Primary Children's Hospital

About: Primary Children's Hospital is a healthcare organization based out in Salt Lake City, Utah, United States. It is known for research contribution in the topics: Population & Health care. The organization has 1770 authors who have published 2594 publications receiving 107857 citations. The organization is also known as: Intermountain Primary Children's Medical Center & Intermountain Primary Children's Hospital.

Long-term sustained disease control in patients with mantle cell lymphoma with or without active disease after treatment with allogeneic hematopoietic cell transplantation after nonmyeloablative conditioning.

Abstract: BACKGROUND Previously, early results were reported for allogeneic hematopoietic cell transplantation (HCT) after nonmyeloablative conditioning with 2 Gy of total body irradiation with or without fludarabine and/or rituximab in 33 patients with mantle cell lymphoma (MCL). METHODS This study examined the outcomes of 70 patients with MCL and included extended follow-up (median, 10 years) for the 33 initial patients. Grafts were obtained from human leukocyte antigen (HLA)–matched, related donors (47%), unrelated donors (41%), and HLA antigen–mismatched donors (11%). RESULTS The 5-year incidence of nonrelapse mortality was 28%. The relapse rate was 26%. The 5-year rates of overall survival (OS) and progression-free survival (PFS) were 55% and 46%, respectively. The 10-year rates of OS and PFS were 44% and 41%, respectively. Eighty percent of surviving patients were off immunosuppression at the last follow-up. The presence of relapsed or refractory disease at the time of HCT predicted a higher rate of relapse (hazard ratio [HR], 2.94; P = .05). Despite this, OS rates at 5 (51% vs 58%) and 10 years (43% vs 45%) were comparable between those with relapsed/refractory disease and those undergoing transplantation with partial or complete remission. A high-risk cytomegalovirus (CMV) status was the only independent predictor of worse OS (HR, 2.32; P = .02). A high-risk CMV status and a low CD3 dose predicted PFS (HR, 2.22; P = .03). CONCLUSIONS Nonmyeloablative allogeneic HCT provides a long-term survival benefit for patients with relapsed MCL, including those with refractory disease or multiple relapses. Cancer 2015;121:3709–3716. © 2015 American Cancer Society.

The Use of Haloperidol in Agitated Critically Ill Children

Abstract: Sedation, analgesia, and muscle relaxants are often used to control agitation and facilitate mechanical ventilation in children with acute hypoxic respiratory failure. In most children, adequate sedation can be achieved with a combination of parenteral benzodiazepines and opioids. However, long-term continuous infusions of opioids and benzodiazepines often result in physiologic tolerance and the need for dose escalation in order to maintain an appropriate level of sedation. Occasionally, mechanically ventilated children remain anxious, agitated, or combative despite escalating doses of opioids and benzodiazepines. In some children, additional doses of these agents seem to exacerbate agitation and combative-

Regional and global myocardial deformation of the fetal right ventricle in hypoplastic left heart syndrome

Abstract: Objective Quantification of fetal right ventricular (RV) function by 2D-echocardiography is challenging. Velocity vector imaging (VVI) is an angle independent speckle tracking technique that assesses regional myocardial mechanics. Alteration in the deformation of the fetal RV in hypoplastic left heart syndrome (HLHS) is unknown. This study aimed to evaluate the regional mechanics of the fetal RV in HLHS. Methods We retrospectively analyzed HLHS fetuses imaged at our center. Velocity, strain and strain rate were obtained using VVI. Global and regional parameters of RV deformation were compared between HLHS fetuses and gestational age matched controls. Results A total of 30 HLHS fetuses and 30 gestational age matched controls were analyzed. The mean gestational age was 30.5 ±3.5 weeks. Global myocardial strain was significantly decreased in HLHS RVs compared to controls (−1.9% vs. -4.1%, p = 0.003). The volume of the hypoplastic left ventricle did not affect RV deformation in HLHS. Conclusions Compared to controls, the RV in HLHS fetuses has decreased systolic strain. Prenatal abnormalities in RV myocardial deformation may be responsible for inefficient cardiac performance and output. RV myocardial performance in HLHS can be measured prenatally and may be predictive of post-natal RV dysfunction. © 2012 John Wiley & Sons, Ltd.

Optimization of anti-pseudomonal antibiotics for cystic fibrosis pulmonary exacerbations: VI. Executive summary.

Abstract: Acute pulmonary exacerbations (APE) are well-described complications of cystic fibrosis (CF) and are associated with progressive morbidity and mortality. Despite aggressive management with two or more intravenous anti-pseudomonal agents, approximately 25% of exacerbations will result in a loss of lung function. The aim of this review is to provide an overview of the classes of intravenous anti-pseudomonal antibiotics, the findings of anti-pseudomonal antibiotic utilization surveys, the current antibiotic dosing recommendations from the U.S. and Europe, and the pharmacokinetic (PK) and pharmacodynamic (PD) differences between CF and non-CF individuals. Anti-pseudomonal antibiotic classes include beta-lactams, aminoglycosides, fluoroquinolones, and colistimethate sodium. Recent surveys of antibiotic utilization in CF Foundation-accredited care centers have shown that a large number of centers are not following recommended dosing strategies despite published recommendations in the U.S. and Europe. The recommended doses for anti-pseudomonal antibiotics may be higher than FDA-approved doses due to PK and PD differences. As a large portion of CF patients will not regain their lung function following an APE, it seems possible that currently available anti-pseudomonal agents are being used sub-optimally. As new anti-pseudomonal agents are not currently available, we suggest the need to optimize antibiotic dosing and dosing regimens used to treat pulmonary exacerbations in an effort to improve outcomes for CF patients infected with Pseudomonas aeruginosa.

Association of cigarette smoking and CRP levels with DNA methylation in α-1 antitrypsin deficiency

Abstract: Alpha-1 antitrypsin (AAT) deficiency and tobacco smoking are confirmed risk factors for Chronic Obstructive Pulmonary Disease. We hypothesized that variable DNA methylation would be associated with smoking and inflammation, as reflected by the level of C-Reactive Protein (CRP) in AAT-deficient subjects. Methylation levels of 1,411 autosomal CpG sites from the Illumina GoldenGate Methylation Cancer Panel I were analyzed in 316 subjects. Associations of five smoking behaviors and CRP levels with individual CpG sites and average methylation levels were assessed using non-parametric testing, linear regression and linear mixed effect models, with and without adjustment for age and gender. Univariate linear regression analysis revealed that methylation levels of 16 CpG sites significantly associated with ever-smoking status. A CpG site in the TGFBI gene was the only site associated with ever-smoking after adjustment for age and gender. No highly significant associations existed between age at smoking initiation...