Desmoid tumour of the external ear and mastoid following middle-ear surgery.

BACKGROUND: Desmoid tumours (aggressive fibromatosis) are rare, locally invasive, benign tumours. The following case represented a diagnostic challenge, because of the uncommon nature of the lesion. CASE REPORT: A 26-year-old woman, who had previously undergone middle-ear surgery for cholesteatoma, presented with a painful swelling involving the post-auricular area and the conchal bowl. Initially, it was believed to be an infective process related to the surgery or an unusual cholesteatoma recurrence. Following investigations, which involved imaging and histology, the swelling was diagnosed as a desmoid tumour, and the patient received chemotherapy. CONCLUSION: Two incidences of paediatric desmoid tumours affecting the ear have been described in the literature, but there is no previous report of a desmoid tumour related to ear surgery. Desmoid tumours have, however, been reported following trauma, including surgery.

Reference Determinant Dependence of the Random Phase Approximation in 3d Transition Metal Chemistry.

Without extensive fitting, accurate prediction of transition metal chemistry is a challenge for semilocal and hybrid density funcitonals. The Random Phase Approximation (RPA) has been shown to yield superior results to semilocal functionals for main group thermochemistry, but much less is known about its performance for transition metals. We have therefore analyzed the behavior of reaction energies, barrier heights, and ligand dissociation energies obtained with RPA and compare our results to several semilocal and hybrid functionals. Particular attention is paid to the reference determinant dependence of RPA. We find that typically the results do not vary much between semilocal or hybrid functionals as a reference, as long as the fraction of exact exchange (EXX) mixing in the hybrid functional is small. For large fractions of EXX mixing, however, the Hartree-Fock-like nature of the determinant can severely degrade the performance. Overall, RPA systematically reduces the errors of semilocal functionals and delivers excellent performance from a single reference determinant for inherently multireference reactions. The behavior of dual hybrids that combine RPA correlation with a hybrid exchange energy was also explored, but ultimately did not lead to a systematic improvement compared to traditional RPA for these systems. We rationalize this conclusion by decomposing the contributions to the reaction energies, and briefly discuss the possible implications for double-hybrid functionals based on RPA. The correlation between EXX mixing and spin-symmetry breaking is also discussed.

Horner syndrome in glandular fever: a case report.

OBJECTIVE: This study aimed to present and discuss the case of a patient with known glandular fever who presented with Horner syndrome. CASE REPORT: A 35-year-old patient with known glandular fever developed acute unilateral Horner syndrome, a previously undescribed complication of this common illness. Magnetic resonance imaging and magnetic resonance angiography showed that enlarged intra-carotid sheath lymphoid tissue was likely to be the underlying cause of sympathetic nerve disruption. The case is described, the anatomy of the sympathetic chain is discussed and possible alternative pathophysiological mechanisms are reviewed. CONCLUSION: This is the first report in the worldwide literature of Horner syndrome arising as a result of compression from enlarged lymph nodes in glandular fever.

Infantile haemangiopericytoma: a rare congenital cervical tumour.

OBJECTIVE: Infantile haemangiopericytoma is a rare childhood vascular tumour of borderline malignancy and unpredictable clinical course. It can present a diagnostic challenge due to indeterminate clinical, radiological and pathological features. This report presents the case of a large congenital haemangiopericytoma of the neck in a neonate, and discusses diagnosis, imaging, pathology and surgical management. CLINICAL PRESENTATION: A full-term neonate presented with a large posterior neck mass at birth. Pre-operative radiological appearances were suggestive of teratoma, but following surgical excision the diagnosis of infantile haemangiopericytoma was confirmed on histological analysis. There were no signs of recurrence at 12-month follow up. CONCLUSION: Haemangiopericytoma can follow an aggressive course in adults, including local recurrence and metastasis. The infantile variant is rare but typically follows a distinct clinical course, and is associated with more benign behaviour compared with similar tumours in adults and children over one year. Congenital haemangiopericytoma can be effectively treated with surgery, without requiring adjuvant therapy.

