Self-reported psychological wellbeing in adolescents: the role of intellectual/developmental disability and gender.

BACKGROUND: The Wellbeing in Special Education Questionnaire was developed to assess subjective wellbeing in young persons with intellectual and developmental disabilities (ID/DD) as this perspective is rarely included in research. The present study explored how ID/DD and gender are related to self-reported wellbeing among adolescents. METHOD: Students with (n = 110) or without (n = 110) ID/DD, aged 12-16 years, completed the Wellbeing in Special Education Questionnaire. Analyses of the effects of gender and disability status on peer relations and conflict, mental health, mental ill-health, school environment and family relations were carried out. RESULTS: The experiences of the school environment and of positive mental health aspects did not differ between students with and without ID/DD, but those with ID/DD reported more mental health problems and less positive experiences of peer relations and family. Generally, boys reported more positive experiences of school and less mental health problems than girls. CONCLUSIONS: Including the subjective perspective of young persons with ID/DD through self-reports can provide essential information about wellbeing that cannot be gained from proxy ratings. The results suggest both differences and similarities in self-reported wellbeing between boys and girls with and without ID/DD and potentially also in how they perceived the concepts measured.

Endodontic regeneration and tooth revitalization in immature infected sheep teeth.

AIM: To examine the response of immature sheep teeth with infected root canal systems to a commonly used pulp regeneration/revitalization protocol. METHODOLOGY: Immature mandibular right first incisors in four sheep were mechanically exposed and the pulps infected. The mandibular left first incisors remained intact as controls. Five weeks later, the experimental root canals were chemo-mechanically cleaned and dressed with a triple-antibiotic paste for 4 weeks, before bleeding was induced inside the canal by mechanically irritating the periapical tissues. A collagen dressing was packed coronally onto the blood clot and the canal orifice sealed with mineral trioxide aggregate and glass ionomer cement. Six months later, the mandibles were collected and the teeth with associated periapical tissues were analysed radiographically, with CT scanning, and by histology. The changes in root length, dentine thickness in the apical third (mesially and distally), and apical diameter were analysed using Student's t-test. RESULTS: Radiographs revealed significant increases in root length, root wall thickness and narrowing of the apical diameter of the canals after treatment (P < 0.05), with no significant differences in root diameters found between the experimental and the control teeth (P > 0.05) on both radiographic and CT results. Root maturation and thickening of the walls due to hard tissue deposition was confirmed by histology in all experimental teeth. Hard tissues in the apical portion of the root were more developed than in the coronal portion. CONCLUSION: Endodontic regeneration and tooth revitalization procedures in immature infected sheep teeth showed positive outcomes with similar increases in root length and development to the control teeth.

The global response regulator ExpA controls virulence gene expression through RsmA-mediated and RsmA-independent pathways in Pectobacterium wasabiae SCC3193.

ExpA (GacA) is a global response regulator that controls the expression of major virulence genes, such as those encoding plant cell wall-degrading enzymes (PCWDEs) in the model soft rot phytopathogen Pectobacterium wasabiae SCC3193. Several studies with pectobacteria as well as related phytopathogenic gammaproteobacteria, such as Dickeya and Pseudomonas, suggest that the control of virulence by ExpA and its homologues is executed partly by modulating the activity of RsmA, an RNA-binding posttranscriptional regulator. To elucidate the extent of the overlap between the ExpA and RsmA regulons in P. wasabiae, we characterized both regulons by microarray analysis. To do this, we compared the transcriptomes of the wild-type strain, an expA mutant, an rsmA mutant, and an expA rsmA double mutant. The microarray data for selected virulence-related genes were confirmed through quantitative reverse transcription (qRT-PCR). Subsequently, assays were performed to link the observed transcriptome differences to changes in bacterial phenotypes such as growth, motility, PCWDE production, and virulence in planta. An extensive overlap between the ExpA and RsmA regulons was observed, suggesting that a substantial portion of ExpA regulation appears to be mediated through RsmA. However, a number of genes involved in the electron transport chain and oligogalacturonide metabolism, among other processes, were identified as being regulated by ExpA independently of RsmA. These results suggest that ExpA may only partially impact fitness and virulence via RsmA.

