Metabolic patterns in brain 18F-fluorodeoxyglucose PET relate to aetiology in paediatric dystonia.

There is a lack of imaging markers revealing the functional characteristics of different brain regions in paediatric dystonia. In this observational study, we assessed the utility of [18F]2-fluoro-2-deoxy-D-glucose (FDG)-PET in understanding dystonia pathophysiology by revealing specific resting awake brain glucose metabolism patterns in different childhood dystonia subgroups. PET scans from 267 children with dystonia being evaluated for possible deep brain stimulation surgery between September 2007 and February 2018 at Evelina London Children's Hospital (ELCH), UK, were examined. Scans without gross anatomical abnormality (e.g. large cysts, significant ventriculomegaly; n = 240) were analysed with Statistical Parametric Mapping (SPM12). Glucose metabolism patterns were examined in the 144/240 (60%) cases with the 10 commonest childhood-onset dystonias, focusing on nine anatomical regions. A group of 39 adult controls was used for comparisons. The genetic dystonias were associated with the following genes: TOR1A, THAP1, SGCE, KMT2B, HPRT1 (Lesch Nyhan disease), PANK2 and GCDH (Glutaric Aciduria type 1). The acquired cerebral palsy (CP) cases were divided into those related to prematurity (CP-Preterm), neonatal jaundice/kernicterus (CP-Kernicterus) and hypoxic-ischaemic encephalopathy (CP-Term). Each dystonia subgroup had distinct patterns of altered FDG-PET uptake. Focal glucose hypometabolism of the pallidi, putamina or both, was the commonest finding, except in PANK2, where basal ganglia metabolism appeared normal. HPRT1 uniquely showed glucose hypometabolism across all nine cerebral regions. Temporal lobe glucose hypometabolism was found in KMT2B, HPRT1 and CP-Kernicterus. Frontal lobe hypometabolism was found in SGCE, HPRT1 and PANK2. Thalamic and brainstem hypometabolism were seen only in HPRT1, CP-Preterm and CP-term dystonia cases. The combination of frontal and parietal lobe hypermetabolism was uniquely found in CP-term cases. PANK2 cases showed a distinct combination of parietal hypermetabolism with cerebellar hypometabolism but intact putaminal-pallidal glucose metabolism. HPRT1, PANK2, CP-kernicterus and CP-preterm cases had cerebellar and insula glucose hypometabolism as well as parietal glucose hypermetabolism. The study findings offer insights into the pathophysiology of dystonia and support the network theory for dystonia pathogenesis. 'Signature' patterns for each dystonia subgroup could be a useful biomarker to guide differential diagnosis and inform personalized management strategies.

Health literacy of parents/caregivers of paediatric surgical patients: A study on 1000 individuals.

PURPOSE: There is a paucity of studies concerning health literacy (HL) of parents/guardians of patients in paediatric surgery. The purpose of our study is to measure HL levels of parents/guardians of paediatric surgery patients and to explore the determinants of low HL levels in this population. MATERIALS AND METHODS: We conducted a cross-sectional study from December 2016 to July 2018 through in-person interviews of parents/guardians of paediatric surgical patients. Sociodemographic and clinical data were recorded, and HL levels were calculated using a validated tool (HLS-EU-Q16). In order to examine the impact of various sociodemographic variables and clinical data on HL, a multivariate regression model was run. RESULTS: A total of 1000 participants were recruited (recruitment rate 93.5%). Slightly less than half (44.2%) presented problematic or inadequate HL levels. The results of the regression analysis showed that nationality other than Greek (ß = -2.180, P < 0.001) and lower health insurance status (ß = -0.461, P < 0.05) were associated with lower HL levels. HL was found positively associated with the educational level of the parent (ß = -0.775, P < 0.001) and being a health professional (ß = 1.791, P < 0.001). CONCLUSION: The prevalence of low HL levels in the parents/guardians of paediatric surgical patients is high and should not be neglected both in the pre-operative and post-operative setting. Communication should be tailored to the specific needs of each individual to achieve better engagement and quality of care.

Parental anxiety in pediatric surgery consultations: the role of health literacy and need for information.

INTRODUCTION: Although important, parental anxiety, health literacy and need-for-information in pediatric surgery outpatient clinics have not been extensively studied. Lower educational attainments, minorities and lower socioeconomic status have been associated with limited health literacy. Parental anxiety has been related to health literacy, sex, education and information needs. The aim of this study is to investigate health literacy and need-for-information and their association to parental anxiety in consultations of pediatric surgery. MATERIALS & METHODS: We conducted an observational, cross-sectional study in the outpatient pediatric surgery clinic from December 2016 to October 2017. Health literacy, anxiety and need-for-information of parents/guardians of children waiting for pediatric surgical consultation were evaluated. Multivariate regression analysis was used to examine the impact of health literacy and need-for-information on parental/guardian anxiety considering sociodemographic and clinical characteristics of the participants. RESULTS: Almost half (46.1%) of the 664 parents/guardians recruited had limited or problematic health literacy and 79.8% of the sample was classified as being anxious. Parental/guardian anxiety was associated at the multiple regression analysis with parental health literacy level (ß = -0.282, p < 0.001), need-for-information preoperatively (ß = 0.907, p < 0.001), educational level (ß = -0.716, p = 0.001), sex (ß = 1.563, p < 0.001), and severity of the condition of the child (ß = 0.379, p < 0.001). CONCLUSION: Parents/guardians experience high levels of anxiety, which is associated to health literacy and need-for-information. These factors should be considered in pediatric surgical consultations, aiming to reduce parental anxiety. TYPE OF STUDY: Retrospective Study. LEVEL OF EVIDENCE: Level II.