6,388,596 results match your criteria Diseases of Tetrapyrrole Metabolism: Refsum Disease and the Hepatic Porphyrias

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Limberg flap reconstruction for sacrococcygeal pilonidal sinus disease with and without acute abscess: Our experience and a review of the literature.

Arch Plast Surg 2019 Apr 20. Epub 2019 Apr 20.

Long Island Plastic Surgical Group, Garden City, NY, USA.

Background: The efficacy of Limberg flap reconstruction for pilonidal sinus with acute abscess remains unclear. This study aimed to compare outcomes after Limberg flap reconstruction for pilonidal sinus disease with and without acute abscess. A secondary objective was to perform a review of the literature on the topic. Read More

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http://dx.doi.org/10.5999/aps.2018.01312DOI Listing

Management of severe hidradenitis suppurativa with biologic therapy and wide excision.

Arch Plast Surg 2019 Apr 20. Epub 2019 Apr 20.

National Skin Centre, Singapore.

Hidradenitis suppurativa (HS) is a chronic inflammatory follicular occlusive disease that involves the intertriginous areas. Treatment methods include conventional topical and systemic medication, radiotherapy, biologic agents, and surgical excision. Of late, there has been an increased focus on the use of biologic agents in patients with moderate to severe HS. Read More

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http://dx.doi.org/10.5999/aps.2018.00339DOI Listing

Theracurmin Ameliorates Cognitive Dysfunctions in 5XFAD Mice by Improving Synaptic Function and Mitigating Oxidative Stress.

Biomol Ther (Seoul) 2019 Apr 19. Epub 2019 Apr 19.

Departments of Life and Nanopharmaceutical Science, Kyung Hee University, Seoul 02447, Republic of Korea.

As the elderly population is increasing, Alzheimer’s disease (AD) has become a global issue and many clinical trials have been conducted to evaluate treatments for AD. As these clinical trials have been conducted and have failed, the development of new theraphies for AD with fewer adverse effects remains a challenge. In this study, we examined the effects of Theracurmin on cognitive decline using 5XFAD mice, an AD mouse model. Read More

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http://dx.doi.org/10.4062/biomolther.2019.046DOI Listing

Disseminated Trichosporon asahii infection in a combined liver-kidney transplant recipient successfully treated with voriconazole.

Immun Inflamm Dis 2019 Apr 20. Epub 2019 Apr 20.

Department of Infectious Disease, Medanta The Medicity, Gurgaon, Haryana, India.

Introduction: Trichosporon asahii is an emerging cause of systemic fungal infection in an immunocompromised host. Several life threatening disseminated T. asahii infection in single solid organ (liver or kidney) transplant recipients, in neutropenic and hematological malignancy patients have been reported. Read More

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http://dx.doi.org/10.1002/iid3.250DOI Listing

Urokinase-type plasminogen activator (uPA) is not essential for epithelial sodium channel (ENaC)-mediated sodium retention in experimental nephrotic syndrome.

Acta Physiol (Oxf) 2019 Apr 21:e13286. Epub 2019 Apr 21.

Department of Internal Medicine, Division of Endocrinology Division of Endocrinology Diabetology, Vascular Disease, Nephrology and Clinical Chemistry, University Hospital Tübingen, Germany.

Aim: In nephrotic syndrome, aberrantly filtered plasminogen (plg) is converted to active plasmin by tubular urokinase-type plasminogen activator (uPA) and thought to lead to sodium retention by proteolytic activation of the epithelial sodium channel (ENaC). This concept predicts that uPA is an important factor for sodium retention and that inhibition of uPA might be protective in nephrotic syndrome.

Methods: Activation of amiloride-sensitive currents by uPA and plg were studied in Xenopus laevis oocytes expressing murine ENaC. Read More

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http://dx.doi.org/10.1111/apha.13286DOI Listing

MiR-204-5p: a novel candidate urinary biomarker of Xp11.2 translocation renal cell carcinoma.

Cancer Sci 2019 Apr 21. Epub 2019 Apr 21.

Department of Molecular Genetics.

Xp11.2 translocation renal cell carcinoma (Xp11 tRCC) is a rare sporadic pediatric kidney cancer caused by constitutively active TFE3 fusion proteins. Tumors in patients with Xp11 tRCC tend to recur and undergo frequent metastasis, in part due to lack of methods available to detect early stage disease. Read More

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http://dx.doi.org/10.1111/cas.14026DOI Listing
April 2019
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Burden of Neurodegenerative Diseases in the Eastern Mediterranean Region, 1990-2016: Findings from the Global Burden of Disease 2016 Study.

Eur J Neurol 2019 Apr 21. Epub 2019 Apr 21.

Department of Community Medicine, Preventive Medicine and Public Health Research Center, Iran University of Medical Sciences, Tehran, Iran.

Background And Purpose: The Eastern Mediterranean Region (EMR) is experiencing a demographic shift towards rapid ageing at a time of political unrest. We aimed to estimate the burden of neurodegenerative disorders, and its relationship with sociodemographic indicators (SDI) in the EMR countries from 1990 to 2016.