Openness and avoidance--a longitudinal study of fathers of children with intellectual disability.

BACKGROUND: Fathers' interactions with children who have intellectual disabilities (ID) or developmental delays (DD) have increased over the past few decades and may be expected to continue to increase as maternal and paternal roles, along with other gender roles, become more equal. The aim of the present study was to explore fathers' experiences of parenthood in relation to a child with ID/DD from the initial discovery of the disability to 5 years later. METHODS: Fathers' experiences of parenting children with ID/DD were explored in a longitudinal framework. Seven Swedish fathers of young children with ID/DD participated in a series of semi-structured interviews from 2005 to 2010, and their accounts were subjected to interpretative phenomenological analysis. RESULTS: The analysis revealed three themes: (1) An interrupted path - no longer taking things for granted, which describes the fathers' reactions to their children's diagnosis; (2) Being a good father, which describes the fathers' overall perceptions of their parenting of a child with ID/DD; and (3) Dealing with the unexpected, which describes fathers' individual ways of integrating, managing, and living with the knowledge of their child's disability over the 5 years during which fathers were interviewed. CONCLUSIONS: Fathers' individual paths need to be taken into consideration when offering psychological support to families of children with ID/DD.

Parents of children with and without intellectual disability: couple relationship and individual well-being.

BACKGROUND: Research on parents of children with intellectual disability (ID) has identified a range of risk and protective factors for parental well-being. In family research, the association between marital quality and depression is a vital field of investigation. Still little research has addressed how aspects of the couple relationship affect the adaptation of parents of children with ID. The present study examined predictive links between couple relationship factors (marital quality and coparenting quality) and individual well-being. METHODS: Data were obtained through self-report questionnaires completed by parents of children with ID (mothers, n = 58; and fathers, n = 46) and control children (mothers, n = 178; and fathers, n = 141). To test the hypothesis that couple relationship factors predicted individual well-being, multiple regression analyses were performed controlling for the following risk factors identified by previous research: child self-injury/stereotypic behaviour, parenting stress, and economic risk. RESULTS: Marital quality predicted concurrent well-being, and coparenting quality predicted prospective well-being. Mothers of children with ID reported lower well-being than other parents. CONCLUSIONS: There is a continued need for investigation of the details of the links between couple relationship and individual well-being in parents of children with ID. Couple relationship factors should be given consideration in clinical interventions.

Expectations of and reactions to disability and normality experienced by parents of children with intellectual disability in Sweden.

BACKGROUND: Much research has been devoted to the emotional burdens of having a child with intellectual disability (ID), but very little of this has focused on the burdens imposed by negative public attitudes towards disability. Adjustment has primarily been thought of as adjustment to the actual disability rather than adjustment to attitudes or prejudices towards disability. AIM: The aim of the present study was to study expectations of and reactions to disability and normality experienced by parents of children with ID in their everyday life in Sweden. RESEARCH QUESTIONS: Do the parents relate to the tragedy discourse and, if so, how? Do parents relate to other expectations and disability discourses and, if so, how? What subject positions do the discourses offer? METHOD: The analysis was based on 17 semi-structured interviews with nine fathers and eight mothers of nine children with different IDs. The children were newly diagnosed and were from 6 months to 5 years of age. RESULTS: Four discourses (tragedy and the lived experience, tragedy and the outsider perspective, the clinical gaze and the super-parent ideal) and subject positions relating to these discourses are discussed. DISCUSSION: The results of this study show that parents of young children with disabilities relate to a wide range of social processes and practices, which could be thought to affect their emotional well-being and parental identity negatively.

Different, difficult or distinct? Mothers' and fathers' perceptions of temperament in children with and without intellectual disabilities.