Impulsivity and genetic variants in DRD2 and ANKK1 moderate longitudinal associations between sleep problems and overweight from ages 5 to 11.

OBJECTIVE: Short sleep duration and sleep problems increase risks of overweight and weight gain. Few previous studies have examined sleep and weight repeatedly over development. This study examined the associations between yearly reports of sleep problems and weight status from ages 5 to 11. Although, previous studies have shown that inter-individual differences moderate the effect of short sleep duration on weight, it is not known whether inter-individual differences also moderate the effect of sleep problems on weight. We tested how the longitudinal associations between sleep problems and weight status were moderated by impulsivity and genetic variants in DRD2 and ANKK1. DESIGN: Seven-year longitudinal study. PARTICIPANTS: A total of 567 children from the Child Development Project for the analysis with impulsivity and 363 for the analysis with genetic variants. MEASUREMENTS AND RESULTS: Sleep problems and weight status were measured by mothers' reports yearly. Impulsivity was measured by teachers' reports yearly. Six single-nucleotide polymorphisms located in DRD2 and ANKK1 were genotyped. Data were analyzed using multilevel modeling. Higher average levels of sleep deprivation across years were associated with greater increases in overweight (P=0.0024). Sleep problems and overweight were associated at both within-person across time (P<0.0001) and between-person levels (P<0.0001). Impulsivity and two polymorphisms, rs1799978 and rs4245149 in DRD2, moderated the association between sleep problems and overweight; the association was stronger in children who were more impulsive (P=0.0022), in G allele carriers for rs1799978 (P=0.0007) and in A allele carriers for rs4245149 (P=0.0002). CONCLUSIONS: This study provided incremental evidence for the influence of sleep problems on weight. Findings of DRD2, ANKK1 and impulsivity are novel; they suggest that reward sensitivity and self-regulatory abilities might modulate the influences of sleep on weight gain. The analysis of polymorphisms was restricted to European Americans and hence the results might not generalize to other populations.

Cochlear implantation in a child with CINCA syndrome who also has wide vestibular aqueducts.

Chronic infantile neurological cutaneous and articular (CINCA) syndrome is a severe auto-inflammatory disease, due to mutation of the CIAS1 gene. CINCA syndrome should be considered the most severe of a spectrum of three disorders all due to mutation of the CIAS1 gene. CINCA syndrome produces a triad of symptoms of neonatal onset: maculopapular urticarial rash, chronic meningitis, and chronic non-inflammatory arthropathy with recurrent fever. CINCA syndrome is also associated with sensory organ damage, especially progressive hearing loss and loss of vision. In this case report, we present the first case of cochlear implantation in a 13-year-old child with CINCA syndrome. Cochlear implantation was successful at rehabilitating the hearing loss with the child able to continue mainstream education, with her academic performance and speech discrimination both showing marked improvement. Anakinra (an interleukin 1 receptor antagonist) is now in widespread use to treat CINCA syndrome and is known to rapidly reverse the inflammatory features of CINCA syndrome. However, current evidence suggests that anakinra has limited effectiveness in reversing the sensorineural hearing loss seen in CINCA syndrome. We therefore propose that cochlear implantation is a viable treatment option in this rare yet severe auto-inflammatory disease, if the patient has failed to respond to anakinra. Owing to the unknown pathogenesis of the progressive hearing loss seen in CINCA syndrome and the limited effectiveness of anakinra in reversing the progressive hearing loss, we suggest that cochlear implantation is the modality of choice in rehabilitating severe-to-profound hearing loss not responsive to anakinra.

Global and domain-specific cognitive impairment and outcome after subarachnoid hemorrhage.

