Affiliations: [a] Children's Hospital Colorado, Aurora, CO, USA | [b] Department of Pediatrics, University of Colorado Denver School of Medicine, Aurora, CO, USA
Correspondence:
[*]
Corresponding author: Laura Watne, Children's Hospital Colorado, 13123 East 16th Avenue, Box 270, Aurora, CO 80045, USA. Tel.: +1 720 777 5369; Fax: +1 720 777 7282; E-mail:Laura.Watne@childrenscolorado.org
Abstract: OBJECTIVE: To assess the effect of nutritional interventions on
growth and on respiratory status in patients with congenital myopathy (CM),
congenital muscular dystrophy (CMD), and congenital myasthenic syndrome
(CMS). METHODS: Retrospective cohort study based on case-note review of 18
patients affected by CM, CMD, and CMS, followed at a single pediatric
neuromuscular center, between 2006 and 2014. RESULTS: Seventy-two percent of patients required placement of a
gastrostomy tube for bulbar weakness or for growth failure. Of those
patients, 10 had 1 year follow up anthropometric data and 6 had 2 year
follow up anthropometric data. Height percentiles and z-scores were
significantly improved in patients after 1 year, while weight and BMI
percentiles and z-scores were not. Weight and height percentiles and
z-scores were significantly improved in patients at 2 year follow up, while
BMI percentiles and z-scores were not. The number of respiratory illnesses
was not significantly different before or after placement of the feeding
tube. Of the patients who did not have placement of a gastrostomy tube, 4
had 1 year follow up anthropometric data and 3 had 2 year follow up
anthropometric data. Gastrostomy tube fed patients had significantly higher
mean weight percentiles and z-scores compared to orally fed patients. There
was no significant difference in height or BMI between the gastrostomy fed
and orally fed groups. Individual growth curves highlight the effect of
intervention on weight and height. CONCLUSIONS: This is a single multidisciplinary center experience
describing the effect of nutritional interventions on growth in patients
with congenital neuromuscular disorders. While the number of patients and
their data in this report are limited, it highlights that the growth in this
group of patients is unique but that the low weight and short stature
respond to nutritional interventions with changes typically seen after 2
years of intervention.
