Changes in Child Behaviour Following a Brief Parenting Intervention (Triple P) for Families of Children with Phenylketonuria (PKU): A Case Series

Observations of teachers' use of noncontingent stimulation to elicit behavior from young children with multiple disabilities and profound developmental delays led us to evaluate the relative efficiency of response-contingent and response-independent stimulation to affect child behavior change. Data from three children (2 females, 1 male) with multiple disabilities and delays were analyzed to determine how many child contingency, visual attention, and social affective behavior would be produced per 100 learning opportunities under contrasting stimulus conditions (contingent vs. noncontingent). Results showed for all three child behaviors, response-contingent stimulation was overwhelmingly more efficient in affecting behavior change compared to response-independent stimulation. Implications for intervention are described. Keywords: Response-contingent stimulation, response-independent stimulation, child operant learning, concomitant child behavior, efficiency ********** More than 25 years ago, we initiated a line of research and practice investigating the value of response-contingent learning opportunities with young children with multiple disabilities and profound developmental delays (e.g., Dunst, 1981; Dunst, Cushing, & Vance, 1985; Dunst & Didoha, 1976; Laub & Dunst, 1974). This work was begun in response to experience showing that traditional early intervention and therapy was not effective in promoting the learning and development of these children. Observations of many practitioners over many years have found that the interventions they used with young children with profound developmental delays more often than not involved response-independent or noncontingent stimulation to elicit or evoke child behavior. In most cases, the more disabled and delayed the children, the greater the likelihood that practitioners would spend large amounts of time using noncontingent stimulation to attempt to affect changes in child behavior. The influences of response-contingent and response-independent stimulation on child learning and behavior has been the focus of investigation for many years (e.g., Dunst, 1984; O'Brien, 1992; Utley, Duncan, Strain, & Scanlon, 1983; Vietze, Foster, & Friedman, 1974). Response-contingent stimulation involves the provision of stimulation contingent upon a child's behavior, whereas response-independent stimulation involves the provision of stimulation that is noncontingent or nondependent upon a child's behavior. Findings from studies indicate that noncontingent stimulation at least initially elicits child attention and increased behavior responding, but that children habituate to the response-independent stimulation the longer it is available. In contrast, response-contingent stimulation elicits and maintains child behavior responding for longer periods of time and is often associated with positive social-emotional behavior following contingency detection and awareness (see e.g., Dunst, 2003). The purpose of the analyses in this brief report was to determine the relative efficiency of response-contingent and response-independent stimulation on child operant learning and two concomitant child behavior (visual attention and affective behavior). The analyses were completed on data collected as part of a study promoting teachers' use of contingency games as a form of early childhood intervention with young children with multiple disabilities and profound developmental delays (Raab, Dunst, Wilson, & Parkey, 2007). During the baseline condition of the study, teachers were observed using noncontingent stimulation to elicit child behavior. The effect appeared to be behavior suppression rather than behavior enhancement. The extent to which this observation was confirmed in a secondary analysis of the study data was the focus of this report. The conduct of the original study was guided by a conceptual and operational framework that postulated both immediate and extended benefits of contingency learning opportunities (Dunst et al. …

Sanofi Pasteur’s new quadrivalent formulation of Fluzone, for example, sold out 24 hours after the vaccine’s availability was announced, Foster told Pharmacy Today. All of the 2013–14 infl uenza vaccines contain antigens for the following three strains: A/California/7/2009 (H1N1) pdm09like virus A(H3N2) virus antigenically like the cell–propagated prototype virus A/Victoria/361/2011 B/Massachusetts/2/2012-like virus The quadrivalents—Fluarix Quadrivalent (GlaxoSmithKline), Fluzone Quadrivalent (Sanofi Pasteur), and FluMist Quadrivalent (MedImmune)— also contain B/Brisbane/60/2008-likevirus antigen. Vaccine choices for healthy adults Table 1 on the next page summarizes available infl uenza vaccines for the 2013–14 season. Not all of these fl u vaccines are right for everyone, however. For most healthy adults younger than 65 years, any of the inactivated infl uenza vaccines listed in the table can be used. Healthy, nonpregnant patients aged 2 years to 49 years can receive FluMist Quadrivalent, a live, attenuated nasal spray infl uenza vaccine that replaces the trivalent formulation used in the previous fl u season. For patients 65 years or older, there’s an alternative: Fluzone HighDose (Sanofi Pasteur), a trivalent formulation with four times as much antigen as regular infl uenza vaccine. It is the fi rst and only vaccine designed specifi cally for older adults. Adults aged 18 years to 49 years with serious egg allergies have a new option in FluBlok (Protein Sciences), a trivalent product made using recombinant processes. If FluBlok supplies run out, the cell culture–derived trivalent Flucelvax may be used instead, according to Foster, because it contains only a miniscule amount of egg protein. FDA has not licensed Flucelvax for use with patients who have egg allergies, however, since the seed virus was grown in eggs, Foster said.

