Feeding difficulties and orofacial myofunctional disorder in patients with hepatic glycogen storage diseases
Table Of Contents
Chapter ONE
1.1 Introduction1.2 Background of Study
1.3 Problem Statement
1.4 Objective of Study
1.5 Limitation of Study
1.6 Scope of Study
1.7 Significance of Study
1.8 Structure of the Research
1.9 Definition of Terms
Chapter TWO
2.1 Overview of Orofacial Myofunctional Disorders2.2 Hepatic Glycogen Storage Diseases: An Overview
2.3 Feeding Difficulties in Patients with Hepatic Glycogen Storage Diseases
2.4 Orofacial Myofunctional Disorders in Patients with Hepatic Glycogen Storage Diseases
2.5 Relationship Between Feeding Difficulties and Orofacial Myofunctional Disorders
2.6 Diagnosis of Orofacial Myofunctional Disorders
2.7 Treatment Approaches for Orofacial Myofunctional Disorders
2.8 Impact of Orofacial Myofunctional Disorders on Quality of Life
2.9 Current Research on Feeding Difficulties and Orofacial Myofunctional Disorders
2.10 Gaps in Literature Review
Chapter THREE
3.1 Research Design and Methodology3.2 Sampling Techniques
3.3 Data Collection Methods
3.4 Data Analysis Techniques
3.5 Ethical Considerations
3.6 Instrumentation and Tools
3.7 Reliability and Validity
3.8 Limitations of the Methodology
Chapter FOUR
4.1 Overview of Research Findings4.2 Analysis of Feeding Difficulties in Patients with Hepatic Glycogen Storage Diseases
4.3 Analysis of Orofacial Myofunctional Disorders in Patients with Hepatic Glycogen Storage Diseases
4.4 Correlation Between Feeding Difficulties and Orofacial Myofunctional Disorders
4.5 Comparison of Treatment Approaches
4.6 Impact of Orofacial Myofunctional Disorders on Quality of Life
4.7 Discussion on Research Findings
4.8 Recommendations for Future Research
Chapter FIVE
5.1 Conclusion and Summary of the Research5.2 Implications of the Findings
5.3 Contributions to the Field
5.4 Practical Applications
5.5 Suggestions for Clinical Practice
Project 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 taste performance (Sniffin’ Sticks and Taste Strips tests), and facial anthropometry. Results Nine (25%) patients had decreased olfactory perception, and four (11%) had decreased taste perception for all flavours. Eight patients (22.2%) had decreased perception for sour taste. Twenty-six patients (72.2%) had FD, and 18 (50%) had OMD. OMD was significantly associated with FD, tube feeding, selective intake, preference for fluid and semisolid foods, and mealtime stress (p < 0.05). Thirteen patients (36.1%) exhibited mouth or oronasal breathing, which was significantly associated with selective intake (p = 0.011) and not eating together with the rest of the family (p = 0.041). Lower swallowing and chewing scores were associated with FD and with specific issues related to eating behavior (p < 0.05). Conclusion There is a high prevalence of FDs and OMDs in patients with GSD. Eating behavior, decreased taste and smell perception, and orofacial myofunctional issues are associated with GSD.
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