variables that can influence salivary cortisol from a pre-analytic, physiopathological and finally analytical point of view.

12 Review Acute adrenal crisis and mortality in adrenal insufficiency: Still a concern in 2018! 2018

Hahner, Stefanie. ·Department of medicine I, endocrinology and diabetology, Würzburg University Hospital, Oberdürrbacher street, 6, 97080 Würzburg, Germany. Electronic address: hahner_s@ukw.de. ·Ann Endocrinol (Paris) · Pubmed #29716733.

ABSTRACT: Despite established replacement therapy, mortality in patients suffering from chronic adrenal insufficiency is increasing. This may be partly explained by the fact that lack of adrenal stress hormones impairs the body's capacity to deal adequately with stress situations, resulting in life-threatening adrenal crises. Since many such situations are of rapid onset, concepts that allow for quick response to emergencies are particularly important. Optimal education for patients and relatives, improved awareness on the part of health professionals and the development of new easy-to-use drugs for acute therapy are of prime importance.

13 Review News about the genetics of congenital primary adrenal insufficiency. 2018

Roucher-Boulez, Florence / Mallet-Motak, Delphine / Tardy-Guidollet, Véronique / Menassa, Rita / Goursaud, Claire / Plotton, Ingrid / Morel, Yves. ·Laboratoire de biochimie et biologie moléculaire Grand Est, UM pathologies endocriniennes rénales musculaires et mucoviscidose, groupement hospitalier Est, hospices civils de Lyon, 69677 Bron, France; Université de Lyon, université Claude-Bernard Lyon 1, 69008 Lyon, France. Electronic address: florence.roucher@chu-lyon.fr. · Laboratoire de biochimie et biologie moléculaire Grand Est, UM pathologies endocriniennes rénales musculaires et mucoviscidose, groupement hospitalier Est, hospices civils de Lyon, 69677 Bron, France. · Laboratoire de biochimie et biologie moléculaire Grand Est, UM pathologies endocriniennes rénales musculaires et mucoviscidose, groupement hospitalier Est, hospices civils de Lyon, 69677 Bron, France; Université de Lyon, université Claude-Bernard Lyon 1, 69008 Lyon, France. ·Ann Endocrinol (Paris) · Pubmed #29661472.

ABSTRACT: Primary adrenal insufficiency (PAI) is characterized by impaired production of steroid hormones due to an adrenal cortex defect. This condition incurs a risk of acute insufficiency which may be life-threatening. Today, 80% of pediatric forms of PAI have a genetic origin but 5% have no clear genetic support. Recently discovered mutations in genes relating to oxidative stress have opened the way to research on genes unrelated to the adrenal gland. Identification of causal mutations in a gene responsible for PAI allows genetic counseling, guidance of follow-up and prevention of complications. This is particularly true for stress oxidative anomalies, as extra-adrenal manifestations may occur due to the sensitivity to oxidative stress of other organs such as the heart, thyroid, liver, kidney and pancreas.

14 Review Autoimmune Addison's disease - An update on pathogenesis. 2018

Hellesen, Alexander / Bratland, Eirik / Husebye, Eystein S. ·Department of Clinical Science, University of Bergen, 5021 Bergen, Norway; K.G. Jebsen Senter for Autoimmune Sykdommer, University of Bergen, 5021 Bergen, Norway. · Department of Clinical Science, University of Bergen, 5021 Bergen, Norway; K.G. Jebsen Senter for Autoimmune Sykdommer, University of Bergen, 5021 Bergen, Norway; Department of Medicine, Haukeland University Hospital, 5021 Bergen, Norway; Department of Medicine (Solna), Karolinska Institutet, 17176 Stockholm, Sweden. Electronic address: Eystein.Husebye@uib.no. ·Ann Endocrinol (Paris) · Pubmed #29631795.

ABSTRACT: Autoimmunity against the adrenal cortex is the leading cause of Addison's disease in industrialized countries, with prevalence estimates ranging from 93-220 per million in Europe. The immune-mediated attack on adrenocortical cells cripples their ability to synthesize vital steroid hormones and necessitates life-long hormone replacement therapy. The autoimmune disease etiology is multifactorial involving variants in immune genes and environmental factors. Recently, we have come to appreciate that the adrenocortical cell itself is an active player in the autoimmune process. Here we summarize the complex interplay between the immune system and the adrenal cortex and highlight unanswered questions and gaps in our current understanding of the disease.