Εισαγωγή: Η παιδική παχυσαρκία αποτελεί μία επιδημία του σύγχρονου δυτικού κόσμου και ορίζεται λειτουργικά ως η υπέρμετρη αύξηση του λιπώδους ιστού. Η παχυσαρκία αποτελεί ανεξάρτητο παράγοντα κινδύνου για την ανάπτυξη μίας πληθώρας συνοσηροτήτων όπως την αντίσταση στην ινσουλίνη, το σακχαρώδη διαβήτη τύπου 2, καρδιοαγγειακά νοσήματα και μεταβολικό σύνδρομο. Ο λιπώδης ιστός είναι ένα παρακρινές και ενδοκρινές όργανο, το οποίο μέσω της έκκρισης κυτταροκινών και φλεγμονογόνων παραγόντων έχει την ικανότητα να ρυθμίσει το ενεργειακό ισοζύγιο του οργανισμού. Η αντιπονεκτίνη μία από τις πιο σημαντικές κυτταροκίνες του λιπώδους ιστού, μέσω των υποδοχέων της AdipoR1 και AdipoR2, ενεργοποιεί την ινσουλινοεπαγώμενη πρόσληψη της γλυκόζης από το λιποκύτταρο, ενώ έχει αντι-φλεγμονώδης και αντι-αθηρωματική δράση σε άλλους ιστούς του οργανισμού. Ο PPAR-γ, ανήκει στην υπερ-οικογένεια των πυρηνικών υποδοχέων PPARs (peroxisome proliferative-activated receptors) και είναι ένας μεταγραφικός παράγοντας, ο οποίος σε ανταπόκριση στα κυκλοφορούντα ελεύθερα λιπαρά οξέα, ενεργοποιεί τη διαφοροποίηση των προλιποκυττάρων σε ώριμα λιποκύτταρα μικρού μεγέθους με πολλά λιποσταγονίδια. Το PPAR-γ μέσω της ενεργοποίησης του από τους ενδογενείς υποκαταστάτες του, τις θειαζολιδινεδιόνες, επάγει την ινσουλινοευαισθησία και αυξάνει την έκφραση της αντιπονεκτίνης. Τα ενδοκανναβινοειδή, μέσω των υποδοχέων τους CB1 και CB2, ρυθμίζουν την όρεξη στο κεντρικό νευρικό σύστημα, ενώ μπορούν να ενεργοποιήσουν περιφερικά τη λιπογένεση και να μειώσουν τη γονιδιακή έκφραση της αντιπονεκτίνης. Τα ενδοκανναβινοειδή βρίσκονται υπερενεργοποιημένα σε ενήλικες παχύσαρκους, ενώ τα επίπεδα της αντιπονεκτίνης μειώνονται σημαντικά. Σκοπός: Να μελετηθούν τα επίπεδα έκφρασης του AdipoR1, του PPAR-γ, του CB1 και των ενζύμων των ενδοκανναβινοειδών FAAH και DAGL-α, σε λεπτόσωμα και παχύσαρκα προεφηβικά παιδιά και να συσχετιστούν με τα κυκλοφορούντα επίπεδα της αντιπονεκτίνης και της ινσουλίνης. Μεθοδολογία: Για το σκοπό αυτό αναπτύχθηκαν πρωτογενείς καλλιέργειες προλιποκυττάρων και ώριμων λιποκυττάρων από βιοψίες κοιλιακού υποδόριου λιπώδους ιστού 17 παχύσαρκων (BMI>95%) και 36 λεπτόσωμων (BMI<85%) προεφηβικών παιδιών. Τα παιδιά χωρίστηκαν σε δύο ηλικιακές ομάδες, ομάδα Α: 2 μηνών-7 ετών και ομάδα Β: 9-12 ετών. Η γονιδιακή και πρωτεϊνική έκφραση του AdipoR1, PPAR-γ και CB1 μελετήθηκαν με τη μέθοδο RT-PCR και Western Immunoblotting. Επίσης, η γονιδιακή έκφραση των ενζύμων των ενδοκανναβινοειδών FAAH και DAGL-α, μελετήθηκαν με Real-Time PCR. Τα κυκλοφορούντα επίπεδα της ολικής και HMW αντιπονεκτίνης όπως και της ινσουλίνης μετρήθηκαν με ELISA, ενώ υπολογίστηκε ο δείκτης ινσουλινοαντίστασης HOMA-IR και μετρήθηκε η περίμετρος κοιλίας σε κάθε παιδί. Αποτελέσματα: Η πρωτεϊνική έκφραση του AdipoR1 βρέθηκε μειωμένη στα προλιποκύτταρα και ώριμα λιποκύτταρα των μικρότερων παχύσαρκων παιδιών της ομάδας Α, σε