Do determinants of burden and emotional distress in dementia caregivers change over time?

OBJECTIVES: Caring for a patient with dementia is a real challenge and can have considerable psychological consequences in the long run. Many caregivers, mostly relatives, feel highly burdened. To develop effective caregiver support to prevent caregivers from getting overburdened, insight is needed into the determinants of burden. The objective of this study is to explore which patient and caregiver characteristics determine the different kinds of caregiver burden over time, both in the short and in the long run. METHOD: The study was longitudinal. Data on patients and caregivers, general burden and emotional distress were collected at three times: at baseline, at the end of treatment and at nine months. The study was conducted in a psychiatric skilled nursing home with a unit for integrative reactivation and rehabilitation (IRR) and at different sites of home-/day care, assisted living arrangements and nursing home wards (usual care). RESULTS: General burden is shown to be determined by severity of patient's neuropsychiatric symptoms, caregiver's sense of competence, health-related quality of life. Emotional distress is determined by severity of patient's neuropsychiatric symptoms, caregiver's sense of competence, high affiliation and patient gender. CONCLUSION: In preventing or treating caregiver burden, professional interventions need to aim specifically at diminishing the neuropsychiatric symptoms in dementia patients and improving the sense of competence in caregivers.

Implication of UGT2B15 Genotype Polymorphism on Postoperative Anxiety Levels in Patients Receiving Lorazepam Premedication.

BACKGROUND: Lorazepam is used as premedication for its anxiolytic properties. The UGT2B15 genotype is of importance for the metabolism of lorazepam. The clinical effect of genetic polymorphisms in UGT2B15 genotype on the treatment of anxiety levels in same-day surgery patients receiving lorazepam, however, is unknown. METHODS: Three hundred ninety-eight same-day surgery patients of mixed sex (from a previous double-blinded randomized controlled trial who were assigned to either lorazepam [n = 198] or placebo [n = 200]) were assessed for the UGT2B15*2 variant allele. Anxiety was measured preoperatively and postoperatively by the State part of the State-Trait Anxiety Inventory. The difference between these 2 measurements served as outcome of the study. Analysis of variance was used to assess the State part of the State-Trait Anxiety Inventory difference for interactions among the following factors: UGT2B15 genotype status, treatment condition (lorazepam or placebo), patient sex, and preoperative anxiety score. RESULTS: The anxiety difference was complex in that the interaction of lorazepam and UGT2B15 genotype status also was dependent on the joint effect of patient sex and preoperative anxiety score (F = 7.15, P = .008). Further exploration showed clinical relevant results in patients with high preoperative anxiety scores. Striking was that females with high preoperative anxiety scores and genetically reduced lorazepam glucuronidation (UGT2B15*2 homozygotes) showed more postoperative anxiety reduction than males with the same genotype. CONCLUSIONS: UGT2B15 genotype contributes to postoperative anxiety reduction after lorazepam premedication. Future research that focuses on patients with high preoperative anxiety scores could help to gain a deeper understanding in the clinical relevance of the interaction between lorazepam and UGT2B15 genotype on postoperative anxiety levels.

Clinical prediction model to identify vulnerable patients in ambulatory surgery: towards optimal medical decision-making.

BACKGROUND: Ambulatory surgery patients are at risk of adverse psychological outcomes such as anxiety, aggression, fatigue, and depression. We developed and validated a clinical prediction model to identify patients who were vulnerable to these psychological outcome parameters. METHODS: We prospectively assessed 383 mixed ambulatory surgery patients for psychological vulnerability, defined as the presence of anxiety (state/trait), aggression (state/trait), fatigue, and depression seven days after surgery. Three psychological vulnerability categories were considered-i.e., none, one, or multiple poor scores, defined as a score exceeding one standard deviation above the mean for each single outcome according to normative data. The following determinants were assessed preoperatively: sociodemographic (age, sex, level of education, employment status, marital status, having children, religion, nationality), medical (heart rate and body mass index), and psychological variables (self-esteem and self-efficacy), in addition to anxiety, aggression, fatigue, and depression. A prediction model was constructed using ordinal polytomous logistic regression analysis, and bootstrapping was applied for internal validation. The ordinal c-index (ORC) quantified the discriminative ability of the model, in addition to measures for overall model performance (Nagelkerke's R (2) ). RESULTS: In this population, 137 (36%) patients were identified as being psychologically vulnerable after surgery for at least one of the psychological outcomes. The most parsimonious and optimal prediction model combined sociodemographic variables (level of education, having children, and nationality) with psychological variables (trait anxiety, state/trait aggression, fatigue, and depression). Model performance was promising: R (2)  = 30% and ORC = 0.76 after correction for optimism. CONCLUSION: This study identified a substantial group of vulnerable patients in ambulatory surgery. The proposed clinical prediction model could allow healthcare professionals the opportunity to identify vulnerable patients in ambulatory surgery, although additional modification and validation are needed. (ClinicalTrials.gov number, NCT01441843).