BACKGROUND: Can ratings of temperament be a way of identifying young children with intellectual disabilities (ID) who are at risk for being experienced as difficult? We aimed to explore parents' reports of temperament in their young children with or without ID, as well as positive and negative impact of the child on parents. METHOD: Mothers and fathers of 55 children recently diagnosed with ID and 183 age-matched typically developing (TD) children completed the EASI Temperamental Survey and two scales of the Family Impact Questionnaire measuring positive and negative impact of the child on parents. RESULTS: Parents rated children with mixed ID/DD (developmental delay) as shyer and more impulsive, and less active and sociable when compared with TD children. Children with mixed ID/DD were also reported to have more negative and less positive impact on the family compared with the TD group. In subgroup analyses, children with Down syndrome and cerebral palsy/motor impairment were described as having less negative impact on parents and were described as low in negative emotionality. Children with autism spectrum disorder (ASD), ID/DD nos and other less common diagnoses had a similar pattern of temperament with high emotionality, shyness and impulsivity, and low activity and sociability. Parents of children with ASD and ID/DD reported the highest level of negative impact. CONCLUSIONS: Temperamental characteristics such as high negative emotionality and impulsivity, which can be identified earlier than behavioural problems, could be indicators of negative impact on parents of young children with ID. Despite great variability in temperament among children with mixed ID/DD, results indicated common temperamental characteristics among children with ASD, ID/DD and other diagnosis.

Parents' descriptions and experiences of young children recently diagnosed with intellectual disability.

AIM: The aim of the present study was to explore the variation of parents' descriptions and experiences of their child that was recently identified to have an intellectual disability (ID). METHODS: The study applied interpretative phenomenological analysis and analysis of narrative style looking at content and form of parental narratives. Data was collected from nine fathers and eight mothers through semi-structured interviews within 6 months following diagnosis. RESULTS: Analysis revealed three factors indicating the parents' level of processing: (1) emotional expressions regarding the child - varying between limited (distanced or idealized) and balanced/affectionate; (2) experience of the disability - varying between preoccupation and acceptance; and (3) time orientation - varying in terms of flexibility and temporal focus. CONCLUSIONS: Although parents of children with ID describe negative emotions in relation to the child and the disability, most of these parents also describe positive emotions that seemed to balance the negative experiences.

Cell swelling, seizures and spreading depression: an impedance study.

The cellular processes that take place during the transition from pre-seizure state to seizure remain to be defined. In this study in awake, paralyzed rats, we used an electrical impedance measure of changes in extra-cellular intracranial volume to estimate changes in cell size in acute models of epilepsy. Animals were prepared with extradural electroencephalographic (EEG)/impedance electrodes and a venous catheter. On a subsequent day, animals were paralyzed, ventilated and treated with picrotoxin, kainic acid or fluorocitrate in doses that usually induce epileptiform discharges. We now report that increases in baseline impedance were induced by kainic acid and smaller increases by picrotoxin. We also demonstrated that epileptiform discharges were preceded by small, accelerated increases in impedance. Increases in baseline impedance were highly correlated with increases in power of non-ictal high frequency EEG activity. Seizures were accompanied by increases in impedance and all treatments induced transient, relatively large, increases in impedance often associated with unilateral reductions in low frequency EEG, likely periods of spreading depression. We conclude: cerebral cells swell in convulsant models of epilepsy, that there are pre-ictal accelerations in cell swelling, and that spreading depression-like events are frequently associated with seizures.

Novel, high incidence exercise-induced muscle bleeding model in hemophilia B mice: rationale, development and prophylactic intervention.

