16,392 results match your criteria Adrenal Insufficiency & Adrenal Crisis


Retention of aberrant cortisol secretion in a patient with bilateral macronodular adrenal hyperplasia after unilateral adrenalectomy.

Ther Clin Risk Manag 2019 27;15:337-342. Epub 2019 Feb 27.

Division of Nephrology, Hypertension and Endocrinology, Department of Internal Medicine, Nihon University School of Medicine, Tokyo, Japan,

Aberrant cortisol secretion responses after exogenous stimuli such as upright posture, eating a mixed meal or receiving agents influencing aberrant G-protein-coupled receptors in adrenal glands, are often observed in patients with bilateral macronodular adrenal hyperplasia (BMAH). However, little is known about whether this aberrant response is retained after unilateral adrenalectomy. Here, we describe a 61-year-old postmenopausal Japanese woman with unsatisfactorily controlled hypertension who was referred to us for further investigation due to her pre-obesity characteristics (body mass index 28. Read More

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http://dx.doi.org/10.2147/TCRM.S196171DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6398404PMC
February 2019

Comparative assessment of hypothalamic-pituitary-adrenal axis suppression secondary to intrabursal injection of different glucocorticoids: a pilot study.

J Endocrinol Invest 2019 Mar 15. Epub 2019 Mar 15.

Division of Diagnostic and Interventional Radiology, IRCCS Rizzoli Orthopaedic Institute, Bologna, Italy.

Background: Hypothalamic-pituitary-adrenal axis (HPAA) suppression is the most common and dangerous, although often unrecognized and untreated, side effect of glucocorticoid administration. The risk and duration depend both on patient and treatment characteristics. High-performance liquid chromatography-tandem mass spectrometry (HPLC-MS/MS) currently represents the gold standard method to evaluate the metabolism of endogenous and exogenous steroids. Read More

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http://link.springer.com/10.1007/s40618-019-01033-6
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http://dx.doi.org/10.1007/s40618-019-01033-6DOI Listing
March 2019
1 Read

A rare cause of postpartum acute hyponatremia.

Endocrinol Diabetes Metab Case Rep 2019 Mar 15;2019. Epub 2019 Mar 15.

Department of Internal Medicine F, Soroka University Medical Center, Beer Sheva, Israel.

A previously healthy 24-year-old female underwent an emergent caesarean section without a major bleeding described. During the first post-operative days (POD) she complained of fatigue, headache and a failure to lactate with no specific and conclusive findings on head CT. On the following days, fever rose with a suspicion of an obstetric surgery-related infection, again with no evidence to support the diagnosis. Read More

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http://dx.doi.org/10.1530/EDM-18-0124DOI Listing

Tuberculous Addison's disease with increased hydrocortisone requirements due to administration of rifampicin.

BMJ Case Rep 2019 Mar 14;12(3). Epub 2019 Mar 14.

Metabolism and Endocrinology, Kanto Central Hospital of the Mutual Aid Association of Public School Teachers, Tokyo, Japan.

A 58-year-old man was referred to our hospital for darkened skin, general fatigue and weight loss. His adrenocorticotropic hormone and cortisol levels indicated a primary adrenal insufficiency. Fluorodeoxyglucose positron emission tomography/CT showed bilateral enlargement of the adrenal glands, with fluorodeoxyglucose accumulation. Read More

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http://dx.doi.org/10.1136/bcr-2018-228293DOI Listing

Opioid induced adrenal insufficiency: what is new?

Authors:
Diane Donegan

Curr Opin Endocrinol Diabetes Obes 2019 Mar 11. Epub 2019 Mar 11.

Department of Endocrinology and Diabetes, Indiana University School of Medicine, Indiana, USA.

Purpose Of Review: Despite the declaration of an opioid epidemic, opioid use remains prevalent. Side-effects of chronic opioid use continue to be problematic. Opioid-induced endocrinopathies have been well documented, yet opioid-induced adrenal insufficiency (OIAI) remains underappreciated. Read More

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http://dx.doi.org/10.1097/MED.0000000000000474DOI Listing

Adrenal insufficiency in neonates undergoing cardiopulmonary bypass and postoperative hypothalamic-pituitary-adrenal function after prophylactic glucocorticoids.

J Perinatol 2019 Mar 12. Epub 2019 Mar 12.

Division of Critical Care Medicine, Department of Pediatrics, University of Texas Southwestern Medical School, Dallas, TX, USA.

Objectives: Determine incidence of preoperative adrenal insufficiency in neonates >35 weeks gestation with congenital heart disease undergoing cardiothoracic surgery with bypass and effects of prophylactic methylprednisolone on postoperative hypothalamic-pituitary-adrenal function and hemodynamic stability.

Design: Prospective observational study in 36 neonates with preoperative adrenocorticotrophic hormone stimulation tests and serial total cortisol and adrenocorticotrophic hormone measurements before and after surgery. Data analyses: analysis of variance and regression. Read More

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http://dx.doi.org/10.1038/s41372-019-0344-7DOI Listing
March 2019
1 Read

Evaluation of a low-dose desoxycorticosterone pivalate treatment protocol for long-term management of dogs with primary hypoadrenocorticism.

J Vet Intern Med 2019 Mar 13. Epub 2019 Mar 13.

Clinic for Small Animal Internal Medicine, Vetsuisse Faculty, University of Zurich, Zurich, Switzerland.

Background: Lowering the dose of desoxycorticosterone pivalate (DOCP) for the treatment of dogs with primary hypoadrenocorticism (PH) decreases costs and could lead to increased owner motivation to treat their affected dogs.

Objective: To evaluate the efficacy of a low-dose DOCP treatment protocol in dogs with PH.

Animals: Prospective study, 17 client-owned dogs with naturally occurring PH (12 newly diagnosed, 5 previously treated with fludrocortisone acetate [FC]). Read More

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http://doi.wiley.com/10.1111/jvim.15475
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http://dx.doi.org/10.1111/jvim.15475DOI Listing
March 2019
1 Read

A Systematic Review and Meta-Analysis of Endocrine-Related Adverse Events Associated with Immune Checkpoint Inhibitors.

Horm Metab Res 2019 Mar 12;51(3):145-156. Epub 2019 Mar 12.

Department of Endocrinology, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.

Monoclonal antibodies targeting cytotoxic T-lymphocyte antigen-4 (CTLA-4), programed cell death 1 (PD-1), or its ligand (PD-L1) have become the mainstay for advanced malignancies. The incidence of endocrine adverse events provoked by these immune checkpoint inhibitors (ICI) is based on data from randomized controlled trials, which have their drawbacks. PubMed was searched through August 22nd, 2017, by 2 reviewers independently (J. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/a-0843-3366
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http://dx.doi.org/10.1055/a-0843-3366DOI Listing
March 2019
5 Reads

Pitfalls in the interpretation of the cosyntropin stimulation test for the diagnosis of adrenal insufficiency.

Curr Opin Endocrinol Diabetes Obes 2019 Mar 6. Epub 2019 Mar 6.

Department of Medicine, Division of Endocrinology, Diabetes, & Metabolism, University of Florida, Gainesville, Florida, USA.

Purpose Of Review: Adrenal insufficiency is a rare disease characterized by cortisol deficiency. The evaluation of patients suspected of having adrenal insufficiency can be challenging because of the rarity of the disease and limitations in the biochemical assessment of the cortisol status by either basal or dynamic testing [adrenocorticotropic hormone (ACTH) stimulation test]. Prompt and adequate diagnosis is of paramount importance to avoid adverse outcomes. Read More

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http://dx.doi.org/10.1097/MED.0000000000000473DOI Listing

Management of Endocrine Emergencies in the ICU.

Authors:
Judith Jacobi

J Pharm Pract 2019 Mar 10:897190019834771. Epub 2019 Mar 10.

1 Pharmacy Department, Indiana University Health Methodist Hospital, Indianapolis, IN, USA.

Endocrine emergencies are frequent in critically ill patients and may be the cause of admission or can be secondary to other critical illness. The ability to anticipate endocrine abnormalities such as adrenal excess or , hypothyroidism, can mitigate their duration and severity. Hyperglycemic crisis may trigger hospital and intensive care unit (ICU) admission and may be life threatening. Read More

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http://dx.doi.org/10.1177/0897190019834771DOI Listing

Low-dose corticosteroids in septic shock: Has the pendulum shifted?

Am J Health Syst Pharm 2019 Mar 9. Epub 2019 Mar 9.

University of Saint Joseph School of Pharmacy and Physician Assistant Studies, Hartford, CT, and Department of Pharmacy, Saint Francis Hospital and Medical Center, Hartford, CT.

Purpose: The utility of low-dose corticosteroids in septic shock is reviewed.

Summary: Low-dose corticosteroids are suggested as treatment for septic shock patients who remain hemodynamically unstable despite adequate fluid resuscitation and vasopressor therapy. However, the risks and benefits of corticosteroids are unclear in this patient population. Read More

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http://dx.doi.org/10.1093/ajhp/zxz017DOI Listing
March 2019
3 Reads

BLT-immune humanized mice as a model for nivolumab induced immune-mediated adverse events: Comparison of the NOG and NOG-EXL strains.

Toxicol Sci 2019 Mar 8. Epub 2019 Mar 8.

Division of Applied Regulatory Science, Office of Clinical Pharmacology, Office of Translational Sciences, Center for Drug Evaluation and Research, U.S. Food & Drug Administration, 10903 New Hampshire Ave., Silver Spring, Maryland.

Checkpoint inhibitors represent a new class of therapeutics in the treatment of cancer that have demonstrated remarkable clinical effectiveness. However, some patients have experienced serious immune-mediated adverse effects including pneumonitis, hepatitis, colitis, nephritis, dermatitis, encephalitis, and adrenal or pituitary insufficiency. These adverse events were not predicted by nonclinical studies. Read More

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http://dx.doi.org/10.1093/toxsci/kfz045DOI Listing
March 2019
3 Reads

Adrenal function and dysfunction in critically ill patients.

Nat Rev Endocrinol 2019 Mar 8. Epub 2019 Mar 8.

Clinical Division and Laboratory of Intensive Care Medicine, Department of Cellular and Molecular Medicine, KU Leuven University, Leuven, Belgium.

Critical illnesses are characterized by increased systemic cortisol availability, which is a vital part of the stress response. Relative adrenal failure (later termed critical-illness-related corticosteroid insufficiency (CIRCI)) is a condition in which the systemic availability of cortisol is assumed to be insufficiently high to face the stress of the illness and is most typically thought to occur in the acute phase of septic shock. Researchers suggested that CIRCI could be diagnosed by a suppressed incremental cortisol response to an injection of adrenocorticotropic hormone, irrespective of the baseline plasma cortisol. Read More

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http://dx.doi.org/10.1038/s41574-019-0185-7DOI Listing
March 2019
3 Reads

Nivolumab-induced hypophysitis leading to hypopituitarism and secondary empty sella syndrome in a patient with non-small cell lung cancer.

BMJ Case Rep 2019 Mar 7;12(3). Epub 2019 Mar 7.

Hematology/Oncology, University of Southern California-Norris Comprehensive Cancer Center and Hospital, Los Angeles, California, USA.

We describe the clinical course of a 64-year-old woman with stage IVa lung adenocarcinoma who presented with over 1 month of fatigue, unintentional weight loss and emesis. She initiated treatment with nivolumab immunotherapy 1 year prior and had been tolerating the treatment well. A comprehensive workup revealed multiple endocrinological abnormalities consistent with hypophysitis leading to hypopituitarism in the form of central adrenal insufficiency and hypogonadism as well as a partially empty sella on imaging. Read More

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http://dx.doi.org/10.1136/bcr-2018-228135DOI Listing
March 2019
2 Reads

Hyponatraemia and hyperpigmentation in primary adrenal insufficiency.

BMJ Case Rep 2019 Mar 7;12(3). Epub 2019 Mar 7.

Department of Internal Medicine, Erasmus University Medical Center, Rotterdam, The Netherlands.

Hyponatraemia is a common electrolyte disturbance with multiple causes. We present a case of a 49-year-old Caucasian female with cholangiocarcinoma, who had a hyponatraemia which was initially assumed to be based on a syndrome of inappropriate antidiuretic hormone secretion as paraneoplastic phenomenon. At physical examination, hyperpigmentation was seen and multiple episodes with syncope were reported. Read More

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http://dx.doi.org/10.1136/bcr-2018-227200DOI Listing
March 2019
2 Reads

[Hypophysitis and retroperitoneal fibrosis associated with autoimmune polyglandular syndrome and IgG4-related disease. Report of one case].

Rev Med Chil 2018 Dec;146(12):1486-1492

Servicio de Medicina, Hospital Naval A. Nef., Viña del Mar, Chile.

We report a 23 year old woman presenting with a nephrotic syndrome due to minimal change disease, central diabetes insipidus, primary hypothyroidism, vitiligo and universal alopecia. Eleven years later, she presented secondary amenorrhea due to hypogonadotropic hypogonadism, with mild hyperprolactinemia and central adrenal insufficiency. A magnetic resonance imaging of the sella turcica showed a pituitary mass with suprasellar extension that was resected using a transsphenoidal approach. Read More

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http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S
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http://dx.doi.org/10.4067/s0034-98872018001201486DOI Listing
December 2018
6 Reads
0.373 Impact Factor

Clinical utility of routine postoperative morning cortisol monitoring in detecting new hypothalamic-pituitary-adrenal axis insufficiency following endoscopic transsphenoidal surgery for sellar lesions.

J Neurosurg 2019 Mar 1:1-5. Epub 2019 Mar 1.

1Department of Neurological Surgery and.

OBJECTIVEHypothalamic-pituitary-adrenal (HPA) axis dysfunction is a well-documented complication of transsphenoidal craniotomy (TSC) for sellar lesions. The authors aimed to assess their multidisciplinary approach to the diagnosis and treatment of postoperative hypocortisolemia utilizing conservative screening methods.METHODSThe authors performed a retrospective review of 257 patients who underwent TSC for pituitary adenoma (PA) or Rathke cleft cyst (RCC) at the University of Southern California between 2012 and 2017. Read More

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http://dx.doi.org/10.3171/2018.11.JNS182521DOI Listing
March 2019
4 Reads

Emergency management of adrenal insufficiency in children: advocating for treatment options in outpatient and field settings.

J Investig Med 2019 Feb 28. Epub 2019 Feb 28.

Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota, USA.

Adrenal insufficiency (AI) remains a significant cause of morbidity and mortality in children with 1 in 200 episodes of adrenal crisis resulting in death. The goal of this working group of the Pediatric Endocrine Society Drug and Therapeutics Committee was to raise awareness on the importance of early recognition of AI, to advocate for the availability of hydrocortisone sodium succinate (HSS) on emergency medical service (EMS) ambulances or allow EMS personnel to administer patient's HSS home supply to avoid delay in administration of life-saving stress dosing, and to provide guidance on the emergency management of children in adrenal crisis. Currently, hydrocortisone, or an equivalent synthetic glucocorticoid, is not available on most ambulances for emergency stress dose administration by EMS personnel to a child in adrenal crisis. Read More

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http://dx.doi.org/10.1136/jim-2019-000999DOI Listing
February 2019
3 Reads

Isolated glucocorticoid deficiency: Genetic causes and animal models.

J Steroid Biochem Mol Biol 2019 Feb 25;189:73-80. Epub 2019 Feb 25.

Centre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary, University of London, Charterhouse Square, London, United Kingdom. Electronic address:

Hereditary adrenocorticotropin (ACTH) resistance syndromes encompass the genetically heterogeneous isolated or Familial Glucocorticoid Deficiency (FGD) and the distinct clinical entity known as Triple A syndrome. The molecular basis of adrenal resistance to ACTH includes defects in ligand binding, MC2R/MRAP receptor trafficking, cellular redox balance, cholesterol synthesis and sphingolipid metabolism. Biochemically, this manifests as ACTH excess in the setting of hypocortisolaemia. Read More

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http://dx.doi.org/10.1016/j.jsbmb.2019.02.012DOI Listing
February 2019
5 Reads

Case of autoimmune polyglandular syndrome type 2: how we uncovered the diagnosis.

BMJ Case Rep 2019 Feb 26;12(2). Epub 2019 Feb 26.

Department of Internal Medicine, Monmouth Medical Center, Long Branch, New Jersey, USA.

A 24-year-old man with no significant medical history presented to the medical clinic with vomiting and giddiness for 2 days, loss of appetite for 1 month and progressive fatigability for the preceding 4 months. On examination, he was found to be hypotensive and was admitted to the hospital for work-up. Considering his abnormal labs and physical findings, he was worked up and was diagnosed with primary adrenal insufficiency. Read More

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http://dx.doi.org/10.1136/bcr-2018-227187DOI Listing
February 2019
5 Reads

Adrenal crises in children with adrenal insufficiency: epidemiology and risk factors.

Eur J Pediatr 2019 Feb 26. Epub 2019 Feb 26.

Pediatric Endocrinology and Diabetes Unit, Dana-Dwek Children's Hospital, Tel-Aviv Sourasky Medical Center, 6 Weizmann St, 6423906, Tel Aviv, Israel.

The aim of the study was to assess the epidemiology and risk factors of adrenal crises (AC) in children with adrenal insufficiency (AI). Children diagnosed with AI between 1990 and 2017 at four Israeli pediatric endocrinology units were studied. Demographic and clinical data were retrieved retrospectively from their files. Read More

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http://dx.doi.org/10.1007/s00431-019-03348-1DOI Listing
February 2019
3 Reads

Acute spontaneous unilateral adrenal hemorrhage: etiology and imaging findings in six cases.

Radiol Bras 2019 Jan-Feb;52(1):12-16

Hospital Universitário Maria Aparecida Pedrossian da Universidade Federal de Mato Grosso do Sul (HUMAP-UFMS), Campo Grande, MS, Brazil.

Objective: To evaluate the computed tomography characteristics of patients admitted with clinical suspicion of acute adrenal hemorrhage at three centers over a 3-year period and in whom that diagnosis was ultimately confirmed.

Materials And Methods: This was a retrospective analysis of computed tomography findings in patients with suspected acute adrenal hemorrhage. We included only those cases in which the diagnosis was confirmed. Read More

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http://dx.doi.org/10.1590/0100-3984.2018.0041DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6383530PMC
February 2019
3 Reads

Increased androgen secretion in patients with prolactinomas: the impact of altered HPA function.

Pituitary 2019 Apr;22(2):170-178

Division of Clinical and Molecular Endocrinology, UH Cleveland Medical Center and Case Western University, 11100 Euclid Avenue, Cleveland, OH, 44106, USA.

Background And Objectives: Earlier studies suggested that hyperprolactinemia was associated with elevated serum DHEA-S levels. The importance of DHEA-S measurements in the diagnosis of adrenal insufficiency prompted us to assess adrenal androgen levels in hyperprolactinemic subjects with normal or impaired function.

Methods: Prospective study including 122 medically treated and 26 surgically patients with prolactinomas. Read More

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http://dx.doi.org/10.1007/s11102-019-00948-wDOI Listing
April 2019
2 Reads

Differential activity of the corticosteroidogenic enzymes in normal cycling women and women with polycystic ovary syndrome.

Rev Endocr Metab Disord 2019 Feb 23. Epub 2019 Feb 23.

Tropical Institute of Reproductive Medicine, Cuiabá, MT, Brazil.

The phenotypic complex of patients with definitive diagnosis of polycystic ovary syndrome may include patients with normal and high serum androgen levels. Patients with hyperandrogenemia seem to present higher risk of changes to the glucose and lipid metabolism and, eventually, of earlier development of cardiovascular diseases than normoandrogenemic patients or healthy women. From a laboratory and clinical point of view, it is important to check androgen levels in patients with polycystic ovary syndrome. Read More

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http://dx.doi.org/10.1007/s11154-019-09482-3DOI Listing
February 2019
2 Reads

Hydrocortisone in Vasodilatory Shock.

Crit Care Clin 2019 Apr 28;35(2):263-275. Epub 2019 Jan 28.

Department of Intensive Care, The Wesley Hospital, Coronation Drive, QLD 4066, Australia; Department of Intensive Care, The Royal Brisbane and Women's Hospital, University of Queensland, Herston Road, QLD 4066, Australia; Division of Critical Care, The George Institute for Global Health, King Street, Sydney, NSW 2050, Australia.

Vasodilatory shock is the most common type of circulatory shock in critically ill patients; sepsis the predominant cause. Steroid use in septic shock gained favor in the 1970s; however, studies of high-dose steroids demonstrated excess morbidity and mortality. Lower dosage steroid use was driven by trials demonstrating improved hemodynamic status and the possibility of relative adrenal insufficiency; however, divergent results led to uncertainty about hydrocortisone treatment. Read More

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http://dx.doi.org/10.1016/j.ccc.2018.11.005DOI Listing
April 2019
2 Reads

Late-onset isolated adrenocorticotropic hormone deficiency caused by nivolumab: a case report.

BMC Endocr Disord 2019 Feb 19;19(1):25. Epub 2019 Feb 19.

Internal Medicine 1, Shimane University Faculty of Medicine, 89-1, Enya-cho, Izumo, Shimane, 693-8501, Japan.

Background: Immune checkpoint inhibitors including nivolumab, an anti-programmed cell death protein 1 antibody, are recently developed cancer immunotherapy agents. Immune checkpoint inhibitors are known to cause autoimmune-related side effects including endocrine dysfunctions. However, there are few reports on late-onset isolated adrenocorticotropic hormone (ACTH) deficiency caused by nivolumab. Read More

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http://dx.doi.org/10.1186/s12902-019-0335-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6381704PMC
February 2019
1 Read

Clinical indicators of adrenal insufficiency following discontinuation of oral glucocorticoid therapy: A Danish population-based self-controlled case series analysis.

PLoS One 2019 19;14(2):e0212259. Epub 2019 Feb 19.

Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark.

Background: Biochemical adrenal insufficiency induced by glucocorticoid treatment is prevalent, but data on the clinical implications are sparse. We investigated clinical consequences of glucocorticoid-induced adrenal insufficiency after oral glucocorticoid cessation.

Methods: We conducted a Danish population-based self-controlled case series utilizing medical registries. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0212259PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6380588PMC
February 2019
2 Reads

Small Cell Lung Cancer with Pituitary Metastasis Presenting as Secondary Adrenal Insufficiency: A Case Report and Literature Review.

Am J Case Rep 2019 Feb 17;20:207-211. Epub 2019 Feb 17.

Department of Endocrinology, University of California San Francisco, San Francisco, CA, USA.

BACKGROUND Pituitary gland metastasis is rarely the initial presentation of metastatic cancer. Most cases of pituitary gland metastasis are asymptomatic with diabetes insipidus being the most common symptomatic presentation. It can rarely present with symptoms of hormone underproduction such as secondary adrenal insufficiency. Read More

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http://dx.doi.org/10.12659/AJCR.913388DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6388645PMC
February 2019
2 Reads

Autoimmune Regulator (AIRE) is required in female mice for optimal embryonic development and implantation1.

Biol Reprod 2019 Feb 16. Epub 2019 Feb 16.

Dept. Pathobiology & Diagnostic Investigation, and.

Autoimmune Regulator (AIRE) regulates central immune tolerance by inducing expression of tissue-restricted antigens in thymic medullary epithelial cells, thereby ensuring elimination of autoreactive T cells. Aire mutations in humans and targeted Aire deletion in mice results in multi-organ autoimmune disease, known in humans as Autoimmune Polyglandular Syndrome Type 1 (APS-1). APS-1 is characterized by presence of adrenal insufficiency, chronic mucosal candidiasis, and/or hypoparathyroidism. Read More

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https://academic.oup.com/biolreprod/advance-article/doi/10.1
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http://dx.doi.org/10.1093/biolre/ioz023DOI Listing
February 2019
6 Reads

ACTH Resistance Syndrome: An Experience of Three Cases.

Indian J Endocrinol Metab 2018 Nov-Dec;22(6):843-847

Department of Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi, India.

The term adrenocorticotropin (ACTH) resistance syndrome is used for a group of rare inherited disorders, which present with primary adrenal insufficiency during childhood. The syndrome includes two disorders inherited in an autosomal recessive fashion - familial glucocorticoid deficiency and triple A syndrome. Herein, we report our experience of three cases with ACTH resistance syndrome, highlighting the approach to diagnosis and management in such patients. Read More

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http://dx.doi.org/10.4103/ijem.IJEM_501_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6330879PMC
February 2019

Alternative (backdoor) androgen production and masculinization in the human fetus.

PLoS Biol 2019 Feb 14;17(2):e3000002. Epub 2019 Feb 14.

Institute of Medical Sciences, School of Medicine, Medical Sciences and Nutrition, University of Aberdeen, Aberdeen, United Kingdom.

Masculinization of the external genitalia in humans is dependent on formation of 5α-dihydrotestosterone (DHT) through both the canonical androgenic pathway and an alternative (backdoor) pathway. The fetal testes are essential for canonical androgen production, but little is known about the synthesis of backdoor androgens, despite their known critical role in masculinization. In this study, we have measured plasma and tissue levels of endogenous steroids in second trimester human fetuses using multidimensional and high-resolution mass spectrometry. Read More

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http://dx.doi.org/10.1371/journal.pbio.3000002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6375548PMC
February 2019
1 Read

Addison's Disease Caused by Tuberculosis: Diagnostic and Therapeutic Difficulties.

Eur J Case Rep Intern Med 2018 28;5(8):000911. Epub 2018 Aug 28.

Department of Pulmonary Diseases and Tuberculosis, Diakonessenhuis Utrecht, Utrecht, The Netherlands.

Objectives: To demonstrate difficulties in diagnosing and treating Addison's disease caused by tuberculosis.

Materials And Methods: We present a clinical case and review of the literature.

Results: A 62-year-old man presented with gastrointestinal symptoms, weight loss and enlarged adrenal glands. Read More

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http://dx.doi.org/10.12890/2018_000911DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346831PMC
August 2018
2 Reads

Iatrogenic Takotsubo Cardiomyopathy Secondary to Norepinephrine by Continuous Infusion for Shock.

Eur J Case Rep Intern Med 2018 26;5(7):000894. Epub 2018 Jul 26.

Serviço de Medicina 1, Hospital do Espírito Santo Évora EPE, Évora, Portugal.

Takotsubo cardiomyopathy is a condition characterized by transient left ventricular systolic and diastolic dysfunction, with a possible direct causal role of catecholamine in its pathophysiology. We present a case of a woman with shock and adrenal insufficiency in whom Takotsubo cardiomyopathy developed after treatment with norepinephrine. This case confirms the direct causal role of catecholamine in the pathophysiology of Takotsubo cardiomyopathy. Read More

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https://www.ejcrim.com/index.php/EJCRIM/article/view/894
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http://dx.doi.org/10.12890/2018_000894DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346780PMC
July 2018
7 Reads

Evaluation of growth and puberty in a child with a novel TBX19 gene mutation and review of the literature.

Hormones (Athens) 2019 Feb 12. Epub 2019 Feb 12.

Department of Pediatric Endocrinology and Diabetes, Marmara University School of Medicine, Istanbul, Turkey.

Background: Biallelic mutations in the TBX19 gene cause severe early-onset adrenal failure due to isolated ACTH deficiency (IAD). This rare disease is characterized by low plasma ACTH and cortisol levels, with normal secretion of other pituitary hormones. Herein, we report a patient with IAD due to a novel TBX19 gene mutation, who is also of tall stature. Read More

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http://dx.doi.org/10.1007/s42000-019-00096-7DOI Listing
February 2019
2 Reads

Novel insights into glucocorticoid replacement therapy for pediatric and adult adrenal insufficiency.

Ther Adv Endocrinol Metab 2019 2;10:2042018818821294. Epub 2019 Feb 2.

Pediatric Endocrinology Unit, Cliniques Universitaires Saint Luc, Pôle PEDI, Institut de Recherche Expérimentale et Clinique, Université Catholique de Louvain, Av. Hippocrate 10, B-1200 Brussels, Belgium.

Adrenal insufficiency is defined as impaired adrenocortical hormone synthesis. According to its source, the deficit is classified as primary (adrenal steroidogenesis impairment), secondary (pituitary adrenocorticotropic hormone deficit) or tertiary (hypothalamic corticotropin-releasing hormone deficit). The management of adrenal insufficiency resides primarily in physiological replacement of glucocorticoid secretion. Read More

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http://dx.doi.org/10.1177/2042018818821294DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360643PMC
February 2019
4 Reads

Unexplained death in patients with NGLY1 mutations may be explained by adrenal insufficiency.

Physiol Rep 2019 Feb;7(3):e13979

Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Pediatric Endocrinology, Amsterdam, The Netherlands.

Homozygous mutations in NGLY1 were recently found to cause a condition characterized by a complex neurological syndrome, hypo- or alacrimia, and elevated liver transaminases. For yet unknown reasons, mortality is increased in patients with this condition. NGLY1 encodes the cytosolic enzyme N-glycanase 1, which is responsible for the deglycosylation of misfolded N-glycosylated proteins. Read More

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http://dx.doi.org/10.14814/phy2.13979DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6369059PMC
February 2019

Bilateral adrenal myelolipomas presenting as acute adrenal insufficiency in an adult with congenital adrenal hyperplasia.

BMJ Case Rep 2019 Feb 9;12(2). Epub 2019 Feb 9.

Department of Internal Medicine, division of Endocrinology, Texas Tech University Health Sciences Centre School of Medicine, Lubbock, Texas, USA.

Adrenal myelolipomas are relatively rare tumours composed of adipocytes and myeloid cells that arise in response to chronic adrenocorticotropic hormone stimulation. We present the case of bilateral adrenal myelolipomas in a 39-year-old man with untreated congenital adrenal hyperplasia (CAH) presenting with acute adrenal insufficiency and severe virilisation. Phenotypically, he is a man of short stature and has hyperpigmentation of the skin, gingiva and nail beds. Read More

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http://dx.doi.org/10.1136/bcr-2018-226826DOI Listing
February 2019
3 Reads

Bifocal germinoma in a patient with 16p11.2 microdeletion syndrome.

Endocrinol Diabetes Metab Case Rep 2019 Feb 7;2019. Epub 2019 Feb 7.

Department of Endocrinology, Diabetes and Metabolism.

Intracranial germinomas are rare tumors affecting mostly patients at young age. Therefore, molecular data on its etiopathogenesis are scarce. We present a clinical case of a male patient of 25 years with an intracranial germinoma and a 16p11. Read More

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http://dx.doi.org/10.1530/EDM-18-0149DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6373620PMC
February 2019
11 Reads

Sellar Metastasis of Cervical Adenocarcinoma.

Case Rep Neurol Med 2019 10;2019:9769657. Epub 2019 Jan 10.

Department of Neurological Surgery, Loyola University Medical Center, Maywood, Illinois, USA.

Background: Pituitary metastasis of cervical adenocarcinoma is an exceedingly rare phenomenon.

Case Description: The authors present a case of a 66-year-old female with cervical adenocarcinoma who was discovered to have a rapidly growing intrasellar mass in the work-up of adrenal insufficiency and hypothyroidism. The patient underwent subsequent endoscopic endonasal subtotal resection of the mass. Read More

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http://dx.doi.org/10.1155/2019/9769657DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6348813PMC
January 2019
3 Reads

Hypopituitarism following Cranial Irradiation for Meningiomas: A single-institution experience.

Pract Radiat Oncol 2019 Feb 4. Epub 2019 Feb 4.

Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts. Electronic address:

Background: Patients undergoing cranial irradiation are at high risk for development of subsequent pituitary deficiencies. Patients with meningiomas can expect to live many years following treatment and are therefore particularly vulnerable to long-term sequalae of radiation therapy (RT). The purpose of this study was to determine the rates and timing of onset of pituitary dysfunction across each hypothalamic-pituitary axis in patients with meningiomas in the sellar region. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S18798500193004
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http://dx.doi.org/10.1016/j.prro.2019.01.009DOI Listing
February 2019
7 Reads

SIADH versus adrenal insufficiency: a life-threatening misdiagnosis.

Ital J Pediatr 2019 Feb 6;45(1):23. Epub 2019 Feb 6.

University of Trieste, Piazzale Europa, 1, 34127, Trieste, Italy.

Background: Primary adrenal insufficiency (PAI) in children is an uncommon but severe condition which can be either inherited or acquired. It consists in clinical manifestation of defective production or ineffective action of endogenous glucocorticoids; deficiency in mineralocorticoids and adrenal androgens may coexist. Diagnosis of PAI in children and young people can be challenging; while adrenal crisis (acute decompensation) is a life-threatening condition, with patient presenting with characteristic features of hypoglycemia, hypotension, collapse and coma, chronic adrenal insufficiency may present with vague and non-specific symptoms, making the diagnosis more difficult. Read More

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http://dx.doi.org/10.1186/s13052-019-0614-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6366080PMC
February 2019
7 Reads

Diabetic Ketoacidosis Revealing a Severe Hypertriglyceridemia and Acute Pancreatitis in Type 1 Diabetes Mellitus.

Case Rep Endocrinol 2019 6;2019:8974619. Epub 2019 Jan 6.

Department of Endocrinology, Diabetes, Metabolic Diseases and Nutrition, Mohammed VI University Hospital, Marrakech, Morocco.

Diabetic ketoacidosis (DKA) is a life-threatening acute metabolic complication occurring in patients with diabetes, especially in patients with type 1 diabetes (T1D), due to an insulin deficiency. Moderate hypertriglyceridemia is commonly observed in DKA but severe hypertriglyceridemia with a triglyceride level exceeding 10g/L is very rarely reported. We report a case of a 14-year-old boy who had type 1 diabetes for 4 years treated with insulin therapy, also having adrenal insufficiency treated with hydrocortisone who presented with ketoacidosis and excruciating abdominal pain. Read More

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http://dx.doi.org/10.1155/2019/8974619DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6339756PMC
January 2019
3 Reads

A Phase II Study to Determine the Safety and Efficacy of the Oral Inhibitor of Indoleamine 2,3-Dioxygenase (IDO) Enzyme INCB024360 in Patients with Myelodysplastic Syndromes.

Clin Lymphoma Myeloma Leuk 2019 Mar 20;19(3):157-161. Epub 2018 Dec 20.

MD Moffitt Cancer Center and Research Institute, Tampa, FL.