Different Perspectives of Clinicians and Patients with Severe Mental Illness on Motivation for Treatment.

The present study assessed motivation for engaging in treatment as rated by clinicians (n = 57) and patients with severe mental illness (SMI, n = 294) using measures based on three different motivation theories. Questionnaires were derived from self-determination theory, the transtheoretical model and the integral model of treatment motivation. It was investigated to which extent clinicians of patients with SMI were able to estimate their patient's perspective on motivation for engaging in treatment, to which extent they agreed on the patient's motivation and which factors were associated with estimation and agreement on treatment motivation. It was found that clinicians were poorly to moderately capable of estimating their patient's type of motivation and readiness for change. Further, agreement on the level of motivation between patients and clinicians was moderate. These findings were consistent across diagnostic groups (psychotic and personality disorders). A higher quality therapeutic relationship was generally associated with higher clinician-rated motivation. The patient's ethnicity and socially desirable responding were factors that differentiated between scales of different motivation theories. It is concluded that patients with SMI and their clinicians have different perceptions on the patient's motivation for engaging in psychiatric treatment, regardless of the theoretical framework that is used to measure motivation. The findings imply that a negotiated approach is needed where both perceptions of clinicians and patients on motivation for treatment are considered to ensure effective mental health interventions. Copyright © 2015 John Wiley & Sons, Ltd. KEY PRACTITIONER MESSAGE: Clinicians show poor to moderate capability in estimating how patients perceive their motivation for engaging in treatment, especially so when the patient's motives revolve around feelings of shame and guilt. Clinicians generally give higher motivation ratings for patients where they experience a higher quality therapeutic relationships with, whereas-depending on the scale that is used to measure motivation-they give lower ratings to patients who respond in socially desirable ways and to ethnic minority patients. As patients with SMI and their clinicians have different perceptions on the patient's motivation for engaging in psychiatric treatment (regardless of the theoretical framework that is used to assess motivation), this implies that a negotiated approach is needed where both perceptions of clinicians and patients on motivation for treatment are considered to ensure effective mental health interventions.

Long-term survival after resection for non-pancreatic periampullary cancer followed by adjuvant intra-arterial chemotherapy and concomitant radiotherapy.

BACKGROUND: There is no consensus regarding the optimal adjuvant treatment after resection of non-pancreatic periampullary adenocarcinoma (NPPC; distal common bile duct, ampulla, duodenum). OBJECTIVES: The present study was conducted to evaluate the impacts on longterm survival and recurrence of adjuvant intra-arterial chemotherapy (IAC) and concomitant radiotherapy (RT) in patients submitted to resection for NPPC or pancreatic ductal adenocarcinoma (PDAC) in a randomized controlled trial. METHODS: A total of 120 patients with PDAC (n = 62) or NPPC (n = 58) were prestratified at a ratio of 1:1 for tumour origin and randomized. Half of these patients were treated with adjuvant IAC/RT and the other half were treated with surgery alone. Follow-up was completed for all patients up to 5 years after resection or until death. RESULTS: There was no survival benefit in either the whole group (primary endpoint) or the PDAC group after IAC/RT. In the NPPC group, longterm survival was observed in 10 patients in the IAC/RT group and five patients in the control group: median survival was 37 months and 28 months, respectively. The occurrence of liver metastases was reduced by IAC/RT from 57% to 29% (P = 0.038). Cox regression analysis revealed a substantial effect of IAC/RT on survival (hazard ratio: 0.44, 95% confidence interval 0.23-0.83; P = 0.011). CONCLUSIONS: This longterm analysis shows that median and longterm survival were improved after IAC/RT in patients with NPPC, probably because of the effective and sustained reduction of liver metastases. The present results illustrate that NPPC requires an adjuvant approach distinct from that in pancreatic cancer and indicate that further investigation of this issue is warranted.

