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Title: Gastrostomy placement favorably alters the natural history of growth failure and undernutrition in 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: Abstract Only
Publication Acceptance Date: 8/1/2007
Publication Date: 10/1/2007
Citation: Motil, K.J., Morrissey, M., Caeg, E., Barrish, J.O., Glaze, D.G. 2007. Gastrostomy placement favorably alters the natural history of growth failure and undernutrition in Rett syndrome. Journal of Pediatric Gastroenterology and Nutrition [abstract]. Journal of Pediatric Gastroenterology and Nutrition. 45(4):E17.

Interpretive Summary:

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. Our objective was to determine if gastrostomy placement for nutritional therapy alters the natural history of growth failure and undernutrition in RTT. We hypothesized that aggressive nutritional therapy with gastrostomy feedings normalizes height, weight, and body mass index (BMI) z-scores in RTT. Height and weight were measured in a cohort of girls (n = 84) with RTT pre- (age 6.1+/-3.9 y) and post- (age 11.7+/-6.1 y) gastrostomy placement with or without fundoplication. BMI was calculated from height and weight measures. Birth length and weight were recorded from parental recall. Height (length), weight, and BMI measures were converted to z-scores based on the NCHS standards. Paired t-tests were used to detect differences in the change in height and weight z-scores pre- and post-gastrostomy placement. Birth length and weight z-scores were 0.2+/-1.1 and 0.4+/-0.9, respectively. Differences in the change in height (n = 56) and weight (n = 69) z-scores between birth to pre-gastrostomy and pre- to post-gastrostomy were 1.31+/-2.06 (P<0.001) and 2.38+/-3.18 (P<0.001), respectively. BMI z-scores (1.24+/-1.76, P<0.001) increased post-gastrostomy. BMI zscores post-gastrostomy were higher in the subset of RTT girls (n = 58) with fundoplication than in those (n = 26) without fundoplication (0.4+/-1.23 vs. 1.13+/-1.47, P<0.05). Aggressive nutritional therapy with Gastrostomy feeding alters the natural history of growth failure and undernutrition in RTT. Fundoplication supports the reversal of undernutrition in RTT. Further improvement in the genetic growth potential of individuals with RTT is yet to be determined.