The present study demonstrated that psychological symptoms (e.g. anxiety) are frequent symptoms in the end-of-life care period and cause severe suffering in the children. Questions in terms of benefits and costs of cancer-directed therapy in the end-of-life care period need to be addressed in future prospective studies. Parents' perspective on their child's death and related end-of-life decisions highlighted the importance of communication between parents and the health care team. Future studies need to investigate potential barriers in the communication between parents and the team to optimise end-of-life decisions and hence, reduce parents' long-term distress. In line with the previous, the present data demonstrated that there is still a lack of routine contact from the health care team following the child's death despite existing guidelines. Research is therefore needed into the implementation of guidelines for routine contact into clinical practice following a child's death.

The Quantitative Sensory Testing (QST) protocol of the German research network on neuropathic pain (DFNS) encompassing all somatosensory modalities assesses the functioning of different nerve fibers and of central pathways. The aim of our study was: (1) to explore, whether this QST protocol is feasible for children, (2) to detect distribution properties of QST data and the impact of body site, age and gender and (3) to establish reference values for QST in children and adolescents. The QST protocol of the DFNS with modification of instructions and pain rating was used in 176 children aged 6.12-16.12years for six body sites. QST was feasible for children over 5years of age. ANOVAs revealed developmental, gender and body site differences of somatosensory functions similar to adults. The face was more sensitive than the hand and/or foot. Younger children (6-8years) were generally less sensitive to all thermal and mechanical detection stimuli but more sensitive to all pain stimuli than older (9-12years) children, whereas there were little differences between older children and adolescents (13-17years). Girls were more sensitive to thermal detection and pain stimuli, but not to mechanical detection and pain stimuli. Reference values differ from adults, but distribution properties (range, variance, and side differences) were similar and plausible for statistical factors. Our results demonstrate that the full QST protocol is feasible and valid for children over 5years of age with their own reference values.

To accurately localize a visual target in space despite eye movement-induced shifts of its retinal image, the brain must take into account both its retinal location and information about current eye position or at least the preceding eye displacement. We examined this ability with respect to saccadic eye movements by applying "double-step" stimuli, where the locations of two sequentially flashed target lights have to be fixated by two successive saccades performed after their disappearance. As the 2nd saccade will not start at the spatial location from which the 2nd target was seen, a dissonance arises between its retinal coordinates and the motor coordinates of the required 2nd saccade. Nevertheless, these saccades were performed quite accurately by 32 healthy human adults. To investigate the contribution of the cerebral cortex, we recorded horizontal double-step saccades in 35 patients with focal unilateral hemispheric lesions. Whereas frontal lesions impaired temporal properties, posterior parietal lesions caused spatial dysmetria or failure of even ipsiversive 2nd saccades following contraversive 1st saccades. This reflects an inability to compensate for retinospatial dissonance by using nonretinal information (corollary discharge) about eye displacement associated with a previous saccade into the contralesional hemifield. In conclusion, the parietal cortex is crucial for spatial constancy across saccades.

BackgroundPrevalence of pain as a recurrent symptom in children is known to be high, but little is known about children with high impairment from chronic pain seeking specialized treatment. The purpose of this study was the precise description of children with high impairment from chronic pain referred to the German Paediatric Pain Centre over a 5-year period.MethodsDemographic variables, pain characteristics and psychometric measures were assessed at the first evaluation. Subgroup analysis for sex, age and pain location was conducted and multivariate logistic regression applied to identify parameters associated with extremely high impairment.ResultsThe retrospective study consisted of 2249 children assessed at the first evaluation. Tension type headache (48%), migraine (43%) and functional abdominal pain (11%) were the most common diagnoses with a high rate of co-occurrence; 18% had some form of musculoskeletal pain disease. Irrespective of pain location, chronic pain disorder with somatic and psychological factors was diagnosed frequently (43%). 55% of the children suffered from more than one distinct pain diagnosis. Clinically significant depression and general anxiety scores were expressed by 24% and 19% of the patients, respectively. Girls over the age of 13 were more likely to seek tertiary treatment compared to boys. Nearly half of children suffered from daily or constant pain with a mean pain value of 6/10. Extremely high pain-related impairment, operationalized as a comprehensive measure of pain duration, frequency, intensity, pain-related school absence and disability, was associated with older age, multiple locations of pain, increased depression and prior hospital stays. 43% of the children taking analgesics had no indication for pharmacological treatment.ConclusionChildren with chronic pain are a diagnostic and therapeutic challenge as they often have two or more different pain diagnoses, are prone to misuse of analgesics and are severely impaired. They are at increased risk for developmental stagnation. Adequate treatment and referral are essential to interrupt progression of the chronic pain process into adulthood.