Methods: Using data from the Global Burden of Disease (GBD) 2016 study, we calculated country-specific trends for prevalence, mortality, disability-adjusted life-years (DALYs), years of life lost (YLLs), and years lived with disability (YLDs) for Alzheimer's disease/other dementias and Parkinson's disease in the EMR during 1990-2016. Read More

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http://dx.doi.org/10.1111/ene.13972DOI Listing

Phosphorylated NUB1 distinguishes α-synuclein in Lewy bodies from that in glial cytoplasmic inclusions in multiple system atrophy.

Brain Pathol 2019 Apr 21. Epub 2019 Apr 21.

Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki, 036-8562, Japan.

Posttranslational modifications by phosphorylation, ubiquitination, neddylation, and other pathways have emerged as major regulators of cellular functions. NEDD8 ultimate buster 1, NUB1, is an adaptor protein, which negatively regulates the levels of the ubiquitin-like protein NEDD8 as well as neddylated proteins through proteasomal degradation. We previously reported that NUB1 is highly involved in the pathogenesis of synucleinopathy including Parkinson's disease (PD), dementia with Lewy bodies (DLB) and multiple system atrophy (MSA). Read More

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http://dx.doi.org/10.1111/bpa.12728DOI Listing

Expression and biological functions of the CCL5-CCR5 axis in oral lichen planus.

Exp Dermatol 2019 Apr 21. Epub 2019 Apr 21.

Jiangsu Key Laboratory of Oral Diseases, Nanjing Medical University, Nanjing, Jiangsu, China.

Oral lichen planus (OLP) is a chronic inflammatory oral mucosal disorder with T cell-mediated immunological pathogenesis. C-C motif chemokine ligand 5 (CCL5) and its receptor C-C motif chemokine receptor 5 (CCR5) play important roles in the activation and recruitment of T cells. This study sought to explore the expression and biological functions of the CCL5-CCR5 axis in OLP. Read More

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http://dx.doi.org/10.1111/exd.13946DOI Listing

Novel Oral Lichen Planus Symptom Severity Measure for assessing patients' daily symptom experience.

Oral Dis 2019 Apr 21. Epub 2019 Apr 21.

AFYX Therapeutics, formerly known as Dermtreat, 2300, Copenhagen S, Denmark.

Objective: A novel Oral Lichen Planus Symptom Severity Measure was developed as a clinical outcome assessment of the daily symptom experience of patients with oral lichen planus.

Methods: A literature review and expert input were followed by open-ended concept elicitation interviews with 17 adults with oral lichen planus in the US and Ireland. Item content was generated and the interviews continued until input saturation was reached. Read More

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http://dx.doi.org/10.1111/odi.13109DOI Listing

Dermoscopy of Pagetoid reticulosis, with dermoscopic-pathologic correlation.

Photodermatol Photoimmunol Photomed 2019 Apr 21. Epub 2019 Apr 21.

First Dermatology Department, Aristotle University of Thessaloniki, Greece.

Pagetoid reticulosis (PR), also known as Woringer-Kolopp disease, is a rare variant of mycosis fungoides with distinctive clinicopathologic features. It clinically manifests as a solitary, erythematous, gradually enlarging, scaly or verrucous plaque on the lower extremities, and due to its indolent course and non-specific clinical features, may remain undiagnosed for years. In the current we describe the clinical and dermoscopic characteristics of a rare case of PR disease, and correlate them with the corresponding histopathologic findings. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/phpp.12472
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http://dx.doi.org/10.1111/phpp.12472DOI Listing
April 2019
4 Reads

Bibliometric analysis of the top-cited articles on oral erythroplakia and leukoplakia.

Authors:
Xi Yang Wei Liu

J Oral Pathol Med 2019 Apr 21. Epub 2019 Apr 21.

Department of Oral and Maxillofacial-Head and Neck Oncology, Shanghai Ninth People's Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine, National Clinical Research Center for Oral Diseases, Shanghai Key Laboratory of Stomatology & Shanghai Research Institute of Stomatology, Shanghai, China.

We read with interest the recent article (jop12683) by Foy et al. [1] entitled "Bibliometric analysis of a century of research on oral erythroplakia and leukoplakia". We congratulate the authors on conducting the first bibliometric analysis on oral erythroplakia and leukoplakia (OEL). Read More

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http://dx.doi.org/10.1111/jop.12863DOI Listing

An updated review of lipid-modifying therapy.

Authors:
Leon A Simons

Med J Aust 2019 Apr 21. Epub 2019 Apr 21.

UNSW Sydney, Sydney, NSW.

Statin drugs reduce low-density lipoprotein (LDL)-cholesterol (LDL-C) and cardiovascular risk. Ezetimibe may be used to supplement statin therapy, or used alone in cases of statin intolerance. Statin-associated side effects do occur, especially muscle symptoms and new onset diabetes, but they do not detract from the benefits of statin therapy. Read More

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http://dx.doi.org/10.5694/mja2.50142DOI Listing

HIV Status Does Not Worsen Oral Health Outcomes.

