Abstract Background Dental anesthetic management in implantable cardioverter defibrillator (ICD) recipients with cardiac channelopathies (CCh) can be challenging due to the potential risk of life-threatening arrhythmias and appropriate ICD therapies during procedural time. Objectives The present study assessed the hypothesis that the use of local dental anesthesia with 2% lidocaine with 1:100,000 epinephrine or without a vasoconstrictor can be safe in selected ICD and CCh patients, not resulting in life-threatening events (LTE). Methods Restorative dental treatment under local dental anesthesia was made in two sessions, with a wash-out period of 7 days (cross-over trial), conducting with a 28h - Holter monitoring, and 12-lead electrocardiography, digital sphygmomanometry, and anxiety scale assessments in 3 time periods. Statistical analysis carried out the paired Student’s t test and the Wilcoxon signed-rank test. In all cases, a significance level of 5% was adopted. All patients were in stable condition with no recent events before dental care. Results Twenty-four consecutive procedures were performed in 12 patients (9 women, 3 men) with CCh and ICD: 7 (58.3%) had long QT syndrome (LQTS), 4 (33.3%) Brugada syndrome (BrS), and 1 (8.3%) Catecholaminergic polymorphic ventricular tachycardia (CPVT). Holter analysis showed no increased heart rate (HR) or sustained arrhythmias. Blood pressure (BP), electrocardiographic changes and anxiety measurement showed no statistically significant differences. No LTE occurred during dental treatment, regardless of the type of anesthesia. Conclusion Lidocaine administration, with or without epinephrine, can be safely used in selected CCh-ICD patients without LTE. These preliminary findings need to be confirmed in a larger population with ICD and CCh.

Abstract Mucopolysaccharidosis VI (MPS VI) is a progressive lysosomal storage disorder with multiorgan and multisystemic pathology. Currently, galsulfase enzyme replacement therapy (ERT) is the only approved treatment for MPS VI. A crosssectional survey study of 121 patients with MPS VI conducted in 2001 to 2002 and a 10-year follow-up study of the same patients (resurvey study; ClinicalTrials.gov NCT01387854) found that those receiving galsulfase at any time showed physical improvements and a lower mortality rate (16.5%) versus treatment-naive patients (50%). After *15 years, galsulfasetreated patients (n ¼ 104) continue to have a survival advantage over treatment-naive patients (n ¼ 14), as demonstrated by a 24% versus 57% mortality rate. This survival advantage is further supported by data from the commercial use of galsulfase (2005-2016), which show a 5-year mortality rate for galsulfase-treated patients of 12.5%. Together, these findings suggest that galsulfase ERT can increase life expectancies for patients with MPS VI over a period of at least 15 years.

Abstract Objective: To assess the feasibility of clinical pharmacist-led CYP2C19 genotype-guided P2Y12 inhibitor antiplatelet drug therapy recommendations to cardiologists in an outpatient cardiology practice. Methods: This was a prospective, open-labeled, single-arm study conducted in an integrated healthcare delivery system between March 1, 2013 and January 23, 2014. Patients requiring non-emergent cardiac catheterization were included. A clinical pharmacist provided interpretation and recommendations from genotyping results. The feasibility of implementing CYP2C19 genotype-guided antiplatelet therapy was assessed by the: 1) percentage of patients approached who consented to CYP2C19 genotyping, 2) percentage of patients with CYP2C19 genotyping results available prior to cardiac catheterization, and 3) percentage of clinical pharmacist CYP2C19 genotype-based antiplatelet recommendations accepted by cardiologists. Results: Of the 43 patients identified for potential recruitment, 22 of these were eligible for study enrollment and 6 (27%) patients consented and received CYP2C19 genotyping. All patients had genotyping results available prior to catheterization and all clinical pharmacists’ antiplatelet therapy recommendations were accepted by the patients’ cardiologists. Three patients had the CYP2C19 wild-type (*1/*1) genotype and the clinical pharmacist recommended clopidogrel therapy. CYP2C19 variant genotypes (i.e., *1/*2, *1/*17, and *2/*17) were found in the other three patients; alternative antiplatelet therapy was recommended for the patient with the *1/*2 genotype, while clopidogrel was recommended for those with *1/*17 and *2/*17 genotypes. Conclusion: A relatively small proportion of patients undergoing non-emergent cardiac catheterization consented to pharmacogenetic testing; however, their cardiologists were receptive to clinical pharmacists conducting such testing and providing corresponding pharmacotherapy recommendations. Future studies should identify patient barriers to pharmacogenetic testing.

