2,532 results match your criteria: "All Children's Hospital[Affiliation]"

Objective: To delineate the mechanism behind insurance-related disparities in the prenatal diagnosis of a congenital heart defect (CHD).

Methods: This was a retrospective analysis of electronic health records of pregnant individuals whose infants received CHD surgery between 2019 and 2020 in the third-largest United States metropolitan area. The outcome was whether a prenatal diagnosis was received.

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  • Fibrinolysis is the process of breaking down fibrin in blood clots, initiated by plasminogen activation through tPA and uPA, while antifibrinolytics like PAI-1 and αAP help regulate this process.
  • Rare inherited disorders of the fibrinolytic pathway often lead to excessive bleeding, with conditions such as PAI-1 deficiency being treated with antifibrinolytic medications like aminocaproic acid during surgeries.
  • Diagnosing these disorders is challenging, often requiring specialized testing for protein activity and genetic mutations, as routine tests typically lack specificity and sensitivity for such conditions.
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  • - The study evaluates barriers to care for patients with orofacial clefts by surveying Florida orthodontists and families, and analyzing the Pediatric Health Information System (PHIS) database for age-related factors in alveolar bone grafting (ABG).
  • - Results show that a significant number of orthodontists treat cleft patients, but financial issues like poor reimbursement and reliance on Medicaid create barriers to care, with many patients facing out-of-pocket costs.
  • - The average age for ABG is found to be 10.3 years, with certain funding and residency factors contributing to delays in the procedure, highlighting ongoing challenges in accessing orthodontic care despite legal insurance requirements.
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Objective: To investigate the effectiveness of intraoperative nerve monitoring at decreasing vocal fold movement impairment in children undergoing at-risk procedures.

Background: Children undergoing aerodigestive or cardiovascular procedures are at risk for recurrent laryngeal nerve injury, leading to vocal fold movement impairment. Although intraoperative nerve monitoring has been shown to decrease recurrent laryngeal nerve injury in adults, there is paucity of data in children.

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  • β-adrenergic (β-AR) signaling triggers fight-or-flight responses, enhancing functions like heart and lung performance, metabolism, and muscle contraction.
  • Recent studies challenge the traditional view of β-AR signaling as short-lived, revealing its role in long-term effects like cell differentiation and epigenomic changes.
  • The chapter examines how cold stress influences adipose tissues through β-AR signaling, emphasizing the dual role of the histone demethylase JMJD1A in managing both immediate and prolonged thermogenic responses.
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  • * The study identifies a five-gene signature linked to N6-methyladenosine (m6A) RNA modification that is associated with patient survival and can improve risk assessment in medulloblastoma through a machine learning classification model.
  • * Findings indicate that m6A-associated long non-coding RNAs influence tumor progression and immune responses, suggesting potential new targets for treatment and the development of clinical tools for better management of the disease.
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High symptom burden in female X-linked chronic granulomatous disease carriers.

Clin Immunol

November 2024

Division of Allergy and Immunology, Department of Pediatrics, Johns Hopkins University, Baltimore, MD, United States of America; Institute for Clinical and Translational Research, St. Petersburg, FL, United States of America; Cancer and Blood Disorders Institute, Johns Hopkins All Children's Hospital, St. Petersburg, FL, United States of America.

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Are paediatric cardiology textbooks obsolete in the current digital era?

Cardiol Young

August 2024

Department of Paediatric Cardiology, Children's Health Ireland at Crumlin, Dublin, IE-L, Ireland.

Introduction: With the rise of online references, podcasts, webinars, self-test tools, and social media, it is worthwhile to understand whether textbooks continue to provide value in medical education, and to assess the capacity they serve during fellowship training.

Methods: A prospective mixed-methods study based on surveys that were disseminated to seven paediatric cardiology fellowship programmes around the world. Participants were asked to read an assigned chapter of textbook, followed by the completion of the survey.

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Velopharyngeal dysfunction (VPD) is the inability to achieve proper closure of the velopharyngeal (VP) port, affecting speech and swallowing. After an auditory-perceptual speech evaluation by a speech-language pathologist, objective assessment of the VP port is required to determine the need for surgical intervention. This 3-part series provides a comprehensive discussion on (1) the anatomy and physiology of the velopharyngeal mechanism; (2) fundamental speech terminology and principles of perceptual speech assessment for VPD; and (3) techniques for objective evaluation of the VP port and surgical decision-making process.

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The velopharyngeal (VP) port separates the nasopharynx from the oropharynx and is bordered by the velum, lateral pharyngeal walls, and posterior pharyngeal wall. Velopharyngeal dysfunction (VPD) is the inability to achieve proper closure of the VP port, affecting speech and swallowing. This 3-part series provides a comprehensive discussion on (1) the anatomy and physiology of the velopharyngeal mechanism; (2) fundamental speech terminology and principles of perceptual speech assessment for VPD; and (3) techniques for objective evaluation of the VP port and surgical decision-making process.

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Velopharyngeal dysfunction (VPD) is the inability to achieve proper closure of the velopharyngeal (VP) port, affecting speech and swallowing. The gold standard for diagnosis is auditory-perceptual speech evaluation by a specialized speech-language pathologist. This 3-part series provides a comprehensive discussion on (1) the anatomy and physiology of the velopharyngeal mechanism, (2) fundamental speech terminology and principles of perceptual speech assessment for VPD, and (3) techniques for objective evaluation of the VP port and surgical decision-making process.

