Hepatic mesenchymal hamartoma and infantile hemangioma: a rare association.

Background/purpose: The association between liver infantile hemangioma and mesenchymal hamartoma is rare. This communication reports the clinical, radiologic, and pathologic features of 3 infants with concurrent liver hemangioma and mesenchymal hamartoma.

Methods: We conducted a comprehensive search of the database of our Vascular Anomalies Center over the past 12 years (1999-2010) for the keywords mesenchymal hamartoma, liver, and hemangioma in a large cohort of patients with liver hemangioma.

Pleiotropic effects of miR-183~96~182 converge to regulate cell survival, proliferation and migration in medulloblastoma.

Medulloblastomas are the most common malignant brain tumors in children. Several large-scale genomic studies have detailed their heterogeneity, defining multiple subtypes with unique molecular profiles and clinical behavior. Increased expression of the miR-183~96~182 cluster of microRNAs has been noted in several subgroups, including the most clinically aggressive subgroup associated with genetic amplification of MYC.

iCanPlot: visual exploration of high-throughput omics data using interactive Canvas plotting.

Increasing use of high throughput genomic scale assays requires effective visualization and analysis techniques to facilitate data interpretation. Moreover, existing tools often require programming skills, which discourages bench scientists from examining their own data. We have created iCanPlot, a compelling platform for visual data exploration based on the latest technologies.

Four-year longitudinal performance of a population-based sample of healthy children on a neuropsychological battery: the NIH MRI study of normal brain development.

The National Institutes of Health (NIH) Magnetic Resonance Imaging (MRI) Study of Normal Brain Development is a landmark study in which structural and metabolic brain development and behavior are followed longitudinally from birth to young adulthood in a population-based sample of healthy children. Cross-sectional findings from the neuropsychological test battery have been previously described (Waber et al., 2007).

Serotype-independent pneumococcal experimental vaccines that induce cellular as well as humoral immunity.

For prevention of Streptococcus pneumoniae (pneumococcus) infections in infancy, protein-conjugated capsular polysaccharide vaccines provide serotype-specific, antibody-mediated immunity but do not cover all of the 90+ capsule serotypes. Therefore, microbiologists have sought protective noncapsular antigens common to all strains. Alternatively, we investigated killed cells of a noncapsulated strain, which expose many such common antigens.

NIDCAP improves brain function and structure in preterm infants with severe intrauterine growth restriction.

Objective: The effect of NIDCAP (Newborn Individualized Developmental Care and Assessment Program) was examined on the neurobehavioral, electrophysiological and neurostructural development of preterm infants with severe intrauterine growth restriction (IUGR).

Study Design: A total of 30 infants, 27-33 weeks gestation, were randomized to control (C; N=17) or NIDCAP/experimental (E; N=13) care. Baseline health and demographics were assessed at intake; electroencephalography (EEG) and magnetic resonance imaging (MRI) at 35 and 42 weeks postmenstrual age; and health, growth and neurobehavior at 42 weeks and 9 months corrected age (9 months).

A system for coreference resolution for the clinical narrative.

Objective: To research computational methods for coreference resolution in the clinical narrative and build a system implementing the best methods.

Methods: The Ontology Development and Information Extraction corpus annotated for coreference relations consists of 7214 coreferential markables, forming 5992 pairs and 1304 chains. We trained classifiers with semantic, syntactic, and surface features pruned by feature selection.

Fourth-dimensional changes in nasolabial dimensions following rotation-advancement repair of unilateral cleft lip.

Background: Repair of unilateral cleft lip requires three-dimensional craftsmanship and understanding four-dimensional changes.

Methods: Ninety-nine children with unilateral complete or incomplete cleft lip were measured by direct anthropometry following rotation-advancement repair (intraoperatively) and again in childhood. Changes in heminasal width, labial height, and labial width were analyzed and compared measures depending on whether the cleft was incomplete/complete or involved left/right side.

Current pharmacologic approaches for prevention and treatment of bronchopulmonary dysplasia.

Bronchopulmonary dysplasia (BPD) is a major complication of preterm birth and has serious adverse long-term health consequences. The etiology of BPD is complex, multifactorial, and incompletely understood. Contributing factors include ventilator-induced lung injury, exposure to toxic oxygen levels, and infection.

Multiscale information for network characterization in epilepsy.

We have developed a multiscale approach for the estimation of neuronal network coordination in the epileptic brain, from continuous (long-term) non-invasive electroencephalograms (EEG). The proposed approach specifically assesses the effect of large-scale network behavior on local network coordination, at individual dominant frequencies (modes) of the EEG spectrum. For this purpose a set of conditional information parameters is proposed to explicitly quantify the effect of global network correlation in the brain on pairwise (local) mutual information, via conditioning.