σύγκριση με τα αντίστοιχα λεπτόσωμά τους. To PPAR-γ βρέθηκε αυξημένο στα ώριμα λιποκύτταρα των λεπτόσωμων και παχύσαρκων παιδιών, σε σύγκριση με τα προλιποκύτταρά τους, ενώ ήταν και σημαντικά αυξημένο στα ώριμα λιποκύτταρα των μικρότερων παχύσαρκων παιδιών, σε σύγκριση με τα αντίστοιχα λεπτόσωμά τους. Ο υποδοχέας των ενδοκανναβινοειδών, CB1, ήταν σημαντικά μειωμένος στα ώριμα λιποκύτταρα των παχύσαρκων παιδιών και των δύο ηλικιακών ομάδων, σε σύγκριση με τα αντίστοιχα λεπτόσωμά τους, ενώ παρουσίασε μία σημαντική αύξηση με την ηλικία. Επιπρόσθετα, το ένζυμο αποδόμησης FAAH (για την ανανδαμίδη) μειώθηκε με την ηλικία στα μεγαλύτερα λεπτόσωμα παιδιά της ομάδας Β, ενώ στα μεγαλύτερα παχύσαρκα παιδιά ήταν αυξημένο σε σύγκριση με τα αντίστοιχα λεπτόσωμά τους. Το ένζυμο βιοσύνθεσης DAGL-α (για το 2-AG) βρέθηκε αυξημένο στα μεγαλύτερα λεπτόσωμα και παχύσαρκα παιδιά της ομάδας Β σε σύγκριση με τα λεπτόσωμα και παχύσαρκα παιδιά της ομάδας Α. Η ινσουλίνη και το HOMA-IR ήταν σημαντικά αυξημένα στα μεγαλύτερα παιδιά, λεπτόσωμα και παχύσαρκα, σε σύγκριση με τα μικρότερα παιδιά. Η HMW αντιπονεκτίνη βρέθηκε μειωμένη στα λεπτόσωμα και παχύσαρκα παιδιά της ομάδας Β σε σύγκριση με τα αντίστοιχα παιδιά της ομάδας Α, ενώ ήταν σημαντικά αυξημένη στα μικρότερα παχύσαρκα παιδιά σε σύγκριση με τα αντίστοιχα λεπτόσωμά τους. Η περίμετρος κοιλίας ήταν σημαντικά αυξημένη στα μεγαλύτερα παχύσαρκα αγόρια σε σύγκριση με τα αντίστοιχα λεπτόσωμά τους. Συμπεράσματα: Η μειωμένη έκφραση του CB1 και η αυξημένη έκφραση του PPAR-γ στα μικρότερα παχύσαρκα προεφηβικά παιδιά της ομάδας Α, σε συνάφεια με τα αυξημένα επίπεδα της HMW αντιπονεκτίνης, πιθανόν να αντικατοπτρίζουν έναν προστατευτικό μηχανισμό ελεγχόμενης λιπογένεσης και διατήρησης της ινσουλινοευαισθησίας στα παιδιά αυτά που ήδη παρουσιάζουν μειωμένα επίπεδα έκφρασης του υποδοχέα της αντιπονεκτίνης, AdipoR1. Επιπλέον, τα μειωμένα επίπεδα της HMW αντιπονεκτίνης και τα αυξημένα επίπεδα της ινσουλίνης στα μεγαλύτερα παιδιά πιθανόν απεικονίζει την προετοιμασία των παιδιών αυτών για την «φυσιολογική» ινσουλινοαντίσταση της εφηβείας. Η αύξηση των ενζύμων FAAH και DAGL-α στα μεγαλύτερα παχύσαρκα παιδιά της ομάδας Β, μπορεί έμμεσα να μας δείχνει ότι τα επίπεδα της ανανδαμίδης στα παιδιά αυτά είναι μειωμένα, ενώ τα επίπεδα του ενδοκανναβινοειδούς 2-AG αυξάνονται, θέτοντας πιθανόν τα παχύσαρκα παιδιά σε μεγαλύτερο κίνδυνο για λιπογένεση. Η μειωμένη έκφραση του CB1 στα μεγαλύτερα παχύσαρκα παιδιά όμως, μπορεί να απεικονίζει είτε την προσπάθεια του οργανισμού να περιορίσει την λιπογένεση στα παιδιά αυτά, που ήδη βρίσκονται σε κίνδυνο λόγω της παχυσαρκίας τους, είτε αντικατοπτρίζει τη μειωμένη ικανότητα του υποδόριου λιπώδους ιστού να αποθηκεύσει λίπος αυξάνοντας τον κίνδυνο εναπόθεσης λίπους ενδοκοιλιακά, το οποίο μπορεί να διαταράξει την ενεργειακή ισορροπία του οργανισμού τους προκαλώντας διαταραγμένη ανοχή στη γλυκόζη.