Results of the study are promising in at least 2 ways: (1) a multimodal inpatient program might stop the negative effects of chronic pain, disability, and emotional distress in children and adolescents, and (2) the exploration of clinical significance testing has demonstrated utility and can be applied to future effectiveness studies in pediatric pain.

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Impaired fetal movement causes malformations, summarized as fetal akinesia deformation sequence (FADS), and is triggered by environmental and genetic factors. Acetylcholine receptor (AChR) components are suspects because mutations in the fetally expressed gamma subunit (CHRNG) of AChR were found in two FADS disorders, lethal multiple pterygium syndrome (LMPS) and Escobar syndrome. Other AChR subunits alpha1, beta1, and delta (CHRNA1, CHRNB1, CHRND) as well as receptor-associated protein of the synapse (RAPSN) previously revealed missense or compound nonsense-missense mutations in viable congenital myasthenic syndrome; lethality of homozygous null mutations was predicted but never shown. We provide the first report to our knowledge of homozygous nonsense mutations in CHRNA1 and CHRND and show that they were lethal, whereas novel recessive missense mutations in RAPSN caused a severe but not necessarily lethal phenotype. To elucidate disease-associated malformations such as frequent abortions, fetal edema, cystic hygroma, or cardiac defects, we studied Chrna1, Chrnb1, Chrnd, Chrng, and Rapsn in mouse embryos and found expression in skeletal muscles but also in early somite development. This indicates that early developmental defects might be due to somite expression in addition to solely muscle-specific effects. We conclude that complete or severe functional disruption of fetal AChR causes lethal multiple pterygium syndrome whereas milder alterations result in fetal hypokinesia with inborn contractures or a myasthenic syndrome later in life.

Preliminary evidence suggests that parental catastrophizing about their child's pain may be important in understanding both parental responses to their child's pain and the child's pain experience. However, little is known about potential differences between mothers and fathers. There were three aims of the present study addressing this lack of knowledge: i) to investigate the three-factor structure of the German version of the Parental Pain Catastrophizing Scale (PCS-P) (Goubert et al., 2006) in mothers and fathers of children with chronic pain, ii) to explore differences between mothers and fathers in parental catastrophizing, iii) to investigate the contribution of parental catastrophizing on the child's chronic pain problem and pain-related parent behavior.In a sample of 128 mothers and fathers of paediatric chronic pain patients, the invariance of the PCS-P was evaluated. Results replicated the previously established three-factor structure (i.e. rumination, magnification and helplessness) in both groups. Mothers reported higher levels of catastrophizing as compared to fathers.Specifically, mothers and fathers differed on levels of rumination; the two groups did not differ in magnification and helplessness. Maternal but not paternal catastrophizing contributed significantly in explaining the child's pain intensity whereas neither mothers' nor fathers' catastrophizing were significantly related to the child's disability. Both maternal and paternal catastrophizing contributed significantly to heightened parental solicitous responses. Fathers' but not mothers' catastrophizing also contributed to heightened distracting responses. The present findings attest to the importance of maternal and paternal catastrophizing for the child's pain characteristics and pain-related parent behavior, which are both relevant for treatment conceptualization.3