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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

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.

BackgroundHidradenitis suppurativa were associated with comorbidities in various organ systems. Inflammatory dermatological diseases such as pyoderma gangrenosum were reported to be associated with hidradenitis suppurativa. Nevertheless, as for the association between hidradenitis suppurativa and psoriasis, evidences were insufficient. In many studies, the association between psoriasis and hidradenitis suppurativa has been reported. However, some evidence seems to be controversial. The purpose of the systematic review and meta-analysis was to assess whether there was significant association between HS and psoriasis.MethodsOn June 01, 2022, we appraised 2,795 articles from databases including PubMed, Web of Science and Embase. Search syntaxes were based on ‘hidradenitis suppurativa’ or ‘acne inversa’ with “psoriasis”, “comorbidities” or ‘epidemiology’. Synonyms were determined based on MeSH terms and Emtree. Observational results that evaluated the odds ratio for people with hidradenitis suppurativa who had psoriasis were extracted for qualitative synthesis.ResultsAfter the selection process of the initial 2,795 studies, ten observational studies, including 3 cohort studies, 1 case-control study, and 6 cross-sectional studies, were extracted for critical appraisal. Based on the integration of 7 studies (with more than 560,000 participants included), people with hidradenitis suppurativa had a higher risk of having psoriasis, with a 2.67-fold risk (95% CI, 1.84, 3.87). The association remained in the sensitivity analyses utilizing strict adjustment models. In the analysis that only included studies with a similar study design and adjustments in obesity-related factors, the risk of people with hidradenitis suppurativa having psoriasis was 3.24 (95% CI, 2.27, 4.62). In male patients with HS, the risk of having psoriasis was 4.30-fold higher than male patients without HS (95% CI, 2.37, 7.78). Likewise, in an analysis including 3 cross-sectional studies, the risk of female HS patients having psoriasis was 3.94-fold higher than female HS-free patients (95% CI, 2.34, 6.63).ConclusionsThe co-occurrence of hidradenitis suppurativa and psoriasis can greatly increase the burden of the disease. Psoriasis could be one of the critical comorbidities of hidradenitis suppurativa and should be recommended for future screening and follow up. The association between the two diseases should be kept in mind in managing hidradenitis suppurativa patients. More prospective studies are needed to establish the true magnitude of the association between psoriasis and hidradenitis suppurativa.

Adverse drug reactions (ADR) remain the major problems in healthcare. Most severe ADR are unpredictable, dose-independent and termed as type B idiosyncratic reactions. Recent pharmacogenomic studies have demonstrated the strong associations between severe ADR and genetic markers, including specific HLA alleles (e.g., HLA-B*15:02/HLA-B*57:01/HLA-A*31:01 for carbamazepine-induced severe cutaneous adverse drug reactions [SCAR], HLA-B*58:01 for allopurinol-SCAR, HLA-B*57:01 for abacavir-hypersensitivity, HLA-B*13:01 for dapsone/co-trimoxazole-induced SCAR, and HLA-A*33:01 for terbinafine-induced liver injury), drug metabolism enzymes (such as CYP2C9*3 for phenytoin-induced SCAR and missense variant of TPMT/NUDT15 for thiopurine-induced leukopenia), drug transporters (e.g., SLCO1B1 polymorphism for statin-induced myopathy), and T cell receptors (Sulfanilamide binding into the CDR3/Vα of the TCR 1.3). This mini review article aims to summarize the current knowledge of pharmacogenomics of severe ADR, and the potentially clinical use of these genetic markers for avoidance of ADR.

As the world deals with the COVID-19 pandemic, vaccination remains vital to successfully end this crisis. However, COVID-19-vaccine-induced immediate hypersensitivity reactions presenting with potentially life-threatening systemic anaphylactic reactions are one of the reasons for vaccine hesitancy. Recent studies have suggested that different mechanisms, including IgE-mediated and non-IgE-mediated mast cell activation, may be involved in immediate hypersensitivity. The main culprits triggering hypersensitivity reactions have been suggested to be the excipients of vaccines, including polyethylene glycol and polysorbate 80. Patients with a history of allergic reactions to drugs, foods, or other vaccines may have an increased risk of hypersensitivity reactions to COVID-19 vaccines. Various strategies have been suggested to prevent hypersensitivity reactions, including performing skin tests or in vitro tests before vaccination, administering different vaccines for the primary and following boosters, changing the fractionated doses, or pretreating the anti-IgE antibody. This review discusses the current trends, potential mechanisms, and prevention strategies for COVID-19-vaccine-induced immediate hypersensitivity reactions.