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Neuropediatrics 2011; 42(05): 179-182
DOI: 10.1055/s-0031-1287841
Original Article
© Georg Thieme Verlag KG Stuttgart · New York

Increased Resting Metabolic Rate in Girls with Rett Syndrome Compared to Girls with Developmental Disabilities

P. Platte
1   Department of Psychology, Biological Psychology, Clinical Psychology and Psychotherapy, University of Würzburg, Würzburg, Germany
,
H. Jaschke
2   Department of Neuropediatrics, Children’s Hospital, Klinikum Oldenburg, Oldenburg, Germany
,
C. Herbert
1   Department of Psychology, Biological Psychology, Clinical Psychology and Psychotherapy, University of Würzburg, Würzburg, Germany
,
G. C. Korenke
2   Department of Neuropediatrics, Children’s Hospital, Klinikum Oldenburg, Oldenburg, Germany
› Author Affiliations
Further Information

Publication History

received 05 April 2011

accepted 09 September 2011

Publication Date:
17 October 2011 (online)

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Abstract

The aim of this study was to determine the body composition and resting metabolic rate (RMR) of girls with Rett syndrome (RS) (n=15) and to compare them with an equally handicapped group of girls with developmental disabilities (DD) (n=13). Body composition was measured by bioelectrical impedance analysis and RMR  −  the amount of energy expended while at rest  −  by indirect calorimetry. Weight, height, body mass index (BMI), BMI percentiles and food intake were all measured and calculated by standardized procedures. Feeding time, ambulatory status and ability to self-feed were also assessed. Due to the sampling, there were no significant differences in age, height, weight, BMI, BMI percentiles and ambulatory status. Significant differences between groups were found for lower percentage lean body mass (LBM) (64.2±14.6 vs. 84.4±24.6) and higher absolute and relative fat mass (FM) in RS. RMR values adjusted for LBM were significantly higher in the group of girls with RS (approximately 160 kcal/day), indicating that higher energy expenditure is a component of increased risk of severely low body weight.

Key words

Rett syndrome - resting metabolic rate - body composition
 

  • References

  • 1 Amir RE, Van den Veyver IB, Wan M et al. Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2. Nat Genet 1999; 23: 185-188
    CrossrefPubMedGoogle Scholar
  • 2 Björntorp P. Do stress reactions cause abdominal obesity and comorbidities?. Obesity Rev 2001; 2: 73-86
    CrossrefPubMedGoogle Scholar
  • 3 Blardi P, de Lalla A, D’Ambrogio T et al. Long-term plasma levels of leptin and adiponectin in Rett syndrome. Clin Endocrinol (Oxf) 2009; 70: 706-709
    CrossrefPubMedGoogle Scholar
  • 4 Fyffe SL, Neul JF, Samaco RC et al. Deletion of Mecp2 in Sim1-expressing neurons reveals a critical role for Mecp2 in feeding behavior, aggression, and the response to stress. Neuron 2008; 59: 947-958
    CrossrefPubMedGoogle Scholar
  • 5 Halbach NS, Smeets EE, Schrander-Stumpel CT et al. Aging in people with specific genetic syndromes: Rett syndrome. Am J Med Genet A 2008; 146: 1925-1932
    PubMedGoogle Scholar
  • 6 Harris HA, Benedict FG. A biometric study of basal metabolism in man. 1919. Washington, DC: Carnegie Institute of Wahington; Publ. No. 279
    Google Scholar
  • 7 Huppke P, Roth C, Christen HJ et al. Endocrinological study on growth retardation in Rett syndrome. Acta Paediatr 2001; 90: 1257-1261
    CrossrefPubMedGoogle Scholar
  • 8 Kromeyer-Hauschild K, Wabitsch M, Kunze D et al. Perzentilen für den Body-mass-Index für das Kindes- und Jugendalter unter Heranziehung verschiedener deutscher Stichproben. Monatsschr Kinderheilkd 2001; 149: 807-818
    CrossrefPubMedGoogle Scholar
  • 9 Isaacs JS, Murdock M, Lane J et al. Eating difficulties in girls with Rett syndrome compared with other developmental disabilities. J Am Diet Assoc 2003; 103: 224-230
    CrossrefPubMedGoogle Scholar
  • 10 Landig J, Erhardt JG, Bode JC et al. Validation and comparison of two computerized methods of obtaining a diet history. Clin Nutr 1998; 17: 113-117
    CrossrefPubMedGoogle Scholar
  • 11 Leonard H, Thomson R, Glasson E et al. A population based approach to the investigation of osteopenia in Rett syndrome. Dev Med Child Neurol 1999; 41: 323-328
    CrossrefPubMedGoogle Scholar
  • 12 Motil KJ, Schultz R, Brown B et al. Altered energy balance may account for growth failure in Rett syndrome. J Child Neurol 1994; 9: 315-319
    CrossrefPubMedGoogle Scholar
  • 13 Motil KJ, Schultz RJ, Wong WW et al. Increased energy expenditure associated with repetitive involuntary movement does not contribute to growth failure in girls with Rett syndrome. J Pediatr 1998; 132: 228-233
    CrossrefPubMedGoogle Scholar
  • 14 Nuber UA, Kriaucionis S, Roloff TC et al. Up-regulation of glucocorticoid-regulated genes in a mouse model of Rett syndrome. Hum Mol Gen 2005; 14: 2247-2256
    CrossrefPubMedGoogle Scholar
  • 15 Oddy WH, Webb KG, Baikie G et al. Feeding experiences and growth status in a Rett syndrome population. J Pediatr Gastroenterol Nutr 2007; 45: 582-590
    CrossrefPubMedGoogle Scholar
  • 16 Platte P, Wade SE, Pirke KM et al. Physical activity, total energy expenditure, and food intake in grossly obese and normal weight women. Int J Eat Dis 1995; 17: 51-57
    CrossrefPubMedGoogle Scholar
  • 17 Reilly S, Cass H. Growth and nutrition in Rett syndrome. Disabil Rehabil 2001; 23: 118-128
    CrossrefPubMedGoogle Scholar
  • 18 Schultz RJ, Glaze DG, Motil KJ et al. The pattern of growth failure in Rett syndrome. Am J Dis Child 1993; 147: 633-637
    PubMedGoogle Scholar
  • 19 Sert C, Aldingdag O, Sirmatel F. Determination of basal metabolic rate and body composition with bioelectrical impedance method in children with cerebral palsy. J Child Neurol 2009; 24: 237-240
    CrossrefPubMedGoogle Scholar
  • 20 Stallings VA, Zemel BS, Davies JC et al. Energy expenditure of children and adolescents with severe disabilities: a cerebral palsy model. Am J Clin Nutr 1996; 64: 627-634
    PubMedGoogle Scholar
  • 21 Trevathan E, Naidu S. The clinical recognition and differential diagnosis of Rett syndrome. J Child Neurol 1988; 3 (Suppl) S6-S16
    CrossrefPubMedGoogle Scholar
  • 22 Venthama JC, Reillya JJ. Reproducibility of resting metabolic rate measurement in children. Br J Nutr 1999; 81: 435-437
    PubMedGoogle Scholar
  • 23 Weir J. New methods for calculating metabolic rate with special reference to protein metabolism. J Physiol 1949; 109: 1-9
    PubMedGoogle Scholar