7,313,253 results match your criteria Diseases of Tetrapyrrole Metabolism - Refsum Disease and the Hepatic Porphyrias


Deep transcriptomic profiling of Dahl salt-sensitive rat kidneys with mutant form of Resp18.

Biochem Biophys Res Commun 2021 Jul 29;572:35-40. Epub 2021 Jul 29.

Department of Physiology and Pharmacology, University of Toledo College of Medicine and Life Sciences, Toledo, OH, 43614, USA; Department of Biomedical Sciences, Ohio University, Athens, OH, 45701, USA; Diabetes Institute, Heritage College of Osteopathic Medicine, Ohio University, Athens, OH, 45701, USA. Electronic address:

Expression of Regulated endocrine specific protein 18 (Resp18) is localized in numerous tissues and cell types; however, its exact cellular function is unknown. We previously showed that targeted disruption of the Resp18 locus in the Dahl SS (SS) rat (Resp18) results in higher blood pressure (BP), increased renal fibrosis, increased urinary protein excretion, and decreased mean survival time following a chronic (6 weeks) 2% high salt (HS) diet compared with the SS rat. Based on this prominent renal injury phenotype, we hypothesized that targeted disruption of Resp18 in the SS rat promotes an early onset hypertensive-signaling event through altered signatures of the renal transcriptome in response to HS. Read More

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Proton beam therapy in rectal cancer: A systematic review and meta-analysis.

Surg Oncol 2021 Jul 27;38:101638. Epub 2021 Jul 27.

Department of Colorectal Surgery, Countess of Chester NHS Foundation Trust, UK; Institute of Systems, Molecular and Integrative Biology, University of Liverpool, 200 London Road, Liverpool, L3 9TA, UK. Electronic address:

Introduction: Locally advanced rectal cancer is often treated with neoadjuvant chemoradiotherapy and surgery. Radiotherapy carries significant risk of toxicity to organs at risk (OAR). Proton beam therapy (PBT) has demonstrated to be effective in other cancers, delivering equivalent dosimetric radiation but with the benefit of improved sparing of OAR. Read More

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An adverse outcome pathway based in vitro characterization of novel flame retardants-induced hepatic steatosis.

Environ Pollut 2021 Jul 28;289:117855. Epub 2021 Jul 28.

Faculty of Science, RECETOX, Masaryk University, Kamenice 5, CZ62500, Brno, Czech Republic. Electronic address:

A wide range of novel replacement flame retardants (nFRs) is consistently detected in increasing concentrations in the environment and human matrices. Evidence suggests that nFRs exposure may be associated with disruption of the endocrine system, which has been linked with the etiology of various metabolic disorders, including nonalcoholic fatty liver disease (NAFLD). NAFLD is a multifactorial disease characterized by the uncontrolled accumulation of fats (lipids) in the hepatocytes and involves multiple-hit pathogenesis, including exposure to occupational and environmental chemicals such as organophosphate flame retardants (OPFRs). Read More

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CT-defined emphysema in COPD patients and risk for change in desaturation status in 6-min walk test.

Respir Med 2021 Jul 23;187:106542. Epub 2021 Jul 23.

Centre for International Health, Department of Global Public Health and Primary Care, University of Bergen, Bergen, Norway.

Background: Emphysema and exercise induced desaturation (EID) are both related to poorer COPD prognosis. More knowledge of associations between emphysema and desaturation is needed for more efficient disease management.

Research Question: Is emphysema a risk factor for both new and repeated desaturation, and is emphysema of more or less importance than other known risk factors?

Methods: 283 COPD patients completed a 6-min walk test (6MWT) at baseline and one year later in the Bergen COPD cohort study 2006-2011. Read More

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Evaluation and management of pleural sepsis.

Respir Med 2021 Jul 28;187:106553. Epub 2021 Jul 28.

The Pulmonary Center, Boston University School of Medicine, Boston, MA, USA; South Shore Hospital, South Weymouth, MA, USA.

