PubMed | University of Minnesota, Childrens Hospitals and Clinics of Minnesota, The Floating Hospital for Children at Tufts Medical Center and Oral and Maxillofacial Surgery Specialists
Type: Journal Article | Journal: JAMA facial plastic surgery | Year: 2016
Computed tomographic (CT) scans are often obtained before mandibular distraction osteogenesis in patients with isolated Pierre Robin sequence. There is concern regarding the risk of radiation exposure from CT in children.To evaluate whether preoperative CT is necessary for adequate airway, feeding, and aesthetic outcomes following mandibular distraction with external distraction devices in infants with isolated Pierre Robin sequence.In a retrospective review of medical records, infants who underwent mandibular distraction between January 1, 1998, and September 30, 2014, at 2 tertiary childrens hospitals were identified using procedure codes. Data analysis was conducted December 1, 2014, to March 31, 2015. Fifty-two patients fit the inclusion criteria of isolated Pierre Robin sequence or Stickler syndrome, of being younger than 9 months at the time of distraction, and of use of external distractors. Forty-two of these infants did not receive preoperative CT imaging.Mandibular distraction osteogenesis for isolated Pierre Robin sequence or Stickler syndrome.Number of infants who were able to avoid tracheostomy or achieve decannulation, who were able to avoid placement or achieve removal of a gastrostomy tube, and in whom there were no intraoperative complications, no open-bite deformity, no malocclusion, no asymmetry, and no postoperative complications.In comparison with the 10 infants who underwent preoperative CT, all 42 of the infants (100%) who did not receive preoperative CT imaging successfully avoided tracheostomy or achieved decannulation (P=.04) and 40 patients (95%) did not require placement of a gastrostomy tube or were able to undergo gastrostomy tube removal postoperatively (P<.001). There were no significant differences between the CT and non-CT groups in the other 5 outcome measures. Two patients (5%) required postoperative gastrostomy tube placement, 2 patients (5%) had minor intraoperative complications that might have been anticipated with CT, 2 patients (5%) demonstrated malocclusion, and 1 infant (2%) had asymmetry at the end of the distraction phase.This series suggests that the absence of preoperative CT does not compromise functional or aesthetic outcomes in mandibular distraction with external distraction devices in infants with isolated Pierre Robin sequence or Stickler syndrome. This finding has implications for cost containment and reduction of radiation exposure to a vulnerable population.4.
Zhang F.F.,Tufts University |
Rodday A.M.,Tufts University |
Rodday A.M.,Institute for Clinical Research and Health Policy Studies |
Kelly M.J.,Tufts University |
And 7 more authors.
Pediatric Blood and Cancer | Year: 2014
Background: A high prevalence of obesity has been increasingly recognized in survivors of pediatric ALL. However, longitudinal patterns of weight change during and after treatment, and associated factors, are less well elucidated. Procedure: In a retrospective cohort of 83 pediatric patients with ALL diagnosed between 1985 and 2010, we examined body mass index (BMI) status at several key time points: diagnosis; end of induction; end of consolidation; every 6 months during maintenance; and yearly for up to 5 years post-treatment. Results: At diagnosis, 21% were overweight (BMI=85-94.9th percentile) or obese (BMI≥95th percentile). At the end of treatment and 5 years post-treatment, approximately 40% were overweight or obese. The mean BMI z-score was 0.2 (58th percentile) at diagnosis and increased significantly during induction (Δ=0.5, P<0.0001). It increased again during the first 6 months of maintenance (Δ=0.2, P<0.01) and did not significantly change over the remainder of maintenance (BMI z-score at the end of treatment = 0.8, 79th percentile) and 5 years post-treatment (BMI z-score=0.7, 76th percentile). High BMI z-score at diagnosis was associated with an increased risk of being overweight/obese at treatment completion (OR=2.9, 95% CI: 1.6-5.1). Weight gain during treatment was associated with being overweight/obese 5 years post-treatment (OR=3.8, 95% CI: 1.1-12.5). Conclusion: Children with ALL are at risk of becoming overweight/obese early in treatment. Increases in weight are maintained throughout treatment and beyond. Lifestyle interventions are needed targeting weight control early during treatment, particularly for patients overweight/obese at diagnosis and those who experience substantial weight gain during treatment. Pediatr Blood Cancer 2014;61:1263-1269. © 2014 Wiley Periodicals, Inc.