Minor impact of multiple psychiatric symptoms on quality of life (EQ5D) in psychogeriatric patients: a clinical-empirical structural modeling approach.

BACKGROUND: The relationship of clinical variables for cognitive functioning and other variables such as multiple neuropsychiatric symptoms and quality of life are usually analyzed bivariately and multivariately. In randomized controlled trials analyses are mostly performed on individual outcome variables. To unravel interdependencies of determinants and outcome variables, Structural Equation Modeling (SEM) was applied. METHODS: Using SEM, we explored interdependencies of clinical determinants (MMSE, BI, and NPI-sum severity) and quality of life (EQ5D) in psychogeriatric patients (including dementia) suffering from cognitive problems and multiple neuropsychiatric symptoms. RESULTS: MMSE and BI showed direct and indirect impact on EQ5D, but the association with NPI-sum severity was minor. The identified model showed that R(2) of EQ5D varied from 0.21 to 0.48. DISCUSSION: This clinical-empirical study showed that SEM could be utilized to unravel and identify a model of complex direct and indirect effects of MMSE and BI on EQ5D. In relation to NPI-sum severity, however, the validity of EQ5D seemed insufficient in psychogeriatric patients. Consequently, the cost-benefit analyses and cost-effectiveness analyses using quality-adjusted life-year measures of an intervention in psychogeriatric patients with multiple neuropsychiatric symptoms, based on EQ5D, are considered questionable. Construction of a quality of life instrument that is more sensitive with regard to multiple neuropsychiatric symptoms in psychogeriatric patients is highly recommended.

Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis.

Cystic fibrosis (CF) is primarily characterised by bronchiectasis and trapped air on chest computed tomography (CT). The revised Cystic Fibrosis Questionnaire respiratory symptoms scale (CFQ-R RSS) measures health-related quality of life. To validate bronchiectasis, trapped air and CFQ-R RSS as outcome measures, we investigated correlations and predictive values for pulmonary exacerbations. CF patients (aged 6-20 years) underwent CT, CFQ-R RSS and 1-year follow-up. Bronchiectasis and trapped air were scored using the CF-CT scoring system. Correlation coefficients and backward multivariate modelling were used to identify predictors of pulmonary exacerbations. 40 children and 32 adolescents were included. CF-CT bronchiectasis (r = -0.38, p<0.001) and CF-CT trapped air (r = -0.35, p = 0.003) correlated with CFQ-R RSS. Pulmonary exacerbations were associated with: bronchiectasis (rate ratio 1.10, 95% CI 1.02-1.19; p = 0.009), trapped air (rate ratio 1.02, 95% CI 1.00-1.05; p = 0.034) and CFQ-R RSS (rate ratio 0.95, 95% CI 0.91-0.98; p = 0.002). The CFQ-R RSS was an independent predictor of pulmonary exacerbations (rate ratio 0.96, 95% CI 0.94-0.97; p<0.001). Bronchiectasis, trapped air and CFQ-R RSS were associated with pulmonary exacerbations. The CFQ-R RSS was an independent predictor. This study further validated bronchiectasis, trapped air and CFQ-R RSS as outcome measures in CF.

Long-term psychological distress in women at risk for hereditary breast cancer adhering to regular surveillance: a risk profile.

BACKGROUND: Some women at risk for hereditary breast cancer are at increased risk of psychological distress. In order to tailor support for individual women, the availability of a tool enabling the identification of psychologically vulnerable women at an early stage is warranted. The objectives of this study were (1) to explore long-term psychological distress in women at risk for hereditary breast cancer adhering to regular surveillance, and (2) to identify women being vulnerable for long-term psychological distress, defined in terms of a multifactorial risk profile. METHODS: General distress and cancer-related distress were assessed at baseline (T0) and after 5-8 years (T1) in 197 high-risk women adhering to breast cancer surveillance. Coping styles, occurrence of breast cancer in the family of origin, breast cancer risk perception, and frequency of breast self-examination, as assessed at T0, were examined as predictor variables for long-term distress (T1). RESULTS: Across time, women reported a significant reduction in intrusion and avoidance. Intrusion levels were increased among women who had lost a first-degree relative to breast cancer. Predictors of increased long-term distress were passive and palliative coping styles, excessive breast self-examination, and overestimation of breast cancer risk. On the other hand, coping through fostering reassuring thoughts was predictive for decreased long-term distress. CONCLUSION: On the basis of the identified risk profile, it is possible to identify vulnerable women at an early stage, who then may be offered additional and individually tailored support.

