Gerben Sinnema

Social functioning in children with a chronic illness

Behavioural, cognitive, and affective aspects of social functioning of 107 children with a chronic illness were studied. The aim of the study was twofold: (1) to describe peer interaction of children with a chronic illness in comparison with normative data of healthy children; (2) to examine whether peer interaction was related to the illness characteristics physical restrictions and pain. Peer interaction was assessed with measures of social activities (CBCL), parent-reported social skills (CABS), child-reported social skills (MESSY), social self-esteem (SPPC), and social anxiety (SASK). Results showed no differences between diagnosis groups, suggesting that the social consequences of chronic illness are not diagnosis specific. Compared with healthy norms, chronically ill children reported less aggressive behaviour. The parent-report measures suggested a similar trend. Children with chronic illness also tended to display more submissive behaviour than healthy norms, as perceived by their parents. With regard to illness characteristics, both physical restrictions and pain were associated with restricted social activities, but not with other measures of social peer interaction. Children who display submissive behaviour and children who are restricted in their social activities should receive extra attention because they are especially vulnerable for problems in their social development.

Disturbed Neuroendocrine-Immune Interactions in Chronic Fatigue Syndrome

The present study was designed to investigate the interaction between neuroendocrine mediators and the immune system in chronic fatigue syndrome (CFS). We examined the sensitivity of the immune system to the glucocorticoid agonist dexamethasone and the beta2-adrenergic agonist terbutaline in 15 adolescent girls with CFS and 14 age- and sex-matched controls. Dexamethasone inhibits T-cell proliferation in healthy controls and in CFS patients. However, the maximal effect of dexamethasone on T-cell proliferation is significantly reduced in CFS patients as compared with controls. The beta2-adrenergic receptor agonist terbutaline inhibits tumor necrosis factor-alpha production and enhances interleukin-10 production by monocytes. Our data demonstrate that the capacity of a beta2-adrenergic agonist to regulate the production of these two cytokines is also reduced in CFS patients. We did not observe differences in baseline or CRH-induced cortisol and ACTH between CFS patients and controls. Baseline noradrenaline was similar in CFS and controls, whereas baseline adrenaline levels were significantly higher in CFS patients. We conclude that CFS is accompanied by a relative resistance of the immune system to regulation by the neuroendocrine system. Based on these data, we suggest CFS should be viewed as a disease of deficient neuroendocrine-immune communication.

High prevalence of methotrexate intolerance in juvenile idiopathic arthritis: development and validation of a methotrexate intolerance severity score

OBJECTIVE To design and validate a new questionnaire for identifying patients with methotrexate (MTX) intolerance, and to determine the prevalence of MTX intolerance in patients with juvenile idiopathic arthritis (JIA) using this questionnaire. METHODS The MTX Intolerance Severity Score (MISS) questionnaire was constructed, consisting of 5 domains: stomach ache, nausea, vomiting, sore mouth, and behavioral symptoms. The domains each consisted of 3 questions pertaining to the presence of a symptom upon, prior to (anticipatory), and when thinking of (associative) MTX intake. The MISS questionnaire was validated in 86 patients by determining its discriminative power between patients with and those without MTX intolerance, identified as such by a gold standard (physicians opinion). Using the MISS questionnaire, the prevalence of MTX intolerance was determined in 297 JIA patients. RESULTS The MISS questionnaire discriminated well between MTX-intolerant and MTX-tolerant patients. A cutoff score of 6 yielded the best sensitivity (88%) and specificity (80%). MTX intolerance was found in 150 (50.5%) of 297 patients. Of 220 patients receiving oral MTX, 98 (44.5%) experienced MTX intolerance, whereas 67.5% of 77 patients receiving parenteral MTX experienced intolerance to the drug (P = 0.001). CONCLUSION Our findings indicate that the MISS questionnaire is a highly sensitive and specific tool for the diagnosis of MTX intolerance, and that there is a high prevalence of MTX intolerance among JIA patients. The prevalence of intolerance in patients receiving parenteral MTX exceeds that in patients receiving oral MTX. The frequent occurrence of anticipatory and associative symptoms suggests that classic conditioning plays an important role in MTX intolerance.

Quality of life in chronic illness: perceptions of parents and paediatricians

Aims: To investigate the differences in perception of quality of life between parents of chronically ill children and paediatricians at diagnosis and follow up. Quality of life was assessed using the (HUI3). Methods: Longitudinal study (July 1999–January 2002) of 37 paediatricians and 181 parents of patients (children aged 1–17 years) with cystic fibrosis admitted for a pneumonia or patients with newly diagnosed acute lymphatic leukaemia, juvenile idiopathic arthritis, or asthma. Main outcome measure was percentage agreement on the attributes of the HUI3 between parents and paediatricians. Results: Differences in perception of health and wellbeing between paediatricians and parents of children with a chronic disease were found, not only at diagnosis but also after a period of follow up. Differences were particularly clear in the subjective attributes emotion (range of agreement 28–68%) and pain/discomfort (range of agreement 11–33%). In all patient groups, at baseline and follow up, the paediatrician assessed the patient to have less pain/discomfort in comparison to the parents. Despite a prolonged patient- paediatrician relationship, differences at follow up did not decrease compared to baseline. Conclusion: At the onset of a chronic disease, but also after a period of follow up, quality of life of paediatric patients may be misunderstood by healthcare professionals, especially in the subjective attributes. Systematic assessment of quality of life may contribute to better understanding between physicians and parents.

