keyword
https://read.qxmd.com/read/38469429/hlh-syndrome-in-a-community-hospital-the-challenge-of-an-early-diagnosis
#21
JOURNAL ARTICLE
Yuval Wagner, Dganit Adam, Galit Pomeranz Engelberg, Avishalom Pomeranz, Yoav H Messinger
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyperinflammatory cytokine storm. It can be secondary to infections, malignancies, autoimmune diseases, or the manifestation of genetic disorders, including primary immune deficiency. HLH requires a high index of suspicion and is challenging for community hospitals. METHODS: Medical records of children with HLH admitted to the Meir Medical Center in Israel between 2014 and 2017 were reviewed...
2024: Pediatric Health, Medicine and Therapeutics
https://read.qxmd.com/read/38465976/pre-existing-immunocompromising-conditions-and-outcomes-of-acute-covid-19-patients-admitted-for-pediatric-intensive-care
#22
JOURNAL ARTICLE
Courtney M Rowan, Brenna LaBere, Cameron C Young, Laura D Zambrano, Margaret M Newhams, Suden Kucukak, Elizabeth R McNamara, Elizabeth H Mack, Julie C Fitzgerald, Katherine Irby, Aline B Maddux, Jennifer E Schuster, Michele Kong, Heda Dapul, Stephanie P Schwartz, Melania M Bembea, Laura L Loftis, Amanda R Kolmar, Christopher J Babbitt, Ryan A Nofziger, Mark W Hall, Shira J Gertz, Natalie Z Cvijanovich, Matt S Zinter, Natasha B Halasa, Tamara T Bradford, Gwenn E McLaughlin, Aalok R Singh, Charlotte V Hobbs, Kari Wellnitz, Mary A Staat, Bria M Coates, Hillary R Crandall, Mia Maamari, Kevin M Havlin, Adam J Schwarz, Christopher L Carroll, Emily R Levy, Kristin L Moffitt, Angela P Campbell, Adrienne G Randolph, Janet Chou
BACKGROUND: We aimed to determine if pre-existing immunocompromising conditions (ICCs) were associated with the presentation or outcome of patients with acute coronavirus disease 2019 (COVID-19) admitted for pediatric intensive care. METHODS: 55 hospitals in 30 U.S. states reported cases through the Overcoming COVID-19 public health surveillance registry. Patients <21 years admitted March 12, 2020-December 30, 2021 to the pediatric intensive care unit (PICU) or high acuity unit for acute COVID-19 were included...
March 11, 2024: Clinical Infectious Diseases
https://read.qxmd.com/read/38465761/pediatric-psoriasis-associated-with-van-wyk-grumbach-syndrome-a-case-report
#23
JOURNAL ARTICLE
Asma Marzouk, Rania Mezzi, Saida Zelfani, Nour Jelaila, Saad Ayeb, Asma Bouaziz
INTRODUCTION: Psoriasis is a common chronic inflammatory condition, often beginning in childhood in approximately one-third of cases. It can be associated with various other autoimmune diseases such as rheumatoid arthritis, celiac disease, and thyroid disorders. However, its co-occurrence with Van Wyk Grumbach syndrome has not been described in the pediatric population. This syndrome, resulting from untreated hypothyroidism, is characterized by early puberty and ovarian cysts. OBSERVATION: A 15-year-old adolescent with a diagnosis of psoriasis since the age of 9 presented with chronic constipation and headaches...
October 5, 2023: La Tunisie Médicale
https://read.qxmd.com/read/38465049/a-case-of-klippel-trenaunay-syndrome-complicated-by-group-a-streptococcemia-and-multiple-organ-failure
#24
Ramaditya Srinivasmurthy, George Gilles, Tha Sok, Brian Chang
Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder defined as a triad of capillary malformation, venous malformation, and hypertrophy of soft tissue and bones, with or without lymphatic malformation. We report a case of a KTS patient with a hospital course complicated by Group A Streptococcus bacteremia and multiple organ failure. The 39-year-old female with KTS presented to the emergency department with a fever, tachycardia, hypotension, and profuse diarrhea for one week. Blood cultures grew Group A Streptococcus necessitating a multi-antibiotic regimen and intravenous immunoglobulins (IVIG)...
February 2024: Curēus
https://read.qxmd.com/read/38463908/correlation-between-neurofilament-hmgb1-mmp9-ds-dna-blood-levels-and-cognitive-impairment-in-patients-with-neuropsychiatric-systemic-lupus-erythematosus
#25
JOURNAL ARTICLE
Arman Ahmadzade, Leila Simani, Mehrdad Roozbeh, Farane Farsad, Mehdi Sheibani, Omid Negaresh, Mohammad Mehdi Emam, Alireza Rajaei, Muhanna Kazempour, Mahtab Ramezani, Samad Nazarpoor
BACKGROUND: Diagnosis of neuropsychiatric systemic lupus erythematosus (NPSLE) is challenging due to nonspecific biomarkers. High serum levels of neurofilament protein light subunit (NFL), high mobility group box 1 (HMGB1), Matrix metalloproteinase-9 (MMP-9) and have been reported in several autoimmune diseases. The aim of this study was to examine whether their plasma levels could serve as a diagnostic or prognostic biomarker for NPSLE. METHODS: There were 90 SLE patients enrolled in this cross-sectional study (87...
2024: Caspian Journal of Internal Medicine
https://read.qxmd.com/read/38463081/knee-replacement-surgery-in-a-patient-with-acquired-von-willebrand-disease-a-case-study-with-recommendations-for-patient-management
#26
María Teresa Álvarez Román, María Isabel Rivas Pollmar, Hortensia De la Corte-Rodríguez, Primitivo Gómez-Cardero, E Carlos Rodríguez-Merchán, Mar Gutiérrez-Alvariño, Eduardo García-Pérez, Mónica Martín-Salces, Damaris Zagrean, Nora V Butta-Coll, Víctor Jiménez-Yuste
INTRODUCTION AND IMPORTANCE: Acquired von Willebrand disease (AvWD) is a rare underdiagnosed bleeding disorder caused by alterations in the levels of the major blood-clotting protein von Willebrand factor (vWF). The clinical and laboratory parameters of AvWD are similar to congenital vWD, but it is found in individuals with no positive family history with no underlying genetic basis. The disease remains multifactorial and incompletely understood. Proposed mechanisms include the development of autoantibodies to vWF, absorption of high molecular weight vWF multimers that impair normal function, shear stress induced vWF cleavage and increased proteolysis...
March 2024: Annals of Medicine and Surgery
https://read.qxmd.com/read/38462403/exploration-of-potential-immune-mechanisms-in-cervical-dystonia
#27
JOURNAL ARTICLE
Laura M Scorr, Gamze Kilic-Berkmen, Diane J Sutcliffe, Ashok R Dinasarapu, J Lucas McKay, Pritha Bagchi, Michael D Powell, Jeremy M Boss, Nezih Cereb, Marian Little, Loren Gragert, John Hanfelt, Andrew McKeon, William Tyor, H A Jinnah
BACKGROUND: Although there are many possible causes for cervical dystonia (CD), a specific etiology cannot be identified in most cases. Prior studies have suggested a relationship between autoimmune disease and some cases of CD, pointing to possible immunological mechanisms. OBJECTIVE: The goal was to explore the potential role of multiple different immunological mechanisms in CD. METHODS: First, a broad screening test compared neuronal antibodies in controls and CD...
February 17, 2024: Parkinsonism & related Disorders
https://read.qxmd.com/read/38454506/b-cell-receptor-repertoire-analysis-in-primary-sjogren-s-syndrome-salivary-glands-identifies-repertoire-features-associated-with-clinical-activity
#28
JOURNAL ARTICLE
Ling Chang, Zihan Zheng, Yiwen Zhou, Kun Liu, Yinong Li, Bing Zhong, Zihua Zhao, Chengshun Chen, Can Qian, Qingshan Ni, Qinghua Zou, Yuzhang Wu, Jingyi Li, Liyun Zou
BACKGROUND: Primary Sjogren's syndrome (pSS) is a complex autoimmune disease featuring damage to salivary and lacrimal glands, with the possibility of manifestations across multiple organs. Antibody-producing B cells have long been appreciated to play a significant role in pSS pathogenesis, with a number of autoreactive antibody species having been identified to be elevated in pSS patients. While several studies have attempted to characterize the BCR repertoires of peripheral blood B cells in pSS patients, much remains unknown about the repertoire characteristics of gland-infiltrating B cells...
March 7, 2024: Arthritis Research & Therapy
https://read.qxmd.com/read/38453214/hyperactivation-and-altered-selection-of-b-cells-in-patients-with-paediatric-sjogren-s-syndrome
#29
JOURNAL ARTICLE
Alessandra Boni, Rebecca Nicolai, Ivan Caiello, Francesca Marinaro, Luciapia Farina, Denise Pires Marafon, Rita Carsetti, Fabrizio De Benedetti, Claudia Bracaglia, Emiliano Marasco
OBJECTIVES: Paediatric Sjögren's syndrome (pSS) is a rare chronic autoimmune disorder, characterised by inflammation of exocrine glands. B cell hyperactivation plays a central role in adult-onset Sjogren. This study was designed to analyse B cell and T cell phenotype, levels of BAFF, and selection of autoreactive B cells in patients with pSS. METHODS: A total of 17 patients diagnosed with pSS and 13 healthy donors (controls) comparable for age were enrolled in the study...
March 6, 2024: RMD Open
https://read.qxmd.com/read/38451436/interferon-regulatory-factor-5-gene-polymorphisms-and-mrna-expression-levels-are-associated-with-neuromyelitis-optica-spectrum-disorder
#30
JOURNAL ARTICLE
Gaoning Wang, Liu Jing, Ying Wang, Arshad Mehmood, Huining Zhang, Ruoyi Guo, Lu Zhang, Bin Li
Interferon regulatory factor 5 (IRF5) is a critical transcription factor in the toll-like receptor signaling pathway. It is associated with autoimmune disorders, such as rheumatoid arthritis, systemic lupus erythematosus, and inflammatory bowel disease. However, the relationship between the functional single nucleotide polymorphisms (SNPs) of IRF5 and its mRNA expression level in patients with neuromyelitis optica spectrum disorder remains unclear. The present study aimed to investigate the relationship between polymorphisms and mRNA expression levels of the IRF5 gene with the incidence of neuromyelitis optica spectrum disorder (NMOSD) in northern Chinese Han people...
March 7, 2024: Molecular Neurobiology
https://read.qxmd.com/read/38450850/chronic-myelomonocytic-leukemia-2024-update-on-diagnosis-risk-stratification-and-management
#31
JOURNAL ARTICLE
Mrinal M Patnaik, Ayalew Tefferi
DISEASE OVERVIEW: Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder with overlapping features of myelodysplastic syndromes and myeloproliferative neoplasms, characterized by prominent monocytosis and an inherent risk for leukemic transformation (~15%-20% over 3-5 years). DIAGNOSIS: Newly revised diagnostic criteria include sustained (>3 months) peripheral blood (PB) monocytosis (≥0.5 × 109 /L; monocytes ≥10% of leukocyte count), consistent bone marrow (BM) morphology, <20% BM or PB blasts (including promonocytes), and cytogenetic or molecular evidence of clonality...
March 7, 2024: American Journal of Hematology
https://read.qxmd.com/read/38449060/the-gateway-reflex-regulates-tissue-specific-autoimmune-diseases
#32
REVIEW
Yuki Tanaka, Izuru Ohki, Kaoru Murakami, Satoshi Ozawa, Yaze Wang, Masaaki Murakami
The dynamic interaction and movement of substances and cells between the central nervous system (CNS) and peripheral organs are meticulously controlled by a specialized vascular structure, the blood-brain barrier (BBB). Experimental and clinical research has shown that disruptions in the BBB are characteristic of various neuroinflammatory disorders, including multiple sclerosis. We have been elucidating a mechanism termed the "gateway reflex" that details the entry of immune cells, notably autoreactive T cells, into the CNS at the onset of such diseases...
