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https://www.readbyqxmd.com/read/28208686/identification-of-autophagy-related-genes-and-their-regulatory-mirnas-associated-with-celiac-disease-in-children
#1
Sergio Comincini, Federico Manai, Cristina Meazza, Sara Pagani, Carolina Martinelli, Noemi Pasqua, Gloria Pelizzo, Marco Biggiogera, Mauro Bozzola
Celiac disease (CD) is a severe genetic autoimmune disorder, affecting about one in 100 people, where the ingestion of gluten leads to damage in the small intestine. Diagnosing CD is quite complex and requires blood tests and intestinal biopsy examinations. Controversy exists regarding making the diagnosis without biopsy, due to the large spectrum of manifesting symptoms; furthermore, small-intestinal gastroscopy examinations have a relatively complex management in the pediatric population. To identify novel molecular markers useful to increase the sensitivity and specificity in the diagnosis of pediatric CD patients, the expression levels of two key autophagy executor genes (ATG7 and BECN1) and their regulatory validated miRNAs (miR-17 and miR-30a, respectively) were analyzed by relative quantitative real-time-PCR on a cohort of confirmed CD patients compared to age-related controls...
February 12, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28203580/unusual-presentation-of-diffuse-large-b-cell-lymphoma-with-splenic-infarcts
#2
Vivek Kumar, Parita Soni, Vishangi Dave, Jonathan Harris
A 67-year-old man presented with a 3-day history of abdominal pain, fever, and significant weight loss over 2 months. Physical examination revealed left upper quadrant tenderness, hepatomegaly, splenomegaly, and bilateral pitting edema but peripheral lymphadenopathy was absent. Laboratory tests showed anemia, thrombocytopenia, elevated prothrombin time (PT), partial thromboplastin time (PTT), and increased lactate dehydrogenase (LDH). PTT was corrected completely in mixing study. Further workup for the cause of coagulopathy revealed decreased levels of all clotting factors except factor VIII and increase fibrinogen levels, which ruled out disseminated intravascular coagulation (DIC)...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28202821/-pres-posterior-reversible-encephalopathy-syndrome
#3
Kouichirou Okamoto, Kunio Motohashi, Hidemoto Fujiwara, Tomohiko Ishihara, Itaru Ninomiya, Osamu Onodera, Yukihiko Fujii
Posterior reversible encephalopathy syndrome (PRES) is suggested in patients with acute neurological symptoms in the appropriate clinical context, including acute hypertension, blood pressure fluctuations, renal failure, blood transfusion, immunosuppression, autoimmune disorders, and eclampsia. PRES is a clinical syndrome, and refers to a disorder with reversible subcortical vasogenic brain edema caused by endothelial dysfunction, predominantly involving the bilateral parieto-occipital regions. Although the clinical course and prognosis are favorable in most cases, intracranial hemorrhage and/or restricted diffusion similar to acute infarction could be seen in some lesions on brain magnetic resonance imaging (MRI)...
February 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/28199771/thyroid-dysfunction-in-children-with-autism-spectrum-disorder-is-associated-with-folate-receptor-alpha-autoimmune-disorder
#4
Richard E Frye, Rebecca Wynne, Shannon Rose, John Slattery, Leanna Delhey, Marie Tippett, Stephen G Kahler, Sirish C Bennuri, Stepan Melnyk, Jeffrey M Sequeira, Edward Quadros
Folate receptor α (FRα) autoantibodies (FRAAs) are prevalent in Autism Spectrum Disorder (ASD). FRAAs disrupt folate transport across the blood-brain barrier by binding to the FRα. Thyroid dysfunction is frequently found in children with ASD. We measured blocking and binding FRAAs and thyroid stimulating hormone (TSH), free T4 (FT4), total T3 (TT3), reverse T3 (rT3), thyroid releasing hormone (TRH) and other metabolites in 87 children with ASD, 84 of whom also underwent behavior and cognition testing and in 42 of whom FRAAs, TSH and FT4 were measured at two time points...
February 15, 2017: Journal of Neuroendocrinology
https://www.readbyqxmd.com/read/28194493/oral-health-of-adult-patients-undergoing-hematopoietic-cell-transplantation-pre-transplant-assessment-and-care
#5
REVIEW
Agnieszka Bogusławska-Kapała, Kazimierz Hałaburda, Ewa Rusyan, Hubert Gołąbek, Izabela Strużycka
Hematopoietic cell transplantation (HCT) is now one of the frequent procedures used for treatment of malignant and non-malignant blood diseases, autoimmune disorders, and certain solid tumors. Despite improvements of therapeutic protocols, HCT still carries a high risk of non-relapse mortality due to early and late complications. Side effects of the therapy regimen frequently occur in the oral cavity and often significantly decrease the patients' quality of life. The complications may result from or may be exacerbated by improper oral preparation of the patient before transplantation...
