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Addison Disease HELP
Based on 767 articles published since 2010

These are the 767 published articles about Addison Disease that originated from Worldwide during 2010-2020.
+ 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 Review Pathophysiological Role and Therapeutic Implications of Vitamin D in Autoimmunity: Focus on Chronic Autoimmune Diseases. 2020

Bellan, Mattia / Andreoli, Laura / Mele, Chiara / Sainaghi, Pier Paolo / Rigamonti, Cristina / Piantoni, Silvia / De Benedittis, Carla / Aimaretti, Gianluca / Pirisi, Mario / Marzullo, Paolo. ·Department of Translational Medicine, Università del Piemonte Orientale UPO, 28100 Novara, Italy. · Division of Internal Medicine, "AOU Maggiore della Carità", 28100 Novara, Italy. · CAAD, Centre for Autoimmune and Allergic Diseases, 28100 Novara, Italy. · Rheumatology and Clinical Immunology Unit and Department of Clinical and Experimental Sciences, Spedali Civili and University of Brescia, 25128 Brescia, Italy. · Division of General Medicine, Ospedale S. Giuseppe, I.R.C.C.S. Istituto Auxologico Italiano, 28921 Verbania, Italy. ·Nutrients · Pubmed #32192175.

ABSTRACT: Vitamin D is a pleiotropic secosteroid yielding multiple actions in human physiology. Besides the canonical regulatory activity on bone metabolism, several non-classical actions have been described and the ability of vitamin D to partake in the regulation of the immune system is particularly interesting, though far stronger and convincing evidence has been collected in in vitro as compared to in vivo studies. Whether vitamin D is able to regulate at physiological concentrations the human immune system remains unproven to date. Consequently, it is not established if vitamin D status is a factor involved in the pathogenesis of immune-mediated diseases and if cholecalciferol supplementation acts as an adjuvant for autoimmune diseases. The development of autoimmunity is a heterogeneous process, which may involve different organs and systems with a wide range of clinical implications. In the present paper, we reviewed the current evidences regarding vitamin D role in the pathogenesis and management of different autoimmune diseases.

8 Review Primary antiphospholipid syndrome, Addison disease, and adrenal incidentaloma. 2020

Medina, Gabriela / Jiménez-Arellano, María Pilar / Muñoz-Solís, Andrés / Servín-Torres, Erick / Ramírez-Mendoza, Pablo / Jara, Luis J. ·Translational Research Unit, Hospital de Especialidades, Centro Médico Nacional "La Raza," IMSS, Mexico City, Mexico. · Universidad Veracruzana, Xalapa, Veracruz, Mexico. · Endocrinology Department, Hospital de Especialidades, Centro Médico Nacional "La Raza," IMSS, Mexico City, Mexico. · Surgery Department, Hospital de Especialidades, Centro Médico Nacional "La Raza," IMSS, Mexico City, Mexico. · Pathology Department, Hospital de Especialidades, Centro Médico Nacional "La Raza," IMSS, Mexico City, Mexico. · Direction of Education and Research, Hospital de Especialidades, Centro Médico Nacional "La Raza," IMSS, Seris y Zaachila S/N Col. La Raza CP, 02990, Mexico City, Mexico. luis_jara_quezada@hotmail.com. ·Clin Rheumatol · Pubmed #32146613.

ABSTRACT: Primary adrenal failure comprises an insufficient production of mineralocorticoids and glucocorticoids in the adrenal cortex. A rare manifestation of antiphospholipid syndrome (APS) is adrenal failure. The majority of patients with adrenal involvement in APS develop an irreversible cortisol deficiency and atrophy of the adrenal glands. Adrenal incidentalomas are adrenal masses larger than 1 cm that are discovered in the course of diagnostic evaluation or treatment for another medical condition. Its prevalence is calculated in 1.5-9% of individuals. We describe an exceptional case of a 23-year-old male patient with APS with persistent high levels of antiphospholipid antibodies (aPL) from the time of diagnosis, who developed Addison's disease as a manifestation of APS with atrophy of the adrenal glands, in whom an adrenal incidentaloma was developed later and was corroborated as an aldosterone-producing adenoma. Currently, the patient is asymptomatic and without manifestations of tumor recurrence. The protumoral effect of elevated and persistent aPL is discussed.

9 Review Adrenal insufficiency: Physiology, clinical presentation and diagnostic challenges. 2020

Martin-Grace, Julie / Dineen, Rosemary / Sherlock, Mark / Thompson, Christopher J. ·Academic Department of Endocrinology, Beaumont Hospital, Dublin 9, Ireland; Royal College of Surgeons in Ireland, 123 St Stephens' Green, Dublin 2, Ireland; Beacon Hospital, Sandyford, Dublin 18, Ireland. · Academic Department of Endocrinology, Beaumont Hospital, Dublin 9, Ireland; Royal College of Surgeons in Ireland, 123 St Stephens' Green, Dublin 2, Ireland. · Academic Department of Endocrinology, Beaumont Hospital, Dublin 9, Ireland; Royal College of Surgeons in Ireland, 123 St Stephens' Green, Dublin 2, Ireland. Electronic address: Christhompson@beaumont.ie. ·Clin Chim Acta · Pubmed #32035851.

ABSTRACT: Adrenal insufficiency (AI) is a serious condition, which can arise from pathology affecting the adrenal gland itself (primary adrenal insufficiency, PAI), hypothalamic or pituitary pathology (secondary adrenal insufficiency, SAI), or as a result of suppression of the hypothalamic-pituitaryadrenal (HPA) axis by exogenous glucocorticoid therapy (tertiary adrenal insufficiency, TAI). AI is associated with an increase in morbidity and mortality and a reduction in quality of life. In addition, the most common cause of PAI, autoimmune adrenalitis, may be associated with a variety of other autoimmune disorders. Untreated AI can present with chronic fatigue, weight loss and vulnerability to infection. The inability to cope with acute illness or infection can precipitate life-threatening adrenal crisis. It is therefore a critical diagnosis to make in a timely fashion, in order to institute appropriate management, aimed at reversing chronic ill health, preventing acute crises, and restoring quality of life. In this review, we will describe the normal physiology of the HPA axis and explain how knowledge of the physiology of this axis helps us understand the clinical presentation of AI, and forms the basis for the biochemical investigations which lead to the diagnosis of AI.

10 Review Renal involvement in adrenal insufficiency (Addison disease): can we always recognize it? 2020

Fofi, Claudia / Maresca, Barbara / Altieri, Silvia / Menè, Paolo / Festuccia, Francescaromana. ·Nephrology Unit, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, II Faculty of Medicine, "Sapienza" University of Rome, Via di Grottarossa 1035-39, 00189, Rome, Italy. claudiafofi2@gmail.com. · Nephrology Unit, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, II Faculty of Medicine, "Sapienza" University of Rome, Via di Grottarossa 1035-39, 00189, Rome, Italy. ·Intern Emerg Med · Pubmed #31625077.

ABSTRACT: Addison disease is due to the destruction or dysfunction of the entire adrenal cortex. Nowadays, the causes of adrenal insufficiency are autoimmune disease for 70-90% and tuberculosis for 7-20%. Many typical signs and symptoms, such as hyponatremia, hyperkalaemia, or renal insufficiency can represent the reasons for a nephrology consultation, especially in conditions of urgency, and they can easily be confused with other causes. Moreover, the fact that in a short time range we have diagnosed the three cases described as a guide in this review, has aroused our attention as nephrologists on a disease in which we have probably already encountered but without recognizing it. The blood tests showed in all three patients severe electrolyte disorders and acute renal failure which will be discussed in their physiopathogenetic mechanisms. In a peculiar way, these alterations were not controlled with repolarizing solutions, fluid replacement and increased volemia, but only after steroid administration. In conclusion, in this review all the known pathogenic mechanisms causing disorders of nephrological interest in adrenal insufficiency are discussed.