March 7, 2024: Inflammation and Regeneration
https://read.qxmd.com/read/38448070/universal-screening-for-early-detection-of-chronic-autoimmune-metabolic-and-cardiovascular-diseases-in-the-general-population-using-capillary-blood-uniscreen-low-risk-interventional-single-centre-pilot-study-protocol
#33
JOURNAL ARTICLE
Aurora Merolla, Rebecca De Lorenzo, Giulia Ferrannini, Cristina Renzi, Francesca Ulivi, Elena Bazzigaluppi, Vito Lampasona, Emanuele Bosi
INTRODUCTION: Chronic autoimmune (type 1 diabetes and coeliac disease) and metabolic/cardiovascular (type 2 diabetes, dyslipidaemia, hypertension) diseases are highly prevalent across all age ranges representing a major public health burden. Universal screening for prediction/early identification of these conditions is a potential tool for reducing their impact on the general population. The aim of this study is to assess whether universal screening using capillary blood sampling is feasible at a population-based level...
March 5, 2024: BMJ Open
https://read.qxmd.com/read/38444587/animal-models-for-type-1-and-type-2-diabetes-advantages-and-limitations
#34
REVIEW
Raj Singh, Mazaher Gholipourmalekabadi, Sasha H Shafikhani
Diabetes mellitus, commonly referred to as diabetes, is a group of metabolic disorders characterized by chronic elevation in blood glucose levels, resulting from inadequate insulin production, defective cellular response to extracellular insulin, and/or impaired glucose metabolism. The two main types that account for most diabetics are type 1 diabetes mellitus (T1DM) and type 2 diabetes mellitus (T2DM), each with their own pathophysiological features. T1D is an autoimmune condition where the body's immune system attacks and destroys the insulin-producing beta cells in the pancreas...
2024: Frontiers in Endocrinology
https://read.qxmd.com/read/38441379/sex-dependent-expression-levels-of-vav1-and-p2x7-in-pbmc-of-multiple-sclerosis-patients
#35
JOURNAL ARTICLE
Airi Rump, Kristel Ratas, Tuuli Katarina Lepasepp, Jaanus Suurväli, Olli-Pekka Smolander, Katrin Gross-Paju, Toomas Toomsoo, Jean Kanellopoulos, Sirje Rüütel Boudinot
Multiple sclerosis (MS) is an inflammatory autoimmune disorder of the central nervous system and the leading cause of progressive neurological disability in young adults. It decreases the patient's lifespan by about 10 years and affects women more than men. No medication entirely restricts or reverses neurological degradation. However, early diagnosis and treatment increase the possibility of a better outcome. To identify new MS biomarkers, we tested the expression of six potential markers (P2X4, P2X7, CXCR4, RGS1, RGS16 and VAV1) using qPCR in peripheral blood mononuclear cells (PBMC) of MS patients treated with interferon β (IFNβ), with glatiramer acetate (GA) or untreated...
August 2023: Scandinavian Journal of Immunology
https://read.qxmd.com/read/38440735/single-cell-rna-sequencing-reveals-cell-type-specific-immune-regulation-associated-with-human-neuromyelitis-optica-spectrum-disorder
#36
JOURNAL ARTICLE
Yushu Jiang, Shuhua Dai, Rui Pang, Lingzhi Qin, Milan Zhang, Huiqin Liu, Xiaojuan Wang, Jiewen Zhang, Gongxin Peng, Yongchao Wang, Wei Li
INTRODUCTION: One rare type of autoimmune disease is called neuromyelitis optica spectrum disorder (NMOSD) and the peripheral immune characteristics of NMOSD remain unclear. METHODS: Here, single-cell RNA sequencing (scRNA-seq) is used to characterize peripheral blood mononuclear cells from individuals with NMOSD. RESULTS: The differentiation and activation of lymphocytes, expansion of myeloid cells, and an excessive inflammatory response in innate immunity are observed...