BACKGROUND: Cognitive dysfunction is the most common form of neurologic impairment after subarachnoid hemorrhage (SAH). OBJECTIVE: To evaluate the impact of global and domain-specific cognitive impairment on functional recovery and quality of life (QOL) after SAH. METHODS: One hundred thirteen patients (mean age 49 years; 68% women) were evaluated 3 months after SAH. Three simple tests of global mental status and neuropsychological tests to assess seven specific cognitive domains were administered. Four aspects of outcome (global handicap, disability, emotional status, and QOL) were compared between cognitively impaired and unimpaired patients with analysis-of-covariance models controlling for age, race/ethnicity, and education. Multiple linear regression was used to evaluate the relative contribution of global and domain-specific cognitive status for predicting concurrent modified Rankin Scale (mRS) and Sickness Impact Profile (SIP) scores. RESULTS: Impairment of global mental status on the Telephone Interview of Cognitive Status (TICS) was associated with poor performance in all seven cognitive domains (all p < 0.0005) and was the only cognitive measure associated with poor recovery in all four aspects of outcome (all p < or = 0.005). Cognitive impairment in four specific domains was also associated with functional disability or reduced QOL. After accounting for global cognitive impairment with the TICS, however, neuropsychological testing did not contribute additional predictive value for concurrent mRS or SIP total scores. CONCLUSIONS: Cognitive impairment impacts broadly on functional status, emotional health, and QOL after SAH. The TICS may be a useful alternative to more detailed neuropsychological testing for detecting clinically relevant global cognitive impairment after SAH.

Continuous EEG monitoring and midazolam infusion for refractory nonconvulsive status epilepticus.

BACKGROUND: Although cIV-MDZ has emerged as a popular alternative to barbiturate therapy for refractory status epilepticus (RSE), experience with its use for this indication is limited. OBJECTIVE: - To evaluate the efficacy of continuous intravenous midazolam (cIV-MDZ) for attaining sustained seizure control in patients with RSE. METHODS: The authors reviewed 33 episodes of RSE treated with cIV-MDZ in their neurologic intensive care unit over 6 years. All patients were monitored with continuous EEG (cEEG). MDZ infusion rates were titrated to eliminate clinical and EEG seizure activity; cIV-MDZ was discontinued once patients were seizure-free for 24 hours. Acute treatment failures (seizures 1 to 6 hours after starting cIV-MDZ), breakthrough seizures (after 6 hours of therapy), post-treatment seizures (within 48 hours of discontinuing therapy), and ultimate treatment failure (frequent seizures that led to treatment with pentobarbital or propofol) were identified. RESULTS: All patients were in nonconvulsive SE at the time cIV-MDZ was started; the mean duration of SE before treatment was 3.9 days (range 0 to 17 days). In addition to benzodiazepines, 94% of patients had received at least two antiepileptic drugs (AED) before starting cIV-MDZ. The mean loading dose was 0.19 mg/kg, the mean maximal infusion rate was 0.22 mg/kg/h, and the mean duration of cIV-MDZ therapy was 4.2 days (range 1 to 14 days). Acute treatment failure occurred in 18% (6/33) of episodes, breakthrough seizures in 56% (18/32), post-treatment seizures in 68% (19/28), and ultimate treatment failure in 18% (6/33). Breakthrough seizures were clinically subtle or purely electrographic in 89% (16/18) of cases and were associated with an increased risk of developing post-treatment seizures (p = 0.01). CONCLUSIONS: Although most patients with RSE initially responded to cIV-MDZ, over half developed subsequent breakthrough seizures, which were predictive of post-treatment seizures and were often detectable only with cEEG. Titrating cIV-MDZ to burst suppression, more aggressive treatment with concurrent AED, or a longer period of initial treatment may reduce the high proportion of patients with RSE who relapse after cIV-MDZ is discontinued.

Peer rejection in childhood, involvement with antisocial peers in early adolescence, and the development of externalizing behavior problems.

