Adult onset hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome, which can develop as a complication of many disorders. Early diagnosis is essential in order to avoid a fatal outcome. To confirm the diagnosis of acquired HLH made in a single institution series of adult patients...
The insights gained from this information will significantly advance our comprehension of blood vessel function, enabling the development of improved disease treatments, including those for vascular grafts.Rheumatoid arthritis (RA), an autoimmune disorder, presents with debilitating symptoms that prove ...
HLH is a disease entity characterised by an uncontrolled cytokine storm and expansion of tissue macrophages or histiocytes that exhibit haemophagocytic activity.36 HLH can result from genetic defects in cytolytic pathways (familial or primary HLH) or other diseases such as infection, malignancy, and ...
Adult non-neoplastic hyperinsulinemic hypoglycemia (ANHH), also known as adult-onset nesidioblastosis, is a rare cause of endogenous hyperinsulinemic hypoglycemia in adults. This disease is characterized by diffuse hyperplasia of pancreatic endocrine cel
The cytokine release syndrome (CRS) is closely linked to macrophage activation syndrome (MAS), a life-threatening complication of several autoimmune diseases related to hemophagocytic lymphohistiocytosis (HLH) [27]. MAS is characterized by pancytopenia, liver insufficiency, hyperferritinemia, coagulopathy ...
Renal cell carcinoma as a metabolic disease: an update on main pathways, potential biomarkers, and therapeutic targets. Int J Mol Sci. 2022;23:14360. Article PubMed PubMed Central Google Scholar De Marco S, Torsello B, Minutiello E, Morabito I, Grasselli C, Bombelli S, et al. The ...
The authors conclude that due to the identical genetic pattern in the second twin, a late onset of the disease may still be possible in this child. From our studies, we present evidence that the A91V exchange represents a polymorphism in the perforin gene not causative of the HLH phenotype....
(Fig.4d). Moreover, the mutant GC B cells which remained 14 days after immunization exhibited an equivalent somatic hypermutation load in the JH4 intron to that of control cells (Fig.4e), and the frequency of affinity-increasing mutations in the rearranged VH186.2 sequence was not reduced ...
Using Salmonella enterica serotype Typhimurium by oral gavage to mimic naturally-occurring infection in Sv129S6 mice, we characterized the clinical, hematologic and morphologic host responses to disease thereby describing an animal model with the clinico-pathologic features of secondary HLH as set forth ...
Marked by extensive fibrosis, alterations in blood vessels, and compromised immune regulation, systemic sclerosis (SSc, or scleroderma) is an autoimmune disorder. Scutellaria baicalensis Georgi's baicalein, a phenolic flavonoid, has been used to address the pathological processes of diverse fibrotic and...