The American journal of the medical sciences
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IgLON5 autoimmunity is a novel antibody-mediated disorder characterized by serum and/or cerebrospinal fluid (CSF) positivity for IgLON5 antibody. Anti-IgLON5 disease mainly manifests as sleep disturbances, movement disorders and brainstem syndromes. In this study, we report the case of a patient with anti-IgLON5 disease who presented with abdominal distension, abdominal pain, intermittent dysuria and constipation, and intermittent lightning pain in the extremities, which are atypical of anti-IgLON5 disease and could easily lead to misdiagnosis. ⋯ Following treatment, the manifestations nearly resolved. The clinical manifestations of anti-IgLON5 disease are diverse and may present in different combinations, which can easily lead to misdiagnosis. Early recognition and treatment of this autoimmune disease with immunosuppressive agents may lead to better outcomes.
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Inflammatory responses play a central role in myocardial ischemia/reperfusion (I/R) injury. Previous studies have demonstrated that the receptor for advanced glycation end-products (RAGE) is involved in the pro-inflammatory process of myocardial I/R injury by binding to diverse ligands. Thus, the inhibitory effects of soluble receptor for advanced glycation end-products (sRAGE), a decoy receptor for RAGE, on myocardial I/R injury may be associated with a reduced inflammatory state. ⋯ Our findings suggested that sRAGE protects the heart from myocardial I/R injury by inhibiting the infiltration of pro-inflammatory M1-macrophages, and subsequently decreasing IL-6 secretion.
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Metabolic dysfunction-associated steatotic liver disease (MASLD) and risk of malnutrition can coexist in patients with inflammatory bowel disease (IBD). We performed a malnutrition risk assessment as part of the standard follow-up of IBD patients and studied the potential risk factors for being at risk of malnutrition based on the presence or absence of MASLD. ⋯ Malnutrition risk and quality of life are modified by the presence of MASLD in IBD patients.
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Review Case Reports
Autoimmune polyglandular syndrome with shock and high anion gap metabolic acidosis.
Autoimmune polyglandular syndrome (APS) is a rare group of immune-mediated disorders, which are typically, but not exclusively, related to the presence of endocrine abnormalities. APS type 2 is the most common subtype of the syndrome, more often observed in adulthood, with a characteristic clinical triad, which includes adrenal insufficiency, autoimmune thyroiditis and diabetes mellitus type 1. ⋯ Herein, we present a 23 years-old patient with adrenal insufficiency in the context of autoimmune polyglandular syndrome type 2 with coexisting autoimmune thyroiditis and metabolic acidosis with an increased anion gap attributed to prolonged malnutrition. Additionally, we analyze the main clinical features of adrenal insufficiency, which is a central component of autoimmune polyglandular syndrome; highlight characteristics that differentiate the major APS subtypes.
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Review
Recognizing the roles of fever in host survival and in medical intervention in infectious diseases.
Fever has roles both in host defense against infectious challenges and in guidance of medical intervention. These roles remain insufficiently acknowledged and considered by both health care providers and patients and their families. This review cites reports in support of both roles and provides recommendations regarding the clinician's approach to fever, as well as points relevant for education of patients and their families.