Feeding impairments associated with plasma sterols in Smith-Lemli-Opitz syndrome.

ABSTRACT:

Objective

To quantitatively evaluate feeding impairment in children with Smith-Lemli-Opitz syndrome (SLOS) and to correlate feeding impairment with clinical and biochemical indices of disease severity.

Study design

The study subjects were 26 children with SLOS ranging in age from 0.4 to 19 years. Clinical severity was measured using an existing scoring system. We created a tool to quantitatively evaluate feeding. Plasma sterol concentrations were measured, and statistical associations (correlations) with feeding scores were calculated.

Results

Oral hyposensitivity or hypersensitivity, adverse behaviors, and risk for dysphagia were seen in ∼65% of the children with SLOS. Thirteen of the 26 children experienced failure to thrive, and 10 children required gastrostomy. Pl

SUBMITTER: Merkens MJ 

PROVIDER: S-EPMC4177270 | biostudies-literature | 2014 Oct

REPOSITORIES: biostudies-literature