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Health-Related Quality of Life After Pediatric Cochlear Implantation
Objectives: To examine the results of health-related quality of life (HRQoL) questionnaire scores from deaf children fitted with at least one cochlear implant (CI) and compare responses to normal-hearing age-peers (NHP) and to their parents.
Methods: This cross-sectional study included 33 pediatric patients with a hearing experience of at least 1 year with CI and a control group of 21 NHP. The KINDLR questionnaire for measuring HRQoL (generic) in children (7-12years) and adolescents (13-17years) and a CI questionnaire (specific) were completed by CI users and their parents. Scores were transformed to a 100-point scale with 100 representing the most positive response.
Results: The group of children and adolescents with CI reached a similar score in the generic HRQoL (Children:78,6; Adolescents:80,4; p = 0,35) and in the overall CI questionnaire (Children:77,6; Adolescents:75,6; p = 0,53). CI users in both age groups scored generic HRQoL similarly to their NHP (NH children:82,2, p = 0,18; NH adolescents:77,5; p = 0,07) and higher than CI users from other centers (OC) (OC children:75,4; OC adolescents:70,3). CI users scored their school domain lower than their NHP (p = 0,04). Generic and CI questionnaire scores of parents and their children correlated positively (r = 0,66, p = 0,00 and r = 0,73, p = 0,00). The total scores of HRQoL in the self-rating and parent rating correlate with none of the variables at study (gender, cause of deafness, age at implantation, years with a CI, chronological age) except with speech progression (p = 0,007).
Conclusions: Children with CI experience similar quality of life as NHP. Parents are reliable reporters on the status of their child's overall quality of life.info:eu-repo/semantics/publishedVersio
Blunted Coronary Flow Velocity Reserve is Associated With Impairment in Systolic Function and Functional Capacity in Hypertrophic Cardiomyopathy
Background
Coronary microvascular dysfunction constitutes an important pathophysiological feature in hypertrophic cardiomyopathy (HCM).
We aimed to assess the association between impaired coronary flow velocity reserve (CFVR) and ventricular systolic function and functional capacity.
Methods
Eighty-three patients with HCM were enrolled in this prospective cohort study. Patients underwent echocardiogram to evaluate ventricular performance and CFVR in the left anterior descending artery (LAD) and posterior descending artery (PD). Diastolic coronary flow velocity was measured in basal conditions and in hyperemia. CFVR was calculated as the ratio of hyperemic and basal peak diastolic flow velocities. Functional capacity was evaluated by cardiopulmonary exercise testing (CPET). The link between CFVR and biventricular systolic function and peak VO2 was studied.
Results
Age was 55.0(14.4)years, 50 patients (60%) were male; 59 patients (71%) had nonobstructive HCM. Mean CFVR LAD was 1.81(0.49) and CFVR PD was 1.73(0.55). Lower CFVR PD was associated with impaired global longitudinal strain (GLS) 2D (β-estimate:-3.240,95%CI:-4.634;-1.846, p < 0.001), GLS 3D (β-estimate:-2.559,95%CI:-3.932;-1.186, p < 0.001) and area strain (β-estimate:-3.044,95%CI:-5.373;-0.716, p = 0.011). Lower values of CFVR PD related to worse global work index (β-estimate:267.824,95%CI:75.964;459.683, p = 0.007), global constructive work (β-estimate:217.300,95%CI:38.750;395.850, p = 0.018) and global work efficiency (β-estimate:5.656,95%CI:2.229;9.084, p = 0.002). Impaired CFVR LAD (β-estimate:2.826, 95%CI:0.913;4.739, p = 0.004) and CFVR PD (β-estimate:2.801,95%CI:0.657;4.945, p = 0.011) were associated with lower TAPSE. Lower values of CFVR LAD (β-estimate:2.580, 95%CI:0.169;4.991, p = 0.036) and CFVR PD (β-estimate:3.163, 95%CI: 0.721;5.606, p = 0.012) were associated with worse peak VO2.