15 Review Therapeutic patient education in adrenal insufficiency. 2018

Guignat, Laurence. ·Service des maladies endocriniennes et métaboliques, hôpital Cochin, CHU Paris-centre, centre de référence des maladies rares de la surrénale, 75014 Paris, France. Electronic address: laurence.guignat@aphp.fr. ·Ann Endocrinol (Paris) · Pubmed #29606279.

ABSTRACT: It is essential to encourage patient autonomy in the management of their illness, and notably their participation in treatment education programs; specific programs target avoidance or early preventive treatment of acute adrenal insufficiency, which is a life-threatening complication. Therapeutic patient education is recommended by the two international consensus statements on the management of primary adrenal insufficiency and the French consensus on adrenal insufficiency. Although there is no common international reference framework to date, the objective of the French consensus was to provide a frame of reference to facilitate the development of therapeutic education for patients with adrenal insufficiency. The principal educational objectives were: for the patient to always carry the necessary emergency equipment; be able to identify situations of increased risk and the early signs of adrenal crisis; know how to adjust oral glucocorticoid treatment; be capable of administering hydrocortisone by subcutaneous injection; be able to adjust treatment to different situations (heat, physical exercise, travel); and be able to appropriately use the resources of the healthcare services. Other programs could also be developed to respond to patients' needs and expectations, notably concerning hydrocortisone dose adjustment to avoid overdose in the context of chronic fatigue syndrome.

16 Review Addisonian crisis: assessment and management. 2018

Feeney, C / Buell, K G / Avari, P / Buckley, A / Meeran, K / Rees, D A. ·Specialist Registrar in Endocrinology and Diabetes, Department of Medicine, The Diabetes Centre, Watford General Hospital, Watford WD18 0HB and Honorary Clinical Research Fellow, Faculty of Medicine, Imperial College London, London. · Academic Foundation Year 2 Doctor, Department of Primary Care and Public Health, Imperial College London, London. · Specialist Registrar in Endocrinology and Diabetes and Clinical Research Fellow, Faculty of Medicine, Imperial College London, London. · Specialist Registrar in Intensive Care Medicine, Department of Surgery and Cancer, Imperial College Healthcare NHS Trust, London. · Professor of Endocrinology, Faculty of Medicine, Imperial College London, London. · Reader in Neuroendocrinology, School of Medicine, Cardiff University, Cardiff. ·Br J Hosp Med (Lond) · Pubmed #29528741.

ABSTRACT: -- No abstract --

17 Review The natural history of autoimmune Addison's disease with a non-classical presentation: a case report and review of literature. 2018

Manso, Jacopo / Pezzani, Raffaele / Scarpa, Riccardo / Gallo, Nicoletta / Betterle, Corrado. ·Endocrinology Unit, Department of Medicine (DIMED), University of Padova, Padova, Italy. · Laboratory Medicine, Department of Medicine (DIMED), University of Padova, Padova, Italy. ·Clin Chem Lab Med · Pubmed #29478039.

ABSTRACT: Autoimmune Addison's disease (AAD) is the most frequent cause of adrenocortical insufficiency. The natural history of AAD usually comprises five consecutive stages with the first stage characterized by the increase of plasma renin consistent with the impairment of pars glomerulosa, which is usually the first affected layer of the adrenal cortex. We describe a 19-year-old female with Hashimoto's thyroiditis (HT) who underwent an autoantibody screening due to having the personal and family history of other autoimmune diseases in the absence of relevant clinical manifestations. She was positive for adrenal cortex autoantibodies (ACA) and steroid 21-hydroxylase autoantibodies (21-OH Ab) at high titers. She had increased basal levels of ACTH with normal basal cortisol not responding to ACTH stimulation, reduced levels of dehydroepiandrosterone-sulfate but normal levels of orthostatic renin and aldosterone. This scenario was consistent with a subclinical AAD presenting with first impairments in pars fasciculata and reticularis and conserved pars glomerulosa function. Only subsequently, progressive deficiency in pars glomerulosa function has become evident. Review of the literature showed that there was only one case, reported to date, with a similar atypical natural history of AAD. The strategies for screening for ACA/21-OH Ab in patients with HT are discussed.

18 Review Rare monogenic causes of primary adrenal insufficiency. 2018

Narumi, Satoshi. ·Department of Molecular Endocrinology, National Research Institute for Child Health and Development, Tokyo, Japan. ·Curr Opin Endocrinol Diabetes Obes · Pubmed #29373482.