Pleural sepsis stems from an infection within the pleural space typically from an underlying bacterial pneumonia leading to development of a parapneumonic effusion. This effusion is traditionally divided into uncomplicated, complicated, and empyema. Poor clinical outcomes and increased mortality can be associated with the development of parapneumonic effusions, reinforcing the importance of early recognition and diagnosis. Read More

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Targeting immunosuppressor cells with nanoparticles in autoimmunity: How far have we come to?

Cell Immunol 2021 Jul 27;368:104412. Epub 2021 Jul 27.

Department of Immunology, School of Medical Sciences, Universiti Sains Malaysia, 16150 Kubang Kerian, Kelantan, Malaysia. Electronic address:

Autoimmunity is the assault of immune response towards self-antigens, resulting to inflammation and tissue injury. It is staged into three phases and caused by malfunction of immune tolerance. In our body, immune tolerance is synchronized by several immunosuppressor cells such as regulatory T cells and B cells as well as myeloid-derived suppressor cells, which are prominently dysregulated in autoimmunity. Read More

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Uncommon and peculiar soft tissue sarcomas: Multidisciplinary review and practical recommendations. Spanish Group for Sarcoma research (GEIS -GROUP). Part II.

Cancer Treat Rev 2021 Jul 16;99:102260. Epub 2021 Jul 16.

Hospital Vall D'Hebron, Medical Oncology Department, Barcelona, Spain.

Among all Soft Tissue sarcomas there are some subtypes with low incidence and/or peculiar clinical behaviour, that need to be consider separately. Most of them are orphan diseases, whose biological characteristics imply a clearly different diagnostic and therapeutic approach from other more common sarcoma tumors. We present a brief and updated multidiciplinary review, focused on practical issues, aimed at helping clinicians in decision making. Read More

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The association between simple reaction time variability and gait variability: The Tasmanian Study of Cognition and Gait.

Gait Posture 2021 Jul 27;89:206-210. Epub 2021 Jul 27.

Menzies Institute for Medical Research, University of Tasmania, Hobart, TAS, Australia; Peninsula Clinical School, Central Clinical School, Monash University, Melbourne, VIC, Australia. Electronic address:

Introduction: Greater double support time (DST) variability is associated with falls and memory decline. The underlying neurophysiological mechanisms of DST variability are poorly understood. Simple reaction time (SRT) variability, a measure of attention-processing speed is associated with falls and dementia and, may underlie greater DST variability. Read More

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Effects of three essential oils and their nano-emulsions on Listeria monocytogenes and Shigella flexneri in Egyptian Talaga cheese.

Int J Food Microbiol 2021 Jul 27;355:109334. Epub 2021 Jul 27.

Certified Food Lab, Animal Health Research Institute, Agriculture Research Center, Egypt.

Talaga cheese is a soft Egyptian cheese that has been associated with foodborne pathogens such as Listeria monocytogenes and Shigella flexneri. Essential oils (EOs) play a pivotal role in sustainably controlling foodborne diseases and as a potential preservative in soft cheeses. However, limited data is available comparing the antibacterial activity of EOs and their nano-emulsions (NEs) when inoculated into Talaga cheese. Read More

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Distribution and antimicrobial resistance of Vibrio parahaemolyticus isolated from fish and shrimp aquaculture farms along the Korean coast.

Mar Pollut Bull 2021 Jul 30;171:112785. Epub 2021 Jul 30.

West Sea Fisheries Research Institute, National Institute of Fisheries Science, Incheon 22383, Republic of Korea.

The present study investigated the distribution and antimicrobial susceptibility patterns of Vibrio parahaemolyticus in water samples and aquatic animals (fish and shrimp) from major aquaculture farms along the Korean coast in 2018. V. parahaemolyticus is the most common pathogen causing seafood-borne illness. Read More

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Perception of stress and cognitive efficiency in older adults with mild and moderate dementia during the COVID-19-related lockdown.

