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Background Despite a rigorous screening process, including cardiac catheterization, a subset of patients with a single right ventricle (SRV) demonstrates suboptimal short‐term outcomes after the Fontan operation. The goal of this study was to perform a comprehensive assessment of diastolic function in pre‐Fontan patients with an SRV using invasive reference‐standard measures and determine their associations with post‐Fontan outcomes. Methods and Results Children aged 2 to 6 years with SRV physiology undergoing pre‐Fontan heart catheterization were recruited prospectively. Patients were divided into those who had an optimal or suboptimal outcome. A suboptimal outcome was defined as length of stay ≥14 days or heart transplant/cardiac death in first year after Fontan. Patients underwent pressure–volume loop analysis using reference‐standard methods. The measure of ventricular stiffness, β, was obtained via preload reduction. Cardiac magnetic resonance imaging for extracellular volume and serum draws for matrix metalloproteinase activity were performed. Of 19 patients with an SRV, 9 (47%) had a suboptimal outcome. Mean age was 4.2±0.7 years. Patients with suboptimal outcomes had lower ventricular stiffness (0.021 [0.009–0.049] versus 0.090 [0.031–0.118] mL−1; P=0.02), lower extracellular volume (25% [28%–32%] versus 31% [28%–33%]; P=0.02), and lower matrix metalloproteinase‐2 (90 [79–104] versus 108 [79–128] ng/mL; P=0.01) compared with patients with optimal outcomes. The only invasive measure that had an association with suboptimal outcome was β (P=0.038). Conclusions Patients with an SRV with suboptimal outcome after the Fontan operation had lower ventricular stiffness and evidence of maladaptive extracellular matrix metabolism compared with patients with optimal outcome. This appears to be a novel phenotype that may have important clinical implications and requires further study.

Background Heart failure phenotyping in single‐ventricle Fontan patients is challenging, particularly in patients with normal ejection fraction (EF). The objective of this study was to identify Fontan patients with abnormal diastolic function, who are high risk for heart failure with preserved ejection fraction (HFpEF), and characterize their cardiac mechanics, exercise function, and functional health status. Methods and Results Data were obtained from the Pediatric Heart Network Fontan Cross‐sectional Study database. EF was considered abnormal if 90th percentile (≥0.26 mL‐1). Patients were divided into: controls=normal EF and diastolic function; systolic dysfunction (SD) = abnormal EF with normal diastolic function; diastolic dysfunction (DD) = normal EF with abnormal diastolic pressure:volume quotient. Exercise function was quantified as percent predicted peak VO2. Physical Functioning Summary Score (FSS) was reported from the Child Health Questionnaire. A total of 239 patients were included, 177 (74%) control, 36 (15%) SD, and 26 (11%) DD. Median age was 12.2 (5.4) years. Arterial elastance, a measure of arterial stiffness, was higher in DD (3.6±1.1 mm Hg/mL) compared with controls (2.5±0.8 mm Hg/mL), P

Pemphigus is a chronic blistering disorder caused by autoantibodies that target desmosomal proteins in the epidermis. Acantholysis may be absent, and pemphigus may present only with spongiosis and vesiculation, thereby leading to a misdiagnosis of eczema. Herein, we conducted a retrospective, observational, single-center study to establish a pattern of spongiosis in cases of pemphigus confirmed by direct immunofluorescence. Immunopathologically diagnosed pemphigus specimens from 2001 to 2020 were retrieved, and specimens with spongiosis were analyzed for the following features: vesiculation, acantholysis, spongiosis, inflammatory cells in the epidermis, and inflammation in the dermis. Cases of spongiotic dermatitis were used as control. Out of 99 immunopathologically diagnosed pemphigus specimens, 41 samples with spongiosis were identified. About one quarter of the specimens did not have acantholysis. Spongiosis in the middle to lower thirds of the perilesional epidermis (p = 0.030), exocytosis with either neutrophils or eosinophils (p = 0.016), dermal infiltrates composed of lymphocytes, eosinophils, and neutrophils (p = 0.012), and absence of Langerhans cell microabscesses (p < 0.001) were more common in pemphigus than control. Spongiosis in pemphigus may mimic eczema in patients without acantholysis. The subtle histological findings in this study provide diagnostic clues and suggest that further immunofluorescence should be performed to confirm pemphigus diagnosis. [ABSTRACT FROM AUTHOR]

Abstract We report on the observation of a new phenomenon occurring in a fiber ring laser. This phenomenon is about the transition from an initially bidirectional emission of a reciprocal fiber ring laser to a unidirectional emission at a certain pump power threshold. In addition, the final direction is not predefined but appears to be randomly chosen every time the threshold is exceeded. Therefore, we term this new phenomenon direction instability. Furthermore, we provide a first discussion of how the instability threshold is influenced by the length and the loss of the cavity. We show that the threshold follows a power times length scaling, indicating a nonlinear origin.