Parents' Obesity-Related Behavior and Confidence to Support Behavioral Change in Their Obese Child: Data From the STAR Study

The differential effect of meteorological factors and air pollutants on pediatric epistaxis in younger and older children has not been evaluated. We evaluated the distribution of pediatric epistaxis cases between younger (0–5 years) and older children (6–18 years). Subsequently, we assessed and compared the effects of meteorological variables and the concentration of particulate matter measuring ≤ 10 μm in diameter (PM10) on hospital epistaxis presentation in younger and older children. This retrospective study included pediatric patients (n = 326) who presented with spontaneous epistaxis between January 2015 and August 2019. Meteorological conditions and PM10 concentration were the exposure variables, and data were obtained from Korea Meteorological Administration 75. The presence and cumulative number of epistaxis presentations per day were considered outcome variables. Air temperature, wind speed, sunshine duration, and PM10 concentration in younger children, and sunshine duration and air pressure in older children, significantly correlated with the presence of and cumulative number of epistaxis presentations per day. The PM10 concentration was not a significant factor in older children. Thus, meteorological factors and PM10 concentration may differentially affect epistaxis in younger (0–5-year-olds) and older (6–18-year-olds) children. Risk factors for pediatric epistaxis should be considered according to age.

This study estimated how patterns of substance use are related to work status, public program use, and well-being among a sample of female caregivers and children. This study assessed work, public program use, and well-being measures as a function of substance use among 1,623 female caregivers of children aged zero to four or ten to 14 who participated in the Welfare of Children and Families study and lived in low- and moderate-income neighborhoods in Boston, Chicago, and San Antonio. Data were analyzed from baseline interviews that were conducted from March through December 1999 and from follow-up interviews that were conducted 11 to 26 months after baseline (average of 16 months). Substance use patterns were placed into three categories: light or no substance use reported in both interviews, moderate or heavy substance use (that is, moderate or heavy use in both interviews or increased substance use during the study period), and reduced substance use during the study period. Among caregivers who reduced their substance use, measures of work status, receipt of income assistance, mental health symptoms, and reports of child behavior problems were not significantly different at follow-up from those of caregivers with light or no substance use. At follow-up, compared with caregivers with light or no substance use, those with moderate or heavy substance use were significantly less likely to experience improvements in mental health symptoms and to see improvements in their children's behavioral problems. Caregivers with moderate or heavy substance use were more likely to be "detached" (p=.051)--that is, neither working nor collecting income assistance--although this difference was only marginally significant. Caregivers with increased substance use fared poorly on measures of well-being and work. Policies that promote, rather than impede, reductions in substance use are more likely to promote self-sufficiency and well-being.