J Psychosom Res 2021 Jul 27;149:110584. Epub 2021 Jul 27.

Geriatrics Division, Department of Medicine - DIMED, University of Padua, Italy. Electronic address:

Aim: Many studies have been carried out with the aim of understanding the manifold effects of the novel coronavirus (Covid-19) on individuals' clinical and psychological states. This paper deals with perceived stress (PS) and cognitive efficiency (CE) in older adults with dementia during the first wave of the pandemic. The study also investigated the potential effects of PS and CE on participants' cognitive functioning. Read More

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Intact circadian rhythm despite cortisol hypersecretion in Alzheimer's disease: A meta-analysis.

Psychoneuroendocrinology 2021 Jul 26;132:105367. Epub 2021 Jul 26.

Department of Psychology, Georgia Institute of Technology, 648 Cherry St. NW, Atlanta GA 30313, USA. Electronic address:

Hypersecretion of the glucocorticoid steroid hormone cortisol by individuals with Alzheimer's disease (AD) has been suspected for several decades, during which time dozens of examinations of this phenomenon have been conducted and published. The goals of this investigation were to summarize this sizeable body of literature, test whether participant and methodological characteristics modify the magnitude of the AD-associated basal cortisol hypersecretion, and examine whether cortisol circadian rhythmicity is maintained among individuals with AD. To this end, the present meta-analysis and systematic review examined over 300 comparisons of indices of basal HPA-axis functioning between individuals with AD and cognitively normal older adults. Read More

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Identifying molecular insight of synergistic complexities for SARS-CoV-2 infection with pre-existing type 2 diabetes.

Comput Biol Med 2021 Jul 23;136:104668. Epub 2021 Jul 23.

Healthy Ageing Theme, Garvan Institute of Medical Research, Darlinghurst, NSW, 2010, Australia; WHO Collaborating Centre on eHealth, UNSW Digital Health, School of Public Health and Community Medicine, Faculty of Medicine, UNSW Sydney, NSW, 2052, Australia. Electronic address:

The ongoing COVID-19 outbreak, caused by SARS-CoV-2, has posed a massive threat to global public health, especially to people with underlying health conditions. Type 2 diabetes (T2D) is lethal comorbidity of COVID-19. However, its pathogenetic link remains unclear. Read More

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Studies on the weight of the gastrointestinal tract, digesta composition and occurrence of gastro- and enteroliths in adult domesticated ostriches fed different diets.

Poult Sci 2021 Jun 26;100(9):101359. Epub 2021 Jun 26.

Institute for Animal Nutrition, University of Veterinary Medicine Hannover, Foundation, D-30173 Hannover, Germany.

Comminuting the ingested material in the stomach and fermentation in the large intestine of ostriches, allows an efficient utilization of fiber-rich feedstuffs. The entire gastrointestinal tract (GIT) of 61 adult ostriches (both sexes; av. age of 15 mo), which had previously been fed a ration consisting of either haylage and pelleted compound feed (HP) or haylage, corn silage and pelleted compound feed (HCP), was the subject of the present investigations. Read More

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Data fusion and smoothing for probabilistic tracking of viral structures in fluorescence microscopy images.

Med Image Anal 2021 Jul 16;73:102168. Epub 2021 Jul 16.

Biomedical Computer Vision Group, BioQuant, IPMB, Heidelberg University, Im Neuenheimer Feld 267, Heidelberg, Germany. Electronic address:

Automatic tracking of viral structures displayed as small spots in fluorescence microscopy images is an important task to determine quantitative information about cellular processes. We introduce a novel probabilistic approach for tracking multiple particles based on multi-sensor data fusion and Bayesian smoothing methods. The approach exploits multiple measurements as in a particle filter, both detection-based measurements and prediction-based measurements from a Kalman filter using probabilistic data association with elliptical sampling. Read More

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Quantifying Parkinson's disease motor severity under uncertainty using MDS-UPDRS videos.