Psychometric evaluation of the Sophia Observation withdrawal symptoms scale in critically ill children.

OBJECTIVE: The Sophia Observation withdrawal Symptoms scale is an instrument for screening benzodiazepine and opioid withdrawal syndrome in pediatric critical care patients. The objectives of this study were to establish cutoff scores and to test sensitivity to change. Second, risk factors for withdrawal syndrome were explored. DESIGN: Prospective observational study with repeated measures. SETTING: Level IV ICU at a university children's hospital. PATIENTS: A total of 154 children with median age 5 months (interquartile range, 0-42 mo) who received continuous infusion of benzodiazepines and/or opioids for 5 or more days. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Nurses repeatedly applied the Sophia Observation withdrawal Symptoms scale and the Numeric Rating Scale withdrawal when children were weaned off benzodiazepines and opioids. The latter represents the nurse's expert opinion. We analyzed 3,754 paired assessments; the median number per child was 15 (interquartile range, 7-31) over a median of 5 days (interquartile range, 3-11 d). Sensitivity and specificity were 0.83 and 0.93, respectively, for the Sophia Observation withdrawal Symptoms scale cutoff score of 4 or higher against a Numeric Rating Scale-withdrawal score of 4 or higher. Sensitivity to change was determined by comparing 156 Sophia Observation withdrawal Symptoms scale assessments (n = 51 patients) before and after additional sedatives or opioids. Multilevel regression analysis showed a mean decline of 1.5 points (at score range 0-15) after intervention (p < 0.0001). Logistic regression analysis identified duration of preweaning of midazolam, duration of weaning of midazolam, duration of preweaning of morphine, duration of weaning of morphine, and number of additional sedatives/opioids as statistically significant risk factors for withdrawal syndrome in these children. CONCLUSIONS: The Sophia Observation withdrawal Symptoms scale is a valid tool with good psychometric properties to assess withdrawal symptoms in PICU patients.

Benefit of an integrative psychotherapeutic nursing home program to reduce multiple psychiatric symptoms of psychogeriatric patients and caregiver burden after six months of follow-up: a re-analysis of a randomized controlled trial.

BACKGROUND: In this paper, we aim to test the long-term benefit of an integrative reactivation and rehabilitation (IRR) program compared to usual care in terms of improved psychogeriatric patients on multiple psychiatric symptoms (MPS) and of caregivers on burden and competence. Improvement was defined as >30% improvement (≥ a half standard deviation) compared to baseline. METHODS: We used the following outcome variables: difference in the number of improved patients on MPS (Neuropsychiatric Inventory, NPI) and improved caregivers on burden (Caregiver Burden, CB) and competence (Caregiver Competence List, CCL). Assessments were taken after intake (T1) and after six months of follow-up (T3). Risk ratios (RR), number needed to treat (NNT), and odds ratios (ORs) were calculated. RESULTS: IRR had a significant positive effect on NPI-cluster hyperactivity (RR 2.64; 95% CI: 1.26-5.53; NNT 4.07). In the complete cases analysis, IRR showed significant ORs of 2.80 on the number of NPI symptoms and 3.46 on the NPI-sum-severity; up to 76% improved patients. For caregivers, competence was a significant beneficiary in IRR (RR 2.23; 95% CI: 1.07-4.62; NNT 5.07). In the complete cases analysis, the ORs were significantly in favor of IRR on general burden and competence (ORs range: 2.40-4.18), with up to 71% improved caregivers. CONCLUSION: IRR showed a significantly higher probability of improvement with a small NNT of four on multiple psychiatric symptoms in psychogeriatric patients. The same applies to the higher probability to improve general burden and competence of the caregiver with an NNT of five. The results were even more pronounced for those who fully completed the IRR program. (Inter)national psychogeriatric nursing home care and ambulant care programs have to incorporate integrative psychotherapeutic interventions.

