ABSTRACT
PURPOSE:: The neonatologists of Sant'Anna and San Sebastiano Hospital of Caserta have carried out a pilot study investigating the safety, feasibility, and accuracy of intracavitary electrocardiography for neonatal epicutaneous cava catheter tip positioning. PATIENTS AND METHODS:: We enrolled 39 neonates (1-28 days of postnatal age or correct age lower than 41 weeks) requiring epicutaneous cava catheter in the district of superior vena cava (head-neck or upper limbs). Intracavitary electrocardiography was applicable in 38 neonates. RESULTS:: No significant complications related to intracavitary electrocardiography occurred in the studied neonates. The increase in P wave on intracavitary electrocardiography was detected in 30 cases. Of the remaining eight cases, six malpositioned catheters tipped out of cavoatrial junction-target zone (chest x-ray and echocardiographical control) and two were false negative (tip located in target zone). The match between intracavitary electrocardiography and x-ray was observed in 29/38 cases, and the same ratio between intracavitary electrocardiography and echocardiography was detected. CONCLUSION:: We conclude that the intracavitary electrocardiography method is safe and accurate in neonates as demonstrated in pediatric and adult patients. The applicability of the method is 97% and its feasibility is 79%. The overall accuracy is 76% but it rises to 97% if "peak" P wave is detected.
Subject(s)
Catheterization, Central Venous/methods , Catheterization, Peripheral/methods , Electrocardiography , Vena Cava, Superior , Catheterization, Central Venous/adverse effects , Catheterization, Central Venous/instrumentation , Catheterization, Peripheral/adverse effects , Catheterization, Peripheral/instrumentation , Catheters, Indwelling , Central Venous Catheters , Echocardiography , Feasibility Studies , Female , Humans , Infant, Newborn , Italy , Male , Pilot Projects , Predictive Value of Tests , Prospective Studies , Radiography, ThoracicABSTRACT
The overgrowth disorder Beckwith-Wiedemann syndrome (BWS) is associated with dysregulation of imprinted genes at chromosome 11p15.5. The molecular defects are heterogeneous but most of the cases are associated with defective DNA methylation at either one of two Imprinting Control Regions (IC1 and IC2) or Uniparental paternal Disomy (UPD) at 11p15.5. In rare cases, the BWS phenotype has been found associated with maternal transmission of IC1 microdeletions. We describe a family with a novel 1.8 kb deletion that is associated with hypermethylation at IC1. The mutation results from recombination between highly homologous sequences containing target sites for the zinc-finger protein CTCF (CTSs). This finding supports the hypothesis that the function of IC1 and the penetrance of the clinical phenotype depend on the spacing of the CTSs resulting from recombination in the mutant allele.