Conclusion
Lower CFVR was associated with impairment in biventricular systolic function parameters and functional capacity assessed by pVO2.info:eu-repo/semantics/publishedVersio
Dapagliflozin Impact on the Exercise Capacity of Non-Diabetic Heart Failure with Reduced Ejection Fraction Patients
Background: Dapagliflozin has been shown to reduce morbidity and mortality in Heart Failure with reduced Ejection Fraction (HFrEF), but its impact on exercise capacity of non-diabetic HF outpatients is unknown.
Methods: Adult non-diabetic HF patients with a left ventricular ejection fraction (LVEF) <50% were randomized 1:1 to receive dapagliflozin 10 mg or to continue with HF medication. Patients underwent an initial evaluation which was repeated after 6 months. The variation of several clinical parameters was compared, with the primary endpoint being the 6 month peak oxygen uptake (pVO2) variation.
Results: A total of 40 patients were included (mean age 61 ± 13 years, 82.5% male, mean LVEF 34 ± 5%), half being randomized to dapagliflozin, with no significant baseline differences between groups. The reported drug compliance was 100%, with no major safety events. No statistically significant difference in HF events was found (p = 0.609). There was a 24% reduction in the number of patients in New York Heart Association (NYHA) class III in the treatment group as opposed to a 15.8% increase in the control group (p = 0.004). Patients under dapagliflozin had a greater improvement in pVO2 (3.1 vs. 0.1 mL/kg/min, p = 0.030) and a greater reduction in NT-proBNP levels (-217.6 vs. 650.3 pg/mL, p = 0.007).
Conclusion: Dapagliflozin was associated with a significant improvement in cardiopulmonary fitness at 6 months follow-up in non-diabetic HFrEF patients.info:eu-repo/semantics/publishedVersio
Efficiency of the EmERGE Pathway of Care in Five European HIV Centres
Objective: We aimed to calculate the efficiency of the EmERGE Pathway of Care in five European HIV clinics, developed and implemented for medically stable people living with HIV.
Methods: Participants were followed up for 1 year before and after implementation of EmERGE, between April 2016 and October 2019. Micro-costing studies were performed in the outpatient services of the clinics. Unit costs for outpatient services were calculated in national currencies and converted to US purchasing parity prices. Primary and secondary outcome measures of participants did not change during the study.
Conclusions: EmERGE is acceptable and provided cost savings in different socio-economic settings. Antiretroviral drug costs remain the main cost drivers in medically stable people living with HIV. While antiretroviral drug prices in local currencies did not differ that much between countries, conversion to US$ purchasing parity prices revealed antiretroviral drugs were more expensive in the least wealthy countries. This needs to be taken into consideration when countries negotiate drug prices with pharmaceutical vendors. Greater efficiencies can be anticipated by extending the use of the EmERGE Pathway to people with complex HIV infection or other chronic diseases. Extending such use should be systematically monitored, implementation should be evaluated and funding should be provided to monitor and evaluate future changes in service provision.info:eu-repo/semantics/publishedVersio
Intensidad de la Senal ˜ en T1 en el Núcleo Dentado Tras la Administración del Agente de Contraste con Gadolinio Macrocíclico Gadoterato de Meglumina: un Estudio Observacional
Introduction and aims: Contradictory results have been reported about hyperintensity of the globus pallidus and/or dentate nucleus on unenhanced T1-weighted magnetic resonance (MR) images after exposure to various gadolinium-based contrast agents. This change in signal intensity varies with different gadolinium-based contrast agents. We aimed to determine whether signal intensity in the dentate nucleus is increased in unenhanced T1-weighted images in patients who have undergone multiple studies with the macrocyclic gadolinium-based contrast agent gadoterate meglumine. We thoroughly reviewed the literature to corroborate our results.
Materials and methods: We included patients who had undergone more than 10 MR studies with gadoterate meglumine. We quantitatively analyzed the signal intensity in unenhanced T1-weighted MR images measured in regions of interest placed in the dentate nucleus and the pons, and we calculated the dentate nucleus-to-pons signal intensity ratios and the differences between the ratio in the first MR study and the last MR study. We used t-tests to evaluate whether the differences between the signal intensity ratios were different from 0. We also analyzed the subgroups of patients who had been administered <15 and ≥15 doses of gadoterate meglumine. We used Pearson correlation to determine the relationships between the differences in the signal intensity ratios and the number of doses of gadoterate meglumine administered.
