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Mastery motivation in young children with Down's syndrome: relations with cognitive and adaptive competence.

BACKGROUND: Prior research on relations between motivation and competence have focused on cognitive competence, despite theoretical predictions that mastery motivation spurs behaviour that is effective in meeting the demands of one's environment, i.e. adaptive competence. Issues of adaptive competence are especially relevant for children with developmental delay since functional independence is an important long-term goal for these children. METHODS: In the present study, mastery motivation was examined in relation to both cognitive and adaptive competence in 5-year-old children with Down's syndrome (n = 41). RESULTS: Scores on mastery task and parent-report measures of mastery motivation were generally low, but positively related to scores on standardized measures of cognitive competence and adaptive competence. CONCLUSIONS: This is the first study of motivation-competence relations in pre-school-age children. The findings have implications for both developmental theory and early intervention efforts, and the authors hope that they will serve as a stimulus for future research on cross-domain relationships in atypical populations.

Adaptation, Physiological↗

[Relaparotomy. Problems of terminology].

The authors discuss the term "relaparotomy" and such its definitions as "early", "delayed", "late", "elective", "targeted", analyze different classifications of relaparotomies, propose their own definition of this term based on objective criteria of general reaction to surgical stress in the form of Seli[symbol: see text] adaptation syndrome which has definite duration (60 days). This fact may be regarded as a clinical criterion of determination of relaparotomy's terms. Surgery may be named as relaparotomy when it is performed in terms of adaptation syndrome (60 days). If surgery is performed after the end of adaptation syndrome it must be regarded as repeated operation.

Humans↗

Increased tolerance of Litopenaeus vannamei to white spot syndrome virus (WSSV) infection after oral application of the viral envelope protein VP28.

It has been generally accepted that invertebrates such as shrimp do not have an adaptive immune response system comparable to that of vertebrates. However, in the last few years, several studies have suggested the existence of such a response in invertebrates. In one of these studies, the shrimp Penaeus monodon showed increased protection against white spot syndrome virus (WSSV) using a recombinant VP28 envelope protein of WSSV. In an effort to further investigate whether this increased protection is limited to P. monodon or can be extended to other penaeid shrimp, experiments were performed using the Pacific white shrimp Litopenaeus vannamei. As found with P. monodon, a significantly lower cumulative mortality for VP28-fed shrimp was found compared to the controls. These experiments demonstrate that there is potential to use oral application of specific proteins to protect the 2 most important cultured shrimp species, P. monodon and L. vannamei, against WSSV. Most likely, this increased protection is based on a shared and, therefore, general defence mechanism present in all shrimp species. This makes the design of intervention strategies against pathogens based on defined proteins a viable option for shrimp culture.

Animals↗

Subject selection and characterization in clinical trials in children with autism.

The goal of psychopharmacologic research in autism is to provide guidance to clinicians and families on the risks and benefits of specific interventions. Careful subject selection and subject characterization in clinical trials are necessary for replication, to inform clinicians about the sample, and to elucidate the type of patients who might benefit from the treatment. At minimum, subject characterization includes demographic information, diagnosis (autism, Asperger's syndrome, or pervasive developmental disorder-not otherwise specified), intellectual functioning, adaptive functioning, symptom severity, general behavioral profile, health status, pertinent clinical laboratory measures, height, weight, current treatments, and educational placements. Subject selection, sample size, and choice of the primary outcome measure are closely interrelated and linked to the study hypothesis. The magnitude of expected improvement on the primary outcome measure, which can be expressed by effect size, has direct implications for sample size. Large sample sizes are required to detect small effect sizes. To facilitate interpretation of study results, research reports should provide descriptive characteristics of the sample as well as the mean change and standard deviation on the primary outcome measure data to permit calculation of the effect size.

Autistic Disorder↗

Cognitive functioning in adults with Williams syndrome.

This paper reports the results of cognitive, linguistic, and academic assessments in a representative sample of 62 adults with Williams syndrome. The average age of the group was 26 years and their mean full scale IQ was 61. Differences between Verbal and Performance IQ, and between receptive and expressive language skills, were smaller than generally found in studies of children with this condition. However, an examination of subtest scores revealed an almost identical cognitive profile to that found in children. Skills in other areas, such as reading, spelling, arithmetic, and social adaptation remained at a low level, with functioning generally being around a 6-8-year age equivalent. The consistency of reports on intellectual abilities in both child and adult studies of individuals with Williams syndrome lends increased support to the notion of a syndrome specific pattern of cognitive, linguistic, and adaptive functioning.

Adolescent↗

Teenagers with Down syndrome study algebra in High School.