Med Image Anal 2021 Jul 21;73:102179. Epub 2021 Jul 21.

Department of Computer Science, Stanford University, Stanford CA 94305, USA; Department of Psychiatry & Behavioral Sciences, Stanford University, Stanford CA 94305, USA. Electronic address:

Parkinson's disease (PD) is a brain disorder that primarily affects motor function, leading to slow movement, tremor, and stiffness, as well as postural instability and difficulty with walking/balance. The severity of PD motor impairments is clinically assessed by part III of the Movement Disorder Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS), a universally-accepted rating scale. However, experts often disagree on the exact scoring of individuals. Read More

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Promotion on labor process and relief of the low back pain by relaxing pelvic muscle with Shangliao (BL 31) point injection in women using epidural analgesia during labor: A randomized, controlled, clinical trial.

Eur J Obstet Gynecol Reprod Biol 2021 Jul 26;264:259-265. Epub 2021 Jul 26.

Department of Anesthesiology, West China Hospital, Sichuan University, China. Electronic address:

Background: The purpose of this study was to explore the effects of combing Shangliao point injection with epidural analgesia on labor pain and birth process in women with low back pain and the possible mechanisms.

Methods: 93 consecutive women were randomized to receive either Shangliao point injection combined with epidural analgesia or epidural analgesia. Another 14 women were recruited to explore the mechanisms and the transperineal ultrasound was performed accordingly. Read More

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Congenital anomalies associated with ambient temperature variability during fetal organogenesis period of pregnancy: Evidence from 4.78 million births.

Sci Total Environ 2021 Jul 28;798:149305. Epub 2021 Jul 28.

School of Health Management, Southern Medical University, Guangzhou 510515, Guangdong Province, China. Electronic address:

Backgrounds: Evidence for the effects of temperature variability on risk of congenital anomalies is lacking. We aimed to examine the association of temperature variability during fetal organogenesis period (weeks 3-8 post-conception) with major congenital anomalies.

Methods: A retrospective cohort study comprising 4,787,356 singleton live-births and stillbirths in China was performed. Read More

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Diabetic foot self-care practice in women with diabetes in Iran.

Diabetes Metab Syndr 2021 Jul 26;15(5):102225. Epub 2021 Jul 26.

Diabetes Research Center, Endocrinology and Metabolism Clinical Sciences Institute, Tehran University of Medical Sciences, Tehran, Iran. Electronic address:

Background And Aims: The present study was conducted to determine the situation of foot self-care practice among Iranian women with diabetes.

Methods: In this cross-sectional study, 475 women completed the Diabetic Foot Self-Care Questionnaire (DFSQ) along with other questions. The overall and three components scores including personal care, podiatric care, and foot wearing, were calculated and their relationship with other factors was analyzed. Read More

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Male subfertility and oxidative stress.

Redox Biol 2021 Jul 18;46:102071. Epub 2021 Jul 18.

Department of Obstetrics and Gynaecology, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands. Electronic address:

To date 15% of couples are suffering from infertility with 45-50% of males being responsible. With an increase in paternal age as well as various environmental and lifestyle factors worsening these figures are expected to increase. As the so-called free radical theory of infertility suggests, free radicals or reactive oxygen species (ROS) play an essential role in this process. Read More

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TSPO deficiency accelerates amyloid pathology and neuroinflammation by impairing microglial phagocytosis.

Neurobiol Aging 2021 Jul 3;106:292-303. Epub 2021 Jul 3.