J Clin Periodontol 2019 Apr 21. Epub 2019 Apr 21.

Department of Oral Biology, School of Dental Medicine, University of Pittsburgh.

Last January 31 , Journal of Clinical Periodontology just made available the report titled "A Retrospective Analysis of Dental Implant Survival in HIV Patients", which concluded that "implants placed in HIV-positive patients had similar survival rates as HIV-negative patients." These data support our hypothesis that infection by HIV does not lead to worse oral health outcomes, including worse periodontitis. We looked 6,092 individuals and selected all HIV-positive subjects (N=73) and matched them by age, sex, ethnicity, and smoking habits with 261 HIV-negative control subjects. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/jcpe.13116
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http://dx.doi.org/10.1111/jcpe.13116DOI Listing
April 2019
1 Read

Incretin-based Therapies for the Management of NAFLD in Patients with Type 2 Diabetes.

Authors:
Kenneth Cusi

Hepatology 2019 Apr 21. Epub 2019 Apr 21.

Division of Endocrinology, Diabetes and Metabolism, University of Florida, Gainesville, FL.

The prevalence of nonalcoholic fatty liver disease (NAFLD) in type 2 diabetes (T2DM) is believed to be ~70% and that of advanced fibrosis ~20%. Given the magnitude of the problem, there is a real sense of urgency to identify the best pharmacological approach for to treat both diabetes and NASH in this population. Vitamin E is recommended for patients with biopsy-proven NASH without diabetes but did not have similar efficacy in a recent proof-of-concept RCT in patients with T2DM. Read More

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http://dx.doi.org/10.1002/hep.30670DOI Listing

ABCA1 variants rs2230806 (R219K), rs4149313 (M8831I), and rs9282541 (R230C) are associated with susceptibility to coronary heart disease.

J Clin Lab Anal 2019 Apr 20:e22896. Epub 2019 Apr 20.

Department of Cardiovascular Disease, Changzhou No. 2 People's Hospital, Affiliated Nanjing Medical University, Changzhou, China.

Background: To investigate the association between three single nucleotide polymorphisms (SNPs) of ABCA1 gene and susceptibility to coronary heart disease (CHD) in Chinese Han population.

Methods: A total of 484 CHD patients and 488 controls were included in the study. Three SNPs rs2230806 (R219K), rs4149313 (M8831I), and rs9282541 (R230C) in ABCA1 gene were genotyped by SNaPshot. Read More

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http://dx.doi.org/10.1002/jcla.22896DOI Listing

Testosterone deficiency in men with Type 2 diabetes: pathophysiology and treatment.

Diabet Med 2019 Apr 21. Epub 2019 Apr 21.

Department of Medicine Austin Health, University of Melbourne.

Epidemiological studies consistently demonstrate that lowered serum testosterone is not only common in men with established Type 2 diabetes, but also predicts future diabetic risks and increased mortality. Preclinical studies report plausible mechanisms by which low testosterone could mediate dysglycaemia. Exogenous testosterone treatment consistently reduces fat mass, increases muscle mass and improves insulin resistance in some studies, but the majority of currently available randomized controlled trials (RCTs) do not report a consistent glycaemic benefit. Read More

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http://dx.doi.org/10.1111/dme.13977DOI Listing

The basophil-IL-4-mast cell axis is required for food allergy.

Allergy 2019 Apr 21. Epub 2019 Apr 21.

Laboratory for Allergic Disease, Yokohama, 230-0045, Japan.

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http://dx.doi.org/10.1111/all.13834DOI Listing

Influenza and pertussis vaccination of women during pregnancy in Victoria, 2015-2017.

Med J Aust 2019 Apr 21. Epub 2019 Apr 21.

Monash University, Melbourne, VIC.

Objectives: To assess variations by time of year and hospital in the uptake of influenza and pertussis vaccinations by pregnant women in Victoria; to identify factors associated with vaccination uptake.

Design, Setting: Retrospective analysis of data in the Victorian Perinatal Data Collection (VPDC), a population surveillance system for obstetric conditions, procedures, and pregnancy and birth outcomes.

Participants: Women whose pregnancies ended in a live or stillbirth during July 2015 - June 2017. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.5694/mja2.50125
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http://dx.doi.org/10.5694/mja2.50125DOI Listing

Intra-articular triamcinolone acetonide improves lung function in horses with severe asthma.

Equine Vet J 2019 Apr 21. Epub 2019 Apr 21.

Department of Clinical Sciences, Faculté de Médecine Vétérinaire, Université de Montréal, 3200 rue Sicotte, Saint-Hyacinthe, J2S 2M2, Quebec, Canada.

Background: Locally administered corticosteroids are commonly used to treat joint diseases in sport and racehorses. As they are also the most potent drugs for the treatment of equine asthma, we hypothesised that the intra-articular corticosteroids used to treat joint diseases also improve the lung function in horses with severe asthma, thus potentially delaying the diagnosis of this common lung condition.

