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Glucocorticoid deficiency

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https://www.readbyqxmd.com/read/28321652/role-of-plasminogen-activator-inhibitor-1-in-glucocorticoid-induced-muscle-change-in-mice
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
https://www.readbyqxmd.com/read/28224564/increased-glucocorticoid-receptor-expression-in-sepsis-is-related-to-heat-shock-proteins-cytokines-and-cortisol-and-is-associated-with-increased-mortality
#8
Konstantinos Vardas, Stavroula Ilia, Amalia Sertedaki, Evangelia Charmandari, Efrossini Briassouli, Dimitris Goukos, Kleovoulos Apostolou, Katerina Psarra, Efthimia Botoula, Stylianos Tsagarakis, Eleni Magira, Christina Routsi, Constantine A Stratakis, Serafim Nanas, George Briassoulis
BACKGROUND: The purposes of this study are to examine if the human glucocorticoid receptor (hGR) isoform-α mRNA and hGR protein expressions are deficient in the acute phase of sepsis (S) compared to systemic inflammatory response syndrome (SIRS) and healthy subjects (H) and to evaluate if the hGRα and hGR alterations are associated with cortisol changes and if they are related to (1) extracellular and intracellular heat shock proteins (HSP) 72 and 90α; (2) ACTH, prolactin, and interleukins (ILs); and (3) outcome...
December 2017: Intensive Care Medicine Experimental
https://www.readbyqxmd.com/read/28224294/cardio-metabolic-risk-factors-in-youth-with-classical-21-hydroxylase-deficiency
#9
Kansuda Ariyawatkul, Supatporn Tepmongkol, Suphab Aroonparkmongkol, Taninee Sahakitrungruang
Patients with congenital adrenal hyperplasia (CAH) appear to have adverse cardiovascular risk profile and other long-term health problems in adult life, but there are limited data in young CAH patients. We aim to evaluate the cardio-metabolic risk factors in adolescents and young adults with classical 21-hydroxylase deficiency (21-OHD). We performed a cross-sectional study of 21 patients (17 females) with classic CAH detected clinically and not through newborn screening, aged 15.2 ± 5.8 years, and 21 healthy matched controls...
February 21, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28223352/cross-talk-between-the-glucocorticoid-receptor-and-myod-family-inhibitor-domain-containing-protein-provides-a-new-mechanism-for-generating-tissue-specific-responses-to-glucocorticoids
#10
Robert H Oakley, John M Busillo, John A Cidlowski
Glucocorticoids are primary stress hormones that regulate many physiological processes, and synthetic derivatives of these molecules are widely used in the clinic. The molecular factors that govern tissue specificity of glucocorticoids, however, are poorly understood. The actions of glucocorticoids are mediated by the glucocorticoid receptor (GR). To discover new proteins that interact with GR and modulate its function, we performed a yeast 2-hybrid assay. The MyoD family inhibitor domain containing (MDFIC) protein was identified as a binding partner for GR...
February 21, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28220235/successful-discontinuation-of-eculizumab-under-immunosuppressive-therapy-in-deap-hus
#11
Agnes Hackl, Rasmus Ehren, Michael Kirschfink, Peter F Zipfel, Bodo B Beck, Lutz T Weber, Sandra Habbig
BACKGROUND: Deficiency of complement factor H-related plasma proteins and complement factor H autoantibody-positive hemolytic uremic syndrome (DEAP-HUS), which is characterized by the deficiency of complement-factor H-related (CFHR) plasma proteins and the subsequent formation of autoantibodies against complement factor H (CFH), has been reported to have an adverse outcome in one third of patients. Therapy options include prompt removal of antibodies by plasma exchange and immunosuppressive therapy...
