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Mahajerin A.,CHOC Childrens Specialists | Webber E.C.,Indiana University | Morris J.,Indiana University | Taylor K.,Indiana University | Saysana M.,Indiana University
Hospital pediatrics | Year: 2015

OBJECTIVES: Incidence of pediatric venous thromboembolism (VTE) is increasing due to increased survival of children with chronic diseases and use of interventions (eg, central venous lines), with VTE risk. Our objective was to create VTE prophylaxis guidelines with targeted identification of children at high risk to support appropriate mechanical and pharmacologic prophylaxis and integrate into the electronic medical record (EMR) as a hospital-wide quality improvement project.METHODS: Patients aged 12 to 17 years were included. We evaluated institutional data regarding VTE incidence and risk factors. We evaluated literature for populations at high risk for VTE. Guidelines were formulated, and an EMR tool to assess risk and support the guidelines was created and implemented.RESULTS: The EMR tool was used to screen 48% of qualified admissions for the first month and 81% in the final study month. On average, 69.1% of qualified admissions were screened monthly during the first 18 months of the program. No adverse events were reported due to pharmacologic prophylaxis.CONCLUSIONS: Many risk factors are common between children and adults and certain pediatric populations warrant prophylactic consideration. Pediatric VTE prophylaxis guidelines can be successfully implemented into the EMR to identify high-risk populations. Future studies should assess the long-term impact of implementation. Copyright © 2015 by the American Academy of Pediatrics.

Mahajerin A.,CHOC Childrens Specialists | Branchford B.R.,University of Colorado at Denver | Amankwah E.K.,Johns Hopkins Hospital | Raffini L.,University of Pennsylvania | And 4 more authors.
Haematologica | Year: 2015

Hospital-associated venous thromboembolism, including deep vein thrombosis and pulmonary embolism, is increasing in pediatric centers. The objective of this work was to systematically review literature on pediatric hospital- acquired venous thromboembolism risk factors and risk-assessment models, to inform future prevention research. We conducted a literature search on pediatric venous thromboembolism risk via PubMed (1946-2014) and Embase (1980-2014). Data on risk factors and risk-assessment models were extracted from case-control studies, while prevalence data on clinical characteristics were obtained from registries, large (n>40) retrospective case series, and cohort studies. Meta-analyses were conducted for risk factors or clinical characteristics reported in at least three studies. Heterogeneity among studies was assessed with the Cochran Q test and quantified by the I2 statistic. From 394 initial articles, 60 met the final inclusion criteria (20 case-control studies and 40 registries/large case series/cohort studies). Significant risk factors among case-control studies were: Intensive care unit stay (OR: 2.14, 95% CI: 1.97-2.32); central venous catheter (OR: 2.12, 95% CI: 2.00-2.25); mechanical ventilation (OR: 1.56, 95%CI: 1.42-1.72); and length of stay in hospital (per each additional day, OR: 1.03, 95% CI: 1.03-1.03). Three studies developed/applied risk-assessment models from a combination of these risk factors. Fourteen significant clinical characteristics were identified through non-case-control studies. This meta-analysis confirms central venous catheter, intensive care unit stay, mechanical ventilation, and length of stay as risk factors. A few pediatric hospital-acquired venous thromboembolism risk scores have emerged employing these factors. Prospective validation is necessary to inform risk-stratified prevention trials. © 2015 Ferrata Storti Foundation.