Objectives: To compare the effects of intra-articular (IA) and intramuscular (IM) triamcinolone acetonide (TA) on lung function in horses with severe asthma. Read More

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http://dx.doi.org/10.1111/evj.13128DOI Listing

A microbiome analysis of equine peripheral dental caries using next generation sequencing.

Equine Vet J 2019 Apr 21. Epub 2019 Apr 21.

Division of Veterinary Clinical Studies, Royal (Dick) School of Veterinary Studies and Roslin Institute, The University of Edinburgh, Midlothian, UK.

Background: Although, peripheral caries (PC) affects almost half of UK horses, no comprehensive microbiological study has been performed on this disorder. Because a high proportion of oral bacteria cannot be conventionally cultured, a molecular microbiological technique such as Next Generation Sequencing are required to examine the complex oral bacteria community.

Objectives: To identify the microbiota involved in equine PC, including comparing microbiota at the more commonly and severely affected three caudal cheek teeth with the less commonly affected three rostral cheek teeth. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/evj.13126
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http://dx.doi.org/10.1111/evj.13126DOI Listing
April 2019
1 Read

Differential vascular effects of aspirin in people with Type 2 diabetes without cardiovascular disease and matched controls without diabetes.

Diabet Med 2019 Apr 21. Epub 2019 Apr 21.

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

Aim: We investigated whether the effect of low-dose aspirin on endothelium-dependent vasodilation and arterial stiffness in people with Type 2 diabetes is different from a matched control group. We examined acute and chronic effects, and effects over the 24h dosing interval.

Methods: In an open-label parallel group intervention study, we included 21 participants with Type 2 diabetes and 21 age- and sex-matched controls. Read More

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http://dx.doi.org/10.1111/dme.13978DOI Listing

Clinical Pharmacogenetics Implementation Consortium (CPIC) Guideline for CYP2B6 and Efavirenz-containing Antiretroviral Therapy.

Clin Pharmacol Ther 2019 Apr 21. Epub 2019 Apr 21.

Department of Internal Medicine, Meharry Medical College School of Medicine, Nashville, TN, USA.

The human immunodeficiency virus (HIV) type-1 non-nucleoside reverse transcriptase inhibitor, efavirenz, is widely used to treat HIV-1 infection. Efavirenz is predominantly metabolized into inactive metabolites by CYP2B6, and patients with certain CYP2B6 genetic variants may be at increased risk for adverse effects, particularly central nervous system toxicity and treatment discontinuation. We summarize the evidence from the literature and provide therapeutic recommendations for efavirenz prescribing based on CYP2B6 genotypes. Read More

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http://dx.doi.org/10.1002/cpt.1477DOI Listing

A novel RNA aptamer identifies plasma membrane ATP synthase beta subunit as an early marker and therapeutic target in aggressive cancer.

Breast Cancer Res Treat 2019 Apr 20. Epub 2019 Apr 20.

Department of Medicine, Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, USA.

Purpose: Primary breast and prostate cancers can be cured, but metastatic disease cannot. Identifying cell factors that predict metastatic potential could guide both prognosis and treatment.

Methods: We used Cell-SELEX to screen an RNA aptamer library for differential binding to prostate cancer cell lines with high vs. Read More

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http://link.springer.com/10.1007/s10549-019-05174-3
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http://dx.doi.org/10.1007/s10549-019-05174-3DOI Listing
April 2019
1 Read

Comparative transcriptome profiling of resistant and susceptible sugarcane genotypes in response to the airborne pathogen Fusarium verticillioides.

Mol Biol Rep 2019 Apr 20. Epub 2019 Apr 20.

Key Laboratory of Sugarcane Biotechnology and Genetic Improvement (Guangxi), Ministry of Agriculture, Sugarcane Research Institute, Guangxi Academy of Agricultural Sciences, Sugarcane Research Center, Chinese Academy of Agricultural Science, Nanning, 530007, China.

Fusarium verticillioides is the pathogen associated with pokkah boeng disease (PBD), the most significant airborne disease of sugarcane. The molecular mechanisms that regulate the defense responses of sugarcane towards this fungus are not yet fully known. Samples of 'YT 94/128' (resistant, R) and 'GT 37' (susceptible, S) inoculated with F. Read More

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http://link.springer.com/10.1007/s11033-019-04820-9
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http://dx.doi.org/10.1007/s11033-019-04820-9DOI Listing
April 2019
2 Reads

The molecular basis and genotype-phenotype correlations of congenital adrenal hyperplasia (CAH) in Anatolian population.

Mol Biol Rep 2019 Apr 20. Epub 2019 Apr 20.

Department of Medical Genetics, Erciyes University, Kayseri, 38039, Turkey.

