Dysphagia in children with congenital myopathy, underlying mechanisms and advices for assessment and treatment

Dysphagia in children with congenital myopathy, underlying mechanisms and advices for assessment and treatment - Dysphagia in children with congenital myopathy

Status

Active, not recruiting

Phases

Unknown

Study type

Observational

Source

NL-OMON

Registry ID

NL-OMON42147

Enrollment

Registered

2014-12-16

Start date

2017-04-11

Completion date

Unknown

Last updated

2024-04-23

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Slikproblemen (dysfagie) Dysphagia

Interventions

None listed

Eligibility

Age

2 Years to 11 Years

Inclusion criteria

Inclusion criteria: Healthy children, part 1 of the study: - Age between 6 months and 5 years - Healthy children with age appropriate growth;Patients, part 2 of the study: - Diagnosed with congenital myopathy from 6 months until 12 years of age (confirmed or a not yet totally classified myopathy) - Known with dysphagia or feeding problems

Exclusion criteria

Exclusion criteria: Healthy children, part 1 of the study: - Dysphagia - Feeding problems, requiring tube feeding - Neuromuscular disorder - Diagnosis of genetic syndrome;Patients, part 2 of the study: - Totally on tube feeding from birth with no oral feeding experience

Design outcomes

Primary

Measure	Time frame
Part 1 of the study, healthy infants and children - Demographic data: sex, age, length and weight - Phase of development feeding (1 - only breast or bottle feeding; 2- breast or bottle feeding and spoon feeding (pureed food) ; 3- chewing (solid food). - Echo intensity of the various oral muscles (with gray scale analysis, 0-244) - Thickness of the various oral muscles (in cm) Regression analysis will be performed to measure which variables (sex, age, height and weight) are influencing echo intensity and muscle thickness. With these equations normal values can be established. Possible deviations can be described in Z-scores (amount of standard deviations above or under normal). Part 2 of the study, patients with a congenital myopathy - Demographic data: sex, age, length and weight - Functional activities based on the BSID-3 (until 2 years of age) or the Motor Function Measure scale (0-100), (measured by the physical therapist) - Phase of development feeding (1 - only breast or bottle feeding; 2- breast or bottle feeding and spoon feeding (pureed food) ; 3- chewing (solid food). - (1) Observation List Spoon feeding (score 0-35); (2) outcome SOMA per function (drinking, chewing); (3) Score on the Dysphagia Disorder Survey: : 0 - no dysphagia, 1- mild dysphagia, 2 - moderate dysphagia, 3- severe dysphagia, 4 - profound dysphagia. - Activity submental muscle group (sEMG) and nasal flow: percentage differences between thin liquid and thick liquid during swallowing in duration (in sec) and amplitude (in µV). Coordination between swallowing and breathing will be described (1- inspiration - swallow - expiration; 2 - inspiration - swallow - inspiration; 3- expiration - swallow - inspiration; 4 - otherwise) - Data of the analysis of the videofluroscopic swallow study, based on a list of 12 signs which are related to underlying dysphagia (score yes or no) - Echo intensity of the various oral muscles (with gray scale analysis, 0-244), expressed as Z-s	—

Measure

Time frame

Part 1 of the study, healthy infants and children - Demographic data: sex, age, length and weight - Phase of development feeding (1 - only breast or bottle feeding; 2- breast or bottle feeding and spoon feeding (pureed food) ; 3- chewing (solid food). - Echo intensity of the various oral muscles (with gray scale analysis, 0-244) - Thickness of the various oral muscles (in cm) Regression analysis will be performed to measure which variables (sex, age, height and weight) are influencing echo intensity and muscle thickness. With these equations normal values can be established. Possible deviations can be described in Z-scores (amount of standard deviations above or under normal). Part 2 of the study, patients with a congenital myopathy - Demographic data: sex, age, length and weight - Functional activities based on the BSID-3 (until 2 years of age) or the Motor Function Measure scale (0-100), (measured by the physical therapist) - Phase of development feeding (1 - only breast or bottle feeding; 2- breast or bottle feeding and spoon feeding (pureed food) ; 3- chewing (solid food). - (1) Observation List Spoon feeding (score 0-35); (2) outcome SOMA per function (drinking, chewing); (3) Score on the Dysphagia Disorder Survey: : 0 - no dysphagia, 1- mild dysphagia, 2 - moderate dysphagia, 3- severe dysphagia, 4 - profound dysphagia. - Activity submental muscle group (sEMG) and nasal flow: percentage differences between thin liquid and thick liquid during swallowing in duration (in sec) and amplitude (in µV). Coordination between swallowing and breathing will be described (1- inspiration - swallow - expiration; 2 - inspiration - swallow - inspiration; 3- expiration - swallow - inspiration; 4 - otherwise) - Data of the analysis of the videofluroscopic swallow study, based on a list of 12 signs which are related to underlying dysphagia (score yes or no) - Echo intensity of the various oral muscles (with gray scale analysis, 0-244), expressed as Z-s

—

Secondary

Measure	Time frame
-	—

Countries

Netherlands

Outcome results