Results: The 54 patients (26 men) had received a mean of 13.8±3.47 doses (range, 10-23 doses). The difference in the dentate nucleus-pons signal intensity ratio between the first and last MR study was -0.0275±0.1917 (not significantly different from 0; p=0.2968) in the entire group, -0.0357±0.2204 (not significantly different from 0; p = 0.351 in the patients who had received <15 doses (n=34), and -0.0135±0.1332 (not significantly different from 0; p = 0.655) in those who had received ≥15 doses (n=20). Differences in signal intensity ratios did not correlate significantly with the accumulated dose of gadoterate meglumine (P = 0.9064; ρ = -0.0164 [95%]).
Conclusions: Receiving more than 10 doses of gadoterate meglumine was not associated with increased signal intensity in the dentate nucleus.Introducción y objetivo: Se han notificado resultados contradictorios sobre un aumento en la
intensidad de la se˜nal (IS) en las imágenes de resonancia magnética (RM) ponderadas en T1
no realzadas en el globo pálido y/o el núcleo dentado (ND) después de la exposición a varios
agentes de contraste con gadolinio (ACG). Este cambio en la se˜nal varía en función del ACG
específico. Nuestro objetivo fue investigar si existe un aumento en la IS del ND en imágenes
ponderadas en T1 no realzadas en pacientes sometidos a múltiples administraciones del ACG
macrocíclico gadoterato de meglumina. Se realizó una revisión exhaustiva de la bibliografía
para corroborar nuestros resultados.
Materiales y métodos: Se incluyeron pacientes que se habían sometido a más de 10 estudios de
RM con contraste y administración exclusiva de gadoterato de meglumina. Se llevó a cabo un
análisis cuantitativo mediante el uso de mediciones de regiones de interés en el ND y el puente
en imágenes no realzadas ponderadas en T1. Se calcularon las proporciones ND-puente y las
diferencias en las proporciones entre el inicio y la última RM realizada. Se utilizó una prueba
de la t de una muestra para evaluar si las diferencias en la proporción de la IS difieren de 0. Se
realizó un análisis de subgrupos de pacientes con <15 y ≥15 dosis. Se utilizó el análisis de correlación
de Pearson para determinar las correlaciones entre las diferencias de las proporciones
de la IS y el número de administraciones del ACG.
Resultados: 54 pacientes (26 hombres) recibieron una media de 13,8 dosis ± 3,47 (desviación
estándar [DE]) (rango, 10−23 dosis). La diferencia en la proporción de la IS ND-puente entre la
primera y la última exploración de RM fue de -0,0275 ± 0,1917 y no difirió significativamente de
0 P = 0,2968) en el análisis general y en el análisis de subgrupos [(<15 (n = 34), -0,0357 ± 0,2204,
P = 0,351 y ≥15 (n = 20), -0,0135 ± 0,1332, p = 0,655)]. Las diferencias en la proporción de la IS
no se correlacionaron significativamente Pon la dosis acumulada de gadoterato de meglumina
(P = 0,9064; = -0,0164 [95%]).
Conclusiones: Más de 10 administraciones de gadoterato de meglumina no se asoció a un
aumento de la IS en el ND.info:eu-repo/semantics/publishedVersio
Dynamic Prediction of Survival After Curative Resection of Intrahepatic Cholangiocarcinoma: a Landmarking-Based Analysis
Background: The current study aimed to develop a dynamic prognostic model for patients undergoing curative-intent resection for intrahepatic cholangiocarcinoma (ICC) using landmark analysis.
Methods: Patients who underwent curative-intent surgery for ICC from 1999 to 2017 were selected from a multi-institutional international database. A landmark analysis to undertake dynamic overall survival (OS) prediction was performed. A multivariate Cox proportional hazard model was applied to measure the interaction of selected variables with time. The performance of the model was internally cross-validated via bootstrap resampling procedure. Discrimination was evaluated using the Harrell's Concordance Index. Accuracy was evaluated with calibration plots.