Congenital adrenal hyperplasia (CAH) is an autosomal recessive genetic disorder due to presence of mutations in the genes involved in the metabolism of steroid hormones in adrenal gland. There are two main forms of CAH, classic form and non-classic form. While classic form stands for the severe form, the non-classic form stands for the moderate and more frequent form of CAH. Read More

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http://link.springer.com/10.1007/s11033-019-04809-4
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http://dx.doi.org/10.1007/s11033-019-04809-4DOI Listing

Potential risk factors of ovarian cancer and analysis of CA125, a biomarker used for its monitoring and diagnosis.

Mol Biol Rep 2019 Apr 20. Epub 2019 Apr 20.

School of Life and Environmental Sciences, University of Sydney, Sydney, Australia.

Ovarian cancer (OC) is the fourth most common cancer among Pakistani, Scottish and Chinese women. The aim of the present study was to determine the association of potential risk factors with OC and analysis of Cancer Antigen 125 (CA125) in its monitoring and diagnosis. A total of 200 patients diagnosed with OC were included in this study. Read More

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http://dx.doi.org/10.1007/s11033-019-04794-8DOI Listing

Lycopene protects against t-BHP-induced neuronal oxidative damage and apoptosis via activation of the PI3K/Akt pathway.

Mol Biol Rep 2019 Apr 20. Epub 2019 Apr 20.

Department of Pathophysiology, Institute of Brain Science Research, Key Laboratory of State Administration of Traditional Chinese Medicine of the People's Republic of China, School of Medicine, Jinan University, Guangzhou, 510632, Guangdong, China.

Oxidative stress is a key factor of and closely implicated in the pathogenesis of Alzheimer's disease (AD). We herein used tert-butyl hydroperoxide (t-BHP) to induce oxidative stress and mimic oxidative neurotoxicity in vitro. Lycopene is a natural antioxidant that has a strong ability to eliminate free radicals and shows effective protection in some neurodegenerative disease models. Read More

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http://link.springer.com/10.1007/s11033-019-04801-y
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http://dx.doi.org/10.1007/s11033-019-04801-yDOI Listing
April 2019
1 Read

Regulation of Neprilysin Activity and Cognitive Functions in Rats After Prenatal Hypoxia.

Neurochem Res 2019 Apr 20. Epub 2019 Apr 20.

I.M. Sechenov Institute of Evolutionary Physiology and Biochemistry, Russian Academy of Sciences, 44 Thorez av, St. Petersburg, Russia, 194223.

The amyloid-degrading enzyme neprilysin (NEP) is one of the therapeutic targets in prevention and treatment of Alzheimer's disease (AD). As we have shown previously NEP expression in rat parietal cortex (Cx) and hippocampus (Hip) decreases with age and is also significantly reduced after prenatal hypoxia. Following the paradigms for enhancement of NEP expression and activity developed in cell culture, we analysed the efficacy of various compounds able to upregulate NEP using our model of prenatal hypoxia in rats. Read More

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http://link.springer.com/10.1007/s11064-019-02796-3
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http://dx.doi.org/10.1007/s11064-019-02796-3DOI Listing
April 2019
1 Read

A case of X-linked retinoschisis with atypical fundus appearance.

Doc Ophthalmol 2019 Apr 20. Epub 2019 Apr 20.

Institute for Ophthalmic Research, Centre for Ophthalmology, Eberhard Karls University, Elfriede-Aulhorn Strasse 7, 72076, Tübingen, Germany.

Purpose: Mutations in the RS1 gene are known to cause retinoschisis, an X-linked hereditary retinal degeneration. Here, we present a case of atypical retinoschisis with clinical findings of retinoschisis and retinitis pigmentosa.

Methods: This report is an observational case report. Read More

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http://dx.doi.org/10.1007/s10633-019-09698-3DOI Listing

Structure and Function of an Inflammatory Cytokine, Interleukin-2, Analyzed Using the Bioinformatic Approach.

Authors:
Urmi Roy

Protein J 2019 Apr 20. Epub 2019 Apr 20.

Department of Chemistry & Biomolecular Science, Clarkson University, 8 Clarkson Avenue, Potsdam, NY, 13699-5820, USA.

The inflammatory cytokine, interleukin-2 (IL-2), is an important regulator of cellular functions. This relatively less studied member of the interleukin protein family is responsible for multiple immuno-modulatory and immuno-stimulatory tasks, like T cell activation, triggering of natural killer cells, inflammation, as well as proliferation and progression of autoimmune diseases and cancers. In this communication we report the temporally variant structural aspects of the IL-2 ligand and its receptor interfaces, based on the available crystal structures. Read More

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http://dx.doi.org/10.1007/s10930-019-09833-8DOI Listing

Impact of CFTR-modulating drugs on GH-IGF-1 axis impairment in adult patients with cystic fibrosis.

J Endocrinol Invest 2019 Apr 20. Epub 2019 Apr 20.

Section of Medical Pathophysiology, Endocrinology, Department Experimental Medicine, Sapienza University of Rome, 00161, Rome, Italy.

