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BACKGROUND: Although pulmonary artery banding (PAB) has been generally acknowledged as an initial palliative treatment for patients having single ventricle (SV) physiology and unrestrictive pulmonary blood flow (UPBF), it may result in unfavorable outcomes. Performing bidirectional Glenn (BDG) surgery without initial PAB in some selected cases may avoid the complications associated with PAB and reduce the number of operative procedures for these patients. This research aimed to assess the outcome of BDG surgery performed directly without doing initial PAB in patients with SV-UPBF. METHODS: This Multicenter retrospective cohort includes all patients with SV-UPBF who had BDG surgery. Patients were separated into two groups. Patients in Group 1 included patients who survived till they received BDG (20 Patients) after initial PAB (28 patients), whereas patients in Group 2 got direct BDG surgery without first performing PAB (16 patients). Cardiac catheterization was done for all patients before BDG surgery. Patients with indexed pulmonary vascular resistance (PVRi) ≥ 5 WU.m2 at baseline or > 3 WU.m2 after vasoreactivity testing were excluded. RESULTS: Compared with patients who had direct BDG surgery, PAB patients had a higher cumulative mortality rate (32% vs. 0%, P = 0.016), with eight deaths after PAB and one mortality after BDG. There were no statistically significant differences between the patient groups who underwent BDG surgery regarding pulmonary vascular resistance, pulmonary artery pressure, postoperative usage of sildenafil or nitric oxide, intensive care unit stay, or hospital stay after BDG surgery. However, the cumulative durations in the intensive care unit (ICU) and hospital were more prolonged in patients with BDG after PAB (P = 0.003, P = 0.001respectively). CONCLUSION: Direct BDG surgery without the first PAB is related to improved survival and shorter hospital stays in some selected SV-UPBF patients.
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Procedimiento de Fontan , Cardiopatías Congénitas , Corazón Univentricular , Humanos , Lactante , Arteria Pulmonar/cirugía , Procedimiento de Fontan/métodos , Cardiopatías Congénitas/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Cuidados Paliativos/métodos , Ventrículos Cardíacos/cirugíaRESUMEN
Perfluorocarbons (PFCs) are organic liquids derived from hydrocarbons in which some of the hydrogen atoms have been replaced by fluorine atoms. They are chemically and biologically inert substances with a good safety profile. They are stable at room temperature, easy to store, and immiscible in water. Perfluorocarbons have been studied in biomedical research since 1960 for their unique properties as oxygen carriers. In particular, PFCs have been used for liquid ventilation in unusual environments such as deep-sea diving and simulations of zero gravity, and more recently for drug delivery and diagnostic imaging. Additionally, when delivered as emulsions, PFCs have been used as red blood cell substitutes. This narrative review will discuss the multifaceted utilization of PFCs in therapeutics, diagnostics, and research. We will specifically emphasize the potential role of PFCs as red blood cell substitutes, as airway mechanotransducers during artificial placenta procedures, as a means to improve donor organ perfusion during the ex vivo assessment, and as an adjunct in cancer therapies because of their ability to reduce local tissue hypoxia.
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Sustitutos Sanguíneos , Fluorocarburos , Sustitutos Sanguíneos/uso terapéutico , Sustitutos Sanguíneos/química , Emulsiones , OxígenoRESUMEN
Introduction: and importance: Infantile hemangioma, being a benign tumor of the blood vessel, is part of a triad composed of also thrombocytopenia and hypofibrinogenemia as part of Kasabach Merrit Syndrome. Case presentation: We report the case of a 2 months old female Saudi infant referred due to respiratory distress, thrombocytopenia, and enlarging hemangioma on right upper chest, neck, and lower cheek. Diagnosis of kaposiform hemangioendothelioma complicated by Kasabach Merritt thrombocytopenia was done based on the clinical triad of thrombocytopenia, bleeding tendency, and the presence of a vascular tumor. Conclusion: Vincristine and CTA embolization are lines of management that showed to be the most efficient in the improvement of the clinical picture of KMS in our patient.