11 Review Primary adrenal insufficiency: New genetic causes and their long-term consequences. 2020

Buonocore, Federica / Achermann, John C. ·Genetics & Genomic Medicine, UCL Great Ormond Street Institute of Child Health, University College London, London, UK. ·Clin Endocrinol (Oxf) · Pubmed #31610036.

ABSTRACT: Primary adrenal insufficiency (PAI) is a potentially life-threatening condition that requires urgent diagnosis and treatment. Whilst the most common causes are congenital adrenal hyperplasia (CAH) in childhood and autoimmune adrenal insufficiency in adolescence and adulthood, more than 30 other physical and genetics cause of PAI have been reported. Reaching a specific diagnosis can have implications for management and for monitoring associated features, as well as for counselling families about recurrence risk in siblings and relatives. Here, we describe some recent insights into the genetics of adrenal insufficiency and associated molecular mechanisms. We discuss (a) the role of the nuclear receptors DAX-1 (NR0B1) and steroidogenic factor-1 (SF-1, NR5A1) in human adrenal and reproductive dysfunction; (b) multisystem growth restriction syndromes due to gain-of-function in the growth repressors CDKN1C (IMAGE syndrome) and SAMD9 (MIRAGE syndrome), or loss of POLE1; (c) nonclassic forms of STAR and P450scc/CYP11A1 insufficiency that present with a delayed-onset adrenal phenotype and represent a surprisingly prevalent cause of undiagnosed PAI; and (d) a new sphingolipidosis causing PAI due to defects in sphingosine-1-phosphate lyase-1 (SGPL1). Reaching a specific diagnosis can have life-long implications for management. In some situations, milder or nonclassic forms of these conditions can first present in adulthood and may have been labelled, "Addison's disease."

12 Review Epidemiology, pathogenesis, and diagnosis of Addison's disease in adults. 2019

Betterle, C / Presotto, F / Furmaniak, J. ·Endocrine Unit, Department of Medicine (DIMED), University of Padova, Via Ospedale Civile 105, 35128, Padua, Italy. · Endocrine Unit, Department of Medicine (DIMED), University of Padova, Via Ospedale Civile 105, 35128, Padua, Italy. fabio.presotto@unipd.it. · Unit of Internal Medicine, Ospedale dell'Angelo, via Paccagnella 11, 30174, Mestre-Venice, Italy. fabio.presotto@unipd.it. · FIRS Laboratories, Llanishen, Cardiff, UK. ·J Endocrinol Invest · Pubmed #31321757.

ABSTRACT: BACKGROUND: Addison's disease (AD) is a rare disorder and among adult population in developed countries is most commonly caused by autoimmunity. In contrast, in children genetic causes are responsible for AD in the majority of patients. PURPOSE: This review describes epidemiology, pathogenesis, genetics, natural history, clinical manifestations, immunological markers and diagnostic strategies in patients with AD. Standard care treatments including the management of patients during pregnancy and adrenal crises consistent with the recent consensus statement of the European Consortium and the Endocrine Society Clinical Practice Guideline are described. In addition, emerging therapies designed to improve the quality of life and new strategies to modify the natural history of autoimmune AD are discussed. CONCLUSIONS: Progress in optimizing replacement therapy for patients with AD has allowed the patients to lead a normal life. However, continuous education of patients and health care professionals of ever-present danger of adrenal crisis is essential to save lives of patients with AD.

13 Review Adrenal crisis: prevention and management in adult patients. 2019

Dineen, Rosemary / Thompson, Christopher J / Sherlock, Mark. ·Academic Department of Endocrinology, Beaumont Hospital and the Royal College of Surgeons in Ireland, Dublin, Ireland. · Department of Endocrinology, Beaumont Hospital and the Royal College of Surgeons in Ireland, Dublin 9, Ireland. ·Ther Adv Endocrinol Metab · Pubmed #31223468.

