Testing the Integral Model of treatment motivation in outpatients with severe mental illness.

The current study tested the Integral Model of treatment motivation (IM) in a sample of 294 outpatients with severe mental illness, using structural equation modelling. The obtained structural model was not consistent with original theory, nor was the model invariant across time and patient groups (psychotic disorders and personality disorders). The patient's perceived suitability of treatment, perceived costs of treatment and outcome expectancy were most strongly associated with motivation and treatment engagement. The model explained between 22 and 86% of variance in clinical outcomes, depending on the timing of the assessment. Currently, the IM does not constitute a robust framework for patterns through which patients become motivated to engage in treatment, but does explain substantial amounts of variance in clinical outcomes. The future potential of IM as a basis for interventions in the mental health care is discussed, including suggestions for subsequent research and potential alterations of the IM to improve its utility for application in clinical practice.

The COMFORT behaviour scale detects clinically meaningful effects of analgesic and sedative treatment.

BACKGROUND: The COMFORT behaviour scale (COMFORT-B scale) is widely used in paediatric intensive care units to assess young children's pain and distress. It is also used to assess the impact of treatment interventions, but little is known on the scale's sensitivity to detect changes between before and after measurements following an intervention. This study explored the sensitivity to change of the COMFORT-B scale. METHODS: COMFORT-B scores, originally and prospectively collected as part of standard care, were retrieved from the digital patient data management system. We analysed scores obtained in 747 paired observations, i.e., before and after a pharmacological intervention in 180 paediatric intensive care patients between September 2009 and September 2010. RESULTS: The mean scores before and after an intervention were 20.0 [standard deviation (SD) 3.7] and 14.1 (SD 4.7), respectively. Multilevel regression analysis showed a 6-point mean decline after an intervention (p < 0.0001). The magnitude of this decline was not statistically significantly related to number and type of interventions or time between assessments. In almost three-quarters of cases (74%), the COMFORT-B score dropped to below 17 after a pharmacological intervention, indicating good responsiveness. CONCLUSIONS: This is the first study demonstrating that the COMFORT-B scale detects treatment-related changes in pain or distress intensity. This implies that COMFORT-B assessments can effectively guide analgesic and sedation treatment in critically ill children.

[Does routine outcome monitoring have a promising future? An investigation into the use of shared decision-making combined with ROM for patients with a combination of physical and psychiatric symptoms]. / Shared decision making in combinatie met ROM bij patiënten met gecombineerde lichamelijke en psychische klachten; een klinisch-empirische verkenning.

BACKGROUND: Although routine outcome monitoring (ROM) has been developed and widely used in the course of patient centered outcome research in the Netherlands, so far the technique has hardly ever been used to improve the treatment of individual patients. AIM: To describe how a rom technique based on the principles of shared decision-making (SDM) was developed and evaluated at the Center for Body, Mind and Health at GGz Breburg, a specialised mental health institution in the Netherlands. METHOD: We have developed a conceptual model for SDM that involves patient participation and the use of evidence-based decision-aids with cut-off scores. RESULTS: According to the conceptual model for SDM that we developed, the patient and the health professional involved took 'shared' decisions in three phases; the decisions related to triage, the drawing-up of a treatment plan and a follow-up treatment course. At the end of the 6 month intake-phase 7 of the 67 patients who were deemed eligible for ROM/SDM were dropped from the study because they were incapable of performing ROM assessments. Due to diagnostic advice and referral at the end of the intake-phase, 25 patients did not require further treatment. Of the remaining 35 patients, 33 delivered at least one follow-up ROM assessment during the subsequent treatment phases. In these patients somatic and psychiatric symptoms were found to be significantly reduced. CONCLUSION: ROM combined with sdm can be used successfully with patients who have a combination of physical and psychiatric symptoms and the technique can be applied by the professional in charge. Very few patients dropped out of the follow-up measurements and somatic as well as depressive or psychiatric symptoms diminished significantly. These findings indicate that a Randomised Clinical Trial is warranted in order to test the effectiveness of sdm combined with ROM as a decision-making instrument.

Usefulness of F-18-fluorodeoxyglucose positron emission tomography to confirm suspected pancreatic cancer: a meta-analysis.

INTRODUCTION: Pancreatic cancer is among the five most lethal malignancies in the world. Unfortunately, many malignant tumors go undetected by the current primary diagnostic tools. (18)FDG-PET and (18)FDG-PET/CT might be useful to confirm suspected pancreatic cancer. METHODS: A meta-analysis was performed using all major search engines. Methodological quality of included studies was assessed as well as quality of the PET-protocol. The following pooled estimates served as primary outcome measures: sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy. RESULTS: Thirty-five studies were included. Pooled estimates for (18)FDG-PET were: sensitivity 90%, specificity 76%, PPV 90%, NPV 76% and accuracy 86%. Pooled estimates for (18)FDG-PET/CT were: sensitivity 90%, specificity 76%, PPV 89%, NPV 78% and accuracy 86%. The pooled sensitivity and specificity for (18)FDG-PET to differentiate between pancreatic cancer and chronic pancreatitis were 90% and 84%, respectively. CONCLUSION: Both (18)FDG-PET and (18)FDG-PET/CT offer no benefit over the current primary diagnostic tools in diagnosing pancreatic cancer. However, the (18)FDG-PET/CT systems are still improving. We should investigate the sensitivity and specificity of these new systems while reevaluating the tradeoff between false positive and false negative results. Yet, (18)FDG-PET/CT may have a role in the staging of pancreatic cancer, in survival prediction, and may add to other diagnostic information, like histology.

[Prevalence and prognostic importance of riskfactors for long hospital stay within elderly patients admitted to a hospital; a clinical-empirical study]. / Prevalentie en prognostisch belang van risicofactoren voor lange opnameduur onder ouderen bij ziekenhuisopname; een klinisch-empirische studie.

BACKGROUND: Of elderly patients (> 70 years) admitted to a general hospital 35% suffer from loss of self-care abilities compared to the level before admission. Risk of loss of self-care ability increases with age up to 65% after tthe age of 90. In addition, for many of these patients the duration of hospitalisation is relatively long. OBJECTIVE It is important to identify in an early stage frail-elderly patients who are at risk of a relatively long hospital stay. We conducted a study of the prevalence at intake (1st of 2nd admission day) of ten clinically relevant, patient-bound risk factors for a long hospital stay among 158 patients (> 60 years), acute and planned admitted to Vlietland Hospital. In addition, the prognostic value of the dichotomous risk factors for length of hospital stay was estimated as indicator of treatment complications. The ten clinically relevant risk factors were home care, history of falling, medication (> 4), weight loss, cognitive level and functioning, self-care, psychiatric symptoms, health status and quality of life. RESULTS: There was a high prevalence of risk factors; 47.5% of the elderly patients had four or more risk factors at intake. Home care and global cognitive deterioration were significant predictors of longer length of hospital stay. Furthermore, acute admission, weight loss, psychiatric symptoms and health status seemed important. The explained variance of the prognostic model was relatively small. CONCLUSION: The findings in this explorative-observational study showed a high prevalence of clinically relevant, patient-bound risk factors in elderly people in a general hospital. Some risk-factors were of prognostic interest for long hospital stay, although the explained variance was relatively small. This indicates that a more comprehensive study should be designed and conducted to include other patient-bound risk factors like co-morbidity, caregiver issues and social environment. Moreover, non-patient-bound factors should be addressed like intrinsic and logistic factors within the hospital, and the quality of recuperation programmes. Understanding of these factors contributes to timely identification of elderly patients, who are at high risk of a long hospital stay. Future policy is to perform specific treatment programmes for elderly patients identified as being patients at risk. Multidisciplinary person-oriented interventions and case management focussed on risk factors and functional recovery will be provided parallel and after hospital treatment period. Comprehensive scientific research on the cost-effectiveness of such a programme has started at the end of 200oo9 in Vlietland Hospital, Schiedam.

Turkish female immigrants' intentions to participate in preconception carrier screening for hemoglobinopathies in the Netherlands: an empirical study.

BACKGROUND: Preconception carrier screening for hemoglobinopathies (HbPs) is debated in the Netherlands. OBJECTIVES: Intentions to participate in preconception carrier screening for HbPs as well as informed reproductive options were assessed in 109 Turkish female immigrants. METHOD: Participants completed a structured questionnaire. RESULTS: 83.5% of 109 Turkish female immigrants intended to participate in preconception carrier screening. Intention to participate was related to the desire to reduce uncertainty about having offspring affected by HbPs, but not with socio-demographic variables, risk-estimation and worrying. If the tests concerning carrier status for HbPs were confirmative for both partners, 36.3% (n = 33) of the women with the intention of preconception carrier screening would refrain from getting children of their own, including 14.3% (n = 13) opting for adoption, 37.4% (n = 34) would decide to become pregnant and to opt for prenatal testing on HbPs. Finally, among 37.4% of the women opting for prenatal testing, 30.3% (n = 10) would terminate an affected pregnancy, 18.2% (n = 6) not and 51.5% (n = 17) could not decide (1 missing value). CONCLUSION: A large majority of Turkish female immigrants would participate in preconception carrier screening for HbPs.

Psychological well-being and sexarche in women with polycystic ovary syndrome.

BACKGROUND: The characteristics of polycystic ovary syndrome (PCOS) such as hyperandrogenism and anovulation can be highly stressful and might negatively affect psychological well-being and sexuality. The objective of this study was to evaluate the association between PCOS characteristics and psychological well-being as well as sexarche. METHODS: Patients (n = 1148) underwent standardized clinical evaluation. Psychological well-being was investigated in 480 patients with the Rosenberg self-esteem scale (RSES), the body cathexis scale (BCS) and the fear of negative appearance evaluation scale (FNAES). Sexarche was also assessed. RESULTS: Amenorrhoea was associated with lower self-esteem (P = 0.03), greater fear of negative appearance evaluation (P = 0.01) and earlier sexarche (P= 0.004). Hyperandrogenism and acne were associated with poorer body satisfaction (P = 0.03, 0.02, respectively). Hirsutism and BMI were negatively associated with all psychological variables (RSES, P = 0.01; BCS, P = 0.05; FNAES, P = 0.02 and RSES, P = 0.03; BCS, P = 0.001; FNAES, P = 0.03, respectively). CONCLUSIONS: Our results suggest that menstrual irregularities might be related to sexarche. Moreover, this study stresses that the treatment of women with PCOS should notably focus on physical but also on psychological and sexual characteristics.

Satisfaction with facial appearance and its determinants in adults with severe congenital facial disfigurement: a case-referent study.

BACKGROUND: Patients with severe congenital facial disfigurement have a long track record of operations and hospital visits by the time they are 18 years old. The fact that their facial deformity is congenital may have an impact on how satisfied these patients are with their appearance. This study evaluated the level of satisfaction with facial appearance of congenital and of acquired facially disfigured adults, and explored demographic, physical and psychological determinants of this satisfaction. Differences compared with non-disfigured adults were examined. METHODS: Fifty-nine adults with a rare facial cleft, 59 adults with a facial deformity traumatically acquired in adulthood, and a reference group of 201 non-disfigured adults completed standardised demographic, physical and psychological questionnaires. RESULTS: The congenital and acquired groups did not differ significantly in the level of satisfaction with facial appearance, but both were significantly less satisfied than the reference group. In facially disfigured adults, level of education, number of affected facial parts and facial function were determinants of the level of satisfaction. High fear of negative appearance evaluation by others (FNAE) and low self-esteem (SE) were strong psychological determinants. Although FNAE was higher in both patient groups, SE was similar in all three groups. CONCLUSION: Satisfaction with facial appearance of individuals with a congenital or acquired facial deformity is similar and will seldom reach the level of satisfaction of non-disfigured persons. A combination of surgical correction (with attention for facial profile and restoring facial functions) and psychological help (to increase SE and lower FNAE) may improve patient satisfaction.

Stigma moderates the associations of insight with depressed mood, low self-esteem, and low quality of life in patients with schizophrenia spectrum disorders.