This paper deals with the adaptation of an algebra curriculum for two students with Down syndrome who were included in High School. Since the kindergarten, this boy and girl have been fully included in general education classes. This paper examines the rationale for this choice on an algebra program. The adaptation of this program was easy because all that was required was to shorten it and do some additional steps in teaching (a little bit more than in a remedial course). Also, visual prompts were provided to the students. The boy needed a calculator all the time. Both of the students learned to calculate algebraic expressions with parenthesis, with positive and negative numbers and even with powers. The boy was able to do algebraic sum of monomials. The girl performed expressions with fractions. They took written and oral tests at the same time as their classmates, but with different exercises or questions. The girl was able to do some mental arithmetic. Often she was more consistent and careful than her typical classmates. The boy had problems with the integration and he did not attend the school full time. The inclusion, even when it was not perfect, provided the motivation to teach and to learn. In both cases, the crucial point was the daily collaboration of the mathematics teacher with the special educator. Both of the students enjoyed the mathematics program, as many typical students do. Mathematics gave them the fulfilling emotion of succeeding!

Adolescent↗

How relevant are exercise capacity measures for evaluating treatment effects in chronic fatigue syndrome? Results from a prospective, multidisciplinary outcome study.

OBJECTIVE: To evaluate the outcome of a multidisciplinary treatment programme for patients with chronic fatigue syndrome, including health-related quality of life (HRQoL) and psychosocial variables, and exercise capacity measures. DESIGN: A six-month prospective outcome study. SETTING: University outpatient rehabilitation clinic; group setting. SUBJECTS: One hundred and sixteen women fulfilling chronic fatigue syndrome criteria. INTERVENTIONS: Cognitive behaviourally and graded exercise-based strategies; emphasis on adaptive lifestyle changes. MEASURES: Short Form General Health Survey (SF-36); Symptom Checklist (SCL-90); Causal Attribution List (CAL); Self-Efficacy Scale (SE); maximum progressive bicycle ergometer test with respiratory gas analysis; and isokinetic leg strength test, before and after treatment. RESULTS: The total group significantly improved on nearly all reported HRQoL/psychosocial variables. Changes in exercise capacity measures were rather modest and did not correlate or only weakly correlated with HRQoL/psychosocial variables. Subgroup analyses indicated that less fit patients improved significantly more on exercise capacity measures than their more fit counterparts. Patients who were fitter at baseline scored better on pretreatment HRQoL/psychosocial variables, but both subgroups improved similarly on these variables. CONCLUSIONS: Health-related quality of life and psychosocial functioning in patients with chronic fatigue syndrome improves after a six-month cognitive behaviourally and graded exercise-based multidisciplinary treatment programme. Increase in exercise capacity measures is not a necessary condition for reported improvements, except for less fit patients.

Adaptation, Psychological↗

Management of the short-bowel syndrome.

The patient with short-bowel syndrome after massive small-intestinal resection represents one of the greatest clinical challenges a general surgeon must face. Maintaining optimal nutritional and metabolic support until maximum bowel adaptation can occur is the top priority of therapy. Currently, no operative procedure for adjunctive management of the short-bowel syndrome is sufficiently safe and effective to recommend its routine use. Long-term parenteral nutrition remains the cornerstone of successful management.

Antacids↗

Fifteen years of Phormia - on the value of an insect for the study of aging.

The blowfly Phormia is currently used for studying various aspects of aging. In the context of comparative life history studies this fly can be characterized as an example of an 'r' selected type with a short life span and explosive reproduction. This is in contrast to the mecopteran fly Panorpa which shows senescence even under free living conditions and represents the 'K' selected type. A sudden drop of flight performance, low levels of enzyme activities at the end of life and specific kinetic adaptations of single enzymes are part of the 'aging syndrome' of Phormia. In the context of general aging investigations Phormia proved to be a suitable model system. This is shown in several examples covering the age variation of oscillating glycolysis, the influence of the cell redox state on purified enzymes, and treatment with geriatrics.

Journal Article↗

Posttraumatic headache: neuropsychological and psychological effects and treatment implications.

Posttraumatic headache (PTHA) is a frequent occurrence following trauma to the head, brain, and/or neck. Estimates of persistence for 6 months are as high as 44%. Review of available studies examining the effect of headache on neuropsychological test findings reveals that chronic headache pain, and chronic pain generally, exerts a significant and negative effect that poses a challenge to differential diagnostic efforts in the evaluation of mild brain injury. Given that PTHA is the most common postconcussive symptom and most frequent type of posttraumatic pain associated with mild traumatic brain injury (TBI), it follows that resolution of the postconcussion syndrome, and successful posttraumatic adaptation, may frequently rely on success in coping with PTHA symptomatology. Viewing PTHA from a biopsychosocial perspective, a general outline is offered for improving both assessment and treatment of PTHA. In addition, the most promising psychology-based treatment interventions are reviewed.