INTRODUCTION: Muscle hematomas are the second most common complication of hemophilia and insufficient treatment may result in serious and even life-threatening complications. Hemophilic dogs and rats do experience spontaneous muscle bleeding, but currently, no experimental animal model is available specifically investigating spontaneous muscle bleeds in a hemophilic setting. AIM: The objective of this study was to develop a model of spontaneous muscle bleeds in hemophilia B mice. We hypothesized that treadmill exercise would induce muscle bleeds in hemophilia B mice but not in normal non-hemophilic mice and that treatment with recombinant factor IX (rFIX) before treadmill exercise could prevent the occurrence of pathology. METHODS: A total of 203 mice (123 F9-KO and 80 C57BL/6NTac) were included in three separate studies: (i) the model implementation study investigating the bleeding pattern in hemophilia B mice after treadmill exercise; (ii) a study evaluating the pharmacokinetics of recombinant FIX (rFIX) in hemophilia B mice and based on these data; (iii) the treatment study, which tested therapeutic intervention with rFIX. At termination of the treadmill studies the presence of bleeds was evaluated. RESULTS: Treadmill exercise resulted in a high incidence of muscle bleeds in F9-KO mice but not in C57BL/6NTac mice. Treating hemophilia B mice with rFIX before treadmill exercise prevented muscle bleeds. CONCLUSION: A novel model of muscle bleeds in hemophilia B mice, responsive to rFIX, has been developed.

Von Willebrand factor, a key protein in the exposure of CD62P on platelets.

When a biomaterial is introduced into the body water, electrolytes, and proteins adsorb to the surface. Platelets are then the first cells to interact with the surface adsorbed protein layer. We have studied the role of von Willebrand factor (vWF) for platelet-protein interaction by measuring different platelet responses to protein- and plasma-coated hydrophobic glass surfaces. A high exposure of CD62P on the platelet surface was seen after 10 min of incubation on platelets interacting with vWF and normal plasma-coated surfaces (79 and 67%, respectively). On the surfaces coated with albumin and factor VIII deficient plasma, the exposure was low (11 and 27%, respectively). A higher formation of filipodial extensions on the platelets was seen on the surfaces coated with vWF and normal plasma than on the surfaces coated with albumin or factor VIII deficient plasma. No significant differences were seen between the surfaces regarding the platelet release of PF4, ATP, or phospholipids. As shown by these results, vWF is a specific regulator of the exposure of CD62P by platelets and hence important for the interaction between platelets and later arriving neutrophils at biomaterial surfaces.

Results of delayed excision of the radial head after fracture.

Twenty-one patients had a delayed excision of a previously fractured radial head (range, one month to more than twenty years). There were four Mason type-II and seventeen Mason type-III fractures. Five fractures had been associated with a concomitant dislocation of the elbow and four, with an ulnar fracture. All of the patients were evaluated for pain, motion, strength, stability, and function by personal interview, examination, and testing in an upper-extremity-biomechanics laboratory. The average length of follow-up was fifteen years (range, three to thirty-two years). Postoperatively, pain was less severe in 76 per cent of the patients and motion was improved in both flexion and rotation in 81 per cent. An objective functional index showed that 77 per cent of the patients had a good or excellent result while 23 per cent had a fair or poor result. This study demonstrates the effectiveness of delayed excision after failure of closed management of fractures of the radial head. Thus, some justification is offered for the initial closed treatment of these fractures, with delayed excision of the radial head to be considered as needed.

Criminal multilation of the human body in Sweden--a thirty-year medico-legal and forensic psychiatric study.

During the 30-year period 1961-1990, a total of 22 deaths with criminal multilation/dismemberment of the human body were registered in Sweden. The multilations occurred in time clusters, mostly during the summer and winter periods, and increased during the three decades, with incidence rates of 0.05, 0.1, and 0.125 per million inhabitants and year, respectively. Multilation was noted 6.6 times more often in large urban areas than in the rest of Sweden. Defensive mutilation, in order to get rid of the corpse or make its identity more difficult, was noted in ten instances, aggressive mutilation following outrageous overkilling in four, offensive mutilation (lust murder) in seven, and necromanic multilation in one instance. In the last-mentioned case the cause of death was natural, while all deaths in the first three groups were homicidal, or homicide was strongly suspected. All perpetrators were males, in six instances assisted by other persons. In more than half of the cases the perpetrator's occupation was associated with application of anatomical knowledge, e.g., butcher, physician, veterinary assistant, or hunter. The perpetrators of the defensive and aggressive mutilations were mostly disorganized, i.e., alcoholics or drug users with previous psychiatric contacts and criminal histories, while the lust murderers were mostly organized, with a history of violent crimes (including the "serial killing" type), drug abuse and mental disorders with anxiety and schizophrenia, in that order to a diminishing degree. There were differences in mode of mutilation, depending on whether the mutilation was carried out by a layman, a butcher, or a physician. In only one case was the perpetrator convicted for the mutilation act itself; in the remaining instances the manslaughter, as a more serious crime, assimilated the mutilation. When the mutilation made it impossible to establish the cause of death, the perpetrators, despite strong circumstantial evidence indicating murder, were acquitted.