This study aims to find out how to use the physiotherapist's speech strategy at RSU Haji Medan according to the theories of Brown and Levinson. This research uses a qualitative approach with the case study method while data obtained through observation and interviews. This research found that there are 2 speech strategies used by physiotherapists at RSU Haji Medan. First, on record with positive politeness strategy contained in request directive speech acts (100%), question directive speech acts (100%), requirements directive speech acts (100%), agreement directive speech acts (100%), advisories directive speech acts (71%). Second, bald-on record strategy contained in prohibition directive speech acts (100%) and advisories directive speech acts (29%). Abstrak Penelitian ini bertujuan untuk mengetahui bagaimana penggunaan strategi bertutur seorang fisioterapis di RSU Haji Medan menurut teori Brown dan Levinson. Penelitian ini menggunakan pendekatan kualitatif dengan metode studi kasus, sedangkan data diperoleh melalui observasi dan wawancara. Analisis data dalam penelitian ini menggunakan teknik analisis interaktif. Penelitian ini menemukan ada dua strategi bertutur yang digunakan oleh fisioterapis di RSU Haji Medan. Pertama, strategi bertutur terus terang dengan basa basi, berupa kesantunan positif yang terdapat dalam tindak tutur direktif permintaan (100%), tindak tutur direktif pertanyaan (100%), tindak tutur direktif persyaratan (100%), tindak tutur direktif persetujuan (100%), dan tindak tutur direktif nasihat (71%);. Kedua, strategi bertutur terus terang tanpa basa-basi hanya terdapat dalam tindak tutur direktif pelarangan (100%) dan tindak tutur direktif nasihat (29%).

Pyogenic granuloma (PG) is a benign vascular tumor removed surgically or by physical modalities, such as electrocautery and cryotherapy. Other treatment options may be warranted, especially in children, using nonsurgical approach. The study aims to determine the effectiveness and safety of topical treatments on PG in children in the clearance of lesions. Systematic literature search were done in February 2023 in the following database: PubMed, HERDIN, Cochrane Library, Hindawi, Directory of Open Access Journals, ScienceDirect, and Google Scholar. The search included studies involving PG treated with topical medications in the pediatric age group. Twenty-five studies (9 cohort studies, 6 case series, and 10 case reports) were included and utilized the following medications: timolol, propranolol, corticosteroids, common table salt, imiquimod, and ingenol mebutate. Among the medications, timolol was the most reported drug to provide partial to complete resolution in 62 out of 149 patients. All the reviewed topical medications provided partial to complete resolution with minimal adverse effects. Among them, topical timolol, propranolol, and table salt have demonstrated high response rates. Topical therapy in children with PG may be considered as a nonsurgical option. However, further clinical trials are warranted to determine its effectiveness.

Pemphigus is a chronic blistering disorder caused by autoantibodies that target desmosomal proteins in the epidermis. Acantholysis may be absent, and pemphigus may present only with spongiosis and vesiculation, thereby leading to a misdiagnosis of eczema. Herein, we conducted a retrospective, observational, single-center study to establish a pattern of spongiosis in cases of pemphigus confirmed by direct immunofluorescence. Immunopathologically diagnosed pemphigus specimens from 2001 to 2020 were retrieved, and specimens with spongiosis were analyzed for the following features: vesiculation, acantholysis, spongiosis, inflammatory cells in the epidermis, and inflammation in the dermis. Cases of spongiotic dermatitis were used as control. Out of 99 immunopathologically diagnosed pemphigus specimens, 41 samples with spongiosis were identified. About one quarter of the specimens did not have acantholysis. Spongiosis in the middle to lower thirds of the perilesional epidermis (p = 0.030), exocytosis with either neutrophils or eosinophils (p = 0.016), dermal infiltrates composed of lymphocytes, eosinophils, and neutrophils (p = 0.012), and absence of Langerhans cell microabscesses (p < 0.001) were more common in pemphigus than control. Spongiosis in pemphigus may mimic eczema in patients without acantholysis. The subtle histological findings in this study provide diagnostic clues and suggest that further immunofluorescence should be performed to confirm pemphigus diagnosis.

