The main problems of parents of a child with epidermolysis bullosa

Epidermolysis Bullosa (EB) is a rare genetic blistering-skin disorder with varying degrees of severity, ranging from mild forms to severe forms, with chronic progression. The aim of this study was to identify and specify the problems of parents of a child with EB. Qualitative research methodology was used, comprising a series of semistructured interviews with eleven families. The key problems of parents were broken down into three themes, related to the child, the family, and the care providers. These themes comprised nine categories, including (1) the child being different, (2) the child suffering pain, (3) feelings of uncertainty, (4) restrictions on employment and leisure time, (5) difficulties in organization of care, (6) never being off-duty, (7) family problems, (8) ignorance and lack of skills of care providers, and (9) resistance to difficult care. Despite the great variance in clinical pictures of the different (sub)types of EB, the main problems parents experienced appear quite similar. However, the problems did appear to differ in extensiveness, intensity, and gravity

Stump sensibility in children with upper limb reduction deficiency

Objectives: To compare stump sensibility in children with upper limb reduction deficiency with sensibility of the unaffected arm and hand. In addition, to evaluate the associations between stump sensibility, stump length and activity level.Design: Cross-sectional study.Subjects: Children and young adults aged 6-25 years with upper limb reduction deficiency.Methods: Threshold of touch was measured with Semmes-Weinstein monofilaments, stereognosis was measured with the Shape-Texture Identification test and kinaesthesia and activity level was measured with the Child Amputee Prosthetics Project Functional Status Inventory and the Prosthetic Upper Extremity Functional Index.Results: A total of 31 children with upper limb reduction deficiency (mean age 15 years, 3 prosthesis wearers) were investigated. The threshold of touch of the stump circumference was lower (indicating higher sensibility) than of the unaffected arm (p=0.006), hand (p=0.004) and stump end-point (p=Conclusion: Threshold of touch, stereognosis and kinaesthesia of the affected sides were excellent. Threshold of touch of the stump circumference was lower (indicating higher sensibility) than of the unaffected arm and hand. High stump sensibility may clarify good functioning in the children without prostheses and contribute to prosthesis rejection.</p

The effect of cerebral palsy on arithmetic accuracy is mediated by working memory, intelligence, early numeracy and instruction time

Item does not contain fulltextThe development of addition and subtraction accuracy was assessed in first graders with cerebral palsy (CP) in both mainstream (16) and special education (41) and a control group of first graders in mainstream education (16). The control group out-performed the CP groups in addition and subtraction accuracy and this difference could not be fully explained by differences in intelligence. Both CP groups showed evidence of working memory deficits. The three groups exhibited different developmental patterns in the area of early numeracy skills. Children with CP in special education were found to receive less arithmetic instruction and instruction time was positively related to arithmetic accuracy. Structural equation modeling revealed that the effect of CP on arithmetic accuracy is mediated by intelligence, working memory, early numeracy, and instruction time

Reliability of popliteal angle measurement:a study in cerebral palsy patients and healthy controls

The popliteal angle is a widely used clinical measure for hamstring contracture in cerebral palsy (CP) patients and in healthy individuals. The reliability of popliteal angle measurement is being questioned. The aim of this study is to determine the reliability of popliteal angle measurement by means of visual and goniometric assessment. METHODS: Three different observers measured the popliteal angle in 15 CP patients and 15 healthy volunteers. In each subject, popliteal angles were visually estimated and measured with a blinded goniometer twice by all observers with approximately 1 hour between measurement sessions. RESULTS: All intraclass correlation coefficients (ICCs) were lower in the CP group compared with healthy controls. The ICC for intraobserver differences was higher than 0.75 for both groups. The ICC for interobserver reliability of visual estimates and goniometric measurements was low for both groups. Intermethod ICC was higher than 0.75 for both groups. CONCLUSIONS: Measurements in the CP group seemed to be less reliable than measurements in the control group. Intraobserver reliability is reasonable for both groups, but lower in CP patients than in controls. Interobserver reliability of both visual estimates and goniometrical measurements is poor. No significant differences in reliability have been found between visual estimation and goniometric measurement. Because of poor interobserver reliability of popliteal angle measurement, this should not be the only variable in clinical decision making in CP patients