Wachman E.M.,Boston Medical Center |
Hayes M.J.,University of Maine, United States |
Sherva R.,Boston University |
Brown M.S.,Eastern Maine Medical Center |
And 3 more authors.
Drug and Alcohol Dependence | Year: 2015
Background: There is significant variability in the severity of neonatal abstinence syndrome (NAS) due to in-utero opioid exposure. We wanted to determine if single nucleotide polymorphisms (SNPs) in key candidate genes contribute to this variability. Methods: Full-term opioid-exposed newborns and their mothers (. n=. 86 pairs) were studied. DNA was genotyped for 80 SNPs from 14 genes utilizing a custom designed microarray. The association of each SNP with NAS outcomes was evaluated. Results: SNPs in two opioid receptor genes in the infants were associated with worse NAS severity: (1) The PNOC rs732636 A allele (OR. =. 3.8, p=. 0.004) for treatment with 2 medications and a longer hospital stay (LOS) of 5.8 days (. p=. 0.01), and (2) The OPRK1 rs702764 C allele (OR. =. 4.1, p=. 0.003) for treatment with 2 medications. The OPRM1 rs1799971 G allele (. β=. -6.9 days, p=. 0.02) and COMT rs740603 A allele (. β=. -5.3 days, p=. 0.01) were associated with shorter LOS. The OPRD1 rs204076 A allele in the mothers was associated with a longer LOS by 6.6 days (. p=. 0.008). Results were significant point-wise but did not meet the experiment-wide significance level. Conclusions: These findings suggest that SNPs in opioid receptor and the PNOC genes are associated with NAS severity. However, further testing in a large sample is warranted. This has important implications for prenatal prediction and personalized treatment regimens for infants at highest risk for severe NAS. © 2015 Elsevier Ireland Ltd.
Panagos P.G.,The Floating Hospital for Children at Tufts Medical Center
Journal of Perinatology | Year: 2016
Objective:To determine the impact of maternal obesity on breastmilk composition.Study Design:Breastmilk and food records from 21 lean and 21 obese women who delivered full-term infants were analyzed at 2 months post-partum. Infant growth and adiposity were measured at birth and 2 months of age.Result:Breastmilk from obese mothers had higher omega-6 to omega-3 fatty acid ratio and lower concentrations of docosahexaenoic acid, eicosapentaenoic acid, docasapentaenoic acid and lutein compared with lean mothers (P<0.05), which were strongly associated with maternal body mass index. Breastmilk saturated fatty acid and monounsaturated fatty acid concentrations were positively associated with maternal dietary inflammation, as measured by dietary inflammatory index. There were no differences in infant growth measurements.Conclusion:Breastmilk from obese mothers has a pro-inflammatory fatty acid profile and decreased concentrations of fatty acids and carotenoids that have been shown to have a critical role in early visual and neurodevelopment. Studies are needed to determine the link between these early-life influences and subsequent cardiometabolic and neurodevelopmental outcomes.Journal of Perinatology advance online publication, 7 January 2016; doi:10.1038/jp.2015.199. © 2016 Nature America, Inc.
Stover M.W.,Tufts Medical Center |
Davis J.M.,The Floating Hospital for Children at Tufts Medical Center |
Davis J.M.,Clinical Translational Science Institute
Seminars in Perinatology | Year: 2015
Opiate use in pregnancy has increased dramatically over the past decade and now represents a major public health problem. More women are using prescription opioids, illegal opioids, and opioid-substitution therapy. These drugs have been associated with numerous obstetrical complications including intrauterine growth restriction, placental abruption, preterm delivery, oligohydramnios, stillbirth, and maternal death. Neonatal complications are also significant, such as an increased risk of mortality as well as neonatal abstinence syndrome (NAS). NAS is a serious and highly variable condition characterized by central nervous system hyperirritability and autonomic nervous system dysfunction. The present review seeks to define current practices regarding the management of opiate dependence in pregnancy and care of the neonate with prenatal opiate exposure. Since genetic factors appear to be associated with the incidence and severity of NAS, opportunities for "personalized genomic medicine" and unique therapeutic interventions could be developed in the future. © 2015 Elsevier Inc.
