48 research outputs found
Longitudinal Assessment of Growth in Hypoplastic Left Heart Syndrome: Results From the Single Ventricle Reconstruction Trial
Background: We sought to characterize growth between birth and age 3 years in infants with hypoplastic left heart syndrome who underwent the Norwood procedure. Methods and Results: We performed a secondary analysis using the Single Ventricle Reconstruction Trial database after excluding patients 2 SD below normal). Failure to find consistent risk factors supports the strategy of tailoring nutritional therapies to patientâ and stageâspecific targets. Clinical Trial Registration URL: http://clinicaltrials.gov/. Unique identifier: NCT00115934
Mucosal associated invariant T cells are altered in patients with Hidradenitis Suppurativa and contribute to the inflammatory milieu
Mucosal Associated Invariant T cells are a population of âinnateâ T cells, which express
the invariant T cell receptor (TCR) a chain Va7.2-Ja33 and are capable of robust rapid
cytokine secretion, producing a milieu of cytokines including IFN-g and IL-17. MAIT
cells have been reported in multiple human tissues including the gut, periphery and
skin. On-going research has highlighted their involvement in numerous inflammatory
diseases ranging from rheumatoid arthritis and obesity to psoriasis. Hidradenitis
Suppurativa (H.S) is a chronic inflammatory disease of the hair follicles, resulting in
painful lesions of apocrine-bearing skin. Several inflammatory cytokines have been
implicated in the pathogenesis of H.S including IL-17. The role of MAIT cells in H.S is
currently unknown. In this study we show for the first time, that MAIT cells are altered
in the peripheral blood of patients with H.S, with reduced frequencies and an IL-17
cytokine bias. We show that CCL20 expression is elevated in lesions of patients with
H.S, and MAIT cells can actively traffic towards lesions via CCL20. We show that MAIT
cells can accumulate in the lesionsfrom patients with H.S. when compared to adjacent
skin, with an IL-17 bias. We show that elevated IL-17, can be linked to the activation
of dermal fibroblasts, promoting the expression of chemotactic signals including
CCL20 and CXCL1. Finally, we show that targeting the IL-17A transcription factor RORyt
robustly reduces IL-17 production by MAIT cells from patients with H.S. Collectively
our data detailsIL-17 producing MAIT cells as a novel player in the pathogenesis of H.S
and highlights the potential of RORyt inhibition as a novel therapeutic strategy
International Consensus Statement on Rhinology and Allergy: Rhinosinusitis
Background: The 5 years since the publication of the first International Consensus Statement on Allergy and Rhinology: Rhinosinusitis (ICARâRS) has witnessed foundational progress in our understanding and treatment of rhinologic disease. These advances are reflected within the more than 40 new topics covered within the ICARâRSâ2021 as well as updates to the original 140 topics. This executive summary consolidates the evidenceâbased findings of the document. Methods: ICARâRS presents over 180 topics in the forms of evidenceâbased reviews with recommendations (EBRRs), evidenceâbased reviews, and literature reviews. The highest grade structured recommendations of the EBRR sections are summarized in this executive summary. Results: ICARâRSâ2021 covers 22 topics regarding the medical management of RS, which are grade A/B and are presented in the executive summary. Additionally, 4 topics regarding the surgical management of RS are grade A/B and are presented in the executive summary. Finally, a comprehensive evidenceâbased management algorithm is provided. Conclusion: This ICARâRSâ2021 executive summary provides a compilation of the evidenceâbased recommendations for medical and surgical treatment of the most common forms of RS
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Neuropsychological Status and Structural Brain Imaging in Adolescents With Single Ventricle Who Underwent the Fontan Procedure
Background: Few studies have described the neuropsychological outcomes and frequency of structural brain or genetic abnormalities in adolescents with single ventricle who underwent the Fontan procedure. Methods and Results: In a crossâsectional, singleâcenter study, we enrolled 156 subjects with single ventricle, mean age 14.5±2.9 years, who had undergone the Fontan procedure. Scores in the entire cohort on a standard battery of neuropsychological tests were compared with those of normative populations or to those of a group of 111 locally recruited healthy adolescents. They also underwent brain magnetic resonance imaging and were evaluated by a clinical geneticist. Genetic abnormalities were definite in 16 subjects (10%) and possible in 49 subjects (31%). Mean FullâScale IQ was 91.6±16.8, mean Reading Composite score was 91.9±17.2, and mean Mathematics Composite score was 92.0±22.9, each significantly lower than the population means of 100±15. Mean scores on other neuropsychological tests were similarly lower than population norms. In multivariable models, risk factors for worse neuropsychological outcomes were longer total support and circulatory arrest duration at first operation, presence of a genetic abnormality, more operations and operative complications, more catheterization complications, and seizure history. The frequency of any abnormality on magnetic resonance imaging was 11 times higher among Fontan adolescents than referents (66% versus 6%); 19 (13%) patients had evidence of a stroke, previously undiagnosed in 7 patients (40%). Conclusions: The neuropsychological deficits and high frequencies of structural brain abnormalities in adolescents who underwent the Fontan procedure highlight the need for research on interventions to improve the longâterm outcomes in this highârisk group