ABSTRACT: Adrenal crisis is an acute life-threatening emergency contributing to the excess mortality that is reported in patients with adrenal insufficiency. The incidence of adrenal crisis is estimated to be 8 per 100 patient years in patients with adrenal insufficiency. Patients with adrenal crisis present systemically unwell with nonspecific signs and symptoms often leading to misdiagnosis and delayed treatment. An adrenal crisis may be the first presentation of adrenal insufficiency or can occur in patients who have been established on glucocorticoid replacement therapy. Infections are the major precipitating factor, but other causes include physical stress such as a surgical procedure or trauma, forgetting or discontinuing glucocorticoid therapy, pronounced physical activity, and psychological stress. The emergency treatment involves prompt recognition and administration of parenteral hydrocortisone, rehydration and management of electrolyte abnormalities. Prevention is centred around patient education. All patients should be educated on stress dosing and parenteral glucocorticoid administration. They should carry a steroid dependency alert card and wear a medical alert bracelet or similar identification. Despite many improvements in the management of patients with adrenal insufficiency, adrenal crisis continues to occur and represents a major source of morbidity, mortality and distress for patients. Improved patient and clinician education and measures to facilitate parenteral hydrocortisone self-administration in impending crisis are central to the management of this life-threatening event.

14 Review Treatment of gynecomastia with prednisone: case report and literature review. 2019

Jin, Yansheng / Fan, Maoxiao. ·contributed equally to this work. ·J Int Med Res · Pubmed #30958070.

ABSTRACT: This study aimed to report a unique case of primary adrenal insufficiency that was accompanied by painful gynecomastia, which was resolved by treatment with prednisone. Enlargement of the left breast with continuous weakness and generalized nausea in a male was discovered 3 months before admission. Magnetic resonance imaging of the brain was normal 1 month before presentation. A physical examination revealed that the diameter of the left breast was 5 cm and the height was 3 cm. Laboratory investigations revealed hyponatremia, with a low serum cortisol level and an elevated prolactin level. Hyperprolactinemia was suspected because of adrenal deficiency that was directly or indirectly associated with increased prolactin levels. Thus, a diagnosis of hyperprolactinemia was confirmed. Ultrasonography of the left breast showed glandular tissue hyperplasia. In the present study, treating adrenal insufficiency with prednisone relieved both gynecomastia and hyponatremia. However, gynecomastia regression and hyponatremia resolution were observed when prednisone was stopped. Gynecomastia completely resolved by re-administering prednisone. Therefore, treating the underlying disease is essential so that prednisone can be given in a timely manner.

15 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.

16 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.

17 Review The potential role for infections in the pathogenesis of autoimmune Addison's disease. 2019

Hellesen, A / Bratland, E. ·Department of Clinical Science, University of Bergen, Bergen, Norway. · K.G. Jebsen Senter for Autoimmune Sykdommer, University of Bergen, Bergen, Norway. ·Clin Exp Immunol · Pubmed #30144040.

ABSTRACT: Autoimmune Addison's disease (AAD), or primary adrenocortical insufficiency, is a classical organ-specific autoimmune disease with 160 years of history. AAD is remarkably homogeneous with one major dominant self-antigen, the cytochrome P450 21-hydroxylase enzyme, which is targeted by both autoantibodies and autoreactive T cells. Like most autoimmune diseases, AAD is thought to be caused by an unfortunate combination of genetic and environmental factors. While the number of genetic associations with AAD is increasing, almost nothing is known about environmental factors. A major environmental factor commonly proposed for autoimmune diseases, based partly on experimental and clinical data and partly on shared pathways between anti-viral immunity and autoimmunity, is viral infections. However, there are few reports associating viral infections to AAD, and it has proved difficult to establish which immunological processes that could link any viral infection with the initiation or progression of AAD. In this review, we will summarize the current knowledge on the underlying mechanisms of AAD and take a closer look on the potential involvement of viruses.

18 Review Factors impacting on the action of glucocorticoids in patients receiving glucocorticoid therapy. 2019

Dineen, Rosemary / Stewart, Paul M / Sherlock, Mark. ·Academic Department of Endocrinology, Beaumont Hospital and the Royal College of Surgeons in Ireland, Dublin, Ireland. · Department of Endocrinology, University of Leeds, Leeds, UK. ·Clin Endocrinol (Oxf) · Pubmed #30120786.