ABSTRACT: PURPOSE OF REVIEW: Monogenic disorders play significant roles in the pathogenesis of childhood-onset primary adrenal insufficiency (PAI). The most common form of PAI is congenital adrenal hyperplasia (CAH), which includes the enzymatic defects of the steroidogenic pathway. This review focuses on less common forms of monogenic PAI (i.e. non-CAH monogenic PAI) with particular attention on their cause, clinical phenotypes and genetic epidemiology. RECENT FINDINGS: Non-CAH monogenic PAI can be classified into three major categories: first, adrenocorticotropic hormone resistance, second, impaired adrenal redox homeostasis and third, defective organogenesis of the adrenal glands. The clinical phenotypes of the mutation-carrying patients vary depending on the responsible gene, and they are partially explained by the tissue RNA expression patterns. Genetic epidemiology studies conducted in Turkey and Japan showed that about 80% of PAI of unknown cause was monogenic. SUMMARY: Genetic basis of non-CAH monogenic PAI had been less clearly understood than CAH; however, significant advances have been made with use of new research techniques such as next-generation sequencing. Understanding of these rare forms of PAI may contribute to clarifying the physiology and pathology of the adrenal glands.

19 Review Latest Insights on the Etiology and Management of Primary Adrenal Insufficiency in Children. 2017

Güran, Tülay. ·Marmara University Faculty of Medicine, Department of Pediatric Endocrinology and Diabetes, İstanbul, Turkey. ·J Clin Res Pediatr Endocrinol · Pubmed #29280740.

ABSTRACT: Primary adrenal insufficiency (PAI) is a heterogeneous group of disorders characterized by an impaired production of cortisol and other steroid hormones by the adrenal cortex. Most of the causes of PAI in childhood are inherited and monogenic in origin and are associated with significant morbidity and mortality whenever the diagnosis and treatment is delayed. Therefore, early and accurate diagnosis would allow appropriate management for the patients and genetic counselling for the family. Congenital adrenal hyperplasia accounts for most cases of PAI in childhood, followed by abnormalities in the development of the adrenal gland, resistance to adrenocorticotropin hormone action and adrenal destruction. In recent years, the use of genome-wide, next-generation sequencing approaches opened new avenues for identifying novel genetic causes in the PAI spectrum. Understanding the genetic basis of adrenal disorders is key to develop innovative therapies for patients with PAI. The promising progress made in congenital adrenal hyperplasia treatment brings new perspectives for personalized treatment in children with PAI. The aim of this review is to characterize recent advances in the genetics and management of PAI in children.

20 Review [Immune checkpoint inhibitors and endocrinological side effects]. 2017

Jørgensen, Line Bisgaard / Bastholt, Lars / Yderstræde, Knud. ·line.bisgaard.joergensen@rsyd.dk. ·Ugeskr Laeger · Pubmed #29212592.

ABSTRACT: Immune checkpoint inhibitors including anti-cytotoxic T-lymphocyte-associated antigen-4 and anti-programmed cell death-1 have revolutionized cancer therapy but have also induced serious immune-related adverse events including hormonal dysfunction. The objective of this review is to characterize the incidence, clinical presentation, management and prognosis of the endocrine-related adverse events including hypophysitis, thyroid dysfunction and diabetes mellitus. Combination therapy is associated with an increased risk of adverse events. We recommend close monitoring of the hormone levels and glycaemic status during and a year after treatment.

21 Review Group 1. Epidemiology of primary and secondary adrenal insufficiency: Prevalence and incidence, acute adrenal insufficiency, long-term morbidity and mortality. 2017

Chabre, Olivier / Goichot, Bernard / Zenaty, Delphine / Bertherat, Jérôme. ·Service d'endocrinologie diabétologie nutrition, CHU Grenoble-Alpes, CS 10217, boulevard de la Chantourne, 38043 Grenoble cedex 9, France. Electronic address: OlivierChabre@chu-grenoble.fr. · Service de médecine interne, endocrinologie et nutrition, hôpitaux universitaires de Strasbourg, 67098 Strasbourg cedex, France. · Service d'endocrinologie diabétologie nutrition, CHU Grenoble-Alpes, CS 10217, boulevard de la Chantourne, 38043 Grenoble cedex 9, France. · Service des maladies endocriniennes et métaboliques, hôpital Cochin, CHU Paris Centre, 75014 Paris, France. ·Ann Endocrinol (Paris) · Pubmed #29174931.

