The effects of early hydrocephalus and related brain anomalies on cognitive skills are not well understood. In this study, magnetic resonance scans were obtained from 99 children aged from 6 to 13 years with either shunted hydrocephalus (n = 42) or arrested (unshunted) hydrocephalus (n = 19), from patient controls with no hydrocephalus (n = 23), and from normal, nonpatient controls (n = 15). Lateral ventricle volumes and area measurements of the internal capsules and centra semiovale in both hemispheres were obtained from these scans, along with area measurements of the corpus callosum. Results revealed reductions in the size of the corpus callosum in the shunted hydrocephalus group. In addition, lateral ventricle volumes were larger and internal capsule areas were smaller in both hemispheres in children with shunted and arrested hydrocephalus. The centra semiovale measurements did not differentiate the groups. Correlating these measurements with concurrent assessments of verbal and nonverbal cognitive skills, motor abilities, and executive functions revealed robust relationships only between the area of the corpus callosum and nonverbal cognitive skills and motor abilities. These results support the theory of a prominent role for the corpus callosum defects characteristic of many children with shunted hydrocephalus in the spatial cognition deficits commonly observed in these children.

Regional variations in brain tissue composition in children with shunted hydrocephalus correlate with a variety of cognitive and visuomotor functions.

Abstract-The N629D mutation, adjacent to the GFG signature sequence of the HERG1 A K ϩ channel, causes long-QT syndrome (LQTS). Expression of N629D in Xenopus oocytes produces a rapidly activating, noninactivating current. N629D is nonselective among monovalent cations; permeation of K ϩ was similar to that of Na ϩ or Cs ϩ . During repolarization to potentials between Ϫ30 and Ϫ70 mV, N629D manifested an inward tail current, which was abolished by replacement of extracellular Na ϩ (Na ϩ e ) with extracellular N-methyl-D-glucamine (NMG e ). Because LQTS occurs in heterozygous patients, we coexpressed N629D and wild type (WT) at equimolar concentrations. Heteromultimer formation was demonstrated by analyzing the response to 0 [K ϩ ] e . The outward time-dependent current was nearly eliminated for WT at 0 [K ϩ ] e , whereas no reduction was observed for homomultimeric N629D or for the equimolar coexpressed current. To assess physiological significance, dofetilide-sensitive currents were recorded during application of simulated action potential clamps. During phase 3 repolarization, WT manifested outward currents, whereas homomultimeric N629D manifested inward depolarizing currents. During coexpression studies, variable phenotypes were observed ranging from a reduction in outward repolarizing current to net inward depolarizing current during phase 3. In summary, N629D replaces the WT outward repolarizing tail current with an inward depolarizing sodium current, which is expected to delay later stages of repolarization and contribute to arrhythmogenesis. Thus, the consequences of N629D resemble the pathophysiology seen in LQT3 Na ϩ channel mutations and may be considered the first LQTS K Key Words: long-QT syndrome Ⅲ HERG1 Ⅲ K ϩ channel Ⅲ gain of function F amilial long-QT syndrome (LQTS) results from defects in sodium and potassium ion channels that cause prolongation of cardiac repolarization and arrhythmias. 1 LQT2 is associated with mutations of the human ether-a-go-go-related gene, HERG1. [2][3][4][5][6][7][8][9][10] The HERG1 primary transcript is alternatively processed, giving rise to at least 3 functional mRNAs, HERG1 A, HERG1 AЈ, and HERG1 B, encoding proteins with distinct physiological properties. 11,12 There are several mechanisms by which individual mutations in HERG1 produce LQTS. 5-9 Some exert a dominant phenotype through loss of repolarizing current. These include mutations that either cause defects in intracellular transport or result in channels that do not open. Alternatively, V630L forms heterotetramers with wild type (WT) that have reduced open probability due to a negative shift in voltage dependence of inactivation. Abnormally fast deactivation mutations caused by mutations in the N terminus of HERG1 A result in reduction of outward current during phase 3 repolarization and thus LQTS. 10 The recently described N629D mutation 9 is of particular interest, as it alters the pore selectivity signature sequence from GFGN to GFGD. 13 In most K ϩ channels, including the extensively studied Shaker chann...

Research on OI has focused very little on pain experience in children and adolescents, and there is no standard method of assessing pain. To better describe the pain experience of these patients, future research should focus on better characterizing OI pain with the use of age-appropriate valid, reliable, and multidimensional pain assessment tools.

Aims: This integrative review aimed to identify, analyse and synthesise studies investigating the clinical efficacy of virtual reality (VR) distraction for children undergoing varying painful and anxiety-inducing medical procedures across different hospital settings and to identify implications for research and clinical practice.Background: Virtual reality has been leveraged as a distraction tool in the healthcare setting to help patients manage procedural pain and anxiety. Initial studies in the burn wound care setting using VR as a non-pharmacological analgesia led to the use of VR during other medical procedures.Design: An integrative review of the literature was conducted following the PRISMA guidelines across four electronic databases. Published studies between 2000 and 2020 investigating the clinical efficacy of VR in managing paediatric procedural pain or anxiety were included for review.Results: Reviewed studies collectively included 2,174 patients aged 6 months-18 years used VR during burn wound care, post-burn physiotherapy, dental, needle-related and other procedures. Additionally, ten studies included samples with adults, for which paediatric data could not be isolated (n = 507). Overall, studies supported the efficacy of VR in managing procedural pain and anxiety in the paediatric setting. Conclusion:Virtual reality is redefining pain management by immersing children in a virtual world, reducing pain and anxiety at the hospital. A notable gap was the neglected use of VR in children with chronic conditions receiving orthopaedic procedures as part of their standard care.Relevance to Clinical Practice: Ultimately, VR distraction will reduce the fear associated with medical interventions, preventing increased pain sensitivity, exacerbated anxiety and healthcare avoidance in adulthood. Nurses will play an important role in ensuring the smooth integration of VR in clinical practice by championing the technology and transferring evidence-based methods for VR use.