Economic evaluation alongside a single RCT of an integrative psychotherapeutic nursing home programme.

BACKGROUND: There is an 80% prevalence of two or more psychiatric symptoms in psychogeriatric patients. Multiple psychiatric symptoms (MPS) have many negative effects on quality of life of the patient as well as on caregiver burden and competence. Irrespective of the effectiveness of an intervention programme, it is important to take into account its economic aspects. METHODS: The economic evaluation was performed alongside a single open RCT and conducted between 2001 and 2006. The patients who met the selection criteria were asked to participate in the RCT. After the patient or his caregiver signed a written informed consent form, he was then randomly assigned to either IRR or UC.The costs and effects of IRR were compared to those of UC. We assessed the cost-utility of IRR as well as the cost-effectiveness of both conditions. Primary outcome variable: severity of MPS (NPI) of patients; secondary outcome variables: general caregiver burden (CB) and caregiver competence (CCL), quality of life (EQ5D) of the patient, and total medical costs per patient (TiC-P). Cost-utility was evaluated on the basis of differences in total medical costs). Cost-effectiveness was evaluated by comparing differences of total medical costs and effects on NPI, CB and CCL (Incremental Cost-Effectiveness Ratio: ICER). CEAC-analyses were performed for QALY and NPI-severity. All significant testing was fixed at p<0.05 (two-tailed). The data were analyzed according to the intention-to-treat (ITT)-principle. A complete cases approach (CC) was used. RESULTS: IRR turned out to be non-significantly, 10.5% more expensive than UC (€ 36 per day). The number of QALYs was 0.01 higher (non-significant) in IRR, resulting in € 276,290 per QALY. According to the ICER-method, IRR was significantly more cost-effective on NPI-sum-severity of the patient (up to 34%), CB and CCL (up to 50%), with ICERs varying from € 130 to € 540 per additional point of improvement. CONCLUSIONS: No significant differences were found on QALYs. In IRR patients improved significantly more on severity of MPS, and caregivers on general burden and competence, with incremental costs varying from € 130 to € 540 per additional point of improvement. The surplus costs of IRR are considered acceptable, taking into account the high societal costs of suffering from MPS of psychogeriatric patients and the high burden of caregivers. The large discrepancy in economic evaluation between QALYs (based on EQ5D) and ICERs (based on clinically relevant outcomes) demands further research on the validity of EQ5D in psychogeriatric cost-utility studies. (Trial registration nr.: ISRCTN 38916563; December 2004).

Lorazepam does not improve the quality of recovery in day-case surgery patients: a randomised placebo-controlled clinical trial.

BACKGROUND: In day-case surgery, the effects of the anxiolytic lorazepam as premedication on the quality of postoperative recovery are unknown. OBJECTIVE: To evaluate whether lorazepam as a premedication beneficially affects quality of recovery (primary outcome) and psychological manifestations (secondary outcome) after day-case surgery. DESIGN: A randomised, double-blind, placebo-controlled clinical trial. SETTING: Single tertiary centre. INCLUSION CRITERIA: day-case surgery; age at least 18 years. EXCLUSION CRITERIA: insufficient knowledge of the Dutch language; intellectual disability; ophthalmology surgery; extracorporeal shock wave lithotripsy; endoscopy; botulinum toxin A treatment; abortion; chronic pain treatment; preceding use of psychopharmaceuticals; contraindication to lorazepam. INTERVENTION: Lorazepam (1 to 1.5 mg) intravenously vs. NaCl 0.9% as a premedication prior to surgery. MAIN OUTCOME MEASURE: Quality of Recovery-40 (QoR-40) score. SECONDARY OUTCOMES: State-Trait Anxiety Inventory (STAI-State/Trait); State-Trait Anger Scale (STAS-State/Trait); Multidimensional Fatigue Inventory (MFI); Hospital Anxiety and Depression Scale (HADS). Timing of evaluation: T0: preoperatively (all scales); T1: before discharge (STAI-State/Trait); T2: first postoperative working day (QoR-40); T3: 7th day after surgery (all scales). Robust regression analysis was applied. Statistical analyses were adjusted for the corresponding baseline value and sex. RESULTS: Four hundred patients were randomised; 398 patients were analysed. Postoperative mean QoR-40 scores were similar in both groups at T2 (174.5 vs. 176.4, P = 0.34) and T3 (172.8 vs.176.3, P = 0.38). Postoperative mean STAI-State/Trait scores decreased less in the group with lorazepam at T1 (32.3 vs. 29.3, P < 0.0001; 32.7 vs. 30.8, P = 0.0002). STAI-Trait and HADS-Anxiety decreased less in the group with lorazepam at T3 (31.1 vs. 30.0; P = 0.03, 3.3 vs. 2.5, P = 0.003). STAS-State increased in the group with lorazepam at T3 (10.8 vs. 10.3, P = 0.04). CONCLUSION: In day-case surgery, lorazepam as a premedication did not improve quality of recovery. Furthermore, this premedication may delay the decrease in postoperative anxiety and aggression. TRIAL REGISTRATION: ClinicalTrials.gov identifier: NCT01441843.