Lieu M.T.,University of California at Los Angeles | Ng B.G.,Sanford Burnham Institute for Medical Research | Rush J.S.,University of Kentucky | Wood T.,Greenwood Genetic Center | And 6 more authors.
Molecular Genetics and Metabolism | Year: 2013

Congenital disorders of glycosylation are a group of metabolic disorders with an expansive and highly variable clinical presentation caused by abnormal glycosylation of proteins and lipids. Dolichol kinase (DOLK) catalyzes the final step in biosynthesis of dolichol phosphate (Dol-P), which is the oligosaccharide carrier required for protein N-glycosylation. Human DOLK deficiency, also known as DOLK-CDG or CDG-Im, results in a syndrome that has been reported to manifest with dilated cardiomyopathy of variable severity. A male neonate born to non-consanguineous parents of Palestinian origin presented with dysmorphic features, genital abnormalities, talipes equinovarus, and severe, refractory generalized seizures. Additional multi-systemic manifestations developed including dilated cardiomyopathy, hepatomegaly, severe insulin-resistant hyperglycemia, and renal failure, which were ultimately fatal at age 9. months. Electrospray ionization mass spectrometric (ESI-MS) analysis of transferrin identified a type I congenital disorder of glycosylation; next-generation sequencing demonstrated homozygous p.Q483K DOLK mutations that were confirmed in patient fibroblasts to result in severely reduced substrate binding and catalytic activity. This patient expands the phenotype of DOLK-CDG to include anatomic malformations and multi-systemic dysfunction. © 2013 Elsevier Inc.

Wang R.Y.,CHOC Childrens Specialists | Braunlin E.A.,University of Minnesota | Rudser K.D.,University of Minnesota | Dengel D.R.,University of Minnesota | And 6 more authors.
Molecular Genetics and Metabolism | Year: 2014

Background: Treatments for mucopolysaccharidoses (MPSs) have increased longevity, but coronary artery disease (CAD) and cardiovascular complications cause mortality in a high percentage of patients. Non-invasive measures of sub-clinical atherosclerosis, such as carotid intima-media thickness (cIMT) and arterial stiffness, may be useful for prediction of CAD outcomes in MPS patients. Objectives: The aim of the study was to determine if cIMT and arterial stiffness are abnormal in MPS I and II patients compared to healthy controls. Methods: MPS patients underwent carotid artery ultrasonography, and electronic wall-tracking software was used to measure cIMT, carotid artery cross-sectional compliance (cCSC), cross-sectional distensibility (cCSD), and incremental elastic modulus (cIEM). Control data from healthy subjects were obtained from a different study that utilized identical testing within the same laboratory. Results: A total of 406 healthy controls and 25 MPS patients (16 MPS I, 9 MPS II) were studied. All MPS patients had or were receiving treatment: 15 patients (6 MPS I, 9 MPS II) were receiving enzyme replacement therapy (ERT), 9 patients (all MPS I) had received hematopoietic stem cell transplant (HSCT), and 1 patient with MPS I had received HSCT and was receiving enzyme replacement therapy (ERT). MPS patients had significantly higher mean (±SD) cIMT (0.56±0.05mm) compared to controls (0.44±0.04mm; adjusted p<0.001). MPS patients also had increased stiffness compared to controls, showing significantly lower cCSC (0.14±0.09mm2/mmHg versus 0.16±0.05mm2/mmHg; adjusted p=0.019), and higher cIEM (1362±877mmHg versus 942±396mmHg; adjusted p<0.001). cCSD in MPS patients was lower than that of controls (29.7±16.4% versus 32.0±8.2%) but was not statistically significant; p=0.12. Among MPS patients, cCSD showed a significant association with cIMT (p=0.047), while the association between cIEM and cIMT approached significance (p=0.077). No significant differences were observed in cIMT, cCSD, cCSC, and cIEM between MPS I and MPS II patients. Conclusions: Despite treatment, MPS patients had higher cIMT compared to healthy controls, indicating this marker of sub-clinical atherosclerosis may be a useful predictor of CAD outcomes. The association of arterial stiffness measures with cIMT suggests that mechanical and structural changes may occur in concert among MPS patients. Although yet to be confirmed, increased cIMT and arterial stiffness in MPS I and II patients may be a consequence of inflammatory signaling pathways triggered by heparan or dermatan sulfate-derived oligosaccharides. Prospective, longitudinal studies will need to be performed in order to evaluate the usefulness of these carotid measurements as predictors of adverse CAD outcomes in MPS patients. © 2013 Elsevier Inc.

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