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The main aim of this study is therefore to evaluate the impact of Prader Willi Syndrome (PWS) on lung and thoracoabdominal volumes, respiratory muscle strength and awake ventilatory pattern, and to quantify and localize the PWS-induced respiratory restriction.
The aim is to study lung function in obese PWS patients using both volitional (spirometry, nitrogen wash-out, and maximal pressure measurements (MIP and MEP)) and non-volitional (awake ventilatory pattern by opto-electron plethysmography) tests, both in sitting and supine positions.
Spirometry, nitrogen wash-out, and MIP and MEP measurements are comprehensive assessments, while optoelectronic plethysmography allows restriction location through accurate measurement of thoracoabdominal volumes.
The results of the study could provide useful information about the calculation of the volumes enclosed by the grid of markers (in particular, the volume of the rib cage, which reflects the action of the intercostal muscles, the volume of the abdomen, which reflects the action of the diaphragm in the inspiratory phase and of the abdominal muscles during exhalation and their sum, which represents the total variation of the thoraco-abdominal wall. All these finds could be useful for respiratory rehabilitation protocols for obese PWS patients.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Patients with Prader Will syndrome and BMI > 30 kg/m2 |
| ||
| Control subjects with BMI < 25 kg/m2 |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Spirometry and optoelectronic plethysmography | Diagnostic Test | Spirometry for respiratory function and optoelectronic plethysmography for respiratory pattern during wakefulness |
|
| Measure | Description | Time Frame |
|---|---|---|
| Rib cage contribution to ventilation | Percentage rib cage contribution to ventilation as measured by optoelectronic plethysmography | At enrollment |
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Patients with Prader Willi Syndrome
Inclusion Criteria:
Exclusion Criteria:
Control subjects
Inclusion Criteria:
Exclusion Criteria:
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Patients with Prader Willi Syndrome and obesity
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Istituto Auxologico Italiano IRCCS, Site Piancavallo | Oggebbio | Verbania | 28824 | Italy |
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| ID | Term |
|---|---|
| D011218 | Prader-Willi Syndrome |
| ID | Term |
|---|---|
| D008607 | Intellectual Disability |
| D019954 | Neurobehavioral Manifestations |
| D009461 | Neurologic Manifestations |
| D009422 | Nervous System Diseases |
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| ID | Term |
|---|---|
| D013147 | Spirometry |
| ID | Term |
|---|---|
| D012129 | Respiratory Function Tests |
| D003948 | Diagnostic Techniques, Respiratory System |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
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| D000015 | Abnormalities, Multiple |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D025063 | Chromosome Disorders |
| D030342 | Genetic Diseases, Inborn |
| D000096803 | Imprinting Disorders |
| D009765 | Obesity |
| D050177 | Overweight |
| D044343 | Overnutrition |
| D009748 | Nutrition Disorders |
| D009750 | Nutritional and Metabolic Diseases |