Effect of intravenous paracetamol on postoperative morphine requirements in neonates and infants undergoing major noncardiac surgery: a randomized controlled trial.

IMPORTANCE: Continuous morphine infusion as standard postoperative analgesic therapy in young infants is associated with unwanted adverse effects such as respiratory depression. OBJECTIVE: To determine whether intravenous paracetamol (acetaminophen) would significantly (>30%) reduce morphine requirements in neonates and infants after major surgery. DESIGN, SETTING, AND PATIENTS: Single-center, randomized, double-blind study conducted in a level 3 pediatric intensive care unit in Rotterdam, The Netherlands. Patients were 71 neonates or infants younger than 1 year undergoing major thoracic (noncardiac) or abdominal surgery between March 2008 and July 2010, with follow-up of 48 hours. INTERVENTIONS: All patients received a loading dose of morphine 30 minutes before the end of surgery, followed by continuous morphine or intermittent intravenous paracetamol up to 48 hours postsurgery. Infants in both study groups received morphine (boluses and/or continuous infusion) as rescue medication on the guidance of the validated pain assessment instruments. MAIN OUTCOME MEASURES: Primary outcome was cumulative morphine dose (study and rescue dose). Secondary outcomes were pain scores and morphine-related adverse effects. RESULTS: The cumulative median morphine dose in the first 48 hours postoperatively was 121 (interquartile range, 99-264) µg/kg in the paracetamol group (n = 33) and 357 (interquartile range, 220-605) µg/kg in the morphine group (n = 38), P < .001, with a between-group difference that was 66% (95% CI, 34%-109%) lower in the paracetamol group. Pain scores and adverse effects were not significantly different between groups. CONCLUSION AND RELEVANCE: Among infants undergoing major surgery, postoperative use of intermittent intravenous paracetamol compared with continuous morphine resulted in a lower cumulative morphine dose over 48 hours. TRIAL REGISTRATION: trialregister.nl Identifier: NTR1438.

The impact of social and personal resources on psychological distress in women at risk for hereditary breast cancer.

OBJECTIVE: The objectives of the present study were to (1) evaluate whether social and personal resources were independently related to psychological distress and (2) examine the interrelationships of social and personal resources in women at risk for hereditary breast cancer. METHODS: General and breast cancer specific distress, family communication regarding hereditary breast cancer, perceived social support, self-esteem, self-concept, and demographics were assessed in 222 high-risk women, having opted either for regular surveillance or prophylactic surgery. RESULTS: Structural equation modeling showed that (1) both personal and social resources were independently associated with psychological distress and (2) the associations between social resources and psychological distress were partially mediated by personal resources. Support from family and friends was associated with a higher level of self-esteem, which in turn was associated with less general distress. Furthermore, communication regarding cancer within the nuclear family was associated with decreased feelings of stigmatization, which in turn was associated with less general and breast cancer specific distress. Moreover, open communication within the family was associated with a reduced sense of vulnerability. CONCLUSION: Health workers involved in the care of high-risk women should carefully monitor women's personal and social resources, and if compromised refer them for appropriate support.

The quick motor function test: a new tool to rate clinical severity and motor function in Pompe patients.

