Publications by authors named "R K Lindell"
Signal transduction downstream of activating stimuli controls CD8+ T cell biology, however these external inputs can become uncoupled from transcriptional regulation in Primary Immune Regulatory Disorders (PIRDs). Gain-of-function (GOF) variants in STAT3 amplify cytokine signaling and cause a severe PIRD characterized by early onset autoimmunity, lymphoproliferation, recurrent infections, and immune dysregulation. In both primary human and mouse models of STAT3 GOF, CD8+ T cells have been implicated as pathogenic drivers of autoimmunity.
View Article and Find Full Text PDFPulmonary light chain deposition disease is a rare entity characterized by immunoglobulin deposition within the lung parenchyma with pathologic features distinct from pulmonary amyloidosis. Here, the authors present the clinical presentation, associations, and radiologic features of pulmonary light chain deposition disease in a series of 4 patients as well as discuss the distinctions from amyloidosis. The present case series highlights the frequent presence of both cysts and nodules at CT.
View Article and Find Full Text PDFArticle Synopsis
- * A study involving 88 critically ill pediatric patients identified three immune subphenotypes linked to clinical outcomes, indicating meaningful differences in immune dysregulation between patients with and without sepsis.
- * The research highlighted the role of STAT3 hyperactivation in lymphocytes, particularly in the sickest subgroup of patients, suggesting that targeting this dysregulated pathway could improve treatment for severe cases of multiple organ dysfunction syndrome (MODS).
Article Synopsis
- Acute kidney injury (AKI) in critically ill children is linked to high mortality, and the timing of starting continuous kidney replacement therapy (CKRT) during hospitalization is crucial but not well understood.
- In a study of 99 pediatric patients, those who started CKRT more than 2 days after stage 3 AKI onset had a significantly higher mortality rate (65%) compared to those who started within 2 days (5%).
- The findings suggest that initiating CKRT earlier may reduce mortality and enhance kidney recovery, although there is still uncertainty around the best timing for treatment.