ABSTRACT: The prevalence of primary adrenal insufficiency is estimated at between 82-144/million, with auto-immunity being the most common cause in adults and genetic causes, especially enzyme defects, being the most common cause in children. The prevalence of secondary adrenal deficiency is estimated to be between 150-280/million. The most frequent occurrence is believed to be corticosteroid-induced insufficiency, despite the incidence of clinically relevant deficiency after cessation of glucocorticoid treatment being widely debated. Data on mortality in adrenal insufficiency are contradictory, with studies from Sweden suggesting a two-fold increase in comparison to the general population, but this is not consistently reported in all studies. However, increased mortality has been consistently reported in young patients, associated with infection and/or acute adrenal insufficiency. Acute adrenal deficiency (adrenal crisis) occurs in primary as well as secondary adrenal insufficiency. Its incidence, mostly determined in retrospective studies, is estimated in Europe at 6-8/100 patients/year. A prospective study reported 0.5 deaths/100 patient-years from adrenal crisis. Long-term morbidity of adrenal insufficiency is not well-established, the increased cardiovascular risk or bone demineralization which are not consistently reported may also be due to a supraphysiological glucocorticoid replacement therapy. However, alteration in quality of life, both in physical and mental health components, has been demonstrated by several studies in both primary and secondary adrenal insufficiency.

22 Review Failed titration of endocrine replacement therapy: diagnostic significance. 2017

Jolobe, Omp. ·Retired Geriatrician, c/o Manchester Medical Society, Manchester M13 9PL. ·Br J Hosp Med (Lond) · Pubmed #29019734.

ABSTRACT: Unsuccessful titration of endocrine replacement therapy may signify the coexistence of an unrecognized disorder which alters the response to replacement therapy. Examples include recurrent hypoglycaemia when type 1 diabetes mellitus coexists with either Addison's disease or coeliac disease. Recurrent Addisonian crisis occurs when Addison's disease coexists with thyrotoxicosis. Conversely, in a patient with Addison's disease, recognition and treatment of coexisting coeliac disease may facilitate a reduction in corticosteroid dosage.

23 Review [Adrenal crisis]. 2017

Burger-Stritt, S / Hahner, S. ·Medizinische Klinik und Poliklinik, Schwerpunkt Endokrinologie und Diabetologie, Universitätsklinikum Würzburg, Oberdürrbacherstr. 6, 97080, Würzburg, Deutschland. · Medizinische Klinik und Poliklinik, Schwerpunkt Endokrinologie und Diabetologie, Universitätsklinikum Würzburg, Oberdürrbacherstr. 6, 97080, Würzburg, Deutschland. hahner_s@ukw.de. ·Internist (Berl) · Pubmed #28815318.

ABSTRACT: Patients with chronic adrenal insufficiency suffer from reduced quality of life and increased mortality. An association between mortality and adrenal crisis is assumed. The frequency of adrenal crisis is about 8/100 patient years. The main causes are infectious disease. Pathophysiology is poorly understood to date. An association with an exaggerated inflammatory response due to a lack of glucocorticoid modulation as well as mineralocorticoid deficiency and diminished adrenomedullary function are discussed. The therapy of adrenal crisis includes prompt parenteral administration of hydrocortisone combined with isotonic saline. To prevent adrenal crisis, patients are equipped with an emergency card and set and educated in glucocorticoid dose adjustment.

24 Review None 2017

Yalcin, Tülay / Schneemann, Markus / Schmid, Beat. ·1 Klinik für Innere Medizin, Kantonsspital Schaffhausen. · 2 Endokrinologie, Klinik für Innere Medizin, Kantonsspital Schaffhausen. ·Praxis (Bern 1994) · Pubmed #28795626.

ABSTRACT: -- No abstract --

25 Review MECHANISMS IN ENDOCRINOLOGY: Update on pathogenesis of primary adrenal insufficiency: beyond steroid enzyme deficiency and autoimmune adrenal destruction. 2017

Flück, Christa E. ·Departments of Pediatrics and Clinical Research, Bern University Children's Hospital Inselspital, University of Bern, Bern, Switzerland. ·Eur J Endocrinol · Pubmed #28450305.

ABSTRACT: Primary adrenal insufficiency (PAI) is potentially life threatening, but rare. In children, genetic defects prevail whereas adults suffer more often from acquired forms of PAI. The spectrum of genetic defects has increased in recent years with the use of next-generation sequencing methods and now has reached far beyond genetic defects in all known enzymes of adrenal steroidogenesis. Cofactor disorders such as P450 oxidoreductase (