A longitudinal, prospective design was used to examine the roles of peer rejection in middle childhood and antisocial peer involvement in early adolescence in the development of adolescent externalizing behavior problems. Both early starter and late starter pathways were considered. Classroom sociometric interviews from ages 6 through 9 years, adolescent reports of peers' behavior at age 13 years, and parent, teacher, and adolescent self-reports of externalizing behavior problems from age 5 through 14 years were available for 400 adolescents. Results indicate that experiencing peer rejection in elementary school and greater involvement with antisocial peers in early adolescence are correlated but that these peer relationship experiences may represent two different pathways to adolescent externalizing behavior problems. Peer rejection experiences, but not involvement with antisocial peers. predict later externalizing behavior problems when controlling for stability in externalizing behavior. Externalizing problems were most common when rejection was experienced repeatedly. Early externalizing problems did not appear to moderate the relation between peer rejection and later problem behavior. Discussion highlights multiple pathways connecting externalizing behavior problems from early childhood through adolescence with peer relationship experiences in middle childhood and early adolescence.

Antecedents and behavior-problem outcomes of parental monitoring and psychological control in early adolescence.

The early childhood antecedents and behavior-problem correlates of monitoring and psychological control were examined in this prospective, longitudinal, multi-informant study. Parenting data were collected during home visit interviews with 440 mothers and their 13-year-old children. Behavior problems (anxiety/depression and delinquent behavior) were assessed via mother, teacher, and/or adolescent reports at ages 8 through 10 years and again at ages 13 through 14. Home-interview data collected at age 5 years were used to measure antecedent parenting (harsh/reactive, positive/proactive), family background (e.g., socioeconomic status), and mother-rated child behavior problems. Consistent with expectation, monitoring was anteceded by a proactive parenting style and by advantageous family-ecological characteristics, and psychological control was anteceded by harsh parenting and by mothers' earlier reports of child externalizing problems. Consistent with prior research, monitoring was associated with fewer delinquent behavior problems. Links between psychological control and adjustment were more complex: High levels of psychological control were associated with more delinquent problems for girls and for teens who were low in preadolescent delinquent problems, and with more anxiety/depression for girls and for teens who were high in preadolescent anxiety/depression.

The timing of child physical maltreatment: a cross-domain growth analysis of impact on adolescent externalizing and internalizing problems.

In a sample of 578 children assessed in kindergarten through the eighth grade, we used growth modeling to determine the basic developmental trajectories of mother-reported and teacher-reported externalizing and internalizing behaviors for three physical maltreatment groups of children-early-harmed (prior to age 5 years), later-harmed (age 5 years and over), and nonharmed--controlling for SES and gender. Results demonstrated that the earlier children experienced harsh physical treatment by significant adults, the more likely they were to experience adjustment problems in early adolescence. Over multiple domains, early physical maltreatment was related to more negative sequelae than the same type of maltreatment occurring at later periods. In addition, the fitted growth models revealed that the early-harmed group exhibited someswhat higher initial levels of teacher-reported externalizing problems in kindergarten and significantly different rates of change in these problem behaviors than other children, as reported by mothers over the 9 years of this study. The early-harmed children were also seen by teachers, in kindergarten, as exhibiting higher levels of internalizing behaviors. The later-harmed children were seen by their teachers as increasing their externalizing problem behaviors more rapidly over the 9 years than did the early- or nonharmed children. These findings indicate that the timing of maltreatment is a salient factor in examining the developmental effects of physical harm.

Discipline responses: influences of parents' socioeconomic status, ethnicity, beliefs about parenting, stress, and cognitive-emotional processes.

Direct and indirect precursors to parents' harsh discipline responses to hypothetical vignettes about child misbehavior were studied with data from 978 parents (59% mothers; 82% European American and 16% African American) of 585 kindergarten-aged children. SEM analyses showed that parents' beliefs about spanking and child aggression and family stress mediated a negative relation between socioeconomic status and discipline. In turn, perception of the child and cognitive-emotional processes (hostile attributions, emotional upset, worry about child's future, available alternative disciplinary strategies, and available preventive strategies) mediated the effect of stress on discipline. Similar relations between ethnicity and discipline were found (African Americans reported harsher discipline), especially among low-income parents. Societally based experiences may lead some parents to rely on accessible and coherent goals in their discipline, whereas others are more reactive.