Growing up with idiopathic short stature: psychosocial development and hormone treatment; a critical review

To facilitate decisions on interventions in medically referred children with idiopathic short stature, the research on psychosocial functioning of these children, possible risk and protective factors influencing adaptation, and effects of hormone treatment were reviewed. Parents ranked the behaviour of their children on average between normal and below normal. The magnitude of these deviations varied from small to large. Little is known about the children’s self-perceived psychosocial functioning. Some risk factors were found: being teased, being juvenilised, being a boy, having a younger but taller sibling, low intelligence, and low socioeconomic status. There have been few studies on the impact of protective factors including temperament, coping strategies, and social support. On average, hormone treatment did not improve psychosocial functioning. The research shows the advantages and disadvantages of hormone treatment that must be considered when choosing a suitable intervention. It is suggested that psychosocial adjustment can be improved by focusing on factors other than height alone.

Peer interaction in adolescents with a chronic illness

This study examined behavioural, cognitive and affective aspects of peer interaction of adolescents with a chronic illness. The aim of the study was twofold: (1) describe peer interaction of adolescents with a chronic illness in comparison with norms of healthy adolescents; (2) examine the relationship of peer interaction with the illness characteristics functional limitations and pain. Peer interaction of 98 adolescents with a chronic illness was assessed with measures of social activities (CBCL), social self-esteem (SPPA), self-reported social skills (MESSY, SIG-frequency) and social anxiety (SIG-anxiety). Results showed that diagnosis is related to social activities, but not to other aspects of peer interaction. Further, chronically ill girls had a lower level of social activities compared with healthy norms. In addition, girls with cystic fibrosis displayed more assertive behaviour than healthy girls, while chronically ill boys displayed less inadequate social skills than healthy boys. With regard to illness characteristics, functional limitations were not associated with peer interaction. However, pain was associated with restricted social activities in boys. It was concluded that in general, chronically ill adolescents were well adjusted socially and that functional limitations and pain do not seem to be risk factors for social adjustment problems. This healthy functioning might be a result of the presence of protective factors, such as the use of adequate coping strategies.

Long-term psychosocial consequences of hormone treatment for short stature

Aim: To examine psychosocial functioning of young adults with idiopathic short stature or short stature born small for gestational age after growth hormone (GH) and gonadotropin‐releasing hormone agonist (GnRHa) treatment in early adolescence or no intervention.

Quality of life in chronic illness: children, parents and paediatricians have different, but stable perceptions

Aim: Quality of life assessments can be helpful to estimate the well‐being of chronically ill children. The aim of this study was to investigate the differences in perception of health‐related quality of life (HRQoL) among children, parents and paediatricians at the time of diagnosis and after initial treatment in four chronic diseases.

Salivary cortisol and dehydroepiandrosterone sulfate in adolescent rape victims with post traumatic stress disorder.

BACKGROUND In chronic sexual abuse victims with post traumatic stress disorder (PTSD), the hypothalamic pituitary adrenal (HPA) axis can be dysregulated. In single rape victims, PTSD symptoms are hypothesized to function as a chronic stressor leading to similar HPA-axis dysregulation. The objective of the current study was to assess HPA-axis functioning in female adolescents with rape-related PTSD, but no prior sexual trauma, in comparison to non-victimized controls. METHOD Salivary cortisol and dehydroepiandrosterone sulfate (DHEAS) were measured in 52 female adolescent rape victims with PTSD and 37 healthy adolescents at 0, 15, 30, 45 and 60 min after awakening, both under basal conditions and after 0.5 mg dexamethasone administration. RESULTS Compared to age-matched controls, adolescent rape victims with PTSD showed significantly reduced cortisol and DHEAS levels. No group differences for the effect of dexamethasone suppression were found. Both the event of rape and PTSD diagnosis, and not factors such as sleep duration, smoking, education or oral contraceptives, accounted for the neuroendocrine differences between rape victims and controls. CONCLUSIONS The results show evidence for a dysregulated HPA-axis in female adolescent victims of single sexual trauma with PTSD. The finding of hypocortisolism is consistent with endocrine dysfunctioning in chronic sexual abuse victims and may have clinical implications with regard to treatment possibilities.

Impaired Cognitive Functioning in Patients with Tyrosinemia Type I Receiving Nitisinone

OBJECTIVE To examine cognitive functioning in patients with tyrosinemia type I treated with nitisinone and a protein-restricted diet. STUDY DESIGN We performed a cross-sectional study to establish cognitive functioning in children with tyrosinemia type I compared with their unaffected siblings. Intelligence was measured using age-appropriate Wechsler Scales. To assess cognitive development over time, we retrieved sequential IQ scores in a single-center subset of patients. We also evaluated whether plasma phenylalanine and tyrosine levels during treatment was correlated with cognitive development. RESULTS Average total IQ score in 10 patients with tyrosinemia type I receiving nitisinone was significantly lower compared with their unaffected siblings (71 ± 13 vs 91 ± 13; P = .008). Both verbal and performance IQ subscores differed (77 ± 14 vs 95 ± 11; P < .05 and 70 ± 11 vs 87 ± 15; P < .05, respectively). Repeated IQ measurements in a single-center subset of 5 patients revealed a decline in average IQ score over time, from 96 ± 15 to 69 ± 11 (P < .001). No significant association was found between IQ score and either plasma tyrosine or phenylalanine concentration. CONCLUSION Patients with tyrosinemia type I treated with nitisinone are at risk for impaired cognitive function despite a protein-restricted diet.