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  • 1
    Electronic Resource
    Electronic Resource
    Temporäre Amaurose nach Kraniopharyngeomoperation (1998)
    Springer
    Monatsschrift Kinderheilkunde 146 (1998), S. 1067-1069 
    Details
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Kraniopharyngeom ; Amaurose ; Chiasma ; Hormone ; Operation ; Key words Kraniopharyngioma ; Blindness ; Chiasma ; Endocrine dysfunction ; Surgery
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Craniopharyngiomas are the third frequent pediatric brain tumors. Complications after surgical treatment are common. The most frequent complications are endocrine disturbances, vision field defects, deteriorated acuity, cognitive or psychological deficiency as well as significant weight gain. A case of complete vision loss right after surgical removal of craniopharyngioma is reported. Magnetic resonance image showed an edema of the chiasm. No damage of anatomical structure could be detected. Seventeen days after surgery the patient reported intermittent visual impressions for the first time. Over the following weeks his visus improved to 0.5, while the chiasm edema disappeared. Discussion: Even weeks after surgery-induced blindness regeneration of sufficient visus is possible.
    Notes: Zusammenfassung Das Kraniopharyngeom ist der dritthäufigste Hirntumor des Kindesalters. Komplikationen nach Kraniopharyngeomoperationen sind häufig. Zu den häufigsten Komplikationen zählen endokrine Störungen, Visusverschlechterung, Gesichtsfeldausfälle, emotionale und kognitive Veränderungen sowie Gewichtszunahme. Wir berichten über 1 Patientin, bei der unmittelbar nach der Operation eines Kraniopharyngeoms eine vollständige Amaurose auftrat. Bei intakten anatomischen Strukturen zeigte das MRT eine Ödem im Bereich des Chiasmas. 17 Tage nach der Operation traten erstmals wieder Seheindrücke auf, die sich im Verlauf von 6 Wochen bis zu einem Visus von 0,5 steigerten. Parallel dazu wies die Bildgebung einen Rückgang des Chiasmaödems auf. Diskussion: Auch Wochen nach einer eingetretenen operationsbedingten Amaurose kann bei intakten anatomischen Strukturen mit der Wiedererlangung eines ausreichenden Visus gerechnet werden.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001120050366
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Regression of neuropsychological deficits in early-treated phenylketonurics during adolescence (1999)
    Springer
    Journal of inherited metabolic disease 22 (1999), S. 693-705 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Even early-treated phenylketonuric patients suffer from phenylalanine-associated (mild) neuropsychological impairment. To date it is still unclear whether patients' deficits show a progression on ageing. This unsolved question seems to be an important aspect in the still ongoing debate about how long and how strictly the patients should be maintained on diet. Twenty early-treated (20±10, 9–30 days) adolescent phenylketonurics (10 boys, 10 girls) and 20 healthy controls, matched for age, sex and IQ, were inves-tigated twice at a mean ages of 11 and 14 years for their IQ (Culture Fair Intelligence Test–Scale 2; CFT-20), fine motor abilities (Motor PerformanceTask), sustained (Test d2) and selective attention (Stroop-Task). At the first test, examinations revealed significant blood phenylalanine-correlated neuropsychological deficits in PKU patients. In spite of raised blood phenylalanine concentrations during the following 3 years and significantly elevated concurrent blood phenylalanine concentrations, the repeated measurements revealed a significant decrease of patients' deficits compared to controls. Clinical-neurological status of patients and controls was normal at both test times. The results indicate a decreased vulnerability of PKU-patients with respect to their neuropsychological functioning against elevated blood phenylalanine levels on ageing.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1005587915468
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  • 3
    Electronic Resource
    Electronic Resource
    Behavioural and emotional problems in early-treated adolescents with phenylketonuria in comparison with diabetic patients and healthy controls (2000)
    Springer
    Journal of inherited metabolic disease 23 (2000), S. 487-496 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Even early-treated patients with phenylketonuria (PKU) have a higher risk of psychosocial maladjustment. This study was performed to determine whether emotional and behavioural problems are specific in phenylketonurics and whether they depend on the quality of biochemical control. This comparative study covered 42 PKU patients aged 10–18 years (mean 14.7 years) and 42 diabetic patients matched for sex, age and socioeconomic status. Patients' groups were compared with a control sample of healthy controls (n=2900) from an epidemiological study. We used the Child Behavior Check List (CBCL) according to Achenbach, intelligence quotient (IQ) test according to Weiss, and monitoring of blood phenylalanine concentrations and HBA1 concentrations. Internalizing problems such as depressive mood, anxiety, physical complaints or social isolation were significantly elevated in both phenylketonuric and diabetic patients, whereas externalizing problems were not. The two patient groups did not differ significantly either in the degree or in the pattern of their psychological profile. In both groups no significant correlations could be computed between the psychological characteristics and the biochemical control, the IQ, and the socioeconomic status. No patient was undergoing psychiatric treatment or psychotherapy. Our results strongly support a psychological perspective for the development of behavioural and emotional problems in both phenylketonuric and diabetic patients. Thus, medical treatment should be accompanied by psychological support for the families.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1005664231017
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Prolactin, a marker for cerebral dopamine deficiency in patients suffering from phenylketonuria (PKU)? (2000)
    Springer
    Journal of inherited metabolic disease 23 (2000), S. 849-851 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1026725122142
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    Paper (German National Licenses)
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