February 20, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28207417/adrenarche-unmasks-compound-heterozygous-3%C3%AE-hydroxysteroid-dehydrogenase-deficiency-c-244g-a-p-ala82thr-and-the-novel-931c-t-p-gln311-variant-in-a-non-salt-wasting-severely-undervirilised-46xy
#12
Stephanie Louise Teasdale, Adam Morton
3β-Hydroxysteroid dehydrogenase type II deficiency (3βHSD2) congenital adrenal hyperplasia is a rare cause of ambiguous genitalia, resulting in abnormal virilisation in both 46XY and 46XX. We describe a case of 46XY ambiguous genitalia that was misdiagnosed as androgen insensitivity syndrome. The correct diagnosis was made after adrenarche. Genotyping demonstrated compound heterozygosity in two alleles, the previously described c.244G>A (p.Ala82Thr), and a novel 931C>T(p.Gln311*) variant. We suggest that adrenarche unmasked the condition by driving cortisol production to rates that caused the mutant 3bHSD2 enzyme to become rate limiting for cortisol production...
March 1, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28199350/25-hydroxyvitamin-d3-and-1-25-dihydroxyvitamin-d3-exert-distinct-effects-on-human-skeletal-muscle-function-and-gene-expression
#13
Zaki K Hassan-Smith, Carl Jenkinson, David J Smith, Ivan Hernandez, Stuart A Morgan, Nicola J Crabtree, Neil J Gittoes, Brian G Keevil, Paul M Stewart, Martin Hewison
Age-associated decline in muscle function represents a significant public health burden. Vitamin D-deficiency is also prevalent in aging subjects, and has been linked to loss of muscle mass and strength (sarcopenia), but the precise role of specific vitamin D metabolites in determining muscle phenotype and function is still unclear. To address this we quantified serum concentrations of multiple vitamin D metabolites, and assessed the impact of these metabolites on body composition/muscle function parameters, and muscle biopsy gene expression in a retrospective study of a cohort of healthy volunteers...
2017: PloS One
https://www.readbyqxmd.com/read/28188961/high-serum-progesterone-associated-with-infertility-in-a-woman-with-nonclassic-congenital-adrenal-hyperplasia
#14
Yoshimasa Kawarai, Hiroshi Ishikawa, Tomoya Segawa, Shokichi Teramoto, Tomoaki Tanaka, Makio Shozu
Nonclassic congenital adrenal hyperplasia (NCAH) is an autosomal-recessive disorder caused by 21-hydroxylase deficiency and manifests as hirsutism and oligomenorrhea due to excess adrenal androgen and progesterone. We report a case of a woman with NCAH who showed continuous high serum progesterone levels in the follicular phase associated with impaired folliculogenesis. NCAH was diagnosed based on high 17-hydroxyprogesterone levels after rapid adrenocorticotropic hormone loading, and three heterozygous missense mutations in CYP21A2, encoding 21-hydroxylase, were identified...
February 11, 2017: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/28186655/chronic-transgenerational-vitamin-b12-deficiency-of-severe-and-moderate-magnitudes-modulates-adiposity-probable-underlying-mechanisms
#15
Shampa Ghosh, Jitendra Kumar Sinha, Bojanapalli Muralikrishna, Uday Kumar Putcha, Manchala Raghunath
We have demonstrated previously that severe but not moderate vitamin B12 deficiency altered body composition and induced adiposity in female C57BL/6 mice. This study aims to elucidate the effects of chronic transgenerational dietary vitamin B12 restriction on body composition and various biochemical parameters in the F1 generation offspring of our mouse models of severe and moderate vitamin B12 deficiency established earlier. Female weanling C57BL/6 mice received, ad libitum, for 4 weeks a (i) control diet, (ii) vitamin B12-restricted diet with pectin as dietary fiber (severely deficient diet), or (iii) vitamin B12-restricted diet with cellulose as dietary fiber (moderately deficient diet) and then mated with control males...