Test-retest, inter-assessor and intra-assessor reliability of the modified Touwen examination

Interest in the Touwen examination (1979) for the assessment of minor neurological dysfunction (MND) is growing. However, information on psychometric properties of this assessment is scarce. Therefore the present study aimed at assessing the test's test-retest, inter- and intra-assessor reliability. Eleven boys and 14 girls, visiting a mainstream school, aged 4-12 years, were tested neurologically by 3 investigators. Inter- and intra-assessor reliability were based on videotapes of the assessments. To determine test-retest reliability children were reassessed after about 1 month. The various forms of reliability were calculated for neurological classification (normal, simple MND, complex MND), clusters of dysfunction and single items. Twelve girls and 7 boys showed a normal neurological condition; 2 girls and 1 boy were classified as having simple MND and 3 boys as having complex MND. The 3 forms of reliability for neurological classification were good (kappa = 0.71-0.83). Reliability for the majority of cluster scores was good. Test-retest agreement was moderate for the clusters reflexes and coordination and poor for fine manipulation; inter-assessor agreement was moderate for the clusters coordination and fine manipulation; intra-assessor agreement moderate for fine manipulation. Reliability for the majority of items was good. In conclusion, the Touwen examination has a moderate to good reliability when applied in a relatively healthy population. Whether reliability is similarly good in populations of children with minor developmental disorders has to be determined. (C) 2007 European Paediatric Neurology Society Published by Elsevier Ltd. All rights reserved

Does surgical management of the hand in children with spastic unilateral cerebral palsy affect functional outcome?

The aim of this review was to examine the literature on the effects of surgery of the spastic hand in children with cerebral palsy on functional outcome and muscle coordination. We performed a search of the relevant literature in Medline, Embase, and Biological Abstracts from 1966 to June 2006. The search resulted in eight studies on the effect of surgery on functional outcome and three studies on the effect of surgery on muscle coordination. Heterogeneity in outcome measures precluded meta-analysis. The studies revealed that - at the level of impairment - surgery has a positive effect on supination and dorsiflexion of the wrist. At the level of function, surgery might improve grip strategy and induce an increase of the repertoire of grips and spontaneous use of the hand. The evidence for the functional effects is, however, limited. Whether the possibly improved function is mediated by alterations in muscle coordination patterns remains unclear. The muscle coordination studies provided inconclusive results. We concluded that surgery improves the position of the hand and there are indications that it might improve hand function. Future research should address the question: does surgery improve hand function and, if so, is this due solely to a better hand position or does change in muscle coordination play an additional role

Does surgical management of the hand in children with spastic unilateral cerebral palsy affect functional outcome?

The aim of this review was to examine the literature on the effects of surgery of the spastic hand in children with cerebral palsy on functional outcome and muscle coordination. We performed a search of the relevant literature in Medline, Embase, and Biological Abstracts from 1966 to June 2006. The search resulted in eight studies on the effect of surgery on functional outcome and three studies on the effect of surgery on muscle coordination. Heterogeneity in outcome measures precluded meta-analysis. The studies revealed that - at the level of impairment - surgery has a positive effect on supination and dorsiflexion of the wrist. At the level of function, surgery might improve grip strategy and induce an increase of the repertoire of grips and spontaneous use of the hand. The evidence for the functional effects is, however, limited. Whether the possibly improved function is mediated by alterations in muscle coordination patterns remains unclear. The muscle coordination studies provided inconclusive results. We concluded that surgery improves the position of the hand and there are indications that it might improve hand function. Future research should address the question: does surgery improve hand function and, if so, is this due solely to a better hand position or does change in muscle coordination play an additional role