Pompe disease is a lysosomal storage disorder characterized by progressive muscle weakness. With the emergence of new treatment options, psychometrically robust outcome measures are needed to monitor patients' clinical status. We constructed a motor function test that is easy and quick to use. The Quick Motor Function Test (QMFT) was constructed on the basis of the clinical expertise of several physicians involved in the care of Pompe patients; the Gross Motor Function Measure and the IPA/Erasmus MC Pompe survey. The test comprises 16 items. Validity and test reliability were determined in a cohort of 91 Pompe patients (5 to 76 years of age). In addition, responsiveness of the scale to changes in clinical condition over time was examined in a subgroup of 18 patients receiving treatment and 23 untreated patients. Interrater and intrarater reliabilities were good (intraclass correlation coefficients: 0.78 to 0.98 and 0.76 to 0.98). The test correlated strongly with proximal muscle strength assessed by hand held dynamometry and manual muscle testing (rs= 0.81, rs=0.89), and showed significant differences between patient groups with different disease severities. A clinical-empirical exploration to assess responsiveness showed promising results, albeit it should be repeated in a larger group of patients. In conclusion, the Quick Motor Function Test can reliably rate clinical severity and motor function in children and adults with Pompe disease.

The profile of executive function in very preterm children at 4 to 12 years.

AIM: To examine executive functioning in very preterm (gestational age ≤30 wks) children at 4 to 12 years of age. METHOD: Two-hundred very preterm (106 males, 94 females; mean gestational age 28.1wks, SD 1.4; mean age 8y 2mo, SD 2y 6mo) and 230 term children (106 males, 124 females; mean gestational age 39.9wks, SD 1.2; mean age 8y 4mo, SD 2y 3mo) without severe disabilities, born between 1996 and 2004, were assessed on an executive function battery comprising response inhibition, interference control, switching, verbal fluency, verbal and spatial working memory, and planning. Multiple regression analyses examined group differences while adjusting for effects of parental education, age, sex, and speed indices. RESULTS: Relative to children born at term, very preterm children had significant (p(s) <0.02; where p(s) represents p-values) deficits in verbal fluency (0.5 standardized mean differences [SMD]), response inhibition (0.4 SMD), planning (0.4 SMD), and verbal and spatial working memory (0.3 SMD), independent of slow and highly fluctuating processing speed. A significant group by age interaction indicated that group differences for response inhibition decreased between 4 and 12 years. INTERPRETATION: Very preterm birth is associated with a profile of affected and non-affected executive functions independent of impaired speed. Deficits are of small to moderate magnitude and persist over time, except for response inhibition for which very preterm children catch up with peers.

Motivation and treatment engagement intervention trial (MotivaTe-IT): the effects of motivation feedback to clinicians on treatment engagement in patients with severe mental illness.

BACKGROUND: Treatment disengagement and non-completion poses a major problem for the successful treatment of patients with severe mental illness. Motivation for treatment has long been proposed as a major determinant of treatment engagement, but exact mechanisms remain unclear. This current study serves three purposes: 1) to determine whether a feedback intervention based on the patients' motivation for treatment is effective at improving treatment engagement (TE) of severe mentally ill patients in outpatient psychiatric treatment, 2) to gather insight into motivational processes and possible mechanisms regarding treatment motivation (TM) and TE in this patient population and 3) to determine which of three theories of motivation is most plausible for the dynamics of TM and TE in this population. METHODS/DESIGN: The Motivation and Treatment Engagement Intervention Trial (MotivaTe-IT) is a multi-center cluster randomized trial investigating the effectiveness of feedback generated by clinicians regarding their patients' treatment motivation upon the patients' TE. The primary outcome is the patients' TE. Secondary outcomes are TM, psychosocial functioning and quality of life. Patients whose clinicians generate monthly motivation feedback (additional to treatment as usual) will be compared to patients who receive treatment as usual. An estimated 350 patients, aged 18 to 65 years, with psychotic disorders and/or severe personality disorders will be recruited from outpatient community mental health care. The randomization will be performed by a computerized randomization program, with an allocation ratio of 1:1 (team vs. team or clinician vs. clinician) and patients, but not clinicians, will be blind to treatment allocation at baseline assessment. Due to the nature of the trial, follow-up assessment can not be blinded. DISCUSSION: The current study can provide important insights regarding motivational processes and the way in which motivation influences the treatment engagement and clinical outcomes. The identification of possible mechanisms through which changes in the outcomes occur, offers a tool for the development of more effective future interventions to improve TM and TE. TRIAL REGISTRATION: Current Controlled Trials NTR2968.