Introduction: A new class of drugs in the treatment of cystic fibrosis (CF) includes two agents: lumacaftor, which corrects CFTR channel protein, and ivacaftor, which increases CFTR channel activity. In our previous study we recruited 50 stable adults with CF and 16 of them showed growth hormone deficit (GHD): 7 patients severe and 9 patients partial GHD.

Material And Methods: We decided to re-evaluate ten patients with the GHRH + arginine test of whom only five were treated with lumacaftor/ivacaftor. Read More

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http://dx.doi.org/10.1007/s40618-019-01051-4DOI Listing

Selective vulnerability in α-synucleinopathies.

Acta Neuropathol 2019 Apr 20. Epub 2019 Apr 20.

Department of Movement and Clinical Neurosciences, UCL Queen Square Institute of Neurology, University College London, Rowland Hill Street, London, NW3 2PF, UK.

Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy are neurodegenerative disorders resulting in progressive motor/cognitive deficits among other symptoms. They are characterised by stereotypical brain cell loss accompanied by the formation of proteinaceous aggregations of the protein α-synuclein (α-syn), being, therefore, termed α-synucleinopathies. Although the presence of α-syn inclusions is a common hallmark of these disorders, the exact nature of the deposited protein is specific to each disease. Read More

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http://link.springer.com/10.1007/s00401-019-02010-2
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http://dx.doi.org/10.1007/s00401-019-02010-2DOI Listing

Galectin-3, a novel endogenous TREM2 ligand, detrimentally regulates inflammatory response in Alzheimer's disease.

Acta Neuropathol 2019 Apr 20. Epub 2019 Apr 20.

Department of Experimental Medical Science, Experimental Neuroinflammation Laboratory, Lund University, 221 84, Lund, Sweden.

Alzheimer's disease (AD) is a progressive neurodegenerative disease in which the formation of extracellular aggregates of amyloid beta (Aβ) peptide, fibrillary tangles of intraneuronal tau and microglial activation are major pathological hallmarks. One of the key molecules involved in microglial activation is galectin-3 (gal3), and we demonstrate here for the first time a key role of gal3 in AD pathology. Gal3 was highly upregulated in the brains of AD patients and 5xFAD (familial Alzheimer's disease) mice and found specifically expressed in microglia associated with Aβ plaques. Read More

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http://dx.doi.org/10.1007/s00401-019-02013-zDOI Listing

A coupled reaction-diffusion-strain model predicts cranial vault formation in development and disease.

Biomech Model Mechanobiol 2019 Apr 20. Epub 2019 Apr 20.

Department of Mechanical and Nuclear Engineering, Pennsylvania State University, 320 Leonhard Building, University Park, PA, 16802, USA.

How cells utilize instructions provided by genes and integrate mechanical forces generated by tissue growth to produce morphology is a fundamental question of biology. Dermal bones of the vertebrate cranial vault are formed through the direct differentiation of mesenchymal cells on the neural surface into osteoblasts through intramembranous ossification. Here we join a self-organizing Turing mechanism, computational biomechanics, and experimental data to produce a 3D representative model of the growing cerebral surface, cranial vault bones, and sutures. Read More

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http://link.springer.com/10.1007/s10237-019-01139-z
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http://dx.doi.org/10.1007/s10237-019-01139-zDOI Listing
April 2019
1 Read

Micronutrient status and leukocyte telomere length in school-age Colombian children.

Eur J Nutr 2019 Apr 20. Epub 2019 Apr 20.

Department of Epidemiology, University of Michigan School of Public Health, Room 5507 SPH II, 1415 Washington Heights, Ann Arbor, MI, 48109, USA.

Purpose: Leukocyte telomere length (LTL) is a biomarker of inflammation and oxidative stress that predicts chronic disease risk. Nutritional factors are related to LTL in adulthood, but these associations are not well characterized in children. We examined whether micronutrient status biomarkers were associated with LTL in school-age children. Read More

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http://dx.doi.org/10.1007/s00394-019-01966-xDOI Listing

Adherence to Mediterranean diet in Crohn's disease.

Eur J Nutr 2019 Apr 20. Epub 2019 Apr 20.

Department of Dietetics and Nutritional Science, School of Health Science and Education, Harokopio University, 70 El. Venizelou Ave, 17671, Athens, Greece.

Purpose: To assess the adherence to MD in patients with Crohn's disease (CD).

Methods: Outpatients with CD were enrolled in this protocol. Medical history, disease activity, dietary intake, habitual Mediterranean diet (MedDiet) score, anthropometric measurements and Inflammatory Bowel Disease Questionnaire (IBDQ) were recorded. Read More

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http://dx.doi.org/10.1007/s00394-019-01972-zDOI Listing
April 2019
1 Read

A Genome-Wide Association Study of Bisphosphonate-Associated Atypical Femoral Fracture.

Calcif Tissue Int 2019 Apr 20. Epub 2019 Apr 20.

Department of Medical Sciences, Uppsala University, Uppsala, Sweden.

