Results 201 to 210 of about 701,871 (361)

Variants in AKR1D1 and Infant Mortality: Should Bile Acid Screening be a Routine Part of Newborn Screening?

open access: yesAmerican Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Biallelic pathogenic variants in AKR1D1 cause Δ4‐3‐oxosteroid 5β‐reductase deficiency, disrupt bile acid synthesis, and result in Congenital Bile Acid Synthesis defect type 2 (CBAS2). CBAS2 presents in infancy with cholestasis, coagulopathy, and failure to thrive.
Jade Hudson   +3 more
wiley   +1 more source

New approaches to treating chronic obstructive pulmonary disease with Colla corii asini

open access: yesAnimal Models and Experimental Medicine, EarlyView.
Colla corii asini improves chronic obstructive pulmonary disease (COPD) treatment through anti‐inflammatory, antioxidant, immune‐modulatory, and lung‐nourishing effects, addressing current therapeutic challenges via multitarget mechanisms. Abstract Chronic obstructive pulmonary disease (COPD) is one of the leading causes of death and disability ...
Wenchao Zhang   +5 more
wiley   +1 more source

Family history of pulmonary fibrosis impacts prognosis in patients with sarcoidosis

open access: yesERJ Open Research
Thomas Planté-Bordeneuve   +6 more
doaj   +1 more source

Engineering Tertiary Lymphoid Structures: Nanomedicine, Bioengineering, and Biomaterials for Precision Immunotherapy

open access: yesAdvanced NanoBiomed Research, EarlyView.
This review explains how biomaterials and nanoparticles can be used to induce or modulate tertiary lymphoid structures (TLSs), which are ectopic immune hubs that form in nonlymphoid tissues during chronic disease and cancer. By comparing different methods, the article highlights design principles for modeling TLSs or recapitulating specific TLS ...
Shaza Karaman, Mei ElGindi, Jeremy Teo
wiley   +1 more source

Mucosal‐Associated Invariant T Cells in Rheumatic Diseases

open access: yesArthritis &Rheumatology, EarlyView.
Mucosal‐associated invariant T (MAIT) cells are innate‐like T cells defined by their semi‐invariant T cell receptor and restriction by the major histocompatibility complex class I–related molecule (MR1). These cells are primarily activated by microbial‐derived metabolites presented by MR1 or by cytokines.
Manon Lesturgie‐Talarek   +7 more
wiley   +1 more source

American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel

open access: yesArthritis &Rheumatology, EarlyView.
Objective Vacuoles E1 enzyme X‐linked autoinflammatory somatic syndrome (VEXAS) is a recently identified rare genetic disorder associated with somatic mutations in the UBA1 gene. VEXAS presents with a combination of inflammatory and hematologic manifestations, leading to increased morbidity and mortality.
Arsene Mekinian   +111 more
wiley   +1 more source

Case‐Based Immunology: B Cells and Systemic Sclerosis Interstitial Lung Disease

open access: yesArthritis &Rheumatology, EarlyView.
Interstitial lung disease (ILD) is an important complication of systemic sclerosis (SSc), with high mortality and morbidity. Recent clinical studies in SSc‐ILD have led to US Food and Drug Administration–approved therapies in SSc‐ILD. Importantly, evidence from these studies has been extrapolated to guide management of ILDs of other systemic autoimmune
Nina Goldman   +2 more
wiley   +1 more source

From Silicosis to Scleroderma: Erasmus Syndrome

open access: yesOpen Respiratory Archives, 2023
María Belén Noboa   +4 more
doaj   +1 more source

Association of Abatacept With Lower Mortality Risk Compared to Rituximab in Rheumatoid Arthritis–Associated Interstitial Lung Disease: An Emulated Target Trial

open access: yesArthritis &Rheumatology, EarlyView.
Objective The optimal treatment strategy for rheumatoid arthritis–associated interstitial lung disease (RA‐ILD) remains uncertain, and direct comparative data between biologics are limited. This study aimed to evaluate the effectiveness and safety of abatacept compared with rituximab in patients with RA‐ILD.
Po‐Cheng Shih   +3 more
wiley   +1 more source

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