首页|Anthropometric Measures Correspond with Functional Motor Outcomes in Females with Rett Syndrome

Anthropometric Measures Correspond with Functional Motor Outcomes in Females with Rett Syndrome

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  • NSTL
  • Elsevier
? 2022 Elsevier Inc.Objective: To characterize growth and anthropometric measurements in females with Rett syndrome and compare these measurements with functional outcomes. Study design: We obtained longitudinal growth and anthropometric measurements from 1154 females with classic and atypical Rett syndrome seen between 2006 and 2019 in the US Natural History Study. We calculated the Clinical Severity Score, Motor Behavior Assessment score, and arm and leg muscle areas and recorded the functional assessments of arm and hand use and ambulation. We compared growth and anthropometric variables from females with Rett syndrome in regard to normative data. We analyzed Clinical Severity Score, Motor Behavior Assessment, and anthropometric measurements in regard to functional assessments. Results: Growth and anthropometric measurements were significantly lower in females with classic and severe atypical Rett syndrome compared with those classified as mild atypical Rett syndrome and deviated from normative patterns among all 3 groups. Suprailiac skinfold measurements correlated with body mass index measurements in each group. Lower leg muscle area measurements were significantly greater among females in all 3 Rett syndrome groups who ambulated independently compared with those who did not. In females with classic Rett syndrome, arm, thigh, and lower leg muscle area measurements increased significantly over time and were significantly greater among those who had purposeful arm and hand use and independent ambulation compared with those who did not. Conclusions: The pattern of growth and anthropometric measures in females with Rett syndrome differs from normative data and demonstrates clear differences between classic and mild or severe atypical Rett syndrome. Anthropometric measures correspond with functional outcomes and could provide markers supporting efficacy outcomes in clinical trials.

Motil K.J.、Geerts S.、Annese F.、Neul J.L.、Benke T.、Marsh E.、Lieberman D.、Skinner S.A.、Glaze D.G.、Heydemann P.、Beisang A.、Standridge S.、Ryther R.、Lane J.B.、Edwards L.、Percy A.K.

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Children's Nutrition Research Center Baylor College of Medicine

Sparks Clinics/Nutrition University of Alabama at Birmingham

Genetics Center Greenwood Genetic Center

Department of Pediatrics/Child Neurology Vanderbilt Kennedy Center Vanderbilt University Medical

Department of Pediatrics/Child Neurology University of Colorado-Denver

Department of Pediatrics/Child Neurology Children's Hospital of Philadelphia University of

Department of Child Neurology Children's Hospital Boston Harvard University

Department of Pediatrics/Child Neurology Baylor College of Medicine

Department of Pediatrics/Child Neurology Rush Medical Center

Department of Pediatrics Gillette Children's Hospital

Department of Pediatrics/Child Neurology Cincinnati Children's Hospital

Department of Pediatrics/Child Neurology Washington University

Department of Pediatrics/Child Neurology University of Alabama at Birmingham

School of Public Health/Biostatistics University of Alabama at Birmingham

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2022

10.1016/j.jpeds.2022.01.009

The Journal of pediatrics

The Journal of pediatrics

ISSN：0022-3476
年,卷(期)：2022.244
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