February 13, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28190431/posterior-reversible-encephalopathy-in-the-intensive-care-unit
#6
M Toledano, J E Fugate
Posterior reversible encephalopathy syndrome (PRES) is increasingly diagnosed in the emergency department, and medical and surgical intensive care units. PRES is characterized by acute onset of neurologic symptoms in the setting of blood pressure fluctuations, eclampsia, autoimmune disease, transplantation, renal failure, or exposure to immunosuppressive or cytotoxic drugs, triggers known to admit patients to the intensive care unit (ICU). Although the exact pathophysiology remains unknown, there is growing consensus that PRES results from endothelial dysfunction...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28187808/diagnosis-and-management-of-acute-encephalitis
#7
J J Halperin
Encephalitis is typically viral (approximately half of diagnosed cases) or autoimmune (about a quarter) with the remainder remaining undiagnosable at this time. All require general supportive care but only a minority requires intensive care admission - in these intubation, to protect the airway or to treat status epilepticus with anesthetic drugs, may be needed. In some dysautonomia with wide blood pressure fluctuations is the principal concern. Remarkably, in addition to supportive care, specific treatment options are available for the majority - immune-modulating therapy for those with autoimmune disorders, antiviral therapy for herpes simplex 1 and 2, and varicella-zoster encephalitis...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28145671/evaluation-of-jaundice-in-adults
#8
Matthew V Fargo, Scott P Grogan, Aaron Saguil
Jaundice in adults can be an indicator of significant underlying disease. It is caused by elevated serum bilirubin levels in the unconjugated or conjugated form. The evaluation of jaundice relies on the history and physical examination. The initial laboratory evaluation should include fractionated bilirubin, a complete blood count, alanine transaminase, aspartate transaminase, alkaline phosphatase, ?-glutamyltransferase, prothrombin time and/or international normalized ratio, albumin, and protein. Imaging with ultrasonography or computed tomography can differentiate between extrahepatic obstructive and intrahepatic parenchymal disorders...
February 1, 2017: American Family Physician
https://www.readbyqxmd.com/read/28144796/clinical-predictors-and-differential-diagnosis-of-posterior-reversible-encephalopathy-syndrome
#9
Laetitia Della Faille, S Fieuws, W Van Paesschen
The aim of our study is to determine the clinical predictors and the differential diagnosis of posterior reversible encephalopathy syndrome (PRES) in patients presenting with acute neurological symptoms and risk factors for PRES. Using the diagnostic algorithm for PRES from Fugate and Rabinstein (Lancet Neurol 14(9):914-925, 1), we carried out a retrospective study on 220 patients, presenting with acute neurological symptoms such as seizures, encephalopathy, headache, visual disturbances or other focal neurological signs that appear in the clinical setting of risk factors such as hypertension/blood pressure fluctuations, chemotherapy, renal failure, autoimmune disorders, or eclampsia, in whom imaging of the brain was performed to exclude PRES...
January 31, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/28127211/posterior-reversible-encephalopathy-syndrome-in-alcoholic-hepatitis-hepatic-encephalopathy-a-common-theme
#10
Elizabeth S John, Ramy Sedhom, Ishita Dalal, Ranita Sharma
Posterior reversible encephalopathy syndrome (PRES) is a neuro-radiologic diagnosis that has become more widely recognized and reported over the past few decades. As such, there are a number of known risk factors that contribute to the development of this syndrome, including volatile blood pressures, renal failure, cytotoxic drugs, autoimmune disorders, pre-eclampsia, and eclampsia. This report documents the first reported case of PRES in a patient with severe alcoholic hepatitis with hepatic encephalopathy and delves into a molecular pathophysiology of the syndrome...