Early developmental precursors of externalizing behavior in middle childhood and adolescence.

This study examined the infancy- and toddler-age precursors of children's later externalizing problem behavior. Risk constructs included suboptimal patterns of observed caregiver-child interaction and the caregiver's perception of child difficultness and resistance to control. In addition, a novel dimension of caregiver-child relationship quality, the caregiver's perception of her toddler's unresponsiveness to her, was examined as a possible precursor of children's externalizing behavior. Externalizing problem outcomes were assessed throughout the school-age period and again at age 17, using multiple informants. As toddlers, children at risk for later externalizing behavior were perceived as difficult and resistant to control, and relationships with their caregivers were relatively low in warmth and affective enjoyment. Finally, the caregiver's perception of her toddler as emotionally unresponsive to her was a consistent predictor of later externalizing behavior, suggesting that negative maternal cognitions associated with child conduct problems may begin in toddlerhood. These predictive patterns were similar for boys and girls, and with minor exceptions, generalized across different subdimensions of externalizing problem behavior. Our findings underscore the importance of the infancy and toddler periods to children's long-term behavioral adjustment, and indicate the desirability of further research into the nature of caregivers' early perceptions of child unresponsiveness.

A cross-domain growth analysis: externalizing and internalizing behaviors during 8 years of childhood.

In a sample of 405 children assessed in kindergarten through the seventh grade, we determined the basic developmental trajectories of mother-reported and teacher-reported externalizing and internalizing behaviors using cross-domain latent growth modeling techniques. We also investigated the effects of race, socioeconomic level, gender, and sociometric peer-rejection status in kindergarten on these trajectories. The results indicated that, on average, the development of these behaviors was different depending upon the source of the data. We found evidence of the codevelopment of externalizing and internalizing behaviors within and across reporters. In addition, we found that African-American children had lower levels of externalizing behavior in kindergarten as reported by mothers than did European-American children but they had greater increases in these behaviors when reported by teachers. Children from homes with lower SES levels had higher initial levels of externalizing behaviors and teacher-reported internalizing behaviors. Males showed greater increases in teacher-reported externalizing behavior over time than did the females. Rejected children had trajectories of mother-reported externalizing and internalizing behavior that began at higher levels and either remained stable or increased more rapidly than did the trajectories for non-rejected children which decreased over time.

Quality of early family relationships and individual differences in the timing of pubertal maturation in girls: a longitudinal test of an evolutionary model.

In an 8-year prospective study of 173 girls and their families, the authors tested predictions from J. Belsky, L. Steinberg, and P. Draper's (1991) evolutionary model of individual differences in pubertal timing. This model suggests that more negative-coercive (or less positive-harmonious) family relationships in early childhood provoke earlier reproductive development in adolescence. Consistent with the model, fathers' presence in the home, more time spent by fathers in child care, greater supportiveness in the parental dyad, more father-daughter affection, and more mother-daughter affection, as assessed prior to kindergarten, each predicted later pubertal timing by daughters in 7th grade. The positive dimension of family relationships, rather than the negative dimension, accounted for these relations. In total, the quality of fathers' investment in the family emerged as the most important feature of the proximal family environment relative to daughters' pubertal timing.

Early behavior problems as a predictor of later peer group victimization: moderators and mediators in the pathways of social risk.

This study is a prospective investigation of the predictive association between early behavior problems (internalizing, externalizing, hyperactivity-impulsiveness, immaturity-dependency) and later victimization in the peer group. Teacher ratings of the behavioral adjustment of 389 kindergarten and 1st-grade children (approximate age range of 5 to 6 years-old) were obtained, using standardized behavior problem checklists. These ratings predicted peer nomination scores for victimization, obtained 3 years later, even after the prediction associated with concurrent behavior problems was statistically controlled. Further analyses suggested that the relation between early behavior problems and later victimization is mediated by peer rejection and moderated by children's dyadic friendships. Behavior problems appear to play an important role in determining victimization within the peer group, although the relevant pathways are complex and influenced by other aspects of children's social adjustment.

