Maternal responses to child frustration and requests for help in dyads with fragile X syndrome.

BACKGROUND: Variability in behaviour displayed by children with fragile X syndrome (FXS) may be partially attributable to environmental factors such as maternal responsivity. The purpose of this study was to explore variables associated with maternal behaviour during a task designed to elicit frustration in their children with FXS. METHODS: Forty-six mother-child dyads, in which the child had full-mutation FXS, were observed in their homes during a task designed to elicit frustration in the child. Each child was given a wrong set of keys and asked to open a box to retrieve a desired toy. Mothers were provided with the correct set of keys and instructed to intervene when they perceived their child was getting too frustrated. Child-expressed frustration and requests for help and maternal behaviours (comforting, negative control, and encouraging/directing) were observed and coded. Maternal variables (e.g. depression, stress, education levels), child variables (e.g. autistic behaviours, age, medication use) and child behaviours (frustration, requests for help) were explored as predictors of maternal behaviour. RESULTS: Almost all mothers intervened to help their children and most used encouraging/directing behaviours, whereas very few used comforting or negative control. Child age and child behaviours during the frustrating event were significant predictors of encouraging/directing behaviours in the mothers. Children whose mothers reported higher depressive symptomology used fewer requests for help, and mothers of children with more autistic behaviours used more negative control. CONCLUSIONS: The results of this study suggest that child age and immediate behaviours are more strongly related to maternal responsivity than maternal traits such as depression and stress.

Prospective audit of emergency department transit times associated with entonox analgesia for reduction of the acute, traumatic dislocated shoulder.

AIM: To establish current UK practice for the management of the acute traumatic shoulder dislocation with respect to analgesia and reduction manoeuvres. To compare the transit times of patients through an emergency department (ED) after the use of intravenous analgesia and/ or sedation compared to entonox +/- simple oral analgesia. METHODS: A postal questionnaire was sent to 100 UK ED consultants to establish current practice. The treating clinicians were allowed to choose the method of analgesia provided to reduce the patient's dislocated shoulder, provided the patient was happy with it. They administered either (1) traditional intravenous morphine and/or midazolam or (2) entonox +/- simple oral analgesia to facilitate reduction. A prospective audit was conducted to compare the transit times of the two groups of patients. RESULTS: The postal questionnaire revealed that intravenous morphine and midazolam are widely used during reduction of the acute shoulder dislocation in the UK. The audit showed that this was associated with a significantly prolonged transit time through the ED, compared to entonox alone, (mean 77 min versus 177 min, respectively, p<0.001) without compromise in reduction success. CONCLUSION: Entonox +/- simple oral analgesia significantly decreases ED transit times as compared to IV morphine and/or midazolam for the reduction of the acute traumatic dislocated shoulder. Further studies should be done into patient pain scores and into the best combination of oral analgesia and entonox.

The impact of implementing the Ottawa ankle rules on ankle radiography requests in A&E.

This audit was set up to quantify the effect of implementing the Ottawa ankle rules in a district general hospital that relies on both medical and nursing radiography requests. Data were collected prospectively on 207 patients who presented with an acute ankle injury between August 2001 and February 2002. The department's activity was recorded before and after a period of teaching on the Ottawa ankle rules. Before teaching, 71% of patients with an acute ankle injury were sent for radiography; teaching reduced this figure to 56% (p < 0.05). Auditing the activity of our department enabled us to observe a significant decrease in the number of patients sent for ankle radiography following acute ankle injury. This correlates well with research in other settings. The difficulties of rationalising radiology investigations are discussed.

Induction of human tumor-loaded dendritic cells.

A preferred anti-cancer vaccine would be tumor-specific, simple to rapidly construct and safe to administer. It would permit immunization against a spectrum of the tumor's distinctive antigens, without requiring their prior identification. Toward these goals, we describe a modification of standard extracorporeal photopheresis (ECP) which initiates, within a single day, both monocyte-to-dendritic cell (DC) differentiation and malignant cell apoptosis. The transition of monocytes to immature DCs was identified by the expression of cytoplasmic CD83 and membrane CD36 in the absence of membrane CD14 staining, as well as induction of membrane CD83 expression. Differentiating DCs were avidly phagocytic and engulfed apoptotic malignant T cells. Differentiating DCs were capable of stimulating significant proliferation of normal alloreactive lymphocyte responders, indicting increased expression of membrane MHC class II molecules. This approach provides a clinically practical means of developing tumor-loaded cells that have initiated the transition to DCs without the requirement of exogenous cytokines, excessive cellular manipulation or isolation. Construction of DC vaccines using this methodology can be generalized to other diseases and may offer a novel approach for improved cancer immunotherapy.

