Mucopolysaccharidosis II
Conditions
Interventions
A controlled clinical trial will be performed. Patients will be divided into two groups: 10 for intervention group (IMT Group) and 10 for sham group (GS). The guidelines of Resolution 466/12 of the Na
The equipment has a spring loaded valve so that inspiratory resistance can adjusted and adapted according to the maximum inspiratory pressure of the patient. Information will be given about responsibi
the healthy group will only do the evaluations (cross-sectional assessment) and will not participate in respiratory muscle training.
Device
E02.190.525.186
E02.779
Sponsors
Universidade Federal de Pernambuco
Universidade Federal de Pernambuco
Eligibility
Age
6 Years to 60 Years
Inclusion criteria
Inclusion criteria: Patients with a diagnosis of Mucopolysaccharidosis; confirmed by enzymatic dosing by blood test; from 6 to 60 years old; registered at the Breno Bloide Institute for rare diseases in the state of Pernambuco; Brazil; and who are over six years of age who agree to be part of the research and who sign the terms
Exclusion criteria
Exclusion criteria: Patients younger than six years of age and; over 60 years old; those who do not understand the controls for performing the evaluations will be excluded from the study; In relation to the specific evaluation tests; only those that are contraindicated for performing the specific test will be excluded
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Maximum inspiratory pressure (respiratory muscle strength) determined with the manovacuometer before and after Inspiratory Muscular Training, an increase of at least 20% in respiratory muscle strength (at the beginning of the study and at the end of the study) | — |
Secondary
| Measure | Time frame |
|---|---|
| It is expected to improve at least 10% in respiratory capacity, assessed by spirometry examination and optoelectronic plethysmography. Data collected immediately before the intervention and after the intervention.;It is expected to improve the mobility and diaphragmatic thickness, will be evaluated by the method of examination of diaphragmatic ultrasonography. Increases above 10% will be verified. Data collected immediately before the intervention and after the intervention. | — |
Countries
Brazil
Contacts
Public ContactBárbara Bernardo Figueirêdo
Universidade Federal de Pernambuco
Outcome results
None listed