A comparison of the stressors experienced by parents of intubated and non-intubated children

When children are ill enough to require admission to paediatric intensive care, parents may become distressed about their child's medical condition and this distress may be compounded by the unfamiliar nature of the highly technological environment Parents of children who are sick enough to warrant intubation are particularly likely to be exposed to a frightening array of technological equipment Seventy-one parents of intubated and non-intubated children completed the Parental Stressor Scale Paediatnc Intensive Care Unit (PSS PICU) Overall the findings suggest that parents were most distressed (a) by the painful procedures to which their children were subjected, (b) by the sights and sounds of the intensive care unit and (c) by their children's reactions to intensive care The behaviour of staff towards parents and the way that staff communicated with them caused the least distress When the levels of stress reported by parents of intubated children were compared with those reported by parents of non-intubated children, different patterns of stress were found Painful procedures were a source of greater stress to parents of intubated children whereas the behaviour of staff and the children's reactions to the intensive care experience caused greater stress to the parents of the non-intubated children In general the findings suggest that the needs of parents of non-intubated children are being overlooked, with staff focusing more of their attention on the parents of intubated children     

Mapping of the gene for X-linked dominant Charcot-Marie-Tooth neuropathy.

We performed a clinical study and linkage analysis on 278 subjects (66 affected) belonging to eight families with X-linked dominant Charcot-Marie-Tooth (CMT) neuropathy. This form affects 11.8% of CMT patients in Iowa. Motor nerve conduction velocities (MNCVs) were significantly slowed consistent with type 1 CMT. Fifty-six obligate carriers manifested mild distal weakness, localized areflexia, pes cavus, and slowing on MNCVs. Seven X-linked restriction fragment length polymorphisms mapping in the Xp11-q21 region were tested for linkage against CMT. Two-point linkage results showed the highest lod scores with PGK1, DXS159, and DXYS1. Multipoint linkage analysis excluded the CMT gene from being telomeric to either DXS14 or DXYS1, with over 1,000:1 odds. The highest location scores were at PGK1 and 1 cM proximal to DXS159.     

Multipoint linkage analysis of spinocerebellar ataxia and markers on chromosome 6

Heterogeneity among the spinocerebellar ataxias (SCA) has been shown on clinical, biochemical, and genetic criteria. Among the autosomal dominant SCAs, several kindreds have shown loose linkage to the HLA loci on chromosome 6, while linkage in other kindreds has been rejected. The advent of multipoint linkage analysis allows the use of several marker loci simultaneously, thus increasing the amount of usable information. We have reanalyzed linkage data from a large kindred with SCA and provide evidence for a telomeric location of the SCA gene in this family. Knowledge of the relative gene location will ease the identification of the SCA gene by reducing the size of the chromosomal regions that must be examined.     

Congenital supratentorial and infratentorial peripheral neurogenic tumor: a clinical, ultrastructural, and immunohistochemical study

Congenital neoplasms of the central nervous system are extremely rare, although they have been well documented since the earliest reports from the mid-19th century. Medulloblastoma, other primitive neuroectodermal tumors, and various types of gliomas have comprised the majority of cases. This report describes a highly unusual infratentorial and supratentorial tumor presenting as a scalp mass in a neonate who experienced in utero distress. The supratentorial mass extended through a defect in the skull to the parietooccipital lobe, and the infratentorial aspect involved the 9th and 10th cranial nerves in the region of the cerebellopontine angle. A complex spindle cell neoplasm incorporating peripheral nerve sheath and vascular characteristics was further characterized by electron microscopy and immunohistochemistry. Based upon these studies, the tumor was interpreted as a congenital schwannoma with divergent vascular differentiation. The child has been tumor-free for approximately 2 years after the initial operative procedure.     

Promoting clinically effective practice: general practitioners' awareness of sources of research evidence

BACKGROUND: Practitioners are being encouraged to base their clinical practice on research evidence. In order to do this, they must be aware of and use the sources of evidence. METHODS: A questionnaire survey was undertaken to establish GPs' awareness of research evidence in their clinical practice and, in fundholding practices, its influence on purchasing plans. Questionnaires were sent to 360 lead fundholders in North Thames Region and 440 of a random sample of the remaining general practitioners in the region for comparison. RESULTS: Questionnaires were returned by 62% of lead fundholders and 63% of GPs in the random sample. There was limited use of the electronic sources of clinical effectiveness. There was greater reported awareness of published sources of research evidence and fundholding GPs were significantly more likely to have referred to publications summarizing research evidence. CONCLUSIONS: GPs seem to make more use of published clinical effectiveness sources than the electronic databases. Consequently, they need educational and technical support if they are to make full use of the available sources of research evidence available in other media.      

Electrocardiographic abnormalities after radiofrequency catheter ablation of accessory bypass tracts in the Wolff-Parkinson-White syndrome.

Repolarization abnormalities on surface electrocardiograms have been described after loss of ventricular preexcitation in some patients with the Wolff-Parkinson-White syndrome. Radiofrequency catheter ablation of overt accessory pathways provides a unique opportunity to study this phenomenon. In this study, serial electrocardiograms were obtained before and after radiofrequency ablation of manifest accessory pathways in 19 patients, of concealed accessory pathways in 6 and after radiofrequency atrioventricular nodal modification in 12. Seven patients undergoing manifest right-sided accessory pathway ablation had left superior frontal plane T-wave axis deviations after ablation (-42 +/- 13 degrees). No patient with a manifest left-sided or concealed accessory pathway, or atrioventricular nodal modification had T-wave abnormalities after ablation; however, left anterior fascicular block and incomplete right bundle branch block each occurred in 1 patient with left accessory pathway ablation. Repolarization abnormalities observed after ablation were similar to T-wave abnormalities during the absence of preexcitation before ablation and persisted up to 5 weeks after the procedure. Patients with repolarization abnormalities after ablation had significantly longer preexcited QRS durations than those without such changes, suggesting that the initial contribution of the pathway to ventricular activation is an important determinant of T-wave changes after ablation. The proposed mechanism for repolarization abnormalities after ablation is the phenomenon of T-wave "memory."     

Current and future modalities of catheter ablation for the treatment of cardiac arrhythmias

Catheter ablation has proven to be a safe and effective treatment for a wide variety of cardiac arrhythmias. By destroying the critical zone of conductive tissue responsible for impulse generation or propagation, the arrhythmias may be cured. A variety of modalities of catheter ablation have been tested in the past decade. Initially, high energy direct current shocks delivered through a conventional electrode catheter were used. Now, use of radiofrequency energy as a power supply has resulted in higher efficacy and much improved safety of this technique. New approaches including low energy direct current shock ablation, microwave hyperthermic ablation, and laser photocoagulation are being tested, and may result in further refinement of nonsurgical curative therapy of arrhythmias.