Zhang F.F.,Tufts University |
Liu S.,Tufts University |
Chung M.,Tufts University |
Kelly M.J.,The Floating Hospital for Children at Tufts Medical Center |
Kelly M.J.,Tufts University
Pediatric Blood and Cancer | Year: 2015
Background: Survivors of pediatric acute lymphoblastic leukemia (ALL) have a significantly higher body mass index (BMI) than their peers. Understanding the critical time periods in which patients with pediatric ALL are vulnerable to unhealthy weight gain will lay the groundwork for developing effectively timed interventions. Procedure: We determined the growth patterns of patients with pediatric ALL during and after treatment through the conduct of a systematic review and meta-analysis. A search of MEDLINE, Scopus, and Web of Science was performed from its inception through May 2014. Studies met the inclusion criteria if they included at least 10 patients of pediatric ALL, and longitudinally assessed BMI at diagnosis and at least one time point after diagnosis Results: Twenty-one studies met the inclusion criteria for the systematic review and 16 were included in meta-analysis. The mean increase in BMI z-score during treatment in 1,514 patients with pediatric ALL was 0.81 (95% CI: 0.25-1.38). Specifically, patients experienced substantial weight gain in early treatment (Δ=0.41, 95% CI: -0.34, 1.17) and again during maintenance (Δ=0.34, 95% CI:-0.22, 0.90). The mean increase in BMI z-score ranged between 0.52 and 0.89 beyond treatment completion. Subgroup analyses found unhealthy weight gain occurred regardless of patients' receipt of cranial radiation therapy, sex, and, weight status at diagnosis. Conclusions: Patients with pediatric ALL experience unhealthy weight gain early in treatment, and increases in weight are maintained beyond treatment completion. Preventing early onset of obesity is a priority for improving the care and outcomes for patients with pediatric ALL. © 2015 Wiley Periodicals, Inc.
Garg A.,The Floating Hospital for Children at Tufts Medical Center |
Sarkar S.,Project HEALTH |
Marino M.,Project HEALTH |
Onie R.,Project HEALTH |
Solomon B.S.,Johns Hopkins University
Patient Education and Counseling | Year: 2010
Introduction: Pediatric guidelines emphasize the importance for healthcare providers to view children in the context of family and community, and promote community resources at visits. In 2006, a Family Help Desk (FHD) was established in an urban academic-based clinic in Baltimore, MD to assist healthcare providers in educating families about available community-based resources. Methods: A longitudinal cohort pilot study was conducted during a 6-week period in 2007 to evaluate the impact of the FHD in connecting at-risk families to community resources. Results: Overall, 6% of parents (n=59) who brought their child for a scheduled clinic visit accessed the FHD. Parents had a mean of 1.7 social needs, including after-school programs and childcare (29%), employment (13%), housing (12%), and food (11%). Most parents who utilized the FHD (64%) contacted a community resource or service within 6 months of their clinic visit. Nineteen parents (32%) who utilized the FHD enrolled in community programs. Conclusion: A clinic-based multi-disciplinary model can empower families to connect with community-based resources for basic social needs. Practice implications: The Family Help Desk model has great potential for addressing family psychosocial needs, and educating families about community resources within the context of pediatric primary care. © 2009 Elsevier Ireland Ltd.
Kelly M.J.,The Health Institute |
Kelly M.J.,The Floating Hospital for Children at Tufts Medical Center |
Kelly M.J.,Tufts University |
Pennarola B.W.,The Health Institute |
And 4 more authors.
Pediatric Blood and Cancer | Year: 2012
Background: Little is known regarding the health-related quality of life (HRQL) trajectory of children with sickle cell disease or thalassemia ("hemoglobinopathies") following hematopoietic stem cell transplantation (HSCT). Procedures: We serially evaluated the HRQL of 13 children with hemoglobinopathies who received HSCT during two prospective multi-center studies using the Child Health Ratings Inventories (CHRIs). The HRQL scores among children with hemoglobinopathies, as reported separately by the children and their parents were compared using repeated measures models to scores of a comparison group of children receiving HSCT for malignancies or severe aplastic anemia. Results: The sample included 13 children with hemoglobinopathies (median age: 8 years, range 5-18) and 268 children in the comparison group (median age: 11 years, range 5-18). There were similar rates of early infection, chronic graft versus host disease, and all-cause mortality between the two groups. There was no significant difference in recovery to baseline scores for physical, emotional, and role functioning by 3 months for either group. Children with hemoglobinopathies had higher HRQL scores for physical (beta=12, se=5.5, P=0.01) and baseline emotional functioning (beta=11.6, se=5.5, P=0.03) than the comparison group. For all domains for both groups, parent reports demonstrated a nadir at 45 days with recovery to baseline by 3 months following transplant. Children's ratings were higher than those of their parents in both diagnostic groups. Conclusions: Children with hemoglobinopathies had higher physical and emotional functioning scores prior to HSCT and experienced a similar pattern of recovery to their baseline functioning by 3 months post-HSCT when compared to children receiving HSCT for acquired conditions. Pediatr Blood Cancer 2012;59:725-731. © 2011 Wiley Periodicals, Inc.
Park J.,Tufts University |
Wick H.C.,Tufts University |
Kee D.E.,Tufts University |
Noto K.,Tufts University |
And 2 more authors.
PLoS Computational Biology | Year: 2014
Identifying molecular connections between developmental processes and disease can lead to new hypotheses about health risks at all stages of life. Here we introduce a new approach to identifying significant connections between gene sets and disease genes, and apply it to several gene sets related to human development. To overcome the limits of incomplete and imperfect information linking genes to disease, we pool genes within disease subtrees in the MeSH taxonomy, and we demonstrate that such pooling improves the power and accuracy of our approach. Significance is assessed through permutation. We created a web-based visualization tool to facilitate multi-scale exploration of this large collection of significant connections (http://gda.cs.tufts.edu/development). High-level analysis of the results reveals expected connections between tissue-specific developmental processes and diseases linked to those tissues, and widespread connections to developmental disorders and cancers. Yet interesting new hypotheses may be derived from examining the unexpected connections. We highlight and discuss the implications of three such connections, linking dementia with bone development, polycystic ovary syndrome with cardiovascular development, and retinopathy of prematurity with lung development. Our results provide additional evidence that plays a key role in the early pathogenesis of polycystic ovary syndrome. Our evidence also suggests that the VEGF pathway and downstream NFKB signaling may explain the complex relationship between bronchopulmonary dysplasia and retinopathy of prematurity, and may form a bridge between two currently-competing hypotheses about the molecular origins of bronchopulmonary dysplasia. Further data exploration and similar queries about other gene sets may generate a variety of new information about the molecular relationships between additional diseases. © 2014 Park et al.
Hui L.,Mother Infant Research Institute |
Hui L.,The Floating Hospital for Children at Tufts Medical Center |
Hui L.,University of Sydney |
Bianchi D.W.,Mother Infant Research Institute |
Bianchi D.W.,The Floating Hospital for Children at Tufts Medical Center
Prenatal Diagnosis | Year: 2011
Fetal therapy can be defined as any prenatal treatment administered to the mother with the primary indication to improve perinatal or long-term outcomes for the fetus or newborn. This review provides an update of the pharmacological therapies that are solely directed at the fetus with anomalies and outlines a future transcriptomic approach. Fetal anomalies targeted with prenatal pharmacotherapy are a heterogeneous group of structural, endocrine, and metabolic conditions, including congenital cystic adenomatoid malformation (CCAM), congenital adrenal hyperplasia, congenital heart block, fetal tachyarrhythmias, inborn errors of metabolism, fetal thyroid disorders, and polyhydramnios. To date, the majority of pharmacotherapies for fetal anomalies have been evaluated only in retrospective, uncontrolled studies. The way forward will be with an evidence-based approach to prenatal pharmacological interventions. © 2011 John Wiley & Sons, Ltd.