The relation between mothers' hostile attribution tendencies and children's externalizing behavior problems: the mediating role of mothers' harsh discipline practices.

This study examined relations among mothers' hostile attribution tendencies regarding their children's ambiguous problem behaviors, mothers' harsh discipline practices, and children's externalizing behavior problems. A community sample of 277 families (19% minority representation) living in three geographic regions of the United States was followed for over 4 years. Mothers' hostile attribution tendencies were assessed during the summer prior to children's entry into kindergarten through their responses to written vignettes. Mothers' harsh discipline practices were assessed concurrently through ratings by interviewers and reports by spouses. Children's externalizing behavior problems were assessed concurrently through written questionnaires by mothers and fathers and in the spring of kindergarten and first, second, and third grades through reports by teachers and peer sociometric nominations. Results of structural equations models demonstrated that mothers' hostile attribution tendencies predicted children's future externalizing behavior problems at school and that a large proportion of this relation was mediated by mothers' harsh discipline practices. These results remained virtually unchanged when controlling for initial levels of children's prekindergarten externalizing behavior problems at home.

Measurement of impulsivity: construct coherence, longitudinal stability, and relationship with externalizing problems in middle childhood and adolescence.

This study focused on the assessment of impulsivity in nonreferred school-aged children. Children had been participants since infancy in the Bloomington Longitudinal Study. Individual differences in impulsivity were assessed in the laboratory when children were 6 (44 boys, 36 girls) and 8 (50 boys, 39 girls) years of age. Impulsivity constructs derived from these assessments were related to parent and teacher ratings of externalizing problems across the school-age period (ages 7-10) and to parent and self-ratings of these outcomes across adolescence (ages 14-17). Consistent with prior research, individual measures of impulsivity factor-analyzed into subdimensions reflecting children's executive control capabilities, delay of gratification, and ability or willingness to sustain attention and compliance during work tasks. Children's performance on the main interactive task index, inhibitory control, showed a signficant level of stability between ages 6 and 8. During the school-age years, children who performed impulsively on the laboratory measures were perceived by mothers and by teachers as more impulsive, inattentive, and overactive than others, affirming the external validity of the impulsivity constructs. Finally, impulsive behavior in the laboratory at ages 6 and 8 predicted maternal and self-ratings of externalizing problem behavior across adolescence, supporting the long-term predictive value of the laboratory-derived impulsivity measures.

The impact of after-school peer contact on early adolescent externalizing problems is moderated by parental monitoring, perceived neighborhood safety, and prior adjustment.

Unsupervised peer contact in the after-school hours was examined as a risk factor in the development of externalizing problems in a longitudinal sample of early adolescents. Parental monitoring, neighborhood safety, and adolescents' preexisting behavioral problems were considered as possible moderators of the risk relation. Interviews with mothers provided information on monitoring, neighborhood safety, and demographics. Early adolescent (ages 12-13 years) after-school time use was assessed via a telephone interview in grade 6 (N = 438); amount of time spent with peers when no adult was present was tabulated. Teacher ratings of externalizing behavior problems were collected in grades 6 and 7. Unsupervised peer contact, lack of neighborhood safety, and low monitoring incrementally predicted grade 7 externalizing problems, after controlling for family background factors and grade 6 problems. The greatest risk was for those unsupervised adolescents living in low-monitoring homes and comparatively unsafe neighborhoods. The significant relation between unsupervised peer contact and problem behavior in grade 7 held only for those adolescents who already were high in problem behavior in grade 6. These findings point to the need to consider individual, family, and neighborhood factors in evaluating risks associated with young adolescents' after-school care experiences.