Estimation of correlations between copy-number variants in non-coding DNA.

Allelic DNA aberrations across our genome have been associated with normal human genetic heterogeneity as well as with a number of diseases and disorders. When copy-number variations (CNVs) occur in gene-coding regions, known relationships between genes may help us understand correlations between CNVs. However, a large number of these aberrations occur in non-coding, extragenic regions and their correlations may be characterized only quantitatively, e.

Novel zebrafish model reveals a critical role for MAPK in lymphangiogenesis.

Purpose: Lymphatic disorders are poorly understood with few animal models. We designed a novel assay to measure lymphatic development using transgenic zebrafish with fluorescently labeled endothelial cells. Two major branches of the vascular endothelial growth factor receptor (VEGFR) signaling pathway were examined: the MAPK and PI3K pathways.

Outcomes in pediatric atypical spitz tumors treated without sentinel lymph node biopsy.

The diagnosis of atypical Spitz tumor (AST) in a pediatric patient conveys an uncertain potential for malignancy. Although pediatric melanoma is rare, AST may be treated aggressively with sentinel lymph node biopsy (SLNB) and subsequent completion lymphadenectomy. These procedures have unclear therapeutic benefit and potential morbidity.

The clinical presentation and surgical management of adnexal torsion in the pediatric and adolescent population.

Study Objective: To determine the history, clinical presentation, physical exam, and laboratory findings of ovarian and/or tubal torsion in the pediatric and adolescent population and to examine the surgical management of adnexal torsion.

Design: Descriptive, retrospective chart review.

Setting: Academic children's hospital.

Paxillin mediates sensing of physical cues and regulates directional cell motility by controlling lamellipodia positioning.

Physical interactions between cells and the extracellular matrix (ECM) guide directional migration by spatially controlling where cells form focal adhesions (FAs), which in turn regulate the extension of motile processes. Here we show that physical control of directional migration requires the FA scaffold protein paxillin. Using single-cell sized ECM islands to constrain cell shape, we found that fibroblasts cultured on square islands preferentially activated Rac and extended lamellipodia from corner, rather than side regions after 30 min stimulation with PDGF, but that cells lacking paxillin failed to restrict Rac activity to corners and formed small lamellipodia along their entire peripheries.

Developmental regulation of group I metabotropic glutamate receptors in the premature brain and their protective role in a rodent model of periventricular leukomalacia.

Cerebral white matter injury in premature infants, known as periventricular leukomalacia (PVL), is common after hypoxia-ischemia (HI). While ionotropic glutamate receptors (iGluRs) can mediate immature white matter injury, we have previously shown that excitotoxic injury to premyelinating oligodendrocytes (preOLs) in vitro can be attenuated by group I metabotropic glutamate receptor (mGluR) agonists. Thus, we evaluated mGluR expression in developing white matter in rat and human brain, and tested the protective efficacy of a central nervous system (CNS)-penetrating mGluR agonist on injury to developing oligodendrocytes (OLs) in vivo.

Rejection and regulation: a tight balance.

Purpose Of Review: Achieving allograft tolerance is the holy grail of transplantation. However, tolerance and rejection are two extreme ends of a scale that can be tipped in either direction. We review the novel effector and regulatory mechanisms involved and factors that tip the balance in favor of rejection or regulation.

Loss of white matter microstructural integrity is associated with adverse neurological outcome in tuberous sclerosis complex.

Rationale And Objectives: Tuberous sclerosis complex (TSC) is a genetic neurocutaneous syndrome in which cognitive and social-behavioral outcomes for patients vary widely in an unpredictable manner. The cause of adverse neurologic outcome remains unclear. The aim of this study was to investigate the hypothesis that disordered white matter and abnormal neural connectivity are associated with adverse neurologic outcomes.

Relationships among the concentrations of 25 inflammation-associated proteins during the first postnatal weeks in the blood of infants born before the 28th week of gestation.

Background: Inflammation appears to be involved in processes leading to organ damage in preterm newborns, yet little is known about the relationships among elevated concentrations of inflammation-associated proteins in the blood of preterm newborns.

Methods: In this exploratory study, we used an electrochemiluminescence method to measure 25 proteins in blood obtained on postnatal day 1 (range 1-3), day 7 (range 5-8), and day 14 (range 12-15) from 939 children born before the 28th week of gestation and evaluated to what extent those whose concentration of each protein was elevated (defined as in the highest quartile for gestational age and day the specimen was obtained) also had an elevated concentration of every other protein the same day or on a day 1 or 2 weeks later (p<.0001).