OBJECTIVE: To examine the usefulness of urine sodium (Na) excretion in spot or timed urine samples to estimate population dietary Na intake relative to the gold standard of 24-hour (h) urinary Na. METHODS: An electronic literature search was conducted of MEDLINE (from 1950) and EMBASE (from 1980) as well as the Cochrane Library using the terms "sodium," "salt," and "urine." Full publications of studies that examined 30 or more healthy human subjects with both urinary Na excretion in 24-h urine and one alternative method (spot, overnight, timed) were examined. RESULTS: The review included 1 380 130 participants in 20 studies. The main statistical method for comparing 24-h urine collections with alternative methods was the use of a correlation coefficient. Spot, timed, and overnight urine samples were subject to greater intra-individual and interindividual variability than 24-h urine collections. There was a wide range of correlation coefficients between 24-h urine Na and other methods. Some values were high, suggesting usefulness (up to r = 0.94), while some were low (down to r = 0.17), suggesting a lack of usefulness. The best alternative to collecting 24-h urine (overnight, timed, or spot) was not clear, nor was the biological basis for the variability between 24-h and alternative methods. CONCLUSIONS: There is great interest in replacing 24-h urine Na with easier methods to assess dietary Na. However, whether alternative methods are reliable remains uncertain. More research, including the use of an appropriate study design and statistical testing, is required to determine the usefulness of alternative methods.

OBJETIVO: Analizar la utilidad de la medición de la excreción urinaria de sodio a partir de la recolección puntual o cronometrada de muestras de orina para calcular la ingesta de sodio alimentario en la población, en relación con la prueba de referencia que mide la excreción de sodio en orina de 24 horas. MÉTODOS: Se realizó una búsqueda de bibliografía electrónica en MEDLINE (desde 1950) y EMBASE (desde 1980), así como en la Biblioteca Cochrane, empleando los términos "sodium", "salt" y "urine" (sodio, sal y orina). Se examinaron las publicaciones completas de los estudios que incluían 30 o más sujetos humanos sanos en los que se hubiera determinado la excreción de sodio mediante la recolección de orina de 24 horas o un método alternativo (recolección puntual, de toda la noche, cronometrada). RESULTADOS: La revisión incluyó a 1 380 130 participantes de 20 estudios. El principal método estadístico adoptado para comparar las recolecciones de orina de 24 horas con los métodos alternativos fue el uso de un coeficiente de correlación (r). Las muestras de orina recolectadas de forma puntual, cronometrada y de toda la noche estaban sujetas a mayor variabilidad intra e interindividual que las recolecciones de orina de 24 horas. Se obtuvo una amplia gama de coeficientes de correlación entre las determinaciones de sodio en orina de 24 horas y mediante los otros métodos. Algunos valores fueron elevados, lo que indica su utilidad (r de hasta 0,94), mientras que otros fueron bajos (r por debajo de 0,17), lo que indica su falta de utilidad. La mejor alternativa a la obtención de orina de 24 horas (de toda la noche, cronometrada, o puntual) no resultó evidente, ni tampoco la base biológica de la variabilidad entre el método de 24 horas y los alternativos. CONCLUSIONES: Hay mucho interés en remplazar la determinación de sodio en orina de 24 horas por otros métodos más fáciles de evaluación del sodio alimentario. Sin embargo, sigue habiendo incertidumbre sobre la fiabilidad de los métodos alternativos. Es preciso ampliar la investigación, incluido el uso de un diseño de estudio y pruebas estadísticas apropiados, para determinar la utilidad de los métodos alternativos.