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| ID | Type | Description | Link |
|---|---|---|---|
| 2017-A02202-51 | Other Identifier | ID-RCB |
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To compensate for insufficiency of diagnostic tools, the present study propose to look for the predictive factors of an early fitting by noninvasive ventilation.
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease. The diagnosis of this diaphragmatic dysfunction in the ALS subject is done all too often on the occasion of a hospitalization for acute respiratory insufficiency, which aggravates then the prognosis of the patients.
The presence of these factors at diagnosis will allow more surveillance to detect early diaphragmatic insufficiency and establish a NIV (Non Invasive Ventilation), the only therapeutic measure definitely improving the quality of life and survival of patients. A clinical score will be established to determine the risk of early Non Invasive Ventilation (NIV) equipment.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| patients with a NIV equipment | Experimental | Determination of diagnosis variables in a group of patient who benefit of the Non Invasive ventilation equipment one year after the diagnostic |
|
| patients without a NIV equipment | Active Comparator | Determination of diagnosis variables in a group of patient who not benefit of the Non Invasive ventilation equipment one year after the diagnostic |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| diagnosis variables | Diagnostic Test | The study compare the diagnostic variables in patients who received Non invasive ventilation equipment one year after diagnosis with the patient who don't need it. For the independent variables, the present study determine a threshold at the diagnosis for which an early fitting risk appears The diagnostic variable are :
|
| Measure | Description | Time Frame |
|---|---|---|
| Early Surgical Clinical Score | An Early Surgical Clinical Score will be established taking into account the independent variables correlated to diagnosis at early fitting. Clinical Variables :
Functional Variables :
| One year after diagnostic |
| Measure | Description | Time Frame |
|---|---|---|
| Sensitivity, specificity, negative and positive predictive values | Sensitivity, specificity, negative and positive predictive values of the diaphragmatic ultrasound (fraction of shortening and diaphragmatic stroke) compared to the SNIP test and for the values of PSG (Polysomnography) compared to SNIP test | One year after the diagnostic |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Marion Dupuis, MD | Contact | 5 67 77 16 91 | 33 | dupuis.m@chu-toulouse.fr |
| Isabelle Olivier, PhD | Contact | 5 61 77 70 51 | 33 | olivier.i@chu-toulouse.fr |
| Name | Affiliation | Role |
|---|---|---|
| Marion Dupuis, MD | University Hospital, Toulouse | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University Hospital Toulouse | Recruiting | Toulouse | 31052 | France |
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| ID | Term |
|---|---|
| D000690 | Amyotrophic Lateral Sclerosis |
| ID | Term |
|---|---|
| D013118 | Spinal Cord Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D016472 | Motor Neuron Disease |
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The study compare the diagnostic's variables in patients who received NIV one year after diagnosis and those who did not.
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|
| Percentage of decrease |
% decrease in shortening fraction and diaphragmatic stroke between two quarterly reevaluations correlated with early fitting and % decrease in FVC, SNIP test et Pimax |
| One year after the diagnostic |
| Slope of FVC decline | Slope of FVC decline before and after NIV implementation and slope of decline of shortening fraction and diaphragmatic stroke before and after NIV | One year after the diagnostic |
| Quality of life score | Quality of life score by the Severe Respiratory Insufficiency (SRI) questionnaire :before and after implementation of the NIV The Severe Respiratory Insufficiency (SRI) Questionnaire: a specific measure of health-related quality of life in patients receiving home mechanical ventilation. | One year after the diagnostic |
| D019636 | Neurodegenerative Diseases |
| D057177 | TDP-43 Proteinopathies |
| D009468 | Neuromuscular Diseases |
| D057165 | Proteostasis Deficiencies |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |