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Glucocorticoid deficiency

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https://www.readbyqxmd.com/read/28522730/11%C3%AE-hsd1-suppresses-cardiac-fibroblast-cxcl2-cxcl5-and-neutrophil-recruitment-to-the-heart-post-mi
#1
Katie J Mylonas, Neil A Turner, Sumia A Bageghni, Christopher J Kenyon, Christopher I White, Kieran McGregor, Robert A Kimmitt, Richard Sulston, Valerie Kelly, Brian R Walker, Karen E Porter, Karen E Chapman, Gillian A Gray
We have previously demonstrated that neutrophil recruitment to the heart following myocardial infarction (MI) is enhanced in mice lacking 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1) that regenerates active glucocorticoid within cells from intrinsically inert metabolites. The present study aimed to identify the mechanism of regulation. In a mouse model of MI, neutrophil mobilization to blood and recruitment to the heart were higher in 11β-HSD1-deficient (Hsd11b1(-)(/)(-) ) relative to wild-type (WT) mice, despite similar initial injury and circulating glucocorticoid...
June 2017: Journal of Endocrinology
https://www.readbyqxmd.com/read/28502879/genetic-deletion-of-p-glycoprotein-alters-stress-responsivity-and-increases-depression-like-behavior-social-withdrawal-and-microglial-activation-in-the-hippocampus-of-female-mice
#2
Natalia I Brzozowska, Kristie L Smith, Cilla Zhou, Peter M Waters, Ligia Menezes Cavalcante, Sarah V Abelev, Michael Kuligowski, David J Clarke, Stephanie M Todd, Jonathon C Arnold
P-glycoprotein (P-gp) is an ABC transporter expressed at the blood brain barrier and regulates the brain uptake of various xenobiotics and endogenous mediators including glucocorticoid hormones which are critically important to the stress response. Moreover, P-gp is expressed on microglia, the brain's immune cells, which are activated by stressors and have an emerging role in psychiatric disorders. We therefore hypothesised that germline P-gp deletion in mice might alter the behavioral and microglial response to stressors...
May 10, 2017: Brain, Behavior, and Immunity
https://www.readbyqxmd.com/read/28472507/a-case-report-of-hypoglycemia-and-hypogammaglobulinemia-david-syndrome-in-a-patient-with-a-novel-nfkb2-mutation
#3
Rayhan A Lal, Laura K Bachrach, Andrew R Hoffman, Jingga Inlora, Shannon Rego, Michael P Snyder, David B Lewis
Context: DAVID syndrome (Deficient Anterior pituitary with Variable Immune Deficiency) is a rare disorder in which children present with symptomatic ACTH deficiency preceded by hypogammaglobulinemia from B-cell dysfunction with recurrent infections, termed common variable immunodeficiency (CVID). Subsequent whole exome sequencing studies have revealed germline heterozygous C-terminal mutations of NFKB2 as either a cause of DAVID syndrome or of CVID without clinical hypopituitarism. However, to the best of our knowledge there have been no cases in which the endocrinopathy has presented in the absence of a prior clinical history of CVID...
May 3, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28472487/11-oxygenated-androgens-are-biomarkers-of-adrenal-volume-and-testicular-adrenal-rest-tumors-in-21-hydroxylase-deficiency
#4
Adina F Turcu, Ashwini Mallappa, Meredith Elman, Nilo A Avila, Jamie Marko, Hamsini Rao, Alexander Tsodikov, Richard J Auchus, Deborah P Merke
Context: Patients with 21-hydroxylase deficiency (21OHD) suffer from long-term complications, which might result from poor disease control and/or glucocorticoid overtreatment. Lack of optimal biomarkers has made it challenging to tailor therapy and to predict long-term outcomes. Objective: To identify biomarkers of disease control and long-term complications in 21OHD. Setting and participants: Cross-sectional study of 114 patients (70 males), ages 2 to 67 (median, 15) years, seen in a tertiary referral center...
May 1, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28458768/early-diagnosis-in-familial-glucocorticoid-deficiency
#5
Fatima Al Jneibi, Tawfiq Hen, Jaishen Rajah, Rajendran Nair
Familial glucocorticoid deficiency (FGD) is a rare autosomal recessive condition, characterized by marked atrophy of zona fasiculata and reticalaris with preservation of zona glomerulosa. Out of more than 50 published cases, 18 patients died as a result of glucocorticoid insufficiency. The main objective of this report is to emphasize the early diagnosis and treatment in our 17 month-old patient. Her presenting features following an upper respiratory tract infection were hypoglycemia, seizures as well as deep hyperpigmentation of the limbs and lips...
2017: Dermato-endocrinology
https://www.readbyqxmd.com/read/28450305/mechanisms-in-endocrinology-update-on-pathogenesis-of-primary-adrenal-insufficiency-beyond-steroid-enzyme-deficiency-and-autoimmune-adrenal-destruction
#6
Christa Flueck
Primary adrenal insufficiency (PAI) is potentially life threatening, but rare. In children genetic defects prevail, while adults suffer mostly from acquired forms. The spectrum of genetic defects has increased in recent years with the use of next generation sequencing methods and reaches now far beyond genetic defects in known enzymes of steroidogenesis. Cofactor disorders such as P450 oxidoreductase (POR) deficiency manifesting as a complex form of congenital adrenal hyperplasia with a broad clinical phenotype have come to the forth...
April 27, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28439619/association-between-bone-mineral-density-muscle-strength-and-vitamin-d-status-in-patients-with-myasthenia-gravis-a-cross-sectional-study
#7
Y Guan, F Lv, Y Meng, D Ma, X Xu, Y Song, O Wang, Y Jiang, W Xia, X Xing, J Zhang, M Li
Myasthenia gravis (MG) patients had low proximal hip BMD, which could be explained by reduced muscle strength, elevated bone resorption markers, vitamin D deficiency, and increased PTH levels in those with MG compared to controls. INTRODUCTION: Muscle strength is closely correlated with bone mineral density (BMD) and vitamin D status. Here, we evaluated muscle strength, BMD, and vitamin D status in a large sample of Chinese patients with MG. METHODS: In this cross-sectional survey, 86 patients with MG without glucocorticoid treatment and 86 healthy controls were included...
April 24, 2017: Osteoporosis International
https://www.readbyqxmd.com/read/28423475/primary-lymphocytic-hypophysitis-clinical-characteristics-and-treatment-of-50-cases-in-a-single-centre-in-china-over-18-years
#8
Shuchang Wang, Linjie Wang, Yong Yao, Feng Feng, Hongbo Yang, Zhiyong Liang, Kan Deng, Hui You, Jian Sun, Bing Xing, Zimeng Jin, Renzhi Wang, Hui Pan, Huijuan Zhu
OBJECTIVE: Primary lymphocytic hypophysitis (LYH) is rare and it is often evaluated in a small case series. This study aimed to describe the diagnosis and treatment of primary LYH in a larger cohort. DESIGN: A retrospective study of the diagnosis and treatment of primary LYH was conducted at Peking Union Medical College Hospital from 1999 to 2016. PATIENTS: Fifty patients (28 histologically-diagnosed and 22 clinically-diagnosed) were eligible for inclusion...
April 19, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28408345/icariin-protects-against-glucocorticoid-induced-osteoporosis-increases-the-expression-of-the-bone-enhancer-dec1-and-modulates-the-pi3k-akt-gsk3%C3%AE-%C3%AE-catenin-integrated-signaling-pathway
#9
Hu Jinhua, Mao Zhao, He Shuangcheng, Zhan Yuanran, Ning Rui, Liu Wei, Yan Bingfang, Yang Jian
Osteoporosis is a serious public health concern worldwide. Herba epimedii has been used for centuries and even thousands of years to treat osteoporotic conditions. Icariin, a flavonol glycoside, is one of the major active ingredients. In this study, we have shown that icariin protected against glucocorticoid-induced osteoporotic changes in SaoS-2 cells and mice. We have also shown that dexamethasone (a glucocorticoid) suppressed and icariin induced DEC1, a structurally distinct helix-loop-helix protein. DEC1 overexpression promoted whereas DEC1 knockdown decreased osteogenic activity...
April 10, 2017: Biochemical Pharmacology
https://www.readbyqxmd.com/read/28402861/trpv1-regulates-stress-responses-through-hdac2
#10
Sung Eun Wang, Seung Yeon Ko, Sungsin Jo, Miyeon Choi, Seung Hoon Lee, Hye-Ryeong Jo, Jee Young Seo, Sang Hoon Lee, Yong-Seok Kim, Sung Jun Jung, Hyeon Son
Stress causes changes in neurotransmission in the brain, thereby influencing stress-induced behaviors. However, it is unclear how neurotransmission systems orchestrate stress responses at the molecular and cellular levels. Transient receptor potential vanilloid 1 (TRPV1), a non-selective cation channel involved mainly in pain sensation, affects mood and neuroplasticity in the brain, where its role is poorly understood. Here, we show that Trpv1-deficient (Trpv1(-/-)) mice are more stress resilient than control mice after chronic unpredictable stress...
April 11, 2017: Cell Reports
https://www.readbyqxmd.com/read/28400640/a-pilot-study-evaluating-therapeutic-response-of-different-dosage-of-oral-glucocorticoid-in-two-children-with-familial-glucocorticoid-deficiency-presenting-with-diffuse-mucocutaneous-hyperpigmentation
#11
Uttam Kumar Sarkar, Nilendu Sarma, Sambreeta Debbarma, Asok Kumar Mandal, Ashok Kumar Bala
INTRODUCTION: Familial glucocorticoid deficiency (FGD) is a rare autosomal recessive potentially life-threatening condition, characterized by glucocorticoid deficiency, preserved aldosterone/renin secretion, and secondary rise in plasma adrenocorticotropic hormone level. This occurs due to either mutation in adrenocorticotropic receptor (25%, FGD Type-1) or in the MC2 receptor accessory protein (15%-20%). However, in about 50% patients, no identifiable mutations have been identified. Clinically, it manifests with weakness, fatigue, weight loss, anorexia, nausea, vomiting, diarrhea, abdominal pain, hypoglycemia, and hypothermia...
March 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28389565/akt3-kinase-suppresses-pinocytosis-of-low-density-lipoprotein-by-macrophages-via-novel-wnk-sgk1-cdc42-pathway
#12
Liang Ding, Lifang Zhang, Michael Kim, Tatiana Byzova, Eugene Podrez
Fluid-phase pinocytosis of Low-density lipoprotein (LDL) by macrophages is regarded as a novel promising target to reduce macrophage cholesterol accumulation in atherosclerotic lesions. The mechanisms of regulation of fluid-phase pinocytosis in macrophages and specifically the role of Akt kinases are poorly understood. We have previously found that increased lipoprotein uptake via the receptor independent process in Akt3 kinase deficient macrophages contributes to increased atherosclerosis in Akt3-/- mice. The mechanism by which Akt3 deficiency promotes lipoprotein uptake in macrophages is unknown...
April 7, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28368470/11%C3%AE-hsd1-modulates-the-set-point-of-brown-adipose-tissue-response-to-glucocorticoids-in-male-mice
#13
Craig L Doig, Rachel S Fletcher, Stuart A Morgan, Emma L McCabe, Dean P Larner, Jeremy W Tomlinson, Paul M Stewart, Andrew Philp, Gareth G Lavery
Glucocorticoids are potent regulators of energy metabolism. Chronic glucocorticoid exposure supresses brown adipose tissue (BAT) thermogenic capacity in mice, with evidence for a similar effect in humans. Intracellular glucocorticoid levels are regulated by 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1) activity which can amplify circulating glucocorticoid concentrations. Therefore, 11β-HSD1 could modulate the impact of glucocorticoids on BAT function. Here we investigate how 11β-HSD1 regulates the molecular architecture of BAT in the context of glucocorticoid excess and aging...
March 27, 2017: Endocrinology
https://www.readbyqxmd.com/read/28321652/role-of-plasminogen-activator-inhibitor-1-in-glucocorticoid-induced-muscle-change-in-mice
#14
Yukinori Tamura, Naoyuki Kawao, Takeshi Shimoide, Kiyotaka Okada, Osamu Matsuo, Hiroshi Kaji
We recently revealed that plasminogen activator inhibitor-1 (PAI-1), a serine protease inhibitor, is involved in diabetes, osteoporosis and muscle wasting induced by glucocorticoid (GC) treatment in mice. In the present study, we investigated the detailed mechanisms by which GC induces muscle wasting through PAI-1 in vivo and in vitro. PAI-1 deficiency suppressed the mRNA levels of atrogin1 and muscle RING-Finger Protein 1 (MuRF1), ubiquitin ligases leading to muscle degradation, elevated by GC treatment in the gastrocnemius muscle of mice...
March 20, 2017: Journal of Bone and Mineral Metabolism
https://www.readbyqxmd.com/read/28299309/two-siblings-with-the-same-severe-form-of-21-hydroxylase-deficiency-but-different-growth-and-menstrual-cycle-patterns
#15
Mariarosaria Lang-Muritano, Karine Gerster, Susanna Sluka, Daniel Konrad
Congenital adrenal hyperplasia (CAH) is one of the most frequent autosomal recessive diseases in Europe. Treatment is a challenge for pediatric endocrinologists. Important parameters to judge the outcome are adult height and menstrual cycle. We report the follow-up from birth to adulthood of two Caucasian sisters with salt-wasting CAH due to the same mutation, homozygosity c.290-13A>G (I2 splice), in the 21-hydroxylase gene. Their adherence to treatment was excellent. Our objective was to distinguish the effects of treatment with hydrocortisone (HC) and fludrocortisone (FC) on final height (FH) from constitutional factors...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28289431/management-issues-of-congenital-adrenal-hyperplasia-during-the-transition-from-pediatric-to-adult-care
#16
REVIEW
Jin-Ho Choi, Han-Wook Yoo
Steroid 21-hydroxylase deficiency is the most prevalent form of congenital adrenal hyperplasia (CAH), accounting for approximately 95% of cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, tumor, osteoporosis, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and health-care providers...
February 2017: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28267764/ex-vivo-modulation-of-the-foxo1-phosphorylation-state-does-not-lead-to-dysfunction-of-t-regulatory-cells
#17
Kristen Kelley Penberthy, Monica Weaver Buckley, Sanja Arandjelovic, Kodi Ravichandran
Peripheral regulatory CD4+ T cells (Treg cells) prevent maladaptive inflammatory responses to innocuous foreign antigens. Treg cell dysfunction has been linked to many inflammatory diseases, including allergic airway inflammation. Glucocorticoids that are used to treat allergic airway inflammation and asthma are thought to work in part by promoting Treg cell differentiation; patients who are refractory to these drugs have defective induction of anti-inflammatory Treg cells. Previous observations suggest that Treg cells deficient in the transcription factor FoxO1 are pro-inflammatory, and that FoxO1 activity is regulated by its phosphorylation status and nuclear localization...
2017: PloS One
https://www.readbyqxmd.com/read/28259685/endogenous-glucocorticoid-deficiency-in-psoriasis-promotes-inflammation-and-abnormal-differentiation
#18
Mrinal K Sarkar, Nihal Kaplan, Lam C Tsoi, Xianying Xing, Yun Liang, William R Swindell, Paul Hoover, Maya Aravind, Gleb Baida, Matthew Clark, John J Voorhees, Rajan P Nair, James T Elder, Irina Budinova, Spiro Getsios, Johann E Gudjonsson
The factors involved in maintaining a localized inflammatory state in psoriatic skin remain poorly understood. Here, we demonstrate through metabolomic and transcriptomic profiling marked suppression of glucocorticoid biosynthesis in the epidermis of psoriatic skin leading to localized deficiency of cortisol. Utilizing a 3D human epidermis model, we demonstrate that glucocorticoid biosynthesis is suppressed by pro-inflammatory cytokines and that glucocorticoid deficiency promotes inflammatory responses in keratinocytes...
March 1, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28254481/effects-of-the-combined-herba-epimedii-and-fructus-ligustri-lucidi-on-bone-turnover-and-tgf-%C3%AE-1-smads-pathway-in-giop-rats
#19
Yan Yang, Honglei Nian, Xiufeng Tang, Xiujuan Wang, Renhui Liu
ETHNOPHARMACOLOGICAL RELEVANCE: Kidney deficiency is the main pathogenesis of osteoporosis based on the theory of "kidney governing bones" in traditional Chinese medicine (TCM). Combined Herba Epimedii and Fructus Ligustri Lucidi, based on traditional Chinese formula Er-Zhi pills, were frequently used in TCM formulas that were prescribed for kidney tonifying and bone strengthening. However, it is unclear whether the combination of the two herbs may have a protective influence on glucocorticoid-induced osteoporosis (GIOP)...
February 27, 2017: Journal of Ethnopharmacology
https://www.readbyqxmd.com/read/28239861/glucocorticoid-deficient-hypoadrenocorticism-secondary-to-intravascular-lymphoma-in-the-adrenal-glands-of-a-dog
#20
M E Buckley, P S Chapman, A Walsh
CASE REPORT: A 2-year-old neutered male German Shepherd dog was presented with weakness, poor appetite and weight loss. Glucocorticoid-deficient hypoadrenocorticism was diagnosed with undetectable pre- and post-ACTH cortisol concentrations but normal sodium and potassium concentrations. Despite appropriate supplementation with glucocorticoids, the patient's weakness progressed and neurological deficits developed. The patient was euthanased. Histopathological analysis of multiple organs, including the adrenal glands, showed an accumulation of neoplastic lymphocytes within blood vessels, consistent with a diagnosis of intravascular lymphoma...
March 2017: Australian Veterinary Journal
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