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Background: Seafood allergy (SA), including allergy to shellfish (crustacean and mollusks) and fish, is among the 4 most common food allergies causing anaphylaxis, but there are limited data showing SA clinical management in different countries.

Objective: We sought to characterize a large cohort of patients with fish and shellfish allergy and to facilitate standardization of future care for this increasingly common allergic disease.

Methods: We performed a retrospective, observational, noninterventional study from 945 patients from 2015 to 2019 in 7 hospitals in the United States and the United Kingdom to evaluate SA.

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  • Rheumatologic disease-associated hemophagocytic lymphohistiocytosis (HLH) is a rare and serious condition that may arise from underlying autoimmune diseases; real-world data on the use of the monoclonal antibody emapalumab for treatment is limited.
  • A study called REAL-HLH reviewed medical records from 33 hospitals to evaluate the use and outcomes of emapalumab in patients with rheumatologic disease-associated HLH, finding that 14.3% of 105 patients studied had this subtype.
  • Results showed that emapalumab treatment led to improvements in key laboratory parameters, significantly reduced glucocorticoid use, and had a high survival rate, indicating its potential effectiveness in managing
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Introduction: The expansion of tumor-infiltrating lymphocytes (TIL) for adoptive cellular therapy is under investigation in many solid tumors of adulthood. Marrow-infiltrating lymphocytes (MIL) have demonstrated antitumor reactivity preclinically. Successful expansion of TIL/MIL has not been reported across pediatric solid tumor histologies.

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  • The diagnosis and management of biliary dyskinesia in children and adolescents is inconsistent, prompting a systematic review by the American Pediatric Surgical Association to create evidence-based recommendations.
  • The review focused on key areas such as diagnostic criteria, the need for cholecystectomy, and outcomes from surgical vs. non-surgical management, but found that diagnostic criteria are unclear and reliable predictors of treatment success are lacking.
  • Pediatric-specific guidelines are needed to clarify this condition, improve diagnostic processes, and determine effective management, along with calls for more prospective studies to identify which patients could benefit from surgery.
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  • The study investigated how weight loss affects sex hormone levels in individuals with type 2 diabetes (T2D), focusing on sex differences in hormone response.
  • It included 850 postmenopausal women and 890 men, measuring hormones like testosterone and estradiol and observing the impact of an intensive lifestyle intervention (ILI) over time.
  • Results showed that ILI led to significant hormone level changes in both sexes, with women experiencing decreases in estradiol and testosterone while men saw increases in testosterone, highlighting the importance of considering these hormonal shifts in relation to health risks.
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Background: The purpose of this study was to assess adults' perspectives after using the insulin-only Bionic Pancreas (BP) during a 13-week pivotal trial. Automated insulin delivery (AID) systems show promise in improving glycemic outcomes and reducing disease burden for those with type 1 diabetes mellitus (T1D). Understanding the lived experience of those using the BP can help to inform education and uptake of AID devices.

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Background: The management of neonates with long-gap esophageal atresia (LGEA) combined with distal congenital esophageal strictures (CES) is challenging. We sought to review our approach for this rare set of anomalies.

Methods: We reviewed children with LGEA + CES surgically treated at two institutions (2018-2024).

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Polycythemia vera is an indolent myeloproliferative disorder that predisposes patients to venous and arterial thrombosis and can transform into myelofibrosis and acute myeloid leukemia. Consistent phlebotomy prevents life-threatening cerebrovascular and coronary artery disease and prolongs survival in low-risk polycythemia vera (patients under 60 years without thrombosis). However, despite its effectiveness in preventing serious complications, phlebotomy does not necessarily enhance the quality of life (QoL).

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  • * Researchers analyzed 469 tumor samples to discover a five-gene m6A-associated long non-coding RNA (lncRNA) signature (M6LSig) that correlates with overall survival and developed a clinical nomogram to predict patient prognosis.
  • * The study found that the expression of m6A-associated lncRNAs is linked to immune cell types in the tumor environment and demonstrated that silencing key m6A genes slows tumor growth, underscoring the potential of
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In academic craniofacial surgery, gender disparities exist across various metrics including faculty positions, leadership roles, and conference representation. This study benchmarks the academic productivity of craniofacial surgeons in 2022 and surveys their perspectives regarding diversity, equity, and inclusion (DEI). Total, first author, and senior author PubMed publications in 2022 were recorded for 193 craniofacial attendings and fellows.

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Spatiotemporal transcriptomic mapping of regenerative inflammation in skeletal muscle reveals a dynamic multilayered tissue architecture.

J Clin Invest

August 2024

Departments of Medicine and Biological Chemistry, Johns Hopkins University School of Medicine, Institute for Fundamental Biomedical Research, Johns Hopkins All Children's Hospital, St. Petersburg, Florida, USA.

Article Synopsis
  • Macrophages are special cells that help heal damaged tissue by getting rid of dead cells and starting the healing process.
  • Scientists studied these cells in injured and sick muscles to understand how they work together to repair tissue.
  • They found different types of macrophages and discovered that their arrangement helps the body heal better, but certain treatments can mess up this healing process.
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