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Feeding difficulties and orofacial myofunctional disorder in patients with hepatic glycogen storage diseases

 

Table Of Contents


Chapter ONE

1.1 Introduction
1.2 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 Feeding Difficulties
2.2 Understanding Orofacial Myofunctional Disorders
2.3 Relationship Between Hepatic Glycogen Storage Diseases and Feeding Difficulties
2.4 Impact of Orofacial Myofunctional Disorders on Patients
2.5 Diagnosis and Assessment of Feeding Difficulties in Patients
2.6 Treatment Approaches for Orofacial Myofunctional Disorders
2.7 Case Studies on Patients with Hepatic Glycogen Storage Diseases
2.8 Research on Feeding Difficulties and Orofacial Myofunctional Disorders
2.9 Current Trends and Developments in the Field
2.10 Gaps in Literature and Research Needs

Chapter THREE

3.1 Research Methodology Overview
3.2 Research Design and Approach
3.3 Sampling Techniques and Participants
3.4 Data Collection Methods
3.5 Data Analysis Procedures
3.6 Ethical Considerations
3.7 Validity and Reliability of Data
3.8 Limitations of the Research Methodology

Chapter FOUR

4.1 Overview of Findings
4.2 Analysis of Feeding Difficulties in Patients with Hepatic Glycogen Storage Diseases
4.3 Examination of Orofacial Myofunctional Disorders in the Study Population
4.4 Comparison of Treatment Outcomes for Patients with and without Orofacial Myofunctional Disorders
4.5 Factors Influencing Feeding Difficulties and Orofacial Myofunctional Disorders
4.6 Discussion on the Relationship Between Hepatic Glycogen Storage Diseases and Orofacial Myofunctional Disorders
4.7 Recommendations for Clinical Practice and Further Research
4.8 Implications for Healthcare Providers

Chapter FIVE

5.1 Summary of Findings
5.2 Conclusion and Interpretation of Results
5.3 Contributions to the Field of Medicine
5.4 Practical Applications and Recommendations
5.5 Future Research Directions

Thesis 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.

Thesis Overview

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