Results: Variables retained in the multivariable Cox regression OS model included age, tumor size, margin status, morphologic type, histologic grade, T and N category, and tumor recurrence. The effect of several variables on OS changed over time. Results were provided as a survival plot and the predicted probability of OS at the desired time in the future. For example, a 65-year-old patient with an intraductal, T1, grade 3 or 4 ICC measuring 3 cm who underwent an R0 resection had a calculated estimated 3-year OS of 76%. The OS estimate increased if the patient had already survived 1 year (79%). The discrimination ability of the final model was very good (C-index: 0.80).
Conclusion: The long-term outcome for patients undergoing curative-intent surgery for ICC should be adjusted based on follow-up time and intervening events. The model in this study showed excellent discriminative ability and performed well in the validation process.info:eu-repo/semantics/publishedVersio
Suitability and Allocation of Protein-Containing Foods According to Protein Tolerance in PKU: A 2022 UK National Consensus
Introduction: There is little practical guidance about suitable food choices for higher natural protein tolerances in patients with phenylketonuria (PKU). This is particularly important to consider with the introduction of adjunct pharmaceutical treatments that may improve protein tolerance. Aim: To develop a set of guidelines for the introduction of higher protein foods into the diets of patients with PKU who tolerate >10 g/day of protein. Methods: In January 2022, a 26-item food group questionnaire, listing a range of foods containing protein from 5 to >20 g/100 g, was sent to all British Inherited Metabolic Disease Group (BIMDG) dietitians (n = 80; 26 Inherited Metabolic Disease [IMD] centres). They were asked to consider within their IMD dietetic team when they would recommend introducing each of the 26 protein-containing food groups into a patient’s diet who tolerated >10 g to 60 g/day of protein. The patient protein tolerance for each food group that received the majority vote from IMD dietetic teams was chosen as its tolerance threshold for introduction. A virtual meeting was held using Delphi methodology in March 2022 to discuss and agree final consensus. Results: Responses were received from dietitians from 22/26 IMD centres (85%) (11 paediatric, 11 adult). For patients tolerating protein ≥15 g/day, the following foods were agreed for inclusion: gluten-free pastas, gluten-free flours, regular bread, cheese spreads, soft cheese, and lentils in brine; for protein tolerance ≥20 g/day: nuts, hard cheeses, regular flours, meat/fish, and plant-based alternative products (containing 5−10 g/100 g protein), regular pasta, seeds, eggs, dried legumes, and yeast extract spreads were added; for protein tolerance ≥30 g/day: meat/fish and plant-based alternative products (containing >10−20 g/100 g protein) were added; and for protein tolerance ≥40 g/day: meat/fish and plant-based alternatives (containing >20 g/100 g protein) were added. Conclusion: This UK consensus by IMD dietitians from 22 UK centres describes for the first time the suitability and allocation of higher protein foods according to individual patient protein tolerance. It provides valuable guidance for health professionals to enable them to standardize practice and give rational advice to patients.info:eu-repo/semantics/publishedVersio
Patterns of Parental Reactions to Their Children’s Negative Emotions: A Cluster Analysis with a Clinical Sample
Parents' emotion socialization practices are an important source of influence in the development of children's emotional competencies This study examined parental reactions to child negative emotions in a clinical sample using a cluster analysis approach and explored the associations between clusters of parents' reactions and children's and parents' adjustment. The sample comprised 80 parents of Portuguese children (aged 3-13 years) attending a child and adolescent psychiatry unit. Measures to assess parental reactions to children's negative emotions, parents' psychopathological symptoms, parents' emotion dysregulation, and children's adjustment were administered to parents. Model-based cluster analysis resulted in three clusters: low unsupportive, high supportive, and inconsistent reactions clusters. These clusters differed significantly in terms of parents' psychopathological symptoms, emotion dysregulation, and children's adjustment. A pattern characterized by high supportive reactions to the child's emotions was associated with higher levels of children's adjustment. On the other hand, an inconsistent reactions pattern was associated with the worst indicators of children's adjustment and parental emotion dysregulation. These results suggest the importance of supporting parents of children with emotional and behavioural problems so that they can be more responsive to their children's emotional manifestations.info:eu-repo/semantics/publishedVersio