BACKGROUND: Good insight into illness in patients with schizophrenia is related not only to medication compliance and high service engagement, but also to depression, low self-esteem, and low quality of life. The detrimental effects of insight pose a problem for treatment. AIM: To investigate whether the negative associations of good insight are moderated by perceived stigma. METHOD: Respondents were 114 patients with schizophrenia spectrum disorders. We used Analyses of Variance (ANOVA) and Structural Equation Modeling (SEM) to test moderation. RESULTS: Good insight was associated with high service engagement and high compliance. Also, good insight was associated with depressed mood, low quality of life, and negative self-esteem. This association was strong when stigma was high and weak when stigma was low. SEM showed that the constrained model performed significantly worse than the unconstrained model, in which detrimental associations of insight were free to vary across stigma groups (chi(2)=19.082; df=3; p<.001). CONCLUSIONS: Our results suggest that the associations of insight with depression, low quality of life, and negative self-esteem are moderated by stigma. Patients with good insight who do not perceive much stigmatization seem to be best off across various outcome parameters. Those with poor insight have problems with service engagement and medication compliance. Patients with good insight accompanied by stigmatizing beliefs have the highest risk of experiencing low quality of life, negative self-esteem, and depressed mood. A clinical implication is that when it is attempted to increase insight, perceived stigma should also be addressed.

Fewer symptoms vs. more side-effects in schizophrenia? Opposing pathways between antipsychotic medication compliance and quality of life.

BACKGROUND: Non-compliance with medication often has long-term detrimental effects in patients with schizophrenia. However, when patients are compliant, it is not certain whether they experience short-term improved quality of life. By simultaneously reducing symptoms and increasing side-effects, compliance with antipsychotics may have opposing effects on a patient's perceived quality of life. AIM: This study aimed to identify any clinical-empirical evidence for two pathways between compliance and quality of life. METHOD: To evaluate various pathways between compliance (Service Engagement Scale plus a one-item rating), psychotic symptoms (Positive and Negative Syndromes Scale), adverse medication effects (Subjective Wellbeing under Neuroleptics scale), and quality of life (EQ-5D), we used Structural Equation Modeling on cross-sectional data of 114 patients with a psychotic disorder. RESULTS: Compliance was not directly related to quality of life (r=0.004). The best-fitting model (chi(2)=1.08; df=1) indicated that high compliance was associated with fewer psychotic symptoms (beta=-0.23) and more adverse medication effects (beta=0.22). Symptoms (beta=-0.17) and adverse medication effects (beta=-0.48) were both related to lower quality of life. DISCUSSION: Our results suggest that compliance with antipsychotics has two opposing pathways towards quality of life, albeit indirect ones. While compliance was associated with less severe psychotic symptoms, and was thus related to higher quality of life, it was also associated with more adverse medication effects, and was thus related to lower quality of life. However, due to our study design, we cannot draw firm conclusions on causality. Two possible clinical implications of the results for compliance and interventions are discussed.

Metamemory and memory test performance in stroke patients.

Memory Self-Efficacy (MSE) has been shown to be related to memory performance and social participation in a healthy elderly population. This relation is unclear in stroke. As about 30% of all stroke survivors report memory complaints, there is an urgent need for effective treatment strategies. Before implementing MSE as a potential target in memory training, it should be examined whether the association between MSE and memory performance demonstrated in healthy elderly people also applies in stroke patients. This study therefore explored the predictive value of MSE on two kinds of memory tests in stroke patients; adjusted and unadjusted for age, gender, education and location of stroke. In 57 stroke patients, the Metamemory in Adulthood Questionnaire (MIA), an everyday memory test (RBMT) and a more traditional memory test (AVLT) were completed. The results show that MSE significantly predicts memory test performance on both memory tests (RBMT: beta = .34; p = .01 AVLT: beta = .28; p = .04). When adjusted for gender, age, education and location of stroke, the predictive value of MSE remained significant for the AVLT (RBMT: beta = .23; p = .07; AVLT: beta = .23; p = .05). The results support the hypothesis that MSE predicts test performance in stroke patients and, by consequence, enables improving memory performance in post-acute memory rehabilitation after stroke.

[A randomised clinical trial on the effectiveness of a re-integration training after short-term inpatient psychotherapy]. / De effectiviteit van re-integratietraining versus boostersessies na kortdurende klinische psychotherapie: een gerandomiseerd klinisch onderzoek.