The reorganization of sensorimotor function in children after hemispherectomy. A functional MRI and somatosensory evoked potential study.

Children who have suffered extensive unilateral brain injury early in life may show a remarkable degree of residual sensorimotor function. It is generally believed that this reflects the high capacity of the immature brain for cerebral reorganization. In this study, we investigated 17 patients who had undergone hemispherectomy for relief from seizures; eight of the patients had congenital brain damage and nine had sustained their initial insult at the age of 1 year or older. Sensorimotor functions of the hand were investigated using functional MRI (fMRI) during a passive movement task, somatosensory evoked potentials (SEPs) arising from electrical and vibration stimulation, and behavioural tests including grip strength, double simultaneous stimulation and joint position sense. On fMRI, two of the eight patients studied with this technique (one with congenital damage and one with damage acquired at the age of 3 years) showed activation in the sensorimotor cortex of the remaining hemisphere with passive movement of the hemiplegic hand. The location of the ipsilateral brain activation was similar to that found on movement of the normal contralateral hand, although the latter was greater in spatial extent. In one of these patients, a greater role was demonstrated for the ipsilateral secondary sensorimotor area (compared with the ipsilateral primary sensorimotor area) for movement of the hemiplegic hand than for movement of the normal hand. Median nerve stimulation of the hemiplegic hand showed reproducible early-latency ipsilateral SEP components in the remaining sensorimotor cortex in 10 of the 17 patients (five with congenital and five with acquired disease). Five of the patients who demonstrated ipsilateral electrical SEPs also showed ipsilateral vibration SEPs (two with congenital and three with acquired disease). The behavioural tests revealed residual sensorimotor function in 14 of the patients; however, not all of the patients who exhibited ipsilateral SEP or fMRI responses had residual sensorimotor function in the hemiplegic hand. Ipsilateral sensorimotor responses were demonstrated both in patients with congenital disease and those with acquired disease, suggesting that factors additional to aetiology and age at injury may influence the degree of residual sensorimotor function and cerebral reorganization.

Spontaneous pneumothorax: is it under tension?

A diagnosis of tension pneumothorax is usually only considered within the context of trauma, incorrect chest drain insertion or positive pressure ventilation. Four patients are presented who developed spontaneous tension pneumothorax with no precipitating factors. In three of these instances, the diagnosis was only made radiologically and in every case the treating physician was unaware that a spontaneous tension pneumothorax could occur. Previously, emphasis has been placed on tracheal deviation in a tension pneumothorax. However, this is an inconsistent finding as one of the cases highlights. Patients may appear surprisingly clinically well until they decompensate. These cases are highlighted to raise awareness of this potentially life threatening condition.

Accident and emergency department led implementation of ketamine sedation in paediatric practice and parental response.

OBJECTIVE: To evaluate accident and emergency (A&E) department led practice of ketamine sedation for painful, short procedures in the paediatric population and to ascertain parental response. METHODS: Analysis of retrospective data for all children who received ketamine sedation over a 20 month period in a district general hospital. A data extraction form was used to record age, sex, dose, indication, side effects, and outcome. The parents were contacted by telephone afterwards and asked standardised questions about the child's treatment, their progress after discharge, and overall satisfaction with the treatment. RESULTS: Intramuscular ketamine was administered to 100 children under 12 years of age during the study period. The drug caused no adverse events pre-operatively or intraoperatively. The main early postoperative complication was vomiting (14%). Ninety three per cent of patients were discharged the same day. No reattendance or treatment attributable to ketamine related side effects were necessary. Over the 24 hours after discharge, vomiting occurred in 12% and ataxia in 15% of patients. Ninety nine per cent of parents were either very satisfied or satisfied with ketamine sedation and were willing for their child to receive it again, if required. CONCLUSION: This study, while confirming the known safety of ketamine sedation, established its suitability for "independent" use within A&E departments by suitably qualified staff.

Somatomotor fMRI in the pre-surgical evaluation of a case of focal epilepsy.

A child with intractable partial epilepsy who was found to have a focal lesion in the motor cortex underwent detailed pre-surgical and intraoperative investigations which enabled curative surgery without morbidity by minimizing a targeted resection. The pre-surgical assessment included mapping motor cortical function with functional magnetic resonance imaging (fMRI). This was subsequently correlated with the results of pre-surgical and intraoperative invasive corticography.

