Selected Abstracts from the 13th Annual Meeting of the Clinical Immunology Society: 2022 Annual Meeting: Immune Deficiency and Dysregulation North American Conference

Journal of Clinical Immunology - Coronavirus disease 2019 (COVID-19) exhibits a wide spectrum of clinical manifestations, ranging from asymptomatic to critical conditions. Understanding the...     

Compassionate Use of Lumacaftor/Ivacaftor in Cystic Fibrosis: Spanish Experience

Background

The most common cystic fibrosis (CF)-causing mutation is deltaF508 (F508del), which is present in 28% of CF Spanish patients. While the literature based on real-life studies on CF patients homozygous F508del treated with lumacaftor/ivacaftor is limited, it demonstrates the need for better strategies to prevent related adverse events (AEs) as well as the development of newer drugs.

Erratum to: Multivariate study of predictive factors for clearly defined lung lesions without visible endobronchial lesions in transbronchial biopsy

Background  

Although the diagnostic validity and predictive factors for the diagnostic yield of transbronchial biopsy (TBB) of clearly defined pulmonary lesions with no visible endobronchial lesion have been analyzed in numerous studies, very few have used multivariate analysis techniques to evaluate the validity of TBB as a diagnostic tool or to analyze the independent influence of clearly dependent variables, such as the bronchus sign and lesion size.     

Endogenous IL-33 enhances Th2 cytokine production and T-cell responses during allergic airway inflammation

IL-33 is an IL-1-related cytokine which has been implicated in T(h)2-associated biology and allergic diseases in humans and mice. IL-33 stimulates T(h)2 cells, mast cells, eosinophils, basophils, iNKT cells and circulating CD34(+) stem cells to proliferate and produce pro-allergic cytokines such as IL-5 and IL-13. IL-33 mediates its cytokine effects through a receptor consisting of ST2 and IL-1RAcP. Whereas IL-1RAcP is ubiquitously expressed, ST2 expression is cell-type restricted and determines responsiveness to IL-33. Studies employing ST2-deficient mice have reported variable results on the role of this receptor, and consequently IL-33, with regards to allergic lung inflammation. In this study, we demonstrate that IL-33 is important for allergic lung inflammation. Intra-nasal administration of IL-33 triggered an immediate allergic response in the airways, and more importantly, we show that endogenous IL-33 contributes to airway inflammation and peripheral antigen-specific responses in ovalbumin-induced acute allergic lung inflammation using IL-33-deficient mice. Our results suggest that IL-33 is sufficient and required for severe allergic inflammation in the lung and support the concept of IL-33 as a therapeutic target in allergic lung inflammation.     

Semantic-phonologic treatment for noun and verb retrieval impairments in aphasia

Nouns and verbs differ in their neural and psycholinguistic attributes. It is not known whether these differences lead to distinct patterns of response to treatment for individuals with word retrieval impairments associated with aphasia. Eight participants with naming disorders induced by left hemisphere strokes were treated with a semantic-phonologic treatment protocol for nouns and verbs using a single participant multiple baseline design. We measured treatment gains in a picture naming measure and other secondary language and communication measures. Treatment led to improved picture naming for trained nouns and verbs in five of eight patients, with no difference evident between nouns and verbs. Improvements for untrained words were minimal. Improvement in verb retrieval was associated with increases on a functional measure of communicative effectiveness. Improvement for nouns and verbs was associated with severity of word retrieval impairment at onset. Although distinct in neural and psycholinguistic attributes, nouns and verbs were affected by treatment in a similar pattern in this group of individuals. Training-specific effects suggest the need for careful selection of training words to have potential for functional benefit in daily communication.