BACKGROUND: Standardised evaluation studies performed in the Netherlands in a large number of inpatient and day-treatment hospitals providing psychotherapy have shown significant symptomatic improvements in patients between the date of entry to the studies and follow-up after one year. However, the work situation of ex-patients hardly changed and a large number of patients were still receiving psychotherapy. AIM: To examine the effectiveness of a specifically designed course of re-integration training. METHOD: A group of 128 patients were assigned randomly either to a re-integration training course aimed at improved functioning at work and improved relationships, or to booster sessions. Outcome measures were symptom level, work status, absence from work, and further psychotherapy. results After two years the number of patients in paid employment remained the same (76%) in the re-integration training course and increased from 67 to 87% in the booster sessions. Attendance was significantly higher in the booster sessions than in the re-integration training. There were no differences in the other outcome measures. CONCLUSION: We conclude that re-integration training was no more effective than the booster sessions. Our hypothesis is that continuity of care (therapists plus programme) explains the favourable result of the booster sessions.

The Distress Thermometer assessed in women at risk of developing hereditary breast cancer.

OBJECTIVES: The Distress Thermometer (DT) is a promising instrument to get insight into distress experienced by cancer patients. At our Family Cancer Clinic the DT, including an adapted problem list, was completed by 100 women at increased risk of developing hereditary breast cancer (mean age 45.2 years; SD: 10.5). Additionally, the women filled in either the Hospital Anxiety and Depression Scale as psychological component (n=48) or the somatic subscale of the Symptom Checklist-90 as somatic component (n=50) to identify associations with the DT-score. Further, the women filled in an evaluation form. RESULTS: The median score on the DT was 2 (range: 0-9). With regression analysis adjusted for age, the contribution of mood and somatic complaints, respectively, was investigated. The standardized regression coefficient for anxiety was 0.32 (ns), for depression 0.14 (ns) and for the somatic subscale 0.49 (p<0.001). The explained variance for anxiety and depression was 16%, and for somatic complaints 24%. The differences between the coefficients were not significant. Evaluation forms were returned by 73 women. In 50% of the cases, the physician had discussed the DT/problem list, which was appreciated by the majority of these women (80%). Sixty-two percent of the women would recommend the use of the DT for other patients. CONCLUSION: The use of the DT/problem list seems promising for the current population, and was appreciated by the majority of the women. As mood and somatic complaints did not differ significantly in explaining the experienced distress, other candidate factors need to be examined.

Long-term cognitive functioning in children and adolescents who survived septic shock caused by Neisseria meningitidis.

AIMS: To assess long-term cognitive functioning and its predictors, in children and adolescents who survived meningococcal septic shock (MSS) 4 to 16 years ago. METHODS: The Wechsler Intelligence Scale for Children-third edition was used to measure intellectual functioning and neuropsychological tests were used to measure attention, verbal memory, visual-motor integration, and executive skills. RESULTS: Overall, results of the total MSS sample (N=77) as to neuropsychological functioning were similar to those of normative reference groups. On social and practical reasoning and visual-motor integration, however, MSS children obtained poorer outcomes compared to normative data. Two children had mental retardation (estimated IQ<70) due to the MSS. The percentage of children with mental retardation or borderline intellectual functioning (15%) was similar to that in the general population (16%). Eighteen children (23%) had a z score<-2, indicating unusual poor functioning, on one or more domains of neuropsychological functioning (selective attention, sustained attention, and executive functioning). Compared to normative data, significantly more children had received special education services in the past. Older age at time of follow-up was the most important significant predictor of poorer long-term cognitive functioning. CONCLUSION: Overall, long-term outcomes as to cognitive functioning of the total MSS sample were similar to those of normative reference groups, but MSS children showed long-term impairments on social and practical reasoning, visual-motor integration, attention, and executive functioning. Older age at time of follow-up was a significant predictor.

Validation of a questionnaire assessing patient's aesthetic and functional outcome after nasal reconstruction: the patient NAFEQ-score.

