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Title: Gastrostomy placement improves height and weight gain in girls with Rett syndrome

Author
item MOTIL, KATHLEEN - Children'S Nutrition Research Center (CNRC)
item MORRISSEY, MATTHEW - Baylor College Of Medicine
item CAEG, ERWIN - Baylor College Of Medicine
item BARRISH, JUDY - Baylor College Of Medicine
item GLAZE, DANIEL - Baylor College Of Medicine

Submitted to: Journal of Pediatric Gastroenterology and Nutrition
Publication Type: Peer Reviewed Journal
Publication Acceptance Date: 9/26/2008
Publication Date: 8/1/2009
Citation: Motil, K.J., Morrissey, M., Caeg, E., Barrish, J.O., Glaze, D.G. 2009. Gastrostomy placement improves height and weight gain in girls with Rett syndrome. Journal of Pediatric Gastroenterology and Nutrition. 49(2):237-242.

Interpretive Summary: Growth failure and undernutrition complicate the clinical course of girls with Rett syndrome (RTT). These abnormalities are, in part, the consequence of oral motor dysfunction and inadequate dietary intake. We sought to determine if gastrostomy placement for nutritional therapy altered the natural history of growth failure and undernutrition in RTT. We found that gastrostomy placement for nutritional therapy favorably altered the natural history of growth failure and undernutrition in RTT by improving weight and BMI z-scores, as well as the body fat and fat-free mass, of the RTT cohort, regardless of the age at which surgery occurred and in the presence or absence of a fundoplication. Our study is important because it provides evidence-based practice guidelines in support of gastrostomy feeding for neurologically-impaired children. Aggressive nutritional interventions via a feeding gastrostomy improves health and well-being, reverses growth delays, decreases infections, reduces hospitalizations, and enhances quality of life of children with neurological disabilities.

Technical Abstract: Growth failure and undernutrition complicate the clinical course of girls with Rett syndrome (RTT). These abnormalities are, in part, the consequence of oral motor dysfunction and inadequate dietary intake. We hypothesized that gastrostomy placement for nutritional therapy reverses the decline in height, weight, and body mass index (BMI) z scores in RTT. Standard stadiometric and anthropometric measures were obtained to derive height, weight, and BMI z scores and estimates of fat-free mass (FFM) and body fat in a cohort of girls (n = 92) with RTT before and after gastrostomy placement. Methyl-CpG-binding protein two (MECP2) mutations and the presence or absences of a fundoplication were recorded. The differences in height (n = 73), weight (n = 81), and BMI (n = 81) z score slopes before and after gastrostomy placement were 1.31 +/- 2.06 (P < 0.001), 2.38 +/- 3.18 (P < 0.001), and 3.25 +/- 3.32 (P < 0.001), respectively. FFM and body fat (n = 43) increased after gastrostomy by 41 +/- 27 g/cm height (P < 0.001) and 7.5% +/- 5.7% body weight (P < 0.001), respectively. The differences in height, weight, and BMI z score slopes were similar regardless of the age at which the gastrostomy was placed. The differences in height, weight, and BMI z score slopes, as well as the change in FFM and body fat deposition after gastrostomy placement, did not differ between those who did or did not have a fundoplication and among the classes of MECP2 mutations. Gastrostomy placement for aggressive nutritional therapy favorably altered the natural history of growth failure and undernutrition in RTT, but did not restore height and weight z scores to birth values, regardless of the age at which surgery occurred and in the presence or absence of a fundoplication.