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Addison Mizner : architect of fantasy and romance
The go-to architect for the Jazz Age elite of South Florida and beyond, Addison Mizner created a new architectural style and a new lifestyle for the wealthy and socially prominent of Palm Beach America s preeminent winter resort town of the time. Building mansions, clubs, hotels and apartment houses with a bent toward fantasy and romance, Mizner established a design vocabulary and tradition that to this day influences architects, designers, and builders. Evocative of old Spain, Venice, and the Moorish capitals of Granada and Seville, Mizner s work is a dream realized: courtyards with fountains, trellises with climbing bougainvillea, arched windows, glazed tile floors, spiraling marble columns, expansive interiors with grand proportions. This book explores Mizner s legacy through the extraordinary houses and other structures he built, including such storied homes as La Guerida, an 11-bedroom Spanish Revival mansion, best known now as the Kennedy Estate the place where JFK he composed his Inaugural Address. Known for their beauty, opulence, fantastic detail, as well as the stories of those individuals who have lived or played in them, the houses and buildings of Addison Mizner stand as monuments to grand living and romance made in stone and iron, stucco and tile.
Book
Circadian Rhythm of Glucocorticoid Administration Entrains Clock Genes in Immune Cells: A DREAM Trial Ancillary Study
Abstract
Context
Adrenal insufficiency (AI) requires lifelong glucocorticoid (GC) replacement. Conventional therapies do not mimic the endogenous cortisol circadian rhythm. Clock genes are essential components of the machinery controlling circadian functions and are influenced by GCs. However, clock gene expression has never been investigated in patients with AI.
Objective
To evaluate the effect of the timing of GC administration on circadian gene expression in peripheral blood mononuclear cells (PBMCs) of patients from the Dual Release Hydrocortisone vs Conventional Glucocorticoid Replacement in Hypocortisolism (DREAM) trial.
Design
Outcome assessor–blinded, randomized, active comparator clinical trial.
Participants and Intervention
Eighty-nine patients with AI were randomly assigned to continue their multiple daily GC doses or switch to an equivalent dose of once-daily modified-release hydrocortisone and were compared with 25 healthy controls; 65 patients with AI and 18 controls consented to gene expression analysis.
Results
Compared with healthy controls, 19 of the 68 genes were found modulated in patients with AI at baseline, 18 of which were restored to control levels 12 weeks after therapy was switched: ARNTL [BMAL] (P = 0.024), CLOCK (P = 0.016), AANAT (P = 0.021), CREB1 (P = 0.010), CREB3 (P = 0.037), MAT2A (P = 0.013); PRKAR1A, PRKAR2A, and PRKCB (all P < 0.010) and PER3, TIMELESS, CAMK2D, MAPK1, SP1, WEE1, CSNK1A1, ONP3, and PRF1 (all P < 0.001). Changes in WEE1, PRF1, and PER3 expression correlated with glycated hemoglobin, inflammatory monocytes, and CD16+ natural killer cells.
Conclusions
Patients with AI on standard therapy exhibit a dysregulation of circadian genes in PBMCs. The once-daily administration reconditions peripheral tissue gene expression to levels close to controls, paralleling the clinical outcomes of the DREAM trial (NCT02277587).
We compared multiple-times-a-day vs once-daily modified-release hydrocortisone in adrenal insufficiency and found a significant effect on the expression of several clock-related genes.
Journal Article
Improved Urinary Cortisol Metabolome in Addison Disease: A Prospective Trial of Dual-Release Hydrocortisone
Abstract
Context
Oral once-daily dual-release hydrocortisone (DR-HC) replacement therapy has demonstrated an improved metabolic profile compared to conventional 3-times-daily (TID-HC) therapy among patients with primary adrenal insufficiency. This effect might be related to a more physiological cortisol profile, but also to a modified pattern of cortisol metabolism.
Objective
This work aimed to study cortisol metabolism during DR-HC and TID-HC.
Design
A randomized, 12-week, crossover study was conducted.
Intervention and Participants
DC-HC and same daily dose of TID-HC were administered to patients with primary adrenal insufficiency (n = 50) vs healthy individuals (n = 124) as controls.
Main Outcome Measures
Urinary corticosteroid metabolites were measured by gas chromatography/mass spectrometry at 24-hour urinary collections.
Results
Total cortisol metabolites decreased during DR-HC compared to TID-HC (P < .001) and reached control values (P = .089). During DR-HC, 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1) activity measured by tetrahydrocortisol + 5α-tetrahydrocortisol/tetrahydrocortisone ratio was reduced compared to TID-HC (P < .05), but remained increased vs controls (P < .001). 11β-HSD2 activity measured by urinary free cortisone/free cortisol ratio was decreased with TID-HC vs controls (P < .01) but normalized with DR-HC (P = .358). 5α- and 5β-reduced metabolites were decreased with DR-HC compared to TID-HC. Tetrahydrocortisol/5α-tetrahydrocortisol ratio was increased during both treatments, suggesting increased 5β-reductase activity.
Conclusions
The urinary cortisol metabolome shows striking abnormalities in patients receiving conventional TID-HC replacement therapy, with increased 11β-HSD1 activity that may account for the unfavorable metabolic phenotype in primary adrenal insufficiency. Its change toward normalization with DR-HC may mediate beneficial metabolic effects. The urinary cortisol metabolome may serve as a tool to assess optimal cortisol replacement therapy.
Journal Article
Clinical and Immunological Characteristics of Autoimmune Addison Disease: A Nationwide Swedish Multicenter Study
Context:Studies of the clinical and immunological features of autoimmune Addison disease (AAD) are needed to understand the disease burden and increased mortality.Objective:To provide upgraded data on autoimmune comorbidities, replacement therapy, autoantibody profiles, and cardiovascular risk factors.Design, Setting, and Participants:A cross-sectional, population-based study that included 660 AAD patients from the Swedish Addison Registry (2008–2014). When analyzing the cardiovascular risk factors, 3594 individuals from the population-based survey in Northern Sweden, MONICA (monitoring of trends and determinants of cardiovascular disease), served as controls.Main Outcome Measures:The endpoints were the prevalence of autoimmune comorbidities and cardiovascular risk factors. Autoantibodies against 13 autoantigens were determined.Results:The proportion of 21-hydroxylase autoantibody-positive patients was 83%, and 62% of patients had ≥1 associated autoimmune diseases, more frequently coexisting in females (P < 0.0001). AAD patients had a lower body mass index (P < 0.0001) and prevalence of hypertension (P = 0.027) compared with controls. Conventional hydrocortisone tablets were used by 89% of the patients, with a mean dose of 28.1 ± 8.5 mg/d. The mean hydrocortisone equivalent dose normalized to the body surface was 14.8 ± 4.4 mg/m2/d. A greater hydrocortisone equivalent dose was associated with a greater incidence of hypertension (P = 0.046).Conclusions:Careful monitoring of AAD patients is warranted to detect associated autoimmune diseases. Contemporary Swedish AAD patients did not have an increased prevalence of overweight, hypertension, type 2 diabetes mellitus, or hyperlipidemia. However, high glucocorticoid replacement doses could be a risk factor for hypertension.Upgraded data regarding autoimmune comorbidities, autoantibody profiles, replacement therapy and cardiovascular risk factors revealing a need to carefully monitor AAD patients.
Journal Article
Increased Resting-State Functional Connectivity in Patients With Autoimmune Addison Disease
Abstract
Context
Individuals with autoimmune Addison disease (AAD) take replacement medication for the lack of adrenal-derived glucocorticoid (GC) and mineralocorticoid hormones from diagnosis. The brain is highly sensitive to these hormones, but the consequence of having AAD for brain health has not been widely addressed.
Objective
The present study compared resting-state functional connectivity (rs-fc) of the brain between individuals with AAD and healthy controls.
Methods
Fifty-seven patients with AAD (33 female) and 69 healthy controls (39 female), aged 19 to 43 years were scanned with 3-T magnetic resonance imaging (MRI).
Results
Independent component and subsequent dual regression analyses revealed that individuals with AAD had stronger rs-fc compared to controls in 3 networks: the bilateral orbitofrontal cortex (OFC), the left medial visual and left posterior default mode network. A higher GC replacement dose was associated with stronger rs-fc in a small part of the left OFC in patients. We did not find any clear associations between rs-fc and executive functions or mental fatigue.
Conclusion
Our results suggest that having AAD affects the baseline functional organization of the brain and that current treatment strategies of AAD may be one risk factor.
Journal Article
Does vitamin D play a role in autoimmune endocrine disorders? A proof of concept
In the last few years, more attention has been given to the “non-calcemic” effect of vitamin D. Several observational studies and meta-analyses demonstrated an association between circulating levels of vitamin D and outcome of many common diseases, including endocrine diseases, chronic diseases, cancer progression, and autoimmune diseases. In particular, cells of the immune system (B cells, T cells, and antigen presenting cells), due to the expression of 1α-hydroxylase (CYP27B1), are able to synthesize the active metabolite of vitamin D, which shows immunomodulatory properties. Moreover, the expression of the vitamin D receptor (VDR) in these cells suggests a local action of vitamin D in the immune response. These findings are supported by the correlation between the polymorphisms of the
VDR
or the
CYP27B1
gene and the pathogenesis of several autoimmune diseases. Currently, the optimal plasma 25-hydroxyvitamin D concentration that is necessary to prevent or treat autoimmune diseases is still under debate. However, experimental studies in humans have suggested beneficial effects of vitamin D supplementation in reducing the severity of disease activity. In this review, we summarize the evidence regarding the role of vitamin D in the pathogenesis of autoimmune endocrine diseases, including type 1 diabetes mellitus, Addison’s disease, Hashimoto’s thyroiditis, Graves’ disease and autoimmune polyendocrine syndromes. Furthermore, we discuss the supplementation with vitamin D to prevent or treat autoimmune diseases.
Journal Article
Addison’s disease in patients with autoimmune primary ovarian insufficiency: a concise guide for gynecologists and obstetricians
Addison's disease (AD) is a rare condition, but its prevalence in Europe has been steadily rising. Its initial presentation might be a life-threatening event termed adrenal crisis (AC), which is particularly likely to happen during situations of increased biological stress, such as septic illness, general anesthesia, surgery, or trauma.Addison's disease can appear as an isolated clinical entity or together with other autoimmune conditions, such as autoimmune thyroiditis, pernicious anemia, or autoimmune primary ovarian insufficiency (POI). In this review, we underline the relationship between AD and POI with a special regard for patients with POI who might develop AD. Due to the nonspecific symptoms of AD, patients often consult multiple specialists before receiving a proper diagnosis, which delays the recognition of AD Ordering basic screening tests might facilitate an early detection of AD and prevent possibly fatal complications of the disease.
Journal Article
Visceral Fat and Novel Biomarkers of Cardiovascular Disease in Patients With Addison’s Disease: A Case-Control Study
ContextPatients with Addison’s disease (AD) have increased cardiovascular mortality.ObjectiveTo study visceral fat and conventional and exploratory cardiovascular risk factors in patients with AD.DesignA cross-sectional, single-center, case-control study.SubjectsPatients (n = 76; n = 51 women) with AD and 76 healthy control subjects were matched for sex, age, body mass index (BMI), and smoking habits.Main outcome measuresThe primary outcome variable was visceral abdominal adipose tissue (VAT) measured using computed tomography. Secondary outcome variables were prevalence of metabolic syndrome (MetS) and 92 biomarkers of cardiovascular disease.ResultsThe mean ± standard deviation age of all subjects was 53 ± 14 years; mean BMI, 25 ± 4 kg/m2; and mean duration of AD, 17 ± 12 years. The median (range) daily hydrocortisone dose was 30 mg (10 to 50 mg). Median (interquartile range) 24-hour urinary free cortisol excretion was increased in patients vs controls [359 nmol (193 to 601 nmol) vs 175 nmol (140 to 244 nmol); P < 0.001]. VAT did not differ between groups. After correction for multiple testing, 17 of the 92 studied biomarkers differed significantly between patients and control subjects. Inflammatory, proinflammatory, and proatherogenic risk biomarkers were increased in patients [fold change (FC), >1] and vasodilatory protective marker was decreased (FC, <1). Twenty-six patients (34%) vs 12 control subjects (16%) fulfilled the criteria for MetS (P = 0.01).ConclusionDespite higher cortisol exposure, VAT was not increased in patients with AD. The prevalence of MetS was increased and several biomarkers of cardiovascular disease were adversely affected in patients with AD.In this study, patients with Addison’s disease had increased prevalence of the metabolic syndrome compared with matched control subjects, and several cardiovascular biomarkers were adversely affected.
Journal Article