Atypical femoral fracture is a well-documented adverse reaction to bisphosphonates. It is strongly related to duration of bisphosphonate use, and the risk declines rapidly after drug withdrawal. The mechanism behind bisphosphonate-associated atypical femoral fracture is unclear, but a genetic predisposition has been suggested. Read More

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http://dx.doi.org/10.1007/s00223-019-00546-9DOI Listing

On prognostic estimates of radiation risk in medicine and radiation protection.

Radiat Environ Biophys 2019 Apr 20. Epub 2019 Apr 20.

Department of Physics, Science Faculty, University of Zürich, Winterthurerstrasse 190, 8057, Zurich, Switzerland.

The problem of expressing cumulative detrimental effect of radiation exposure is revisited. All conventionally used and computationally complex lifetime or time-integrated risks are based on current population and health statistical data, with unknown future secular trends, that are projected far into the future. It is shown that application of conventionally used lifetime or time-integrated attributable risks (LAR, AR) should be limited to exposures under 1 Gy. Read More

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http://link.springer.com/10.1007/s00411-019-00794-1
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http://dx.doi.org/10.1007/s00411-019-00794-1DOI Listing
April 2019
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Age dependent ultrasound B-mode findings of the elbow joint in healthy children and adolescents.

Rheumatol Int 2019 Apr 20. Epub 2019 Apr 20.

Department of Pediatrics, Asklepios Hospital Weißenfels, Naumburger Strasse 76, 06667, Weissenfels, Germany.

Due to maturation of joints, various changes take place, not only in the field of paediatric rheumatology but also in paediatric orthopaedics musculoskeletal ultrasound plays an important role in both the diagnosis and the follow-up of diseases in this field. To differentiate between physiological and pathological findings, the knowledge of reference values of joint structures is indispensable. The objective was to define B-mode ultrasound age- and sex-related reference values for the elbow joint in healthy children and adolescents during maturation. Read More

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http://dx.doi.org/10.1007/s00296-018-4233-8DOI Listing
April 2019
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Sunitinib in pediatric patients with advanced gastrointestinal stromal tumor: results from a phase I/II trial.

Cancer Chemother Pharmacol 2019 Apr 20. Epub 2019 Apr 20.

Pediatrics, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA, USA.

Background: Sunitinib is approved for treatment of adults with imatinib-resistant gastrointestinal stromal tumor (GIST) or imatinib intolerance.

Methods: This single-arm, multicenter, multinational phase I/II clinical trial (NCT01396148) enrolled eligible patients aged 6 to < 18 years with advanced, unresectable GIST with non-mutant KIT, or who demonstrated disease progression or intolerance to imatinib. Patients received sunitinib 15 mg/m per day, 4-weeks-on/2-weeks-off (schedule 4/2), for ≤ 18 cycles over 24 months. Read More

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http://link.springer.com/10.1007/s00280-019-03814-5
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http://dx.doi.org/10.1007/s00280-019-03814-5DOI Listing
April 2019
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NEAT1 regulates neuroglial cell mediating Aβ clearance via the epigenetic regulation of endocytosis-related genes expression.

Cell Mol Life Sci 2019 Apr 20. Epub 2019 Apr 20.

School of Life Sciences, Tsinghua University, Beijing, 100084, China.

The accumulation of intracellular β-amyloid peptide (Aβ) is important pathological characteristic of Alzheimer's disease (AD). However, the exact underlying molecular mechanism remains to be elucidated. Here, we reported that Nuclear Paraspeckle Assembly Transcript 1 (NEAT1), a long n on-coding RNA, exhibits repressed expression in the early stage of AD and its down-regulation declines neuroglial cell mediating Aβ clearance via inhibiting expression of endocytosis-related genes. Read More

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http://dx.doi.org/10.1007/s00018-019-03074-9DOI Listing

Children Born to Mothers with Rash During Zika Virus Epidemic in Brazil: First 18 Months of Life.

J Trop Pediatr 2019 Apr 21. Epub 2019 Apr 21.

Faculdade de Medicina, Universidade Federal Fluminense, RJ 24.033-900, Brazil.

Objective: To better understand the clinical spectrum and course of congenital Zika syndrome (CZS) during the first 18 months of life of children whose mothers had rash during pregnancy.

Methods: This longitudinal observational study evaluated the clinical progress from birth until 18 months of life of children of mothers who developed rash during or up to 3 months before gestation. Maternal rash occurred from November 2015 to May 2017. Read More

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http://dx.doi.org/10.1093/tropej/fmz019DOI Listing

Non-communicable disease prevention in Mexico: policies, programs and regulations.

Health Promot Int 2019 Apr 21. Epub 2019 Apr 21.

University of Arizona, Mel and Enid Zuckerman College of Public Health, 1296 N. Martin, Tucson, AZ 85724, USA.