In determining patient satisfaction with functional and aesthetic outcome after reconstructive surgery, including nasal reconstruction, standardised assessment instruments are very important. These standardised tools are needed to adequately evaluate and compare outcome results. Since no such instrument existed for nasal reconstruction, a standardised evaluation questionnaire was developed to assess aesthetic and functional outcome after nasal reconstruction. Items of the Nasal Appearance and Function Evaluation Questionnaire (NAFEQ) were derived from both the literature and experiences with patients. The NAFEQ was validated on 30 nasal reconstruction patients and a reference group of 175 people. A factor analysis confirmed the arrangement of the questionnaire in two subscales: functional and aesthetic outcome. High Cronbach's alpha values (>0.70) for both subscales showed that the NAFEQ was an internally consistent instrument. This study demonstrated that the NAFEQ can be used as a standardised questionnaire for detailed evaluation of aesthetic and functional outcome after nasal reconstruction. Its widespread use would enable comparison of results achieved by different techniques, surgeons and centres in a standardised fashion.

Intelligence of very preterm or very low birthweight infants in young adulthood.

OBJECTIVE: To examine the effect of intrauterine and neonatal growth, prematurity and personal and environmental risk factors on intelligence in adulthood in survivors of the early neonatal intensive care era. METHODS: A large geographically based cohort comprised 94% of all babies born alive in the Netherlands in 1983 with a gestational age below 32 weeks and/or a birth weight >1500 g (POPS study). Intelligence was assessed in 596 participants at 19 years of age. Intrauterine and neonatal growth were assessed at birth and 3 months of corrected age. Environmental and personal risk factors were maternal age, education of the parent, sex and origin. RESULTS: The mean (SD) IQ of the cohort was 97.8 (15.6). In multiple regression analysis, participants with highly educated parents had a 14.2-point higher IQ than those with less well-educated parents. A 1 SD increase in birth weight was associated with a 2.6-point higher IQ, and a 1-week increase in gestational age was associated with a 1.3-point higher IQ. Participants born to young mothers (<25 years) had a 2.7-point lower IQ, and men had a 2.1-point higher IQ than women. The effect on intelligence after early (symmetric) intrauterine growth retardation was more pronounced than after later (asymmetric) intrauterine or neonatal growth retardation. These differences in mean IQ remained when participants with overt handicaps were excluded. CONCLUSIONS: Prematurity as well as the timing of growth retardation are important for later intelligence. Parental education, however, best predicted later intelligence in very preterm or very low birthweight infants.

Mental and motor development before and during growth hormone treatment in infants and toddlers with Prader-Willi syndrome.

BACKGROUND: Prader-Willi syndrome (PWS) is a neurogenetic disorder characterized by muscular hypotonia, psychomotor delay, feeding difficulties and failure to thrive in infancy. GH treatment improves growth velocity and body composition. Research on the effects of GH on psychomotor development in infants with PWS is limited. OBJECTIVE: To evaluate psychomotor development in PWS infants and toddlers during GH treatment compared to randomized controls. DESIGN/PATIENTS: Forty-three PWS infants were evaluated at baseline. Twenty-nine of them were randomized into a GH group (n = 15) receiving 1 mg/m(2)/day GH or a non-GH-treated control group (n = 14). At baseline and after 12 months of follow-up, analysis with Bayley Scales of Infant Development II (BSID-II) was performed. Data were converted to percentage of expected development for age (%ed), and changes during follow-up were calculated. RESULTS: Infants in the GH group had a median age of 2.3 years [interquartile range (IQR) 1.7-3.0] and in the control group of 1.5 years (IQR 1.2-2.7) (P = 0.17). Both mental and motor development improved significantly during the first year of study in the GH group vs. the control group: median (IQR) change was +9.3% (-5.3 to 13.3) vs.-2.9% (-8.1 to 4.9) (P < 0.05) in mental development and +11.2% (-4.9 to 22.5) vs.-18.5% (-27.9 to 1.8) (P < 0.05) in motor development, respectively. CONCLUSION: One year of GH treatment significantly improved mental and motor development in PWS infants compared to randomized controls.

Thyroid hormone levels in children with Prader-Willi syndrome before and during growth hormone treatment.

