Introduction: COVID-19 disease resulted in over six million deaths worldwide. Although vaccines against SARS-CoV-2 demonstrated efficacy, breakthrough infections became increasingly common. There is still a lack of data regarding the severity and outcomes of COVID-19 among vaccinated compared to unvaccinated individuals.
View Article and Find Full Text PDFIntroduction: Atherosclerotic cardiovascular disease (ASCVD) is the major cause of morbidity and mortality worldwide. Statins provide primary and secondary ASCVD prevention. Intolerance due to statin-associated myalgias reduces long-term adherence, thus muting potential benefits.
View Article and Find Full Text PDFSingle ventricle congenital heart defect patients have improved survival with Fontan palliation. However, they remain at risk for nephropathy, as indicated by pathologic microalbuminuria. We sought to investigate whether echocardiographic measures of the inferior vena cava diameter (a surrogate for elevated CVP) indexed to the body surface area (iIVC) or cardiac index (CI) can predict the presence of nephropathy in Fontan patients.
View Article and Find Full Text PDFCase Rep Pediatr
December 2019
Introduction: Pompe disease is an autosomal recessive lysosomal storage disorder with marked morbidity and mortality, if untreated. With the advent of enzyme replacement therapy, it is essential to identify the infantile-type as early as possible to mitigate the effects of the enzyme deficiency. Identification is possible prenatally with testing of both parents.
View Article and Find Full Text PDFPurpose: Previous studies have reported that monoclonal antibodies against proprotein convertase subtilisin/kexin type 9 (PCSK9) in clinical practice have been underutilized due to several barriers, including high cost, stringent insurance authorization, patient cost-sharing and insufficient documentation of a patient's medical history. The purpose of our study was to determine if prescribing PCSK9 inhibitors only to patients meeting the established indications would significantly improve the approval rate and utilization.
Methods: We conducted a review and analysis of the medical records of patients referred by their physician to a hospital-based lipid clinic over a 20-month period specifically for initiation of a PCSK9 inhibitor.
Clin Pediatr (Phila)
October 2018
We aimed to determine median cumulative radiation exposure in pediatric intensive care unit (PICU) patients, proportion of patients with high radiation exposure (above annual average radiation per person of 6.2 mSv), and determine risk factors for high exposure. This was a retrospective chart review of PICU patients up to 18 years of age admitted to a large community hospital over 2 years.
View Article and Find Full Text PDFThe yield of outpatient echocardiograms varies based on the indication for the echocardiogram and the age of the patient. The purpose of this study was to determine the cumulative yield of outpatient echocardiograms by age group and reason for the test. A secondary aim was to determine the predictors of a positive echocardiogram in an outpatient cardiology clinic at a large community teaching hospital.
View Article and Find Full Text PDFPseudoaneurysm (PSA) is a known but rare complication of the right ventricle to pulmonary artery (RV-PA) conduits. The patient's clinical presentation can be variable ranging from asymptomatic to potential rupture. We describe an unusual case of a massive PSA in an infant who underwent RV-PA pulmonary homograft placement after relief of right ventricular outflow tract obstruction.
View Article and Find Full Text PDFWe present 4 normal-weight sibling children with lysosomal acid lipase deficiency (LAL-D). LAL-D was considered in the differential diagnosis based on the absence of secondary causes and primary inherited traits for their marked hyperlipidemia, together with unexplained hepatic transaminase elevation. Residual lysosomal acid lipase activity confirmed the diagnosis.
View Article and Find Full Text PDFIntroduction: In anthracycline-induced cardiomyopathy, the onset of diastolic dysfunction occurs before systolic dysfunction. Although, conventional echocardiogram is the standard method to assess cardiac function post anthracycline therapy, Tissue Doppler Imaging (TDI) may detect early onset cardiac diastolic dysfunction among anthracycline-recipient survivors of childhood cancers. There are limited data on the use of TDI in assessing anthracycline-associated cardiotoxicity in children.
View Article and Find Full Text PDFEbstein's anomaly is a rare congenital heart disorder and has a varied clinical course, with detection as late as the seventh decade. We hereby describe an 11-year-old child in whom Ebstein's anomaly was diagnosed. The most common presentation in early second decade is due to an arrhythmia; however, the present case report is about a patient who presented with acute congestive heart failure due to decompensation from an acute upper respiratory tract infection, which is a rather uncommon presentation.
View Article and Find Full Text PDFThis is a case report of a three-month-old male who presented to clinic with a cystic lesion under the tongue. On clinical examination, a cystic lesion was observed in the, floor-of-mouth. The patient was referred to Paediatric Otorhinolaryngology service for further management.
View Article and Find Full Text PDFIntroduction: It is unknown how the conflicting recommendations of the American Academy of Pediatrics and the US Preventive Services Task Force on pediatric hyperlipidemia affect screening.
Objectives: (a) Identify clinical predictors for screening for hyperlipidemia, (b) assess the adherence to the 2008 American Academy of Pediatrics guidelines, and (c) determine the efficacy of reminder cards in increasing the adherence to the aforementioned guidelines.
Methods: Retrospective chart review in the resident, attending, and adolescent medicine clinics during a 3-month period.
Aim: To determine whether heart rate variability parameters vary between in-utero drug-exposed infants and controls. To determine correlations between Finnegan score and heart rate variability parameters. To differentiate those drug-exposed infants who require treatment from those infants who do not.
View Article and Find Full Text PDFBackground: Pompe disease is a lysosomal glycogen storage disease (GSDII) characterized by deficiency of acid glucosidase, resulting in lysosomal glycogen accumulation in multiple tissues, with cardiac and skeletal muscles being the most seriously affected. It manifests itself as a spectrum in multiple age groups including infancy, childhood and adulthood.
Case Report: We present a case of infantile Pompe disease that was detected at a four month well visit in the presence of hypotonia and failure to thrive.
Unlabelled: As the effect of Fontan circulation on the kidneys has not been defined, the purpose of this study was to determine whether it is associated with nephropathy.
Methods: Patients with single ventricle physiology at least 2 years after Fontan procedure with normal cardiac function by echocardiography, history of normal renal ultrasound, and no co-morbidities known to affect the kidneys were evaluated. Patient's demographics, diagnoses, pre- and post-Fontan catheterization data, and current medications were collected.
Fish chromatophores serve as excellent study models for cytoskeleton-dependent organelle translocations because the distribution of pigmentary organelles can be observed against a time frame by microscopy. In this study the distribution of microfilaments along with microtubules in cultured melanophores of the killifish (Fundulus heteroclitus Linneaus) are examined using whole-cell transmission electron microscopy (WCTEM), fluorescence, and laser scanning confocal microscopy. Dispersing, dispersed, aggregating and aggregated states of pigment are induced by adding either caffeine (for dispersion) or epinephrine (for aggregation) to the cells in a standard culture medium.
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