Non-communicable diseases (NCD), such as diabetes and cardiovascular disease, have become a leading cause of the death in Mexico. The federal government has addressed this issue through developing NCD prevention plans, regulations and policies (PRPs) that seek to address social and environmental factors, which was led by the National Institute of Public Health and Ministry of Health in concert with various non-governmental organizations. This review aims to synthesize and summarize national NCD prevention PRPs addressing social and environmental factors passed from 2010 to 2016, and to assess the extent to which these efforts successfully addressed factors contributing to the epidemic. Read More

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http://dx.doi.org/10.1093/heapro/daz029DOI Listing

Family-centred oral health promotion through Victorian child-health services: a pilot.

Health Promot Int 2019 Apr 21. Epub 2019 Apr 21.

Dental Health Services Victoria, 720 Swanston Street, Carlton, Victoria 3053, Australia.

Maternal and Child Health Services (MCHS) provide ideal settings for oral disease prevention. In Victoria (Australia), child mouth-checks (Lift-the-Lip) and oral health promotion (OHP) occur during MCHS child visits. This study trialled Tooth-Packs (OHP resources, toothbrushes, toothpastes) distribution within MCHS to (i) assess the impacts of Tooth-Packs distribution on child and family oral health (OH) behaviours and knowledge, including Maternal and Child Health Nurses (MCHN) child referral practices to dental services, and (ii) determine the feasibility and acceptability of incorporating Tooth-Packs distribution into MCHN OHP practices. Read More

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http://dx.doi.org/10.1093/heapro/daz025DOI Listing

Does the site of coronary endarterectomy have an impact on the clinical outcomes and graft patency?

Interact Cardiovasc Thorac Surg 2019 Apr 21. Epub 2019 Apr 21.

Department of Cardiac Surgery, Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

Objectives: To explore whether coronary endarterectomy (CE) sites have obvious impacts on the clinical outcomes and graft patency in off-pump coronary artery bypass (OPCAB).

Methods: The patients who underwent OPCAB with CE in our unit between January 2009 and December 2016 were included. The patients and the grafts were grouped according to the CE sites. Read More

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http://dx.doi.org/10.1093/icvts/ivz099DOI Listing

Renal handling of zinc in chronic kidney disease patients and the role of circulating zinc levels in renal function decline.

Nephrol Dial Transplant 2019 Apr 21. Epub 2019 Apr 21.

Department of Medicine, Service of Nephrology and Hypertension, Lausanne University Hospital (CHUV) and University of Lausanne Lausanne, Switzerland.

Background: Zinc deficiency is commonly encountered in chronic kidney disease (CKD). The aims of this study were to assess whether zinc deficiency was related to increased renal excretion of zinc and to the progression of CKD.

Methods: Plasma and 24-h urinary zinc levels, urinary electrolytes and uromodulin were measured in 108 CKD patients and 81 individuals without CKD. Read More

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http://dx.doi.org/10.1093/ndt/gfz065DOI Listing

Low on-treatment diastolic blood pressure is not independently associated with increased cardiovascular risk: an analysis of the SPRINT trial.

Eur Heart J 2019 Apr 21. Epub 2019 Apr 21.

Department of Internal Medicine, Hypertension and Vascular Diseases, Medical University of Warsaw, Banacha 1a, 02-097 Warsaw, Poland.

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http://dx.doi.org/10.1093/eurheartj/ehz225DOI Listing

Association of changes in bone mineral parameters with mortality in haemodialysis patients: insights from the ARO cohort.

Nephrol Dial Transplant 2019 Apr 21. Epub 2019 Apr 21.

Department of Nephrology, Division of Nephrology and Clinical Immunology, RWTH University of Aachen, Aachen, Germany.

Background: There is little information in haemodialysis (HD) patients on whether temporal changes in serum calcium, phosphate or intact parathyroid hormone (iPTH) are associated with mortality.

Methods: We analysed associations of phosphate, total calcium and iPTH with all-cause and cardiovascular mortality in 8817 incident HD patients from the European second Analyzing Data, Recognizing Excellence and Optimizing Outcomes (AROii) cohort enrolled in 2007-09, which were prospectively followed for a median of 3 years, using time-dependent Cox proportional hazards models. We evaluated changes in risk over time depending on changes in phosphate, calcium or iPTH. Read More

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https://academic.oup.com/ndt/advance-article/doi/10.1093/ndt
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http://dx.doi.org/10.1093/ndt/gfz060DOI Listing
April 2019
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Impact of pretransplant mitral annular calcification on the incidence of cardiac events after renal transplantation.

Nephrol Dial Transplant 2019 Apr 21. Epub 2019 Apr 21.

Division of Cardiology, Cook County Health, Chicago, IL, USA.

Background: Mitral annular calcification (MAC) is associated with increased risk of major adverse cardiac events. We hypothesized that MAC, identified on a pretransplant transthoracic echocardiography (TTE), is predictive of cardiac events following renal transplantation (RT).

Methods: In a retrospective cohort of consecutive RT recipients, pretransplant MAC presence and severity were determined on TTE performed within 1 year prior to transplant. Read More

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https://academic.oup.com/ndt/advance-article/doi/10.1093/ndt
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http://dx.doi.org/10.1093/ndt/gfz063DOI Listing
April 2019
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