Lung transplantation (LTx) outcomes are impeded by ischemia/reperfusion injury (IRI) and subsequent chronic lung allograft dysfunction (CLAD). We examined the undefined role of receptor Mer tyrosine kinase (MerTK) on monocytic myeloid-derived suppressor cells (M-MDSCs) in efferocytosis to facilitate resolution of lung IRI. Single-cell RNA sequencing of lung tissue and bronchoalveolar lavage (BAL) from patients after LTx were analyzed. Murine lung hilar ligation and allogeneic orthotopic LTx models of IRI were used with BALB/c (WT), Cebpb-/- (MDSC-deficient), Mertk-/-, or MerTK-cleavage-resistant mice. A significant downregulation in MerTK-related efferocytosis genes in M-MDSC populations of patients with CLAD was observed compared with healthy individuals. In the murine IRI model, a significant increase in M-MDSCs, MerTK expression, and efferocytosis and attenuation of lung dysfunction was observed in WT mice during injury resolution that was absent in Cebpb-/- and Mertk-/- mice. Adoptive transfer of M-MDSCs in Cebpb-/- mice significantly attenuated lung dysfunction and inflammation. Additionally, in a murine orthotopic LTx model, increases in M-MDSCs were associated with resolution of lung IRI in the transplant recipients. In vitro studies demonstrated the ability of M-MDSCs to efferocytose apoptotic neutrophils in a MerTK-dependent manner. Our results suggest that MerTK-dependent efferocytosis by M-MDSCs can substantially contribute to the resolution of post-LTx IRI.

The preferred tool for conceptualizing, determining, and claiming relations of kinship, ancestry, and descent among humans are diagrams. For this reason, and at the same time to avoid a reduction to biology as transported by terms such as kinship , ancestry , and descent , we introduce the expression diagrammatics of relatedness . We seek to understand the enormous influence that especially tree diagrams have had as a way to express and engage with human relatedness, but hold that this success can only be adequately understood by attending to what in fact are broader diagrammatic practices. These practices bring to light that diagrams of relatedness do not simply make visible natural connections, but create or deny relations in particular ways and for particular reasons. In this special section, contributors investigate diagrams of relatedness in genealogy, heredity, as well as biological and social anthropology. Conceiving of diagrams as techniques that transcend such binaries as 'thought and action' and 'image and text', we aim at an understanding of how they were constructed and how they functioned in particular epistemic, cultural, and political contexts., Competing Interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

The pathogenesis of abdominal aortic aneurysm (AAA) formation involves vascular inflammation, thrombosis formation and programmed cell death leading to aortic remodeling. Recent studies have suggested that ferroptosis, an excessive iron-mediated cell death, can regulate cardiovascular diseases, including AAAs. However, the role of ferroptosis in immune cells, like macrophages, and ferroptosis-related genes in AAA formation remains to be deciphered. Single cell-RNA sequencing of human aortic tissue from AAA patients demonstrates significant differences in ferroptosis-related genes compared to control aortic tissue. Using two established murine models of AAA and aortic rupture in C57BL/6 (WT) mice, we observed that treatment with liproxstatin-1, a specific ferroptosis inhibitor, significantly attenuated aortic diameter, pro-inflammatory cytokine production, immune cell infiltration (neutrophils and macrophages), increased smooth muscle cell α-actin expression and elastic fiber disruption compared to mice treated with inactivated elastase in both pre-treatment and treatment after a small AAA had already formed. Lipidomic analysis using mass spectrometry shows a significant increase in ceramides and a decrease in intact lipid species levels in murine tissue compared to controls in the chronic AAA model on day 28. Mechanistically, in vitro studies demonstrate that liproxstatin-1 treatment of macrophages mitigated the crosstalk with aortic smooth muscle cells (SMCs) by downregulating MMP2 secretion. Taken together, this study demonstrates that pharmacological inhibition by liproxstatin-1 mitigates macrophage-dependent ferroptosis contributing to inhibition of aortic inflammation and remodeling during AAA formation.

This round table discussion takes the diversity of discourse and practice shaping modern knowledge about childhood as an opportunity to engage with recent historiographical approaches in the history of science. It draws attention to symmetries and references among scientific, material, literary and artistic cultures and their respective forms of knowledge. The five participating scholars come from various fields in the humanities and social sciences and allude to historiographical and methodological questions through a range of examples. Topics include the emergence of children's rooms in US consumer magazines, research on the unborn in nineteenth-century sciences of development, the framing of autism in nascent child psychiatry, German literary discourses about the child's initiation into writing, and the sociopolitics of racial identity in the photographic depiction of African American infant corpses in the early twentieth century. Throughout the course of the paper, childhood emerges as a topic particularly amenable to interdisciplinary perspectives that take the history of science as part of a broader history of knowledge.