Publications by authors named "L Roland"

Background: WHIM (Warts, Hypogammaglobulinemia, Infections, and Myelokathexis) syndrome is an ultra-rare, combined primary immunodeficiency and chronic neutropenic disorder characterized by a range of clinical presentations, including peripheral neutropenia, lymphopenia, and recurrent infections. WHIM syndrome is most often caused by gain-of-function mutations in the gene encoding C-X-C chemokine receptor 4 (CXCR4). As such, inhibition of CXCR4 with XOLREMDI (mavorixafor), an orally bioavailable CXCR4 antagonist, demonstrated clinically meaningful increases in absolute neutrophil and lymphocyte counts and concomitant reduction in infections in patients with WHIM syndrome, resulting in its recent U.

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Article Synopsis
  • Hematopoietic multipotent progenitors (MPPs) in the bone marrow can differentiate into various cell types, influenced by both intrinsic and extrinsic signals, with WHIM syndrome patients exhibiting an excess of myeloid cells due to CXCR4 signaling mutations.
  • Research using knock-in mice with WHIM-associated mutations showed that MPP4 cells, which usually develop into lymphoid cells, instead skewed towards myeloid differentiation due to increased mTOR signaling and altered oxidative phosphorylation.
  • Treatment with CXCR4 antagonist AMD3100 or mTOR inhibitor rapamycin reversed this myeloid bias, indicating that normal CXCR4 function is crucial for maintaining the lymphoid potential of MPP4 cells by regulating
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In patients with chronic rhinosinusitis and comorbid asthma, patients with surgical intervention required less asthma rescue medication, as compared to those who did not undergo surgery. Following sinus surgery, patients with chronic rhinosinusitis and asthma required more asthma medication, as compared to the time period prior to surgery.

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Purpose Of Review: To summarize the evidence surrounding diagnosis, treatment, prognosis, and surveillance of patients with acute invasive fungal sinusitis (AIFS) and discuss future research needs.

Recent Findings: New risk factors for AIFS such as COVID have been identified, and a new prognostic staging system has been developed.

Summary: Most patients who develop AIFS are immunocompromised, with the majority having a history of diabetes or a hematologic malignancy.

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Nasal obstruction is the subjective perception and objective state of insufficient airflow through the nose. Nasal congestion, conversely, describes a state of not just inadequate airflow or obstructive phenomena but also pressure- and mucus-related states to the patient. Nasal receptors belonging to the transient receptor potential (TRP) protein family mediate the sense of nasal patency via the trigeminal nerve.

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