Background: INCB024360 is an oral inhibitor of the enzyme indoleamine 2,3-dioxygenase (IDO), which catalyzes the degradation of tryptophan to kynurenine. Preclinical data suggest that IDO1 inhibition by INCB024360 will increase T cell proliferation, and decrease T regulatory cells and myeloid derived suppressor cells suppressive activity. We conducted a phase II study to explore activity and pharmacodynamics of INCB024360 in patients with myelodysplastic syndromes. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S21522650183119
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http://dx.doi.org/10.1016/j.clml.2018.12.005DOI Listing
March 2019
3 Reads

Endoscopic Transnasal Resection of Suprasellar Teratoma.

J Neurol Surg A Cent Eur Neurosurg 2019 Feb 1. Epub 2019 Feb 1.

Division of Neurosurgery, Department of Surgery, King Saud University, Riyadh, Riyadh Province, Saudi Arabia.

Background:  Intracranial teratomas are rare tumors that usually present during the second decade of life. Among intracranial teratomas, suprasellar germ cell tumors in particular are extremely rare. We present the case of an 18-year-old girl who underwent a successful endoscopic transnasal resection of a suprasellar teratoma with a mixed germ cell component. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0038-1676624
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http://dx.doi.org/10.1055/s-0038-1676624DOI Listing
February 2019
7 Reads
0.493 Impact Factor

The association between the development of autoimmune polyglandular syndrome in adults and polymorphism of HLA class II genes and the predisposition to the development of chronic adrenal insufficiency in the context of these syndromes.

Ter Arkh 2018 Nov;90(10):23-29

National Medical Research Center of Endocrinology, Ministry of Health of Russia, Moscow, Russia.

Aim: To consider association of chronic adrenal insufficiency in patients with APS of adults with polymorphism of class II HLA genes, -CTLA-4 and PTPN-22.

Materials And Methods: The case-control study involved 78 patients with APS 2, 3, 4 types and 109 healthy subjects). Alleles of the HLA class II genes, CTLA-4 and PTPN-22 were identified by the multiprimer allele-specific PCR method. Read More

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http://dx.doi.org/10.26442/terarkh2018901023-29DOI Listing
November 2018
1 Read

Low Risk of Adrenal Insufficiency After Use of Low- to Moderate-Potency Topical Corticosteroids for Children With Atopic Dermatitis.

Clin Pediatr (Phila) 2019 Apr 29;58(4):406-412. Epub 2019 Jan 29.

1 University of Virginia, Charlottesville, VA, USA.

Our objective was to assess the risk of adrenal insufficiency (AI) with short-term use of low- to moderate-potency topical corticosteroids (TCS) for treatment of atopic dermatitis. Our systematic literature search revealed 9 studies (n = 371) that evaluated AI using adrenocorticotropic hormone stimulation testing, with measures of serum cortisol levels at baseline and following at least 2 weeks of TCS application. Biochemical AI was defined by a stimulated cortisol level of ≤18. Read More

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http://dx.doi.org/10.1177/0009922818825154DOI Listing
April 2019
3 Reads

Spectrum of immune checkpoint inhibitors-induced endocrinopathies in cancer patients: a scoping review of case reports.

Clin Diabetes Endocrinol 2019 22;5. Epub 2019 Jan 22.

1Division of Metabolism, Endocrinology & Diabetes, Department of Internal Medicine, University of Michigan, 24 Frank Lloyd Wright Drive, Ann Arbor, MI 48106 USA.

Background: Since 2011 six immune checkpoint inhibitors (ICI) have been approved to treat patients with many advanced solid tumor and hematological malignancies to improve their prognosis. Case reports of their endocrine immune-related adverse events [irAEs]) are increasingly published as more real-world patients with these malignancies are treated with these drugs. They alert physicians of a drug's AEs (which may change during a drug's life cycle) and contribute to post-marketing safety surveillance. Read More

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https://clindiabetesendo.biomedcentral.com/articles/10.1186/
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http://dx.doi.org/10.1186/s40842-018-0073-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343255PMC
January 2019
5 Reads

A Case of Acute Exacerbation of Chronic Adrenal Insufficiency Due to Ipilimumab Treatment for Advanced Melanoma.

Am J Case Rep 2019 Jan 25;20:106-110. Epub 2019 Jan 25.

Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka City, Fukuoka, Japan.

BACKGROUND Ipilimumab is a therapeutic human monoclonal antibody that targets the T-cell inhibitory molecule, cytotoxic T-lymphocyte antigen-4 (CTLA-4), and is classified as an immune checkpoint inhibitor that has been shown to improve prognosis in patients with advanced melanoma. However, several immune-related adverse events have been reported to be associated with ipilimumab Treatment. A case of acute exacerbation of chronic adrenal insufficiency is presented that highlights that glucocorticoid dosage for patients undergoing steroid treatment at the time of ipilimumab treatment has yet to be established. Read More

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https://www.amjcaserep.com/abstract/index/idArt/913021
Publisher Site
http://dx.doi.org/10.12659/AJCR.913021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6354724PMC
January 2019
5 Reads