Department of Immunology, CAMS Key Laboratory for T Cell and Immunotherapy, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences and School of Basic Medicine, Peking Union Medical College, State Key Laboratory of Medical Molecular Biology, Beijing, China. Electronic address:

Increasing evidence has placed inflammation and immune dysfunction at the center of the pathogenesis of Alzheimer's disease (AD). The mitochondrial protein translocator protein (18 kDa) (TSPO) is highly upregulated in microglia and astrocytes in response to inflammatory stimulation. However, the biological action of TSPO in the pathogenesis of AD has not been determined to date. Read More

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Hyper- and hypo-connectivity in sensorimotor network of drug-naïve patients with cervical dystonia.

Parkinsonism Relat Disord 2021 Jul 22;90:15-20. Epub 2021 Jul 22.

Center for Movement Disorders, Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China; China National Clinical Research Center for Neurological Diseases, Beijing, China; Parkinson's Disease Center, Beijing Institute for Brain Disorders, Beijing, China. Electronic address:

Background: Cervical dystonia (CD) is the most common form of focal dystonia with involuntary movements and postures of the head. The pathogenesis and neural mechanisms underlying CD have not been fully elucidated.

Methods: Twenty-seven newly drug-naïve patients with CD and 21 healthy controls (HCs) were recruited with clinical assessment and resting-state functional magnetic resonance imaging (rs-fMRI) scanning. Read More

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Impact of ozone exposure on heart rate variability and stress hormones: A randomized-crossover study.

J Hazard Mater 2021 Jul 26;421:126750. Epub 2021 Jul 26.

Department of Environmental Health, School of Public Health, Key Lab of Public Health Safety of the Ministry of Education and NHC Key Lab of Health Technology Assessment, Fudan University, P.O. Box 249,130 Dong-An Road, Shanghai 200032, China; Children's Hospital of Fudan University, National Center for Children's Health, Shanghai, China. Electronic address:

The biological mechanisms underlying the associations between atmospheric ozone exposure and adverse cardiometabolic outcomes are yet to be identified. Imbalanced autonomic nervous system (ANS) as well as activations of the sympatho-adrenomedullary (SAM) and hypothalamic-pituitary-adrenal (HPA) axes are among possible early biological responses triggered by ozone, and may eventually lead to cardiometabolic abnormalities. To determine whether acute ozone exposure causes ANS imbalance and increases the secretion of neuroendocrine stress hormones, we conducted a randomized, double-blind, crossover trial, under controlled 2-hour exposure to either ozone (200 ppb) or clean air with intermittent exercise among 22 healthy young adults. Read More

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Design, synthesis and evaluation of side-chain hydroxylated derivatives of lithocholic acid as potent agonists of the vitamin D receptor (VDR).

Bioorg Chem 2021 Jul 22;115:105202. Epub 2021 Jul 22.

Departamento de Química Orgánica, Laboratorio de Investigación Ignacio Ribas, Universidad de Santiago de Compostela, Avda das Ciencias s/n, 15782 Santiago de Compostela, Spain. Electronic address:

A high number of biologically active and low-calcemic secosteroidal ligands of the vitamin D receptor (VDR) have been developed, some of which are already used clinically although with limited success in the treatment of hyperproliferative diseases because the required pharmaceutical dosages induce toxicity. We describe here the in silico design, synthesis, structural analysis and biological evaluation of two novel active lithocholic acid derivatives hydroxylated at the side chain as highly potent inhibitors of atopic dermatitis-relevant keratinocyte inflammation of potential therapeutic interest. Read More

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Evaluation of S-RBD and high specificity ACE-2-binding antibodies on SARS-CoV-2 patients after six months from infection.

Int Immunopharmacol 2021 Jul 27;99:108013. Epub 2021 Jul 27.

Department of Laboratory Medicine, Tor Vergata University Hospital, Rome, Italy; Department of Experimental Medicine, University of Tor Vergata, Rome, Italy.