ABSTRACT: Glucocorticoids (GCs) are steroid hormones, which are essential for life. They are secreted by the adrenal cortex under the control of the hypothalamic-pituitary-adrenal (HPA) axis. Glucocorticoids are essential for the normal function of most organ systems and, in both, excess and deficiency can lead to significant adverse consequences. Adrenal insufficiency (AI) is a rare, life-threatening disorder characterized by insufficient production of corticosteroid hormones. Primary AI is defined by the inability of the adrenal cortex to produce sufficient amounts of glucocorticoids and/or mineralocorticoids despite normal or increased adrenocorticotropin hormone (ACTH). Secondary AI is adrenal hypofunction due to insufficient amount of ACTH produced by the pituitary gland. Conventional treatment of both primary and secondary adrenal insufficiencies involves lifelong glucocorticoid replacement therapy. The role of cortisol deficiency and the impact of hydrocortisone replacement on morbidity and mortality in this patient group are under increasing scrutiny. Established glucocorticoid replacement regimens do not completely mirror endogenous hormonal production, and their monitoring to ensure optimum therapy is hampered by the lack of reliable biomarkers of hormone sufficiency. A further confounding issue is the tissue-specific regulation of glucocorticoid through the two isozymes of 11β-hydroxysteroid dehydrogenase (11β-HSD) with research focusing on the role of this prereceptor regulation in the development of adverse metabolic features in patients. This review defines the factors influencing glucocorticoid action in patients with adrenal insufficiency receiving glucocorticoid therapy.

19 Review Primary adrenal insufficiency in children: Diagnosis and management. 2018

Kirkgoz, Tarik / Guran, Tulay. ·Marmara University School of Medicine, Department of Paediatric Endocrinology and Diabetes, Istanbul, Turkey. Electronic address: tarikkrkgz@gmail.com. · Marmara University School of Medicine, Department of Paediatric Endocrinology and Diabetes, Istanbul, Turkey. Electronic address: tulayguran@yahoo.com. ·Best Pract Res Clin Endocrinol Metab · Pubmed #30086866.

ABSTRACT: Primary adrenal insufficiency (PAI) is a life-threatening disorder of adrenal cortex which is characterized by deficient biosynthesis of glucocorticoids, with or without deficiency in mineralocorticoids and adrenal androgens. Typical manifestations of primary adrenal insufficiency include hyperpigmentation, hypotension, hypoglycaemia, hyponatremia with or without hyperkalemia that are generally preceded by nonspecific symptoms at the onset. Recessively inherited monogenic disorders constitute the largest group of primary adrenal insufficiency in children. The diagnostic process of primary adrenal insufficiency includes demonstration of low cortisol concentrations along with high plasma ACTH and identifying the cause of the disorder. Specific molecular diagnosis is achieved in more than 80% of children with PAI by detailed clinical and biochemical characterization integrated with advanced molecular tools. Hormone replacement therapy determined on the type and the severity of deficient adrenocortical hormones is the mainstay of treatment. Optimized methods of steroid hormone delivery, improved monitoring of hormone replacement along with intensive education of patients and families on the rules during intercurrent illness and stress will significantly reduce the morbidity and mortality associated with primary adrenal insufficiency.

20 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.

21 Review Management of hypoadrenocorticism (Addison's disease) in dogs. 2018

Lathan, Patty / Thompson, Ann L. ·College of Veterinary Medicine, Mississippi State University, Starkville, MS, USA, lathan@cvm.msstate.edu. · School of Veterinary Science, The University of Queensland, Gatton, Queensland, Australia. ·Vet Med (Auckl) · Pubmed #30050862.