2024: Frontiers in Immunology
https://read.qxmd.com/read/38435176/t-large-granular-lymphocytic-leukemia-a-rare-diagnosis-in-a-young-woman-with-fever-necrotic-skin-lesions-and-cytopenias
#37
Mário Ferreira, Joana Paulo, Paulo Ramos, Carolina Padrão, Zélia Neves
T-large granular lymphocytic leukemia (T-LGLL) is a rare lymphoproliferative disorder. The diagnosis is established by identifying an abnormally high number of clonal granular T lymphocytes in the peripheral blood and eventually in the bone marrow, in cases with medullary infiltration. The majority of patients present with symptoms related to neutropenia and this condition may be associated with autoimmune diseases in up to a third of cases. The authors describe the case of a 26-year-old patient admitted with subacute high fever and bullous dermatitis with necrotic lesions with central bullae...
February 2024: Curēus
https://read.qxmd.com/read/38434407/glial-fibrillary-acidic-protein-astrocytopathy-presented-as-meningitis-a-case-report
#38
Ya Guo, Jiamin Guo, Xueyu Wang, Aihua Ma, Yuxing Gao, Jiacheng Chen, Cuili Nie, Na Chen
INTRODUCTION: Glial fibrillary acidic protein (GFAP) astrocytopathy is a novel autoimmune neurological disorder and is diagnosed by GFAP-IgG in cerebrospinal fluid (CSF) measurement. CASE REPORT: Herein, we described a 10-year-old boy with abnormal neurological symptoms and signs. GFAP-IgG was detected in CSF using cell-based assay (CBA), and his CSF showed an increase in lymphocytes, a slight decrease in glucose and an increase in protein level in the early stage...
March 15, 2024: Heliyon
https://read.qxmd.com/read/38433036/investigating-viral-and-autoimmune-t-cell-responses-associated-with-post-acute-sequelae-of-covid-19
#39
JOURNAL ARTICLE
Gregory P Williams, Esther Dawen Yu, Kendra Shapiro, Eric Wang, Antoine Freuchet, April Frazier, Cecilia S Lindestam Arlehamn, Alessandro Sette, Ricardo da Silva Antunes
Post-acute sequelae of COVID-19 (PASC), or Long COVID, is a chronic condition following acute SARS-CoV-2 infection. Symptoms include exertion fatigue, respiratory issues, myalgia, and neurological manifestations such as 'brain fog,' posing concern for their debilitating nature and potential role in other neurological disorders. However, the underlying potential pathogenic mechanisms of the neurological complications of PASC is largely unknown. Herein, we investigated differences in antigen-specific T cell responses from the peripheral blood towards SARS-CoV-2, latent viruses, or neuronal antigens in 14 PASC individuals with neurological manifestations (PASC-N) versus 22 individuals fully recovered from COVID-19...
March 2, 2024: Human Immunology
https://read.qxmd.com/read/38427255/promises-of-lipid-based-nanocarriers-for-delivery-of-dimethyl-fumarate-to-multiple-sclerosis-brain
#40
JOURNAL ARTICLE
Sreya Subhash, Nishtha Chaurawal, Kaisar Raza
Multiple sclerosis (MS) is a neurodegenerative autoimmune disorder of the central nervous system (CNS) infecting 2.5 million people worldwide. It is the most common nontraumatic neurological impairment in young adults. The blood-brain barrier rupture for multiple sclerosis pathogenesis has two effects: first, during the onset of the immunological attack, and second, for the CNS self-sustained "inside-out" demyelination and neurodegeneration processes. In addition to genetic variations, environmental and lifestyle variables can also significantly increase the risk of developing MS...
2024: Methods in Molecular Biology
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