January 14, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28124217/elemental-analysis-of-whole-and-protein-separated-blood-serum-of-patients-with-systemic-lupus-erythematosus-and-sj%C3%A3-gren-s-syndrome
#11
Csilla Noémi Tóth, Edina Baranyai, István Csípő, Tünde Tarr, Margit Zeher, József Posta, István Fábián
Systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS) are systemic autoimmune diseases with complex symptoms and pathogenesis that are still not completely understood. Several studies showed that the trace element homeostasis and also the levels of antioxidant plasma proteins are changed in autoimmune disorders; however, these results are controversial. In this study, the potassium (K), calcium (Ca), magnesium (Mg), copper (Cu), zinc (Zn), and iron (Fe) concentrations of the serum and proteins-immunoglobulin G (IgG), transferrin (Trf), albumin (Alb), and ceruloplasmin (Cp)-separated from serum samples by affinity chromatography were determined in patients with SLE and SS...
January 26, 2017: Biological Trace Element Research
https://www.readbyqxmd.com/read/28117328/dietary-cholesterol-promotes-repair-of-demyelinated-lesions-in-the-adult-brain
#12
Stefan A Berghoff, Nina Gerndt, Jan Winchenbach, Sina K Stumpf, Leon Hosang, Francesca Odoardi, Torben Ruhwedel, Carolin Böhler, Benoit Barrette, Ruth Stassart, David Liebetanz, Payam Dibaj, Wiebke Möbius, Julia M Edgar, Gesine Saher
Multiple Sclerosis (MS) is an inflammatory demyelinating disorder in which remyelination failure contributes to persistent disability. Cholesterol is rate-limiting for myelin biogenesis in the developing CNS; however, whether cholesterol insufficiency contributes to remyelination failure in MS, is unclear. Here, we show the relationship between cholesterol, myelination and neurological parameters in mouse models of demyelination and remyelination. In the cuprizone model, acute disease reduces serum cholesterol levels that can be restored by dietary cholesterol...
January 24, 2017: Nature Communications
https://www.readbyqxmd.com/read/28114998/th17-skewed-immune-response-and-cluster-of-differentiation-40-ligand-expression-in-canine-steroid-responsive-meningitis-arteritis-a-large-animal-model-for-neutrophilic-meningitis
#13
Jessica Freundt-Revilla, Arianna Maiolini, Regina Carlson, Martin Beyerbach, Kai Rentmeister, Thomas Flegel, Andrea Fischer, Andrea Tipold
BACKGROUND: Steroid-responsive meningitis-arteritis (SRMA) is an immune-mediated disorder characterized by neutrophilic pleocytosis and an arteritis particularly in the cervical leptomeninges. Previous studies of the disease have shown increased levels of IL-6 and TGF-ß1 in cerebrospinal fluid (CSF). In the presence of these cytokines, naive CD4+ cells differentiate into Th17 lymphocytes which synthesize interleukin 17 (IL-17). It has been shown that IL-17 plays an active role in autoimmune diseases, it induces and mediates inflammatory responses and has an important role in recruitment of neutrophils...
January 23, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28107375/narcolepsy-type-1-is-associated-with-a-systemic-increase-and-activation-of-regulatory-t-cells-and-with-a-systemic-activation-of-global-t-cells
#14
Michel Lecendreux, Guillaume Churlaud, Fabien Pitoiset, Armelle Regnault, Tu Anh Tran, Roland Liblau, David Klatzmann, Michelle Rosenzwajg
Narcolepsy is a rare neurologic disorder characterized by excessive daytime sleepiness, cataplexy and disturbed nocturnal sleep patterns. Narcolepsy type 1 (NT1) has been shown to result from a selective loss of hypothalamic hypocretin-secreting neurons with patients typically showing low CSF-hypocretin levels (<110 pg/ml). This specific loss of hypocretin and the strong association with the HLA-DQB1*06:02 allele led to the hypothesis that NT1 could be an immune-mediated pathology. Moreover, susceptibility to NT1 has recently been associated with several pathogens, particularly with influenza A H1N1 virus either through infection or vaccination...
2017: PloS One
https://www.readbyqxmd.com/read/28107165/interleukin-33-plasma-levels-in-patients-with-relapsing-remitting-multiple-sclerosis
#15
Fereshteh Alsahebfosoul, Ilnaz Rahimmanesh, Mansour Shajarian, Masoud Etemadifar, Nahid Sedaghat, Zahra Hejazi, Shamsi Naderi
Cytokines are implicated in the immunopathogenesis of multiple sclerosis (MS). Interleukin (IL)-33, one of the recently discovered members of the IL-1 superfamily, is a dual functional cytokine involved in various autoimmune disorders. In a case-control study, venous blood was collected from healthy subjects categorized as control group (n=44) and MS patients (n=44). All recruited patients were clinically diagnosed with relapsing-remitting MS (RRMS), including patients without treatment (new identified cases, n=16) and those treated with interferon beta (IFN-β) (n=28)...
January 20, 2017: Biomolecular Concepts
https://www.readbyqxmd.com/read/28094319/helminth-induced-ly6c-hi-monocyte-derived-alternatively-activated-macrophages-suppress-experimental-autoimmune-encephalomyelitis
#16
Cesar Terrazas, Juan de Dios Ruiz-Rosado, Stephanie A Amici, Kyle A Jablonski, Diana Martinez-Saucedo, Lindsay M Webb, Hanna Cortado, Frank Robledo-Avila, Steve Oghumu, Abhay R Satoskar, Miriam Rodriguez-Sosa, Luis I Terrazas, Mireia Guerau-de-Arellano, Santiago Partida-Sánchez
Helminths cause chronic infections and affect the immune response to unrelated inflammatory diseases. Although helminths have been used therapeutically to ameliorate inflammatory conditions, their anti-inflammatory properties are poorly understood. Alternatively activated macrophages (AAMϕs) have been suggested as the anti-inflammatory effector cells during helminth infections. Here, we define the origin of AAMϕs during infection with Taenia crassiceps, and their disease-modulating activity on the Experimental Autoimmune Encephalomyelitis (EAE)...
January 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28094061/vitiligo-like-lesions-occurring-in-patients-receiving-anti-programmed-cell-death-1-therapies-are-clinically-and-biologically-distinct-from-vitiligo
#17
Maiana Larsabal, Aurélie Marti, Clément Jacquemin, Jérôme Rambert, Denis Thiolat, Léa Dousset, Alain Taieb, Caroline Dutriaux, Sorilla Prey, Katia Boniface, Julien Seneschal
BACKGROUND: The use of anti-programmed cell death (PD)-1 therapies in metastatic tumors is associated with cutaneous side effects including vitiligo-like lesions. OBJECTIVE: We sought to characterize clinically and biologically vitiligo-like lesions occurring in patients receiving anti-PD-1 therapies by studying a case series of 8 patients with metastatic tumors and 30 control subjects with vitiligo. METHODS: Eight patients receiving anti-PD-1 therapies with features of vitiligo-like lesions seen in our department were recruited...
January 13, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28079913/intravenous-immunoglobulin-in-the-treatment-of-hematologic-disorders-in-pediatrics
#18
Gabriela Villanueva, Jill L O de Jong, Jennifer L McNeer
Intravenous immunoglobulin (IVIG) is pooled immunoglobulin G derived from human blood donors. It was introduced in the early 1980s to treat immunodeficiency disorders. Since then, its use has expanded to other fields such as neurology, rheumatology, and hematology. IVIG has been used to provide passive immunity in qualitative and quantitative immunoglobulin disorders, to neutralize antibodies in immune-mediated diseases, and as an immune modulatory agent. The difficulty of producing IVIG in high quantities, in addition to a growing list of "off-label" indications, has resulted in a worldwide shortage and increase in cost...
January 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28078733/therapeutic-apheresis-in-immunologic-renal-and-neurological-diseases
#19
REVIEW
Rolf Bambauer, Reinhard Latza, Daniel Burgard, Ralf Schiel
Since the mid 1970s, when membrane modules became available, plasma separation techniques have gained in importance especially in the past few years. The advantages of this method are a complete separation of the corpuscular components from the plasma and due to increased blood flow rate and higher efficacy. Systemic autoimmune diseases based on an immune pathogenesis produce autoantibodies and circulating immune complexes, which cause inflammation in the tissues of various organs. In most cases, these diseases have a poor prognosis without treatment...
February 2017: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/28072956/-clinical-and-immunological-analysis-of-the-patient-with-autoimmunity-due-to-germline-stat3-gain-of-function-mutation
#20
Y Ding, Y Zhang, Y P Wang, H Y Zhao, X M Chen, X H Xue, X M Bai, Y F An, Z Y Zhang, X M Tang, X D Zhao
Objective: To investigate the clinical and immunological laboratory features and gene mutation in a female patient who carried a germline gain-of-function mutation in STAT3. Method: A patient with lymphadenopathy and pancytopenia, visited the Department of Rheumatology and Immunology of Children's Hospital of Chongqing Medical University in May 2016. The clinical and laboratory characteristics, results of immunophenotyping and exome sequencing were analyzed retrospectively and related literature was reviewed...
January 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
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