Development and validation of a neonatal intensive care parent satisfaction instrument.

OBJECTIVE: To develop and test the psychometric properties of the EMPATHIC-N (EMpowerment of PArents in THe Intensive Care-Neonatology) questionnaire measuring parent satisfaction. DESIGN: A psychometric study testing the reliability and validity of a parent satisfaction questionnaire by applying confirmatory factor analysis including standardized factor loadings and subsequently Cronbach's α reliability estimates across time, congruent validity, and nondifferential validity testing. SETTING: A 30-bed neonatal intensive care unit in a university hospital. PATIENTS: Two cohorts with a total of 441 parents whose child was admitted to the neonatal intensive care unit, January to December 2009. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: In the first cohort, 220 of 339 (65%) parents responded; in the second cohort, 59 of 102 (58%) parents responded. Structural equation modeling and confirmatory factor analysis resulted in a sufficient model fit of 57 statements within five domains: Information, Care & Treatment, Organization, Parental Participation, and Professional Attitude. Standardized factor loading of these statements were between 0.58 and 0.91. Reliability measures, Cronbach's α, of the domains ranged from 0.82 to 0.95. Reliability across time showed no evidence of statistically significant differences between the domains. Congruent validity was confirmed by a good correlation (p = .01) between the domains and four general satisfaction questions. Nondifferential validity showed no significant effect sizes between the infants' characteristics and the domains, except between ventilated infants and parent participation statements and infants ≥30 wks gestational age and organizational statements. CONCLUSIONS: The EMPATHIC-N questionnaire is a valid quality performance indicator to measure the delivered care as perceived by parents.

Implementing structured, multiprofessional medical ethical decision-making in a neonatal intensive care unit.

BACKGROUND: In neonatal intensive care, a child's death is often preceded by a medical decision. Nurses, social workers and pastors, however, are often excluded from ethical case deliberation. If multiprofessional ethical case deliberations do take place, participants may not always know how to perform to the fullest. SETTING: A level-IIID neonatal intensive care unit of a paediatric teaching hospital in the Netherlands. METHODS: Structured multiprofessional medical ethical decision-making (MEDM) was implemented to help overcome problems experienced. Important features were: all professionals who are directly involved with the patient contribute to MEDM; a five-step procedure is used: exploration, agreement on the ethical dilemma/investigation of solutions, analysis of solutions, decision-making, planning actions; meetings are chaired by an impartial ethicist. A 15-item questionnaire to survey staff perceptions on this intervention just before and 8 months after implementation was developed. RESULTS: Before and after response rates were 91/105 (87%) and 85/113 (75%). Factor analysis on the questionnaire suggested a four-factor structure: participants' role; structure of MEDM; content of ethical deliberation; and documentation of decisions/conclusions. Effect sizes were 1.67 (p<0.001), 0.69 (p<0.001) and 0.40 (p<0.01) for the first three factors respectively, but only 0.07 (p=0.65) for the fourth factor. Nurses' perceptions of improvement did not significantly exceed those of physicians. CONCLUSION: Professionals involved in ethical case deliberation perceived that the process of decision-making had improved; they were more positive about the structure of meetings, their own role and, to some extent, the content of ethical deliberation. Documentation of decisions/conclusions requires further improvement.

Defense mechanisms in congenital and acquired facial disfigurement: a clinical-empirical study.

It is of clinical interest to investigate the degree to which patients with facial disfigurement use defense styles. Therefore, 59 adults born with rare facial clefts, 59 patients with facial deformities acquired at an adult age, and a reference group of 141 adults without facial disfigurements completed standardized questionnaires. There was a significant difference between the group with and the group without disfigurements on immature defense styles, with the disfigured group using the immature style more frequently. There was a trend for the nondisfigured group to use more mature defense styles. No difference between congenital and acquired groups was seen on individual types of defense style. Self-esteem had the strength to differentiate mature and immature defense styles within our disfigured groups. The association of low self-esteem and the utilization of immature defense styles suggests that professional help may tailor treatment on discussing immature defense style and problems triggering or maintaining this style.