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A novel murine dispersed homeobox gene, designated Gsh-2, is described. Analysis of cDNA sequence, including the full open reading frame, reveals an encoded homeodomain that is surprisingly similar to those of the Antennapedia-type clustered Hox genes. In addition, the encoded protein includes polyhistidine and polyalanine tracts, as observed for several(More)
Duchenne muscular dystrophy (DMD) is a lethal X-linked disorder associated with dystrophin deficiency that results in chronic inflammation and severe skeletal muscle degeneration. In DMD mouse models and patients, we find that IkappaB kinase/NF-kappaB (IKK/NF-kappaB) signaling is persistently elevated in immune cells and regenerative muscle fibers. Ablation(More)
OBJECTIVE The management of parapneumonic effusions in children is controversial. The objective of this study was to evaluate the effectiveness and safety of intrapleural tissue plasminogen activator (tPA) in children who require tube thoracostomy for drainage of a complicated parapneumonic effusion. METHODS An observational cohort study was used to(More)
The Notch pathway plays multiple roles during vertebrate somitogenesis, functioning in the segmentation clock and during rostral/caudal (R/C) somite patterning. Lunatic fringe (Lfng) encodes a glycosyltransferase that modulates Notch signaling, and its expression patterns suggest roles in both of these processes. To dissect the roles played by Lfng during(More)
We describe the second case of congenital disorder of glycosylation type IL (CDG-IL) caused by deficiency of the ALG9 a1,2 mannosyltransferase enzyme. The female infant's features included psychomotor retardation, seizures, hypotonia, diffuse brain atrophy with delayed myelination, failure to thrive, pericardial effusion, cystic renal disease,(More)
BACKGROUND Patients with community-acquired or nosocomial infections are often managed in a hospital with isolation precautions. Given the high prevalence and substantial inconvenience associated with implementation of isolation precautions in pediatric settings, we explored the impact of this intervention on the care provided to children and their(More)
OBJECTIVE To evaluate the efficacy of a program designed to improve adherence to antiretroviral therapy among patients with poor adherence. METHODS A randomized intervention trial was conducted among 90 HIV-positive patients experiencing treatment failure as a result of noncompliance with their medication regimen. Eligible participants were randomly(More)
Cytogenetic studies of bone marrow fibroblasts and blood cells from peripheral blood or bone marrow were performed in 19 patients with myelofibrosis with myeloid metaplasia (group 1), nine patients with other myeloproliferative syndromes without myelofibrosis (group 2), and 12 patients with anaemia secondary to iron deficiency or chronic inflammatory(More)
OBJECTIVE To describe the long-term outcomes of pediatric pleural empyema. DESIGN Prospective observational study from October 2008 to October 2011. SETTING Tertiary care children's hospital. PARTICIPANTS Children with pleural empyema (loculations and/or septations identified on radiologic imaging or frank pus on thoracentesis). MAIN OUTCOME(More)
Members of the fibroblast growth factor (FGF) family of molecules are critical to limb outgrowth. Here, we examine the expression of Fgfs in three types of limbs-embryonic (developing), mature (differentiated), and regenerating-as well as in the surrounding non-limb tissues in the Mexican axolotl, Ambystoma mexicanum. We have previously cloned partial cDNAs(More)