The Danish Patient Insurance Act of 1991: first experiences.

In 1991, Denmark established a Patient Insurance Scheme. The Scheme entered into force on 1 July 1992 and the first experiences with the Scheme have now been gained. Even though it is still too early to draw a clear picture of the significance of the Scheme, it is already safe to conclude that the Scheme provides a much more extensive coverage than did the hitherto prevailing negligence-based liability system. Furthermore, it is safe to conclude that the Scheme works much faster than the fault liability system where it might take years before the courts reached a decision.

The physician's civil liability under Danish law.

The physician's liability in Danish law is based on negligence, which is assessed by the courts largely on the basis of expert opinions. Such opinions are provided primarily by the Medico-Legal Council rather than by experts selected by the parties. The evaluation of negligence is based on a "reasonable man" standard and the performance expected of a competent colleague; a hospital will be responsible for the negligence of its employees. The burden of proof generally lies with the plaintiff; negligence will not be presumed and the assessment of the evidence of negligence will be adapted to the individual situation, e.g. factors such as the degree of specialization involved, the time which the physician had at his disposal to make his decision and the resources available to him will be taken into consideration. The courts have shown themselves willing to allow for the fact that doctors differ, i.e. recognizing that there must be scope for reasonable discretion. Because the culpa principle is central, the standard applied to medical knowledge will be that which pertained at the time of the treatment. Where a non-specialist is confronted with a problem which may go beyond the knowledge of his limits and experience, he is under an obligation to refer the patient. The principle of informed consent to treatment is accepted in Danish law, but such consent will readily be considered to have been given tacitly.

The Danish Patient Insurance Act of 1991 (LOV 1991-06-06 NR 367 OM PATIENTFORSIKRING).

In 1991, Denmark established a Patient Insurance Scheme, the fourth Nordic country to do so. Under the various national Nordic schemes, patients can receive indemnification for the consequences of medical injury even where the injury is not caused by negligent treatment. Instead emphasis is laid on whether the injury, viewed in retrospect, could have been avoided or whether the injury, though unavoidable, clearly exceeds the risk that the patient must accept. Differences in the four national approaches are analyzed and it is concluded that a no-fault scheme presents an elegant alternative to the tort law system, which proves to be particularly unsatisfactory in the field of medical accidents.

Effects of anesthetic agents on seizure-induction with intra-cortical injection of convulsants.

RATIONALE: Studies of partial or generalized seizure pathophysiology often require the use of intact animals. Additionally, anesthesia may be required for ethical reasons or paralysis if instrumental measures require immobilization. We examined three commonly used injected anesthetic for their impact on seizures induced by three convulsant agents. METHODS: We prepared rats, under pentobarbitone anesthesia (65 mg/kg) with a catheter, electrodes and a dural window, for later non-noxious experimentation. Three to seven days later, kainic acid (1.25 µg), picrotoxin (225 ng) or fluorocitrate (0.8 nmol) were injected intra-cortically in animals paralysed with succinylcholine, or anesthetised with pentobarbitone, urethane or fentanyl plus droperidol. We recorded EEG activity, the latencies to seizure discharges, the occurrence of spreading depressions and the presence of movements in response to the convulsants. RESULTS: Fentanyl plus droperidol was the only anesthetic agent permissive for seizure-discharges and spreading depressions. No significant differences in the time for seizure onset for fentanyl plus droperidol compared to paralyzed unanesthetised rats were seen for any of the convulsants (Student's t-test p>0.20). Movements during seizures as well as other drug-induced behaviors continued to be expressed during anesthesia. CONCLUSION: Fentanyl plus droperidol has useful properties as an anesthetic agent in studies of seizure induction with different convulsants.