Neurochemical and behavioural investigations of the NMDA receptor-associated glycine site in the rat striatum: functional implications for treatment of parkinsonian symptoms.

The glutamatergic cortico-striatal and subthalamo-entopeduncular pathways are both overactive in parkinsonism. Previous behavioural investigations have shown that intra-entopeduncular injection of either NMDA-site or glycine-site antagonists results in alleviation of parkinsonian symptoms, although injection of the former is associated with the appearance of anaesthetic-like side effects. These behavioural differences may be mediated by action on different NMDA receptor subtypes. Recent neurochemical and molecular pharmacological studies have indicated the existence of NMDA receptor subtypes which display differential modulation by glycine. In the present study, three potential modes of NMDA antagonism were differentiated in vitro by effects on [3H]-glycine binding to striatal sections. Specific [3H]-glycine binding was totally displaced by the glycine partial agonist (R)-HA-966; the NMDA-site antagonist D-CPP had no effect; and the NMDA-site antagonist D-AP5 displaced [3H]-glycine binding in a subpopulation of glycine sites. The anti-parkinsonian effects of (R)-HA-966, D-CPP and D-AP5 were assessed by intra-striatal injection in reserpine-treated rats and 6-OHDA-lesioned rats. Injection of (R)-HA-966 and D-CPP resulted in alleviation of parkinsonian akinesia, although the latter elicited anaesthetic-like side effects; D-AP5 was ineffective as an anti-parkinsonian agent. (R)-HA-966 was also effective as an anti-parkinsonian agent when administered systemically in the reserpine-treated rat. These data suggest that different classes of NMDA antagonist mediate different behavioural responses within the parkinsonian striatum. The behavioural response produced may depend on the exact nature of the conformational change induced by the antagonist and the location of the subtype most sensitive to that class of compound. Selection of a specific mode of NMDA receptor antagonism or targeting of striatal NMDA receptor subtypes may form the basis of a novel therapeutic approach to Parkinson's disease.

Fluidotherapy and exercise in the management of sickle cell anemia. A clinical report.

Children hospitalized during sickle cell anemia crises suffer restricted range of motion secondary to vasoocclusive-induced pain and are almost always nonambulatory. Texas Children's Hospital, Houston, TX, now refers most of these children to the Physical Therapy Department at St. Luke's Episcopal Hospital, Houston, TX, for Fluidotherapy and general strengthening and increased endurance programs. The Fluidotherapy treatment and the exercise program have resulted in a marked reduction in the length of hospitalization (compared with length of hospitalization by the same patients during previous episodes) and have permitted a major reduction in the dosage of analgesics previously administered. Spine, trunk, and extremity range of motion and gait improved markedly with treatment.

Rapid recognition of methicillin-resistant Staphylococcus aureus by use of automated test systems.

The ability to rapidly recognize methicillin-resistant Staphylococcus aureus by use of two automated instrument systems, the MS-2 system (Abbott Laboratories, Diagnostics Division, Irving, Tex.) and the AutoMicrobic system (Vitek Systems, Hazelwood, Mo.), was evaluated on a collection of 95 methicillin-resistant S. aureus isolates recovered from at least six geographical areas of the United States. Isolates were simultaneously tested with both systems, and the results were compared with MIC tests performed by the National Committee for Clinical Laboratory Standards agar dilution method. Methicillin-resistant S. aureus isolates were defined as those with a methicillin MIC greater than or equal to 8 micrograms/ml by the reference procedure. Overall, with the AutoMicrobic system, 94.7% of 95 methicillin-resistant S. aureus isolates were detected, and with the MS-2 system, 91.6% of the isolates were detected. Isolates with methicillin MICs greater than or equal to 32 micrograms/ml were readily detected with both systems (41 of 42 isolates). Of 53 isolates from three locales with methicillin MICs of 8 or 16 micrograms/ml, 90.6% (48) were detected by the AutoMicrobic system, whereas 86.8% (46) were detected by the MS-2 system. A program update which has been added to the MS-2 system prints a warning message indicating possible methicillin-resistant S. aureus with isolates which demonstrate multiple antibiotic resistance (greater than or equal to four drugs other than methicillin). This warning message would have provided presumptive recognition of six of eight isolates with discrepant results for methicillin by the MS-2 system.