Biomedical subjects

Meinolf Noeker

Publications and source records attributed to Meinolf Noeker.

7 recordsLinked to original sources

PubMed2006

Neurocognitive development in children experiencing intrauterine growth retardation and born small for gestational age: pathological, constitutional and therapeutic pathways.

Interest in the neurocognitive and psychosocial outcomes in children who are born small for gestational age (SGA) has increased since the recent approval of growth hormone (GH) therapy in this indication. The objective of GH treatment in SGA children is to provide a symptomatic treatment for growth retardation. From a patient perspective, the ultimate goals of GH therapy are the reduction in the present or future risk of neurocognitive, psychological, social or occupational impairment, not the accompanying improvements in growth velocity and final height per se. Therefore, from a scientific perspective, neurocognitive and psychosocial endpoints become relevant domains of assessment to determine the final treatment benefit experienced by the patient born SGA. This article reviews recent available studies on developmental risks in SGA, and then transforms the empirical findings into an integrated conceptual framework on the sources and mediators of neurocognitive and psychosocial outcomes in intrauterine growth retardation and SGA. This framework depicts two distinct therapeutic pathways by which GH therapy may improve neurocognitive and behavioural outcomes. The first ('traditional') pathway is the prevention of exposure to short-stature-related stressors via an improvement in growth velocity and final height. The second pathway refers to potential metabolic, and thus neurotropic and psychotropic, effects of GH binding at receptors in the central nervous system, thus changing neuronal activity. To date, the existence and potential mechanisms of such physiologically and not psychologically mediated effects of GH on neurocognitive functioning in SGA patients remain hypothetical.

Adolescent↗

PubMed2006

Gender-associated differences in the psychosocial and developmental outcome in patients affected with the bladder exstrophy-epispadias complex.

OBJECTIVE: To identify problems in the long-term psychosocial and developmental outcome specific to patients with the bladder exstrophy-epispadias complex (BEEC), using a self-developed semi-structured questionnaire, as there are various techniques of reconstruction to repair BEEC but to date neither patients nor surgeons have a clear answer about which type gives the most acceptable long-term results. PATIENTS AND METHODS: Increasingly many patients with BEEC reach adulthood and wish to have sexual relationships and families. To date, no studies have used disease-specific psychological instruments to measure the psychosocial status of patients with BEEC. Thus we contacted 208 patients with BEEC, and 122 were enrolled, covering the complete spectrum of the BEEC. The data assessed included the surgical reconstruction, subjective assessment of continence, developmental milestones, school performance and career, overall satisfaction in life, disease-specific fears and partnership experiences in patients aged >18 years. We compared affected females and males to assess gender-associated differences in quality of life. RESULTS: Affected females had more close friendships, fewer disadvantages in relation to healthy female peers and more partnerships than the males. Family planning seemed to be less of a problem in affected females. There were no gender differences in the adjustments within school and professional career, which was very good in general. CONCLUSION: Future studies are needed to assess the disease-specific anxieties, considering gender-specific differences.

Adolescent↗

PubMed2005

Development of mental health dysfunction in childhood epilepsy.

A marked prevalence of mental health dysfunction in childhood epilepsy has been documented in the literature. While several individual risk factors have been identified, which are statistically associated with an impaired mental health outcome, there is a lack of knowledge on the pathways taken by these risk factors on disease development and treatment. The relevant literature of the last decade will be reviewed in this paper to provide evidence for the conceptual framework presented here. Thus, the emergence of mental health dysfunction in childhood epilepsy is analyzed under three levels. Pathogenetic causes: These involve both the underlying CNS pathology and the associated epilepsy disorder characterized by specific time of onset duration type and severity. Mediators and moderators connecting causes to outcomes: These comprise, firstly, the differentiation between the intervening role of anti-epileptic drugs and their positive psychotropic impact via suppression of seizure activity and transient cognitive impairments, as against their negative psychotropic side-effects; secondly, the psychological processes of adaptation which entail responding to three major demands (adherence to treatment requirements, exercising self-control and lifestyle modification to reduce seizure activity, and coping with the psychosocial stressors secondary to living with epilepsy); thirdly, the age-dependent level of neurocognitive and behavioral functioning; and, fourthly, contextual risks and protective factors within the family and social environment. Mental health outcome: This encompasses three major domains: risks for learning disability, for impairments of health-related quality of life, and for psychopathology. The proposed framework serves the development and validation of hypotheses and can be applied to testing procedures aimed at investigating the emergence of mental health dysfunction in childhood epilepsy. On the scientific level, it provides an appropriate tool to approach childhood epilepsy in general, whereas on the clinical level, it facilitates the assessment and management of individual patients.

Adaptation, Psychological↗

PubMed2004

Epilepsy--improvement of giving the diagnosis between the demands for standardisation versus individualisation.

Starting from Cunningham et al.'s [Seizure 11 (2002) 500] attempt to develop a guideline for giving the diagnosis of childhood epilepsy, the paper discusses the specific difficulties emerging on the way towards a standardisation and development of guidelines for the disclosure of diagnosis. The major objective of disclosure is to enhance positive adaptation towards epilepsy and its associated stressors and treatment demands. Adaptation to a chronic disease, however, depends on subjective processes of stress appraisal and coping response. Supporting adaptation by favourable strategies of disclosure therefore requires to explore and respond to the very personal perception of the medical and psychosocial consequences of the disorder. The broad interindividual variation of subjective anxieties therefore entails the necessity to individualise the procedure of telling the diagnosis in order to maximise its goodness of fit to patient and family characteristics. A procedure is suggested that integrates the individualisation of information provision and counselling, on the one side, and the efforts of standardisation and guideline development, on the other side, in order to improve resulting disclosure practice.

Adaptation, Psychological↗

PubMed2004

Risk analyses for the cognitive phenotype in Turner's syndrome: evidence of familial influence as a decisive factor.

The interindividual varying cognitive performance in female patients with Turner's syndrome has usually been attributed to the interindividual varying mosaicism with a consecutive variable loss of X-chromosome DNA or to secondary risk factors such as estrogen deficiency owing to ovarian failure. The aim of our study was to determine the specific impact of X chromosome-related features and associated risk factors, on the one hand and familial influences, on the other hand on the interindividual variation in the cognitive phenotype. One hundred and one subjects with Turner's syndrome and 53 sisters as controls for familial influences were examined by comparing the cognitive information processing abilities (Kaufmann Assessment Battery for Children [K-ABC]). Subjects with Turner's syndrome performed at a significantly lower level than sisters on all subscales (eg, Mental Processing Composite: Turner's syndrome 86.4 [SD 15.0] versus sisters 99.3 [SD 10.6]; P < .001). For the neurocognitive phenotype in subjects with Turner's syndrome, a significant correlation was found only with the sisters' cognitive abilities (Mental Processing Composite: r = .38, P < .05). In contrast, neither the individual mosaic status nor the known associated risk factors predicted the neurocognitive phenotype in Turner's syndrome. These results are corroborated in the regression analyses in those subjects with Turner's syndrome with a sister (Simultaneous Processing(sister) for Simultaneous Processing(Turner's syndrome): beta = .346, P < .05, corrected R2 = .049; and Mental Processing Composite(sister) for Mental Processing Composite(Turner's syndrome): beta = .354, P < .05, corrected R2 = .033). The interindividual variation of intellectual abilities in Turner's syndrome seems to be primarily related to familial coinfluences and not to the interindividual varying loss of X-chromosome DNA in terms of hidden mosaicism or potential associated risk factors.

Child↗

PubMed2004

Cognitive development in Silver-Russell syndrome: a sibling-controlled study.

This study examined cognitive development in Silver-Russell syndrome (SRS), a condition with intrauterine growth retardation, persisting short stature, and specific stigmata. Neuropsychological function and cognitive abilities were assessed in a sample of 36 children with SRS (21 males, 15 females; mean age 7 years 6 months, SD 2 years 8 months; age range 3 to 12 years) and 25 normally developing siblings (mean age 8 years 6 months, SD 2 years 7 months; age range 3 to 12 years) using the Kaufman Assessment Battery for Children. Special measures were taken to control for confounding factors and sample bias. Mean overall IQ score in the total SRS sample (95.7, SD 10.63), as measured by the Mental Processing Composite Scale, was significantly reduced (p=0.021) compared with test norms (IQ 100, SD 15), indicating a moderate cognitive impairment. Subscale analysis revealed some specific deficiencies. However, these cannot be attributed to an established category of specific learning disorder. The mean score in the Achievement Scale (91.25, SD 14.92), which is more sensitive to educational influences, showed stronger deficits (p=0.001). The sibling control group achieved a slightly better mean IQ score (104.20, SD 12.32) than test norms (p=0.10). Direct analysis of paired differences between the subsample of children with SRS and a sibling among the control group (n=25) revealed a significant mean difference of 8.08 IQ points (p=0.011). Risk factor analysis revealed that cognitive development is not associated with birth length (p=0.404), birthweight (p=0.820), growth hormone therapy (p=0.810), phenotypic severity (p=0.828), or sex (p=0.880). Two children with maternal uniparental disomy for the entire chromosome 7 had markedly lower IQ scores (81 and 84 respectively). In contrast to the few previous findings, children with SRS show only moderate, but significant, impairments in cognitive outcome, which are more striking in our sample when compared with siblings than with test norms.

Adolescent↗

PubMed2004

[Factitious disorder and factitious disorder by proxy].

Similar to the adult patient, a child or adolescent may actively feign or produce artificial symptoms (synonymous: Munchausen syndrome). The more frequent case is that the child suffers from being an object of symptom fabrication induced by a close person caring for the child, regularly the mother (Munchausen syndrome by proxy). This review focuses on psychopathological aspects of the clinically more relevant factitious disorder by proxy. Typical behaviour and personality characteristics are presented that can be taken as clinical warning signs. Doctor-mother-interaction is affectively challenging due to conflicting tasks imposed on the physician. Complementary to pediatric exclusion of genuine disease, psychopathological assessment is required to exclude other sources of deviant illness behaviour. Factious disorder shares particular features (active violation of the child, false report of history, aggravated symptom presentation and increased doctor-hopping, difficulties in conforming maternal report in biomedical data) with other psychopathological entities (child abuse, simulation, dissociative disorders, somatoform disorders including hypochondria, variants of maternal overprotection and infantilization, psychosis or delusion in the mother). Criteria for differentiation are presented. Three concepts on the psychopathological etiology of factitious disorder by proxy are relevant: In some cases, it may be conceived as secondary manifestation of a primary psychopathological entity or personality disorder. Learning theory emphasises operant rewards received from vicarious sick role. Attachment theory provides possible explanations concerning the traumatic impact on the child, early sources of psychopathology in the fabricating mother and risks for intergenerational transmission of factitious disorders.

Adolescent↗