Families of children with phenylketonuria (PKU) report child emotional and behavioral problems, parenting stress, and parenting difficulties, which are associated with worse health-related quality of life. This study aimed to examine acceptability and feasibility of a brief, group-based parenting program (Healthy Living Triple P) for families of children with PKU. An uncontrolled nonrandomized trial design was used. Families of children aged 2-12 years (N = 17) completed questionnaire measures assessing child behavior and impact of PKU on quality of life (primary outcomes), and parenting behavior, self-efficacy and stress, and children's behavioral and emotional adjustment (secondary outcomes). Routinely collected blood phenylalanine (Phe) levels were obtained from the treating team. Parents selected two child behaviors as targets for change. The intervention comprised two, 2-hr group sessions delivered face-to-face or online. Assessment was repeated at 4-week postintervention (T2) and 4-month follow-up (T3). Attrition was low and parent satisfaction with the intervention (face-to-face and online) was high. All families achieved success with one or both child behavior goals, and 75% of families achieved 100% success with both behavior goals by T3; however, there was no change in health-related quality of life. There were moderate improvements in parent-reported ineffective parenting (total score, d = 0.87, 95% CI -1.01 to 2.75) and laxness (d = 0.59, 95% CI -1.27 to 2.46), but no effects on parenting stress or children's adjustment. Phe levels improved by 6month post-intervention for children with elevated preintervention levels. Results support intervention acceptability and feasibility. A randomized controlled trial is warranted to establish intervention efficacy.

Differences between a clinical sample of younger (ages 5 to 11) and older (ages 12 to 19) children meeting DSM-III criteria for overanxious disorder (OAD) were examined. Younger and older children were compared in terms of (1) the rates of OAD diagnoses occurring in the two age groups, (2) sociodemographic characteristics, (3) symptom expression, (4) association with other forms of maladjustment, and (5) self-reported anxiety and depression. The prevalence of OAD diagnoses and sociodemographic characteristics did not differ. Although younger and older OAD children showed similar rates of most specific DSM-III OAD symptoms, older children presented with a higher total number of overanxious symptoms than younger children. Older children more frequently exhibited a concurrent major depression or simple phobia, whereas younger OAD children more commonly had coexisting separation anxiety or attention deficit disorders. Older OAD children reported significantly higher levels of anxiety and depression on self-report measures. Findings indicated that the expression of OAD varies by developmental level.

Parenting and childhood atopic dermatitis: A cross-sectional study of relationships between parenting behaviour, skin care management, and disease severity in young children

To understand how traumatic brain injury (TBI) affects parent-child interactions acutely following injury. Young children hospitalized for TBI (n = 80) and orthopedic injuries (OI; n = 113). Raters coded videotaped interactions during free play and structured tasks for parental warmth/responsiveness and negativity and child warmth, behavior regulation, and cooperation. Raters also counted parental directives, critical/restricting statements, and scaffolds. Parents of children with TBI exhibited less warm responsiveness and made more directive statements during a structured task than parents in the OI group. Children with TBI displayed less behavior regulation than children with OI. Parental warm responsiveness was more strongly related to child cooperativeness in the OI group than in the TBI group. Child behavior also mediated group differences in parental responsiveness and directiveness. TBI accounted for as much variance in parental behaviors as or more than did sociodemographic factors. TBI-related changes in child behavior may negatively influence parent-child interactions and disrupt the reciprocity between parent and child.

Treatment Efficacy of Social Communication Skills Training After Traumatic Brain Injury: A Randomized Treatment and Deferred Treatment Controlled Trial

Exposure to multiple stressors and lack of access to resources place rural children at high risk for adverse consequences. Family Stress Model guided this study to examine relations between two stressors- food insecurity and maternal depressive symptoms, and behavior problems among younger and older rural children. To test associations between food insecurity, maternal depressive symptoms, and behavior problems among younger and older rural low-income children. Cross-sectional data from 370 low-income rural families across 13 states was analyzed using structural equation modeling and multiple group analyses. Mothers’ education level, household income, marital/partner status, and participation in SNAP served as covariates. Among younger children, maternal depressive symptoms partially mediated the relation between food insecurity and child externalizing behaviors, while among older children, maternal depressive symptoms completely mediated the relation between food insecurity and child internalizing and externalizing behaviors. Stress manifested directly from, or indirectly through, maternal depressive symptoms and from food insecurity was related to behavior problems among younger and older rural children; however, the relations varied by age of children. Programs and policies that prevent or lessen both food insecurity and maternal depression may help to lessen problem behaviors among on rural children. Longitudinal studies are needed to rigorously examine causation and directionality among food insecurity, maternal depression and rural child behavior problems, while accounting for influences of child, caregiver and family characteristics.