Adaptation, Psychological↗

[Psychosocial adaptation in children and adolescents with craniofacial malformations].

To determine children's perception of their own psychosocial functioning, to compare it with their parents' perception, and determine the type and rate of their psychopathological syndromes and family functioning, and to investigate correlation among these data. Nineteen children with craniofacial deformities and their parents responded to the Columbia Impairment Scale, the Child Behavior Checklist and the McMaster General Functioning Subscale. Psychosocial impairment is a trend in children with craniofacial deformities. There is a correlation between children's and parents' perception of the patient's psychosocial adaptation, global psychopathology and externalizing and aggressive syndromes. A less strong correlation was found with internalizing, somatization and attention deficit syndromes, and with family functioning. Craniofacial deformities in childhood pose special risks for psychosocial adjustment due to type of illness and environmental and family factors.

Adaptation, Psychological↗

Mothers' lexicon of internal state words in speech to children with Down syndrome and to nonhandicapped children at mealtime.

This study examines internal state words in mothers' speech to children with Down syndrome, and the relation between the use of internal state words and the children's levels of social-adaptive, communicative, and linguistic functioning. Results indicate qualitative differences in mothers' use of internal state words to children with children Down syndrome, compared with a sample of maternal speech to nonhandicapped children who were matched on the Vineland scales for their level of adaptive functioning. Differences include use of fewer internal state words overall to children with Down syndrome, and different kinds of internal state words: more words referring to physiological states, and fewer words referring either to affect or to cognition. In general, child Mean Length of Utterance (MLU) was associated with the pattern of inner state words used by mothers, whereas no associations were found between children's social-adaptive competence and maternal input. Even when controlling for child MLU, there were, however, some qualitative differences in the inner state lexicons used to children with Down syndrome. Results suggest that speech to children with Down syndrome that is calibrated to their MLU may underestimate their competence in domains other than grammar.

Child↗

Longitudinal evaluation of early and late anthracycline cardiotoxicity in children with AML.

BACKGROUND: Anthracyclines are effective antineoplastic drugs in acute myelogenous leukemia (AML). However, their use is limited by cardiomyopathy, which occurs in children already at cumulative doses of 300 mg/m(2) (given as daunorubicin equivalent). PROCEDURE: To evaluate anthracycline-associated cardiomyopathy in pediatric AML-patients, the incidence of early and late (>1 year after intensive AML chemotherapy) clinical and subclinical cardiotoxicity was analyzed out of a total of 1,207 patients <18 years treated between 1993 and 2003 in trials AML-BFM93/98: 1,010 protocol patients with de novo AML, 121 with Down syndrome (DS)-AML, and 76 with secondary AML. The cumulative dose of anthracyclines was generally risk-adapted: 300-450 mg/m(2) using 1-4-hr infusions of anthracyclines with the assumed lowest cardiotoxic potential. Eight hundred eighty-five patients (73%) were eligible for the analysis of early and 547 (45%) of late cardiotoxicity (1,399 follow-up data). RESULTS: Thirty-eight patients (4.3%), including 3 DS-AML and 1 secondary AML, suffered from early cardiomyopathy. After 5 years, four patients showed temporarily or persistently a reduced shortening fraction, which led to death in one DS-AML patient. Including these 4 patients, late cardiomyopathy was seen in 16 patients (cumulative incidence after 11 years: 5% +/- 1%). Nine patients (2.5 +/- 1%) showed clinical symptoms, five of them had persistent abnormal shortening fraction. Late subclinical cardiomyopathy occurred temporarily in seven patients. Late clinical cardiomyopathy mainly affected patients with a second anthracycline therapy (secondary malignancy) and those with early cardiotoxicity. CONCLUSION: In spite of a highly intensive and effective treatment, the frequency of anthracycline-associated cardiomyopathy was low in the AML-BFM studies.

Acute Disease↗

Acute local inflammation causing generalized increased ground substance viscosity: guttate psoriasis, Reiter's syndrome, adjuvant disease, cancer regression.