ABSTRACT: Hypoadrenocorticism (HOAC; Addison's disease) is an endocrine condition seen in small animal practice. Dogs with this disease can present in a variety of ways from acute hypovolemic collapse to vague, chronic, waxing, and waning clinical signs. In the most common form of this disease, animals have both mineralocorticoid and glucocorticoid deficiency, resulting in hyponatremia and hyperkalemia, and signs of cortisol deficiency. The etiology may be immune-mediated destruction of the adrenal cortex, drug-induced adrenocortical necrosis (mitotane), enzyme inhibition (trilostane), or infiltrative processes such as neoplastic or fungal disease. Much less commonly, dogs have signs of cortisol deficiency, but no electrolyte changes. This is referred to as atypical HOAC. The veterinarian needs to have a clinical suspicion for HOAC to make a diagnosis in a timely manner. Treatment of dogs with an acute presentation prioritizes correcting the hypovolemia, hyperkalemia, acidosis, and hypoglycemia. Fluid therapy addresses most of these issues, but other directed therapies may be required in the most severe cases. For chronic management, all patients with Addison's disease will require replacement of glucocorticoids (usually prednisone), and most patients require replacement of mineralocorticoids with either desoxycorticosterone pivalate or fludrocortisone. Atypical Addisonians do not require mineralocorticoid supplementation, but electrolytes should be monitored in case the need arises in the future. The prognosis for dogs treated for HOAC promptly and appropriately is excellent; most patients die from other diseases. However, if the diagnosis is missed, patients may die as a consequence of HOAC. Thus, knowledge of the hallmarks of Addison's disease is imperative.

22 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.

23 Review [CME: Adrenal Insufficiency]. 2018

Fischli, Stefan. ·1 Abteilung Endokrinologie, Diabetologie und Klinische Ernährung, Departement Innere Medizin, Luzerner Kantonsspital. ·Praxis (Bern 1994) · Pubmed #29921185.

ABSTRACT: CME: Adrenal Insufficiency Abstract. Patients suffering from adrenal insufficiency (AI) often present with unspecific symptoms. Therefore, the diagnosis of AI, a potential life-threatening condition, can be missed. Lab tests, especially the ACTH-stimulation test, play a crucial role in the diagnosis of AI. According to the different etiologies, AI can be grouped into a primary (adrenal) or central (hypothalamic or pituitary, respectively) form. However, the most common cause is the treatment with glucocorticoids, which can lead to central AI. Patients suffering from AI are given hydrocortisone. The chronic replacement dose should be as low as possible, in acute situations, a rapid and sufficient increase of the hydrocortisone dose is necessary to prevent adrenal crisis. Replacement therapy with fludrocortisone is only necessary in patients with primary AI.

24 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.

25 Review Quality of life in patients with adrenal disease: A systematic review. 2018

Ho, Winnie / Druce, Maralyn. ·Centre for Endocrinology, Barts and the London School of Medicine & Dentistry, William Harvey Research Institute, Queen Mary University of London, London, UK. · Department of Endocrinology, Western Health, Melbourne, Vic., Australia. ·Clin Endocrinol (Oxf) · Pubmed #29672878.

ABSTRACT: BACKGROUND: Evaluating the patient with adrenal disease is challenging due to the lack of precise clinical and biochemical parameters for disease control. Quality of life (QOL) evaluation aims to measure the patient's subjective experience. OBJECTIVE: To describe how QOL is defined and measured in adrenal disease, critically appraise the use of QOL tools in published literature, discuss the implications of these findings and provide direction for further research in this field. MATERIALS AND METHODS: We searched the Cochrane library, EMBASE, Google Scholar, PsycINFO, PubMed, Web of Science databases to identify only primary studies where self-reported QOL was measured as a parameter in adults with confirmed adrenal disease, and results presented in English. Key data were independently extracted from each study and adherence to reporting guidelines evaluated. RESULTS: A total of 117 studies involving 13 717 subjects were included. The vast majority of studies did not define QOL. The most common approach was to combine generic and domain-specific tools, although disease-specific tools are increasingly being used. Adherence to reporting guidelines was variable. A narrative synthesis of the findings was performed. CONCLUSION: We present the first systematic review of QOL in adrenal disease. Quality of life is reduced in patients with adrenal disease, irrespective of adrenal hyperfunction or hypofunction. Quality of life improved with therapy but was not completely reversed despite biochemical remission. Authors should adhere to consistent reporting practices which are interpretable by clinicians. Further research is required to explain the mechanisms driving impaired QOL and value of QOL evaluations in the clinical context.