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01-07-2009 | Original Article

Intellectual and motor performance, quality of life and psychosocial adjustment in children with cystinosis

Authors: Francis F. Ulmer, Markus A. Landolt, Russia Ha Vinh, Thierry A. G. M. Huisman, Thomas J. Neuhaus, Bea Latal, Guido F. Laube

Published in: Pediatric Nephrology | Issue 7/2009

Abstract

Cystinosis is a rare multisystemic progressive disorder mandating lifelong medical treatment. Knowledge on the intellectual and motor functioning, health-related quality of life and psychosocial adjustment in children with cystinosis is limited. We have investigated nine patients (four after renal transplantation) at a median age of 9.7 years (range 5.3–19.9 years). Intellectual performance (IP) was analysed with the Wechsler Intelligence Scale for Children-III (seven children) and the Kaufman Assessment Battery for Children (two children). Motor performance (MP) was evaluated using the Zurich Neuromotor Assessment Test, and quality of life (QOL) was studied by means of the Netherlands Organization for Applied Scientific Research Academical Medical Center Child Quality of Life Questionnaire. Psychosocial adjustment was assessed by the Child Behavior Checklist. The overall intelligence quotient (IQ) of our patient cohort (median 92, range 71–105) was significantly lower than that of the healthy controls (p = 0.04), with two patients having an IQ < 85. Verbal IQ (93, range 76–118) was significantly higher than performance IQ (90, range 68–97; p = 0.03). The MP was significantly below the norm for pure motor, pegboard and static balance, as well as for movement quality. The patients’ QOL was normal for six of seven dimensions (exception being positive emotions), whereas parents reported significant impairment in positive emotions, autonomy, social and cognitive functions. Significant disturbance was noted in terms of psychosocial adjustment. Based on the results from our small patient cohort, we conclude that intellectual and motor performance, health-related QOL and psychosocial adjustment are significantly impaired in children and adolescents with cystinosis.

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Title: Intellectual and motor performance, quality of life and psychosocial adjustment in children with cystinosis
Authors: Francis F. Ulmer
Markus A. Landolt
Russia Ha Vinh
Thierry A. G. M. Huisman
Thomas J. Neuhaus
Bea Latal
Guido F. Laube
Publication date: 01-07-2009
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Nephrology / Issue 7/2009
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-009-1149-2

At a glance: The STEP trials

Springer Medicine

Intellectual and motor performance, quality of life and psychosocial adjustment in children with cystinosis

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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