February 10, 2017: BioFactors
https://www.readbyqxmd.com/read/28165470/sodium-chloride-promotes-tissue-inflammation-via-osmotic-stimuli-in-subtotal-nephrectomized-mice
#16
Fumiko Sakata, Yasuhiko Ito, Masashi Mizuno, Akiho Sawai, Yasuhiro Suzuki, Takako Tomita, Mitsuhiro Tawada, Akio Tanaka, Akiyoshi Hirayama, Akihiro Sagara, Takashi Wada, Shoichi Maruyama, Tomoyoshi Soga, Seiichi Matsuo, Enyu Imai, Yoshifumi Takei
Chronic inflammation, which is often associated with high all-cause and cardiovascular mortality, is prevalent in patients with renal failure; however, the precise mechanisms remain unclear. High-salt intake was reported to induce lymphangiogenesis and autoimmune diseases via osmotic stimuli with accumulation of sodium or chloride. In addition, sodium was recently reported to be stored in the extremities of dialysis patients. We studied the effects and mechanisms of high salt loading on tissue and systemic inflammation in subtotal-nephrectomized mice (5/6Nx) and in cultured cells...
February 6, 2017: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/28137896/vitamin-d-deficiency-and-impaired-placental-function-potential-regulation-by-glucocorticoids
#17
Nathanael Yates, Rachael Christina Crew, Caitlin Wyrwoll
Maternal vitamin D deficiency has been implicated in a range of pregnancy complications including preeclampsia, preterm birth and intrauterine growth restriction. Some of these adverse outcomes arise from alterations in placental function. Indeed, vitamin D appears critical for implantation, inflammation, immune function and angiogenesis of the placenta. Despite these associations, absence of placental vitamin D receptor in mice provokes little effect. Thus, interactions between maternal and fetal compartments are likely crucial for instigating adverse placental changes...
January 30, 2017: Reproduction: the Official Journal of the Society for the Study of Fertility
https://www.readbyqxmd.com/read/28137450/crf1-receptor-deficiency-increases-cocaine-reward
#18
Angelo Contarino, Pierre Kitchener, Monique Vallée, Francesco Papaleo, Pier-Vincenzo Piazza
Stimulant drugs produce reward but also activate stress-responsive systems. The corticotropin-releasing factor (CRF) and the related hypothalamus-pituitary-adrenal (HPA) axis stress-responsive systems are activated by stimulant drugs. However, their role in stimulant drug-induced reward remains poorly understood. Herein, we report that CRF1 receptor-deficient (CRF1-/-), but not wild-type, mice show conditioned place preference (CPP) responses to a relatively low cocaine dose (5 mg/kg, i.p.). Conversely, wild-type, but not CRF1-/-, mice display CPP responses to a relatively high cocaine dose (20 mg/kg, i...
January 27, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28126912/growth-hormone-with-aromatase-inhibitor-may-improve-height-in-cyp11b1-congenital-adrenal-hyperplasia
#19
Katherine Hawton, Sandra Walton-Betancourth, Gill Rumsby, Joseph Raine, Mehul Dattani
With an estimated prevalence of 1 in 100 000 births, 11β-hydroxylase deficiency is the second most common form of congenital adrenal hyperplasia (CAH) and is caused by mutations in CYP11B1 Clinical features include virilization, early gonadotropin-independent precocious puberty, hypertension, and reduced stature. The current mainstay of management is with glucocorticoids to replace deficient steroids and to minimize adrenal sex hormone overproduction, thus preventing virilization and optimizing growth. We report a patient with CAH who had been suboptimally treated and presented to us at 6 years of age with precocious puberty, hypertension, tall stature, advanced bone age, and a predicted final height of 150 cm...
February 2017: Pediatrics
https://www.readbyqxmd.com/read/28117144/review-alterations-in-placental-glycogen-deposition-in-complicated-pregnancies-current-preclinical-and-clinical-evidence
#20
REVIEW
Lisa K Akison, Marloes Dekker Nitert, Vicki L Clifton, Karen M Moritz, David G Simmons
Normal placental function is essential for optimal fetal growth. Transport of glucose from mother to fetus is critical for fetal nutrient demands and can be stored in the placenta as glycogen. However, the function of this glycogen deposition remains a matter of debate: It could be a source of fuel for the placenta itself or a storage reservoir for later use by the fetus in times of need. While the significance of placental glycogen remains elusive, mounting evidence indicates that altered glycogen metabolism and/or deposition accompanies many pregnancy complications that adversely affect fetal development...
January 11, 2017: Placenta
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