The antibody response to SARS-CoV-2 has not yet fully defined, but the availability of sensitive and specific serological assays is crucial to observe the presence of specific antibodies against the human receptor binding domain (S-RBD) and high specificity ACE-2-binding antibodies or neutralizing antibodies (NT) in response to vaccines. Indeed, these peculiar antibodies should prevent viral interaction between RBD and Angiotensin-Converting Enzyme 2 (ACE2) receptor, located on surface of host cells. In this study, 72 samples from 37 hospitalized COVID-19 patients and 35 not-hospitalized patients were analyzed longitudinally. Read More

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Dioscin prevents DSS-induced colitis in mice with enhancing intestinal barrier function and reducing colon inflammation.

Int Immunopharmacol 2021 Jul 31;99:108015. Epub 2021 Jul 31.

Department of Clinical Veterinary Medicine, College of Veterinary Medicine, Jilin University, Changchun, Jilin Province, China. Electronic address:

Dioscin is a natural steroid saponin derived from plants of the genus Dioscoreaceae. Previous studies have proved its effects of antibacterial, anti-inflammatory and hypolipidemic. In this study, our aim was to explore the protective effect and preliminary mechanism of Dioscin on dextran sulfate sodium (DSS)-induced colitis in mice. Read More

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miR-132-3p promotes the cisplatin-induced apoptosis and inflammatory response of renal tubular epithelial cells by targeting SIRT1 via the NF-κB pathway.

Int Immunopharmacol 2021 Jul 30;99:108022. Epub 2021 Jul 30.

Department of Urology, Renmin Hospital of Wuhan University, Wuhan 430060, China. Electronic address:

Cisplatin is a highly effective and broad-spectrum anticancer drug for the clinical treatment of solid tumors. However, it causes acute kidney injury (AKI) in patients with cancer. Consequently, its clinical application is limited. Read More

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The potential impact of discrepancies between automated susceptibility platforms and other testing metho`dologies for cefazolin in the treatment of Enterobacterales bloodstream infections.

Diagn Microbiol Infect Dis 2021 Jul 9;101(2):115483. Epub 2021 Jul 9.

Department of Pharmacy, University of Maryland School of Pharmacy, Baltimore, MD, USA.

Revised breakpoints for cefazolin (CFZ) against Enterobacterales may be difficult to implement with current automated susceptibility testing platforms and could falsely report organisms as susceptible, leading to inappropriate treatment for bloodstream infections (BSI). This was a retrospective cohort of adult patients with Enterobacterales BSI reported CFZ susceptible per Vitek®2. The primary outcome was the percentage susceptible by minimum inhibitory concentration (MIC) Gradient Test Strips and disk diffusion. Read More

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Impact of Chronic Hepatitis C virus co-infection on outcomes of infective endocarditis in people who inject drugs.

Diagn Microbiol Infect Dis 2021 Jun 12;101(2):115453. Epub 2021 Jun 12.

Section on Infectious Diseases, Department of Internal Medicine, Wake Forest Baptist Health, Winston-Salem, NC, USA.

Injection drug use (IDU) is a risk factor for infective endocarditis (IE) and hepatitis C virus (HCV) infection. This retrospective cohort study assessed HCV's impact on outcomes of adult people who inject drugs (PWID). Those admitted due to IE using modified Duke criteria from January 2012 through May 2018 were identified. Read More

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Artificial intelligence for classification of temporal lobe epilepsy with ROI-level MRI data: A worldwide ENIGMA-Epilepsy study.

Neuroimage Clin 2021 Jul 24;31:102765. Epub 2021 Jul 24.

Institute for Diagnostic Radiology and Neuroradiology, University Medicine Greifswald, Greifswald, Germany; Institute for Diagnostic and Interventional Radiology, Pediatric and Neuroradiology, University Medical Centre Rostock, Rostock, Germany.

Artificial intelligence has recently gained popularity across different medical fields to aid in the detection of diseases based on pathology samples or medical imaging findings. Brain magnetic resonance imaging (MRI) is a key assessment tool for patients with temporal lobe epilepsy (TLE). The role of machine learning and artificial intelligence to increase detection of brain abnormalities in TLE remains inconclusive. Read More

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