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Bar-On L.,University Hospital | Aertbelien E.,Celestijnenlaan 300b | Wambacq H.,Celestijnenlaan 300b | Severijns D.,University Hospital | And 9 more authors.
Gait and Posture | Year: 2013

Most clinical tools for measuring spasticity, such as the Modified Ashworth Scale (MAS) and the Modified Tardieu Scale (MTS), are not sufficiently accurate or reliable. This study investigated the clinimetric properties of an instrumented spasticity assessment. Twenty-eight children with spastic cerebral palsy (CP) and 10 typically developing (TD) children were included. Six of the children with CP were retested to evaluate reliability. To quantify spasticity in the gastrocnemius (GAS) and medial hamstrings (MEH), three synchronized signals were collected and integrated: surface electromyography (sEMG); joint-angle characteristics; and torque. Muscles were manually stretched at low velocity (LV) and high velocity (HV). Spasticity parameters were extracted from the change in sEMG and in torque between LV and HV. Reliability was determined with intraclass-correlation coefficients and the standard error of measurement; validity by assessing group differences and correlating spasticity parameters with the MAS and MTS. Reliability was moderately high for both muscles. Spasticity parameters in both muscles were higher in children with CP than in TD children, showed moderate correlation with the MAS for both muscles and good correlation to the MTS for the MEH. Spasticity assessment based on multidimensional signals therefore provides reliable and clinically relevant measures of spasticity. Moreover, the moderate correlations of the MAS and MTS with the objective parameters further stress the added value of the instrumented measurements to detect and investigate spasticity, especially for the GAS. © 2012 Elsevier B.V.

Luque V.,Rovira i Virgili University | Escribano J.,Rovira i Virgili University | Grote V.,Ludwig Maximilians University of Munich | Ferre N.,Rovira i Virgili University | And 6 more authors.
Pediatric Research | Year: 2013

Background: Animal models have shown that insulin-like growth factor I (IGF-I) may mediate protein-induced kidney growth. Our aim was to analyze the effect of IGF-I on protein-induced kidney growth in healthy infants.Methods: This is a secondary analysis of a randomized trial that compared growth of infants fed with a higher-protein (HP) (n = 169) vs. lower-protein (LP) (n = 182) formula (in the first year of life). Outcome measures were anthropometric parameters, kidney volume (cm 3), and total and free IGF-I (ng/ml).Results: The highest levels of total and free IGF-I were found in the HP group. Both parameters correlated significantly with BMI z-score (r = 0.229, P < 0.001 and r = 0.223, P < 0.001, respectively), kidney volume (r = 0.115, P = 0.006 and r = 0.208, P < 0.001, respectively), and kidney volume/body length (r = 0.109, P = 0.010 and r = 0.194, P < 0.001, respectively) at 6 mo. Linear regression analyses showed a significant effect of free IGF-I on kidney volume in models, including significant effects of HP formula and anthropometry. The structural equation model revealed a significant direct effect of the HP formula on kidney volume and an indirect effect mediated by free IGF-I.Conclusion: This study suggests that IGF-I partly mediates protein-induced kidney growth in healthy infants. IGF-I could be involved in a pathway for the programming of the renal system. © 2013 International Pediatric Research Foundation, Inc.

Rymen D.,Catholic University of Leuven | Rymen D.,University Hospital Gasthuisberg | Keldermans L.,Catholic University of Leuven | Race V.,Catholic University of Leuven | And 9 more authors.
Orphanet Journal of Rare Diseases | Year: 2012

Background: The Conserved Oligomeric Golgi (COG) complex is involved in the retrograde trafficking of Golgi components, thereby affecting the localization of Golgi glycosyltransferases. Deficiency of a COG-subunit leads to defective protein glycosylation, and thus Congenital Disorders of Glycosylation (CDG). Mutations in subunits 1, 4, 5, 6, 7 and 8 have been associated with CDG-II. The first patient with COG5-CDG was recently described (Paesold-Burda et al. Hum Mol Genet 2009; 18:4350-6). Contrary to most other COG-CDG cases, the patient presented a mild/moderate phenotype, i.e. moderate psychomotor retardation with language delay, truncal ataxia and slight hypotonia. Methods. CDG-IIx patients from our database were screened for mutations in COG5. Clinical data were compared. Brefeldin A treatment of fibroblasts and immunoblotting experiments were performed to support the diagnosis. Results and conclusion. We identified five new patients with proven COG5 deficiency. We conclude that the clinical picture is not always as mild as previously described. It rather comprises a broad spectrum with phenotypes ranging from mild to very severe. Interestingly, on a clinical basis some of the patients present a significant overlap with COG7-CDG, a finding which can probably be explained by subunit interactions at the protein level. © 2012 Rymen et al.; licensee BioMed Central Ltd.

Huysentruyt K.,Vrije Universiteit Brussel | Goyens P.,University Childrens Hospital Queen Fabiola | Alliet P.,Jessa Hospital | Bontems P.,Center Hospitalier University Tivoli | And 4 more authors.
Acta Paediatrica, International Journal of Paediatrics | Year: 2015

Aim Reports suggest that 10% of hospitalised children in Europe are undernourished. We investigated whether nutritional screening tools (NST) were used in Belgian secondary-level hospitals, examined strategies for detecting undernutrition and identified barriers preventing the systematic management of undernutrition. Methods A nationwide questionnaire-based survey of paediatric departments in Belgian secondary-level hospitals was carried out from September 2013 to February 2014. Respondents were dived into French-speaking (Walloon + Brussels) and Dutch-speaking (Flemish) departments. Results We received replies from 71 of the 97 (73.2%) departments. Half of the departments - 39.5% Flemish speaking and 71.4% Walloon speaking - carried out nutritional screening. Undernutrition was identified by measuring weight and length or height (92.7% of cases), clinical appraisal (74.7%), mid-upper arm circumference and/or skin fold thickness (19.7%). There was no protocol for undernutrition in many Flemish (60.5%)- and Walloon (28.6%)-speaking departments. Reasons given for not screening were as follows: lack of training (46.9%), ignorance of NST (42.2%) and lack of time (29.7%). Conclusion Half of the paediatric departments in Belgian secondary-level hospitals did not carry out nutritional screening, and differences in current practices and attitudes may be due to cultural and/or educational differences. ©2015 Foundation Acta Pædiatrica. Published by John Wiley & Sons Ltd.

Vandenplas Y.,Vrije Universiteit Brussel | De Ronne N.,Kind en Gezin | Van De Sompel A.,University of Antwerp | Huysentruyt K.,Vrije Universiteit Brussel | And 5 more authors.
European Journal of Pediatrics | Year: 2014

Growing-up milks (GUM) are milk-based drinks with low protein and added minerals and vitamins intended for children 12–36 months. Since the advantages of GUM are heavily debated, we reviewed the literature. A literature search was done using the classic databases (Pubmed, Embase, Cochrane) on the use of GUM in 12- to 36-month-old young children. Only limited data are available. GUM have a highly variable composition as their marketing is not regulated. Nevertheless, all papers conclude that GUM help to cover nutritional requirements of 12- to 36-month-old infants. Conclusion: Appropriate intakes of macro- and micronutrients in 1- to 3-year-old children have long-term health benefits. Present diets offered to toddlers do in general not meet the requirements. Supplemented foods are therefore helpful, of which GUM is a possibility. © 2014, Springer-Verlag Berlin Heidelberg.

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