BACKGROUND: Prader-Willi syndrome (PWS) is a neurogenetic disorder characterized by muscular hypotonia, psychomotor delay, obesity and short stature. Several endocrine abnormalities have been described, including GH deficiency and hypogonadotrophic hypogonadism. Published data on thyroid hormone levels in PWS children are very limited. OBJECTIVE: To evaluate thyroid function in children with PWS, before and during GH treatment. DESIGN/PATIENTS: At baseline, serum levels of T4, free T4 (fT4), T3, reverse T3 (rT3) and TSH were assessed in 75 PWS children. After 1 year, assessments were repeated in 57 of the them. These children participated in a randomized study with two groups: group A (n = 34) treated with 1 mg GH/m(2)/day and group B (n = 23) as controls. RESULTS: Median age (interquartile range, IQR) of the total group at baseline was 4.7 (2.7-7.6) years. Median (IQR) TSH level was -0.1 SDS (-0.5 to 0.5), T4 level -0.6 SDS (-1.7 to 0.0) and fT4 level -0.8 SDS (-1.3 to -0.3), the latter two being significantly lower than 0 SDS. T3 level, at 0.3 SDS (-0.3 to 0.9), was significantly higher than 0 SDS. After 1 year of GH treatment, fT4 decreased significantly from -0.8 SDS (-1.5 to -0.2) to -1.4 SDS (-1.6 to -0.7), compared to no change in untreated PWS children. However, T3 did not change, at 0.3 SDS (-0.1 to 0.8). CONCLUSIONS: We found normal fT4 levels in most PWS children. During GH treatment, fT4 decreased significantly to low-normal levels. TSH levels remained normal. T3 levels were relatively high or normal, both before and during GH treatment, indicating that PWS children have increased T4 to T3 conversion.

Frontal presentation in progressive supranuclear palsy.

BACKGROUND: Progressive supranuclear palsy (PSP) is a progressive hypokinetic rigid disorder with supranuclear gaze palsy and frequent falls. Although clinical consensus criteria are available, an atypical presentation may lead to clinical misdiagnosis in the initial phase. In the present study we investigated the clinical presentation of PSP and its relationship to initial clinical diagnosis and survival. METHODS: We ascertained patients with PSP in a prospective cohort by nationwide referral from neurologists and nursing home physicians. All patients underwent a structural interview and clinical examination before entering the study. Medical records were reviewed for the presence of symptoms during the first 2 years. RESULTS: A total of 152 patients ascertained between 2002 and 2005 fulfilled the international consensus criteria for PSP. Categorical principal component analysis of clinical symptoms within the first 2 years showed apart from a cluster of typical PSP symptoms, the clustering of cognitive dysfunction and behavioral changes. Further analysis showed that 20% of patients had a predominant frontal presentation with less than two other PSP symptoms. Survival analysis showed that this subgroup had a similar prognosis to that of the total group of patients with PSP. CONCLUSIONS: There exists a subgroup of patients with progressive supranuclear palsy (PSP) with a predominant frontal presentation, who progressed into typical PSP over the course of the disease.

Who is prone to high levels of distress after prophylactic mastectomy and/or salpingo-ovariectomy?

BACKGROUND: The present study aimed to assess predictors of distress after 'prophylactic mastectomy (PM) and salpingo-ovariectomy (PSO), in order to enable the early identification of patients who could benefit from psychological support. PATIENTS AND METHODS: General distress and cancer-related distress were assessed in 82 women at increased risk of hereditary breast and/or ovarian cancer undergoing PM and/or PSO, before and 6 and 12 months after prophylactic surgery. Neurotic lability and coping were assessed before surgery. RESULTS: Cancer-related distress and general distress at both follow-up moments were best explained by the level of cancer-related and general distress at baseline. Being a mutation carrier was predictive of increased cancer-related distress at 6-month follow-up (but not at 12 months), and of lower general distress 12 months after prophylactic surgery. Also, coping by having comforting thoughts was predictive of less cancer-related distress at 6-month follow-up. CONCLUSIONS: Genetically predisposed women who are at risk of post-surgical distress can be identified using one or more of the predictors found in this study. Exploration of and/or attention to cancer-related distress and coping style before prophylactic surgery may help physicians and psychosocial workers to identify women who might benefit from additional post-surgical support.