Function and expression of the proton-coupled amino acid transporter PAT1 along the rat gastrointestinal tract: implications for intestinal absorption of gaboxadol.

BACKGROUND AND PURPOSE: Intestinal absorption via membrane transporters may determine the pharmacokinetics of drug compounds. The hypothesis is that oral absorption of gaboxadol (4,5,6,7-tetrahydroisoxazolo [5,4-c] pyridine-3-ol) in rats occurs via the proton-coupled amino acid transporter, rPAT1 (encoded by the gene rSlc36a1). Consequently, we aimed to elucidate the in vivo role of rPAT1 in the absorption of gaboxadol from various intestinal segments obtained from Sprague-Dawley rats. EXPERIMENTAL APPROACH: The absorption of gaboxadol was investigated following its administration into four different intestinal segments. The intestinal expression of rSlc36a1 mRNA was measured by quantitative real-time PCR. Furthermore, the hPAT1-/rPAT1-mediated transport of gaboxadol or L-proline was studied in hPAT1-expressing Xenopus laevis oocytes, Caco-2 cell monolayers and excised segments of the rat intestine. KEY RESULTS: The absorption fraction of gaboxadol was high (81.3-91.3%) following its administration into the stomach, duodenum and jejunum, but low (4.2%) after administration into the colon. The pharmacokinetics of gaboxadol were modified by the co-administration of L-tryptophan (an hPAT1 inhibitor) and L-proline (an hPAT1 substrate). The in vitro carrier-mediated uptake rate of L-proline in the excised intestinal segments was highest in the mid jejunum and lowest in the colon. The in vitro uptake and the in vivo absorption correlated with the expression of rSlc36a1 mRNA along the rat intestine. CONCLUSIONS AND IMPLICATIONS: These results suggest that PAT1 mediates the intestinal absorption of gaboxadol and therefore determines its oral bioavailability. This has implications for the in vivo role of PAT1 and may have an influence on the design of pharmaceutical formulations of PAT1 substrates.

Child's positive and negative impacts on parents--a person-oriented approach to understanding temperament in preschool children with intellectual disabilities.

BACKGROUND: Despite previous efforts to understand temperament in children with intellectual disability (ID), and how child temperament may affect parents, the approach has so far been unidimensional. Child temperament has been considered in relation to diagnosis, with the inherent risk of overlooking individual variation of children's temperament profiles within diagnostic groups. The aim of the present study was to identify temperamental profiles of children with ID, and investigate how these may affect parents in terms of positive and negative impacts. METHOD: Parent-rated temperament in children with ID was explored through a person-oriented approach (cluster analysis). Children with ID (N=49) and typically developing (TD) children (N=82) aged between 4 and 6 years were clustered separately. RESULTS: Variation in temperament profiles was more prominent among children with ID than in TD children. Out of the three clusters found in the ID group, the disruptive, and passive/withdrawn clusters were distinctly different from clusters found in the TD group in terms of temperament, while the cluster active and outgoing was similar in shape and level of temperament ratings of TD children. Children within the disruptive cluster were described to have more negative and less positive impacts on mothers compared to children within the other clusters in the ID group. CONCLUSIONS: Mothers who describe their children as having disruptive temperament may be at particular risk for experiencing higher parenting stress as they report that the child has higher negative and lower positive impacts than other parents describe. The absence of a relationship between child temperament profile and positive or negative impact on fathers may indicate that fathers are less affected by child temperament. However, this relationship needs to be further explored.