Feeding Difficulties and Orofacial Myofunctional Disorder in Patients with Hepatic Glycogen Storage Diseases.

ABSTRACT: Hepatic glycogen storage diseases (GSDs) are inborn errors of metabolism whose dietary treatment involves uncooked cornstarch administration and restriction of simple carbohydrate intake. The prevalence of feeding difficulties (FDs) and orofacial myofunctional disorders (OMDs) in these patients is unknown.

Objective

To ascertain the prevalence of FDs and OMDs in GSD.

Methods

This was a cross-sectional, prospective study of 36 patients (19 males; median age, 12.0 years; range, 8.0-18.7 years) with confirmed diagnoses of GSD (type Ia = 22; Ib = 8; III = 2; IXa = 3; IXc = 1). All patients were being treated by medical geneticists and dietitians. Evaluation included a questionnaire for evaluation of feeding behavior, the orofacial myofunctional evaluation (AMIOFE), olfactory and

SUBMITTER: Martinez CC 

PROVIDER: S-EPMC6336547 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature