Quality of life among children with sickle cell disease receiving chronic transfusion therapy.

Sickle cell disease (SCD) is a genetic disorder that is most prevalent among those of African American and Mediterranean descent. Hemoglobin SS is the most severe form of SCD and carries an increased risk for stroke. Although the initial treatment for stroke is an exchange transfusion, the use of routine, chronic transfusion therapy (CTT) has been shown to help prevent this neurological injury. The treatment plan is rigorous and time consuming, both of which impact one's quality of life (QoL). The purpose of this study was to explore QoL, from the child's perspective, as it is affected by CTT Semistructured interviews were performed on 10 children undergoing CIT: Five themes emerged from the data: (a) pain, (b) school issues, (c) disease knowledge, (d) transfusion therapy, and (e) having a stroke. Data from this study reveal that CTT does have an impact on QoL. This information is important to share with those making CTT treatment decisions.     

A comprehensive strategy for the evaluation and triage of the chest pain patient: A cost comparison study

BACKGROUND: Our objective was to determine the cost-effectiveness of a comprehensive, risk-based triage system, composed of multiple critical pathways, with the use of early myocardial perfusion imaging (MPI) in low-risk patients. We found previously that a chest pain evaluation system that uses MPI in low-risk patients was safe and effective, but the cost-effectiveness of this approach was not studied. METHODS AND RESULTS: We compared two groups. The Acute Cardiac Team (ACT) group (n = 874) was assigned prospectively to 1 of 4 risk levels by emergency department (ED) physicians. Level 1, 2, and 3 patients were admitted; level 4 patients were evaluated in the ED. Level 3 and 4 patients underwent ED MPI. The control group (n = 713) represented consecutive patients evaluated in the prior year according to standard care and assigned retrospectively to an ACT level based on the presenting electrocardiographic and clinical data. Record and hospital administrative data were assessed for clinical variables, outcomes, lengths of stay, and all expenses incurred within 30 days of the index visit. The baseline characteristics of the two groups were similar, including age, sex, myocardial infarction prevalence, and 30-day revascularization rates within each level or between the two groups. Mean costs per encounter were reduced for the ACT patients for each level, which was significant when all patients were compared ($5,030 +/- $7,081 vs $6,044 +/- $10,432, P =.02). Use of MPI in the low-risk patients was associated with reduced costs (level 3, $4,958 +/- $4,948 vs $5,051 +/- $7,036; level 4, $1,529 +/- $2,664 vs $1,794 +/- $6,854) and was associated with a significantly lower angiography rate and shorter length of stay. CONCLUSIONS: Implementation of a comprehensive strategy for chest pain evaluation and triage reduced overall costs for patients with chest pain on presentation. Acute MPI in the ED setting did not increase net cost.     

Cerebral Emboli during Cardiac Surgery in Children

Background: Microemboli occur commonly during cardiac surgery in adults, and, when present, increase the risk of neuropsychological deficits. Their incidence and significance during correction of congenital heart disease is unknown. The authors hypothesized that microemboli would occur before bypass with right-to-left cardiac shunts and would also occur in large numbers when the aortic crossclamp was released in children during repair of congenital heart defects.

Methods: In 25 children studied with carotid artery Doppler, embolic signals were counted and timed in relation to 13 intraoperative events. Patients were classified as either at high risk (obligate right-to-left shunt or uncorrected transposition of the great arteries) or at low risk (net left-to-right shunt or simple obstructive lesions) for paradoxical (venous to arterial) emboli.

Results: The median number of emboli detected was 122 (range, 2-2,664). Forty-two percent of all emboli were detected within 3 min of release of the aortic crossclamp. The high-risk group had significantly more emboli (median, 66; range, 0-116) during the time interval before cardiopulmonary bypass than did the low-risk group (median, 8; range, 0-73), with P < 0.01. There was no significant difference between the high-and low-risk groups in the total number of emboli detected. There was no apparent association between number of emboli and gross neurologic deficits.     

Psychometric Validation of the Eortc Core Quality of Life Questionnaire, 30-Item Version and A Diagnosis-Specific Module for Head and Neck Cancer Patients

A cancer-specific self-reporting quality of life questionnaire has been validated. The questionnaire is designed to assess physical functioning, role functioning, cognitive functioning, emotional functioning, social functioning, pain, fatigue, emesis and quality of life by means of multi-item scales, and other disease- and treatment-related symptoms by means of single items. The questionnaire was completed by 126 head and neck cancer patients with a mean age of 67 years. The internal consistency (scale reliability) was satisfactory for all scales but one. Correlations between scales and items assessing the same underlying dimension were also satisfactory. The questionnaire discriminates between patient subgroups and between acute, subacute and late toxicity. Patient compliance was high. The questionnaire provided valuable information, and most of the scales/items functioned well. A few problems were found, especially with the modified visual analogue scales, and minor modifications will be made.     

