Eosinophilic esophagitis (EoE) and type 2B von Willebrand disease (vWD) are both rare diseases, and the co-occurrence is unlikely. Patients with EoE often need recurrent endoscopic dilations and esophageal biopsies, and the safety of these procedures in the setting of bleeding disorders is not well described in the literature. We describe successful management strategies in a patient with co-existing EoE and type 2B vWD who required multiple dilations and biopsies.
View Article and Find Full Text PDFObjective: Previous studies estimated the incidence of catheter-associated venous thrombosis to be between 2% and 81%. Our goals were to define the incidence rate of catheter-related thrombosis in a hospitalized, noncritically ill, pediatric population and to determine modifiable factors that alter the risk of thrombosis.
Methods: A retrospective cohort study was performed at the North Carolina Children's Hospital from 2009 to 2012.
Central venous catheters and peripherally inserted central catheters are widely used in children with serious chronic diseases. In this report, data about catheters and venous thromboembolic disease (VTE) in children will be reviewed, and the experience of a single academic children's hospital will be described. Two separate data sets that examine overlapping subpopulations will be reported: (1) the proportion of pediatric patients with catheters who develop VTE and (2) the proportion of patients referred to pediatric hematology for VTE who have catheters.
View Article and Find Full Text PDFBackground: The pulmonary phenotype in cystic fibrosis (CF) is variable; thus, environmental and genetic factors likely contribute to clinical heterogeneity. We hypothesized that genetically determined ABO histo-blood group antigen (ABH) differences in glycosylation may lead to differences in microbial binding by airway mucus, and thus predispose to early lung infection and more severe lung disease in a subset of patients with CF.
Methods And Principal Findings: Clinical information and DNA was collected on >800 patients with the DeltaF508/DeltaF508 genotype.
Malignancies of the head and neck are uncommon among children. The most common solid tumors to occur in this region are rhabdomyosarcoma, Hodgkin disease, and non-Hodgkin lymphoma. Two children are described who presented with signs and symptoms consistent with rhabdomyosarcoma, but who were found to have benign spindle cell lesions.
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