Assessing real-world natural history of indolent systemic mastocytosis: A retrospective matched cohort study.
Indolent systemic mastocytosis (ISM) is the most common form of systemic mastocytosis, accounting for more than 80% of cases. Patients with ISM experience severe, unpredictable symptoms, including potentially life-threatening anaphylaxis. As a chronic condition, understanding its longitudinal natura…
Hereditary alpha-tryptasemia demonstrates relative basophil enrichment without signs of cellular hyperreactivity.
Hereditary alpha-tryptasemia (HαT) is an autosomal dominant trait caused by increased tryptase alpha/beta 1 (TPSAB1) copy number, resulting in elevated serum tryptase levels. Although often asymptomatic, HαT is associated with anaphylaxis, flushing, and connective tissue abnormalities. A…
Too high and too loose: dysautonomia and the hypertensive paradox in hypermobility disorders.
Hypertension in hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD) represents an increasingly recognized but poorly understood clinical phenomenon. This review addresses the unique pathophysiology, diagnostic considerations, and management challenges of hypertension…
Tissue-level RBE determination and normal lung responses along the proton SOBP.
To determine proton relative biological effectiveness (RBE) across the spread-out Bragg peak (SOBP) in rat lung using a histological endpoint, with emphasis on changes associated with increasing linear energy transfer (LET). Histological alterations in alveolar epithelial cells (ATI and ATII) and in…
Mast cell specific Cyp11a1 deficiency promotes T cell mediated immunity and suppresses tumour metastasis in a mouse model of melanoma.
Mast cells are emerging players in malignant conditions, but the underlying molecular mechanisms remain poorly defined. Based on previous studies showing that steroids can impact on tumour progression in various settings, we here investigated whether mast cell-derived steroid synthesis can have an i…
Broadening the View: Substance P and Its Metabolism in Pruritus-Related Diseases.
Chronic pruritus is a debilitating symptom accompanying numerous inflammatory skin diseases and remains a major therapeutic challenge. Neurogenic inflammation plays a central role in its pathogenesis, with the tachykinin substance P acting as a key mediator at the interface of the nervous system, im…
Progress in mast cell activation syndrome: the global consensus-2 diagnostic criteria at six years.
Since establishment in 2020 of the global consensus-2 diagnostic criteria for mast cell activation syndrome (MCAS), recognition of this prevalent disease, existing alongside rare cutaneous or systemic mastocytosis, has grown significantly. Despite this progress, some have continued using more restri…