It is known that at sites of acute inflammation there is increased ground substance formation and fibroblast proliferation. There is also increased serum glycoprotein following localized acute inflammation. The author proposes that a severe acute inflammatory response in a localized area of the body induces a generalized increase in ground substance viscosity (generalized ground substance adaptive phenomenon). This would increase host resistance to invasion and heighten the inflammatory response. There are multiple clinical and experimental facts which could be explained by this response. These include guttate psoriasis, pustular psoriasis, pustular bacterid of Andrew's, Reiter's syndrome, and others. This response would include some aspects of adjuvant disease. Increased generalized ground substance viscosity could also explain the beneficial effects of streptococcal cell fractions and streptococcal infections on tumor regression. A number of other clinical observations could be explained. Connective tissue-activating peptide released at the inflammation site could be having a systemic effect. It could be generally beneficial to have generalized increased ground substance viscosity when acute inflammation is occurring in a localized area. A few patients who already have a highly reactive inflammatory level could develop excess inflammation especially at sites of trauma, or preexisting inflammation.

Acute-Phase Reaction↗

When should immunosuppressants be prescribed to treat systemic vasculitides?

Steroids and immunosuppressants are indicated to treat systemic vasculitides. However, the therapeutic strategy is different from one disease to the other. Treatment choice should be adapted to the predictable outcome, severity, pathogenic mechanisms and patient's general condition. In polyarteritis nodosa, Churg Strauss syndrome, and microscopic polyangiitis we have demonstrated that immunosuppressants should not be systematically prescribed. Immunosuppressants should be only prescribed in the most severe patients, when factors of poor prognosis are present. In Wegener's granulomatosis, immunosuppressants should be systematically prescribed together with steroids. The optimal treatment duration is of 12 months for polyarteritis nodosa and Churg-Strauss syndrome. A more prolonged treatment is mandatory in Wegener's granulomatosis, at least 18 months. The new therapeutic strategies comprise also new immunosuppressants and new immunomodulating agents which could replace or be associated to the "older drugs".

Churg-Strauss Syndrome↗

[Fiberscopic intubation under general anesthesia for children with Goldenhar syndrome].

Goldenhar syndrome is characterised by an eye abnormality such as epibulbar dermoid and lipoma, associated with ear, mandibular, and/or vertebral anomalies. It is well documented that difficult intubation in patient with this syndrome may be expected because of mandibular hypoplasia and limitation of neck movement resulting from vertebral anomalies. We report anesthetic management of two children with Goldenhar syndrome, 9 and 14-year-old. By preoperative examination, two children were expected to be difficult in intubating trachea. We used our newly developed mask adapter which enabled us to perform fiberoptic bronchoscopy-aided tracheal intubation under general anesthesia under controlled ventilation. By using this adapter, under sevoflurane/nitrous oxide anesthesia with vecuronium, we could successfully intubate with a 6 mm spiral tube through the nostril without any major problems. Anesthesia and surgery went uneventfully, and no postoperative complications were noted. We confirmed that our newly developed mask adapter for fiberoptic bronchoscopy is as useful for children with difficult intubation as for adults.

Adolescent↗

Transfer of perceptual-motor training and the space adaptation syndrome.

Perceptual cue conflict may be the basis for the symptoms which are experienced by space travelers in microgravity conditions. Recovery has been suggested to take place after perceptual modification or reinterpretation. To elucidate this process, 10 subjects who repeatedly experienced a visual/vestibular conflict (Purkinje) over trials and days, were then tested in a similar but not identical perceptual situation (pseudo-Coriolis) to determine whether any savings in perceptual adaptation had occurred as compared to an unpracticed control group (N = 10). The practiced subjects experienced lessening dizziness and ataxia within and over sessions. Their response to the new perceptual situation was markedly less than the control group (p less than 0.001). Assessment of "adaptability," in addition to the provocative vestibular tests now in use, may improve prediction of susceptibility to the space adaptation syndrome. An adaptability trait may exist in humans which, properly measured, could be used to predict who would adapt more readily to visual/vestibular conflict and perhaps to environmental stressors in general.

Adaptation, Physiological↗

Tolerance of unusually low mixed venous oxygen saturation. Adaptations in the chronic low cardiac output syndrome.

Introduction of a reliable method for continuous monitoring of mixed venous oxygen saturation has focused attention on the therapeutic and prognostic implications of this value. It is generally agreed that mixed venous oxygen saturation in the 40 to 60 percent range is associated with substantial patient morbidity and mortality if not rapidly corrected. However, several patients with chronic low cardiac output syndrome who have had mixed venous oxygen saturation of less than 40 percent for prolonged periods of time and who have not had decompensation were observed. Three representative patients with these findings are described and mechanisms put forth that may account for the observed relations between mixed venous oxygen saturation and mortality in these and other patients.

Adaptation, Physiological↗