Prevention of Secondary Insults in Neurointensive Care of Traumatic Brain Injury

Background: Secondary insults/complications have a major impact on the prognosis after traumatic brain injury (TBI). The aim was to study the occurrence and prognostic value of secondary insults occurring in TBI patients, during standardized neurointensive care (NIC) dedicated to avoiding secondary insults. Material and Methods: 154 patients, 17-79 years, with acute head trauma and pathologic CT, treated during a 2-year period at the NIC unit were studied. The occurrence of defined secondary insults (standard and severe) was recorded during the 1st week of NIC from bedside surveillance charts containing one value per hour and parameter (intracranial pressure, cerebral perfusion pressure, systolic blood pressure, PaO₂, temperature, and blood glucose). The data set was analyzed using univariate and multivariate logistic regression with favorable outcome as the response variable. Both admission variables (Glasgow Coma Scale Motor Score [GCS M], CT class, Injury Severity Score [ISS], age, and gender) and secondary insult variables were included as explanatory variables. Results: In total, 1,570 insults were identified (320 severe). In the univariate analysis, the sum of all insults, blood glucose, GCS M, CT class, and ISS showed significant effects on outcome (p < 0.05). In the multiple regression analysis, GCS M was the only significant explanatory variable. Conclusions: The occurrence of secondary insults in the NIC unit was not negligible, despite the fact that major efforts were made to avoid them. The sum score of all insult categories and high blood glucose had a statistically significant effect on favorable outcome in the univariate analysis, but secondary insults did not add any prognostic information to the neurologic grade in the multivariate analysis. This finding indicates that the insults that occurred were related to the degree of primary injury/neurologic grade.     

Learning and retention of movement sequences in Parkinson's disease.

The purpose of this study was to examine motor learning and retention given extensive practice in two fundamentally different movement sequences. One sequence was a memory-driven task (performing a series of whole body positions from memory) and the other a context-driven task (buttoning). Practice took place over 3 weeks, with performance measured weekly; retention was measured weekly for 3 weeks after practice. There were 7 people with Parkinson's disease (PD) and 7 age-matched neurologically healthy people who participated in this study. Both groups improved performance on both tasks with practice, with the majority of the change for the PD group occurring between 1 and 2 weeks of practice. Although those with PD did not necessarily perform as well as age-matched controls, they learned both sequences in a manner similar to age-matched controls, and exhibited retention across the 3-week retention interval. If people with PD are given sufficient practice they can learn and retain both memory-based and context-driven movement sequences as well as age-matched controls. The results provide support for maintaining physical activity and for intervention through movement therapy.     

Adult outcomes of attention deficit hyperactivity disorder and conduct disorder: are the risks independent or additive?

METHODS: Data were obtained from a longitudinal study sample of 754 adoptees and categorized based on review of the available adoption agency, medical, and psychiatric records of the biological parents. Categorical data were analyzed using chi2 or Fisher's exact tests, as appropriate. Logistic regression analyses were used to assess the relative contribution of variables. RESULTS: There was not a statistically significant difference in the frequency or type of self-reported adult disruptive behavior, arrests, jail stays, felony arrests, or frequency of conduct disorder (CD) when inattentiveness, impulsivity, and hyperactivity were analyzed individually. The contributions of attention deficit hyperactivity disorder (ADHD) were independent and no additional increased risk for future illegal behavior was conferred by the combination of the disorders. While the effect of CD on illegal behavior was correlated with substance abuse and dependence, ADHD continued to be a significant contributor after controlling for substance abuse and dependence. CONCLUSIONS: Data indicated that ADHD and CD are related but different disorders conferring risk for adult illegal behavior or arrest. In this sample, inattention was the most common domain impaired among those with ADHD, followed closely by hyperactivity, with impulsivity reported least often among those endorsing symptoms of ADHD.     

Dissociation of the Medial Prefrontal, Posterior Parietal, and Posterior Temporal Cortex for Spatial Navigation and Recognition Memory in the Rat

Rats with lesions of the medial prefrontal, posterior parietal,or posterior temporal cortex were tested in five spatial navigationtasks, which varied in egocentric or allocentric demands, avisual discrimination task, and two delayed nonmatching-to-sampletasks. Rats with prefrontal lesions were impaired at every spatialnavigation task, whereas rats with posterior parietal lesionshad selective spatial navigation impairments. Rats with prefrontallesions were also impaired at a visual delayed nonmatching-to-sampletask, as they were unable to learn the task, even with no delay.The results are consistent with the idea that the basic planof mammalian cortex includes prefrontal, posterior parietal,and posterior temporal regions, each of which have generallysimilar functions across mammalian taxa. There are, however,species-typical differences that reflect specific ecologicalpressures on the development of the different regions.