Phenylketonuria (PKU) is an inherited metabolic condition that can lead to the onset of intellectual disabilities if not strictly managed through a low-protein diet. Parents are responsible for supervising their child's treatment for PKU, which may impact on their experience of distress. This cross-sectional study aimed to identify the factors that contribute to distress in parents who care for a child with PKU, distinct from parents in the general population. Thirty-eight parents of children and adolescents with PKU and 32 parents in the general population completed the questionnaires measuring parental psychological resilience, child behaviour problems, perceived social support and distress. Parents of children with PKU also completed measures of their child's care dependency and behaviour related to developmental and intellectual disabilities. The findings revealed no statistically significant differences in distress between the groups, but parents of children with PKU reported more child behaviour problems. Multiple regression analysis identified that parental psychological resilience and child anxious behaviour explained 35% of the variance in distress for parents of children with PKU. By comparison, parental psychological resilience and generic child behaviour only accounted for 19% of the variance in distress for parents in the general population. This has implications for developing interventions in clinical settings that aim to reduce parents' distress by enhancing their psychological resilience and supporting them to manage child behaviour difficulties, particularly anxious behaviour. Future research should include larger, more diverse samples and use longitudinal study designs.

Differences in Cyto- and Molecular Genetic Abnormalities between Children <2 Years and Older Children with Acute Myeloid Leukemia.

Introduction: Identifying responsive outcome measures for assessing functional change related to cognition, communication, and quality of life for individuals with neurodegenerative disease is important for intervention design and clinical care. Goal Attainment Scaling (GAS) has been used as an outcome measure to formally develop and systematically measure incremental progress toward functional, patient-centered goals in clinical settings. Evidence suggests that GAS is reliable and feasible for use in older adult populations and in adult populations with cognitive impairment, but no review has assessed the suitability of GAS in older adults with neurodegenerative disease experiencing dementia or cognitive impairment, based on responsiveness. This study conducted a systematic review to evaluate the suitability of GAS as an outcome measure for older adult populations with neurodegenerative disease experiencing dementia or cognitive impairment, based on responsiveness. Methods: The review was registered with PROSPERO and performed by searching ten electronic scientific databases (PubMed, Medline OVID, CINAHL, Cochrane, Embase, Web of Science, PsycINFO, Scopus, OTSeeker, REHABDATA) and four registries (Clinicaltrials.gov, Grey Literature Report, Mednar, OpenGrey). A summary measure of responsiveness (post-intervention minus pre-intervention mean GAS T-score) was compared across eligible studies using a random-effects meta-analysis. Risk of bias in included studies was assessed using the NIH Quality Assessment Tool for Before-After (Pre-Post) Studies with No Control Group. Results: 882 eligible articles were identified and screened by two independent reviewers. Ten studies met inclusion criteria for the final analysis. Of the ten included reports, 3 focus on all-cause dementia, 3 on multiple sclerosis, 1 on Parkinson’s disease, 1 on mild cognitive impairment, 1 on Alzheimer’s disease, and 1 on primary progressive aphasia. Responsiveness analyses showed pre- and post-intervention GAS goals were significantly different from zero (Z = 7.48, p < 0.001), with post-intervention GAS scores being higher than pre-intervention GAS scores. Three included studies showed a high risk of bias, 3 showed a moderate risk of bias, and 4 showed a low risk of bias. Overall risk of bias of included studies was rated as moderate. Conclusion: GAS showed an improvement in goal attainment across different dementia patient populations and intervention types. The overall moderate risk of bias suggests that while bias is present across included studies (e.g., small sample size, unblinded assessors), the observed effect likely represents the true effect. This suggests that GAS is responsive to functional change and may be suitable for use in older adult populations with neurodegenerative disease experiencing dementia or cognitive impairment.