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Background: Williams syndrome (WS) (also as known Williams-Beuren Syndrome) is a neurodevelopmental disorder caused by deletion of chromosomes 7q11.23. WS phenotype is very variable but usually it is associated with a distinctive pattern of cognitive abilities. Case presentation: 9-year-old female patient known case of developmental delay and precocious puberty presented to the emergency department with altered level of consciousness, elevated blood pressure of 230/160 with a provisional diagnosis of hypertensive encephalopathy. Conclusions: In our patient, the strongest indications for establishing the diagnosis included the classic elfin face and multisystemic involvement. Later on genetic analysis confirmed our diagnosis.
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Introduction and importance: Lupus nephritis is particularly a very concerning occurrence due to the susceptibility for potential renal damage and ultimately renal failure. Cardiac involvement was present as well in the form of pericarditis. Our study reports a case of lupus nephritis that has had a very severe course of fluctuations between relapses and improvements which constantly necessitated an MDT interference at various points. Case presentation: We report a case of a 13-year-old female patient who presented with a 5-day history of fever, dizziness, joint pain, menorrhagia, convulsions, and visual disturbances. Essential diagnostic tests took place and a diagnosis of lupus nephritis was confirmed. Conclusion: In conclusion we found that a combination of various treatment modalities and flexibility in decision making in response to changes in the clinical course are vital to treatment success. Utilization of plasma exchange which resulted in an enormous drop in the percentage of fragmented red blood cells (RBCs) from 9.8% to 1.8%.
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Introduction: The hand hygiene practice (HHP) is the most effective and simplest preventive measure to reduce the risk of infection. HHP is more relevant among pediatric physicians in the context of the COVID-19 pandemic since, children are more vulnerable to infection. Therefore, assessment of the COVID-19 impact on HHP could be useful in minimizing lethal virus transmission from pediatric physicians to patients and vice versa. Method: The present cross-sectional, electronically self-administered supplement based survey study was conducted among different professional levels of pediatric physicians involving consultants, specialists, and residents. The supplement includes information related to demography, knowledge, awareness, preventive measures, demonstration and practice of HHP. The information was collected and summarized on a Microsoft excel sheet before being imported to SPSS for statistical analysis. Results: Of the total (N = 404) pediatric physicians, 56.68% male, 43.06% belongs to 25-35 years, 42.32% were consultants, 98.01% respondents were familiar with five moments of HHP. Further, HHP immediately before touching patients (99.26%), clean/aseptic procedure (95.04%), after body fluid exposure (72.28%), after touching patients (98.01%), after touching surrounding of patients (74.75%) may prevent germ transmission to patients whereas HHP after touching patients (98.27%), before clean/aseptic procedure (67.57%), after exposure to immediate surroundings of patients (97.02%) may prevent germ transmission to pediatric physicians. Rubbing hands is preferred before palpation of abdomen (74.25%), before giving injection (56.68%), after removing gloves (61.88%), after making a patient's bed (47.80%), while washing of hands preferred after emptying bedpan (67.82%) and after visible exposure to blood (84.40%), 92.57% believed gloves can't replace HHP, posters display at point of care as reminders (95.30%), received frequent HHP education (82.92%), 50.49% do not need HHP reminder, 51.73% preferred alcohol based sanitizer, 53.46% facilitate daily morning huddle, HHP >10 times per day before COVID-19 (24.62%) while in COIVID-19 (56.44%). HPP is the most effective way to prevent the spread (98.01%) of microbes because it kills germs (90.35%), health care associated infections is the major (38.06%) cause of germ transmission, 86.88% will be remains committed to HHP even after pandemic. In comparison to residents and specialists, consultants gave more importance (p = 0.02) to HHP and were more adherent during (p = 0.007) and even after (p = 0.001) COVID-19 pandemic. Conclusion: Assessing knowledge of pediatric physician, awareness, and adherence to hand hygiene measures could be helpful to reduce the contact transmission of lethal viruses to patients and vice versa. Further increase in the awareness, knowledge and education of HHP are required in order to maximize its utilization.