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Sleep Patterns in Patients Affected by Lymphangioleiomiomatosis

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ClinicalTrials.gov Identifier: NCT04577937
Recruitment Status : Recruiting
First Posted : October 8, 2020
Last Update Posted : October 8, 2020
Sponsor:
Information provided by (Responsible Party):
dr. Stefano Nava, Azienda Ospedaliera Universitaria di Bologna Policlinico S. Orsola Malpighi

Brief Summary:

Lymphangioleiomyomatosis (LAM) is a rare and progressive pulmonary disease of unknown etiology that almost exclusively affects women. It is characterised by cystic radiological lung pattern and by the possible presence of angiomyolipomas in other sites or organs. Functionally LAM is associated with airway obstruction or restriction and progressive hypoxemia up to chronic respiratory failure.

There are no studies, so far, which have investigated whether during sleep these patients show changes in the sleep profile and gas exchange and if these changes are related to disease severity. Aim of the study, prospective and pilot, is to evaluate whether the physiological modification of respiratory mechanics during sleep is associated with polysomnographic alterations in LAM.


Condition or disease Intervention/treatment Phase
Lymphangioleiomyomatosis Sleep Disorder Sleep Apnea Syndromes Hypopnea Syndrome Diagnostic Test: LAM patients underwent PSG Not Applicable

Detailed Description:

Lymphangioleiomiomatosis (LAM) is a rare progressive disease that affects primarily lungs with a cystic radiologic pattern and may be associated with angiomiolipomas in the kidneys or in other sites and an increased frequency of meningioma. Patients with sporadic LAM are usually female (incidence around 1/400.000 adult females). LAM associated with tuberous sclerosis may affect male, female and children.

LAM patients are functionally characterized by obstructive or restrictive syndrome that leads patients to hypoxemia and chronic respiratory failure, so that quality of Life (QoL) of these patients is affected by dyspnea. Hypoxemia contributes to the development of secondary pulmonary hypertension (PH) during progression of LAM history, worsening the damage.

The modifications in the neural control of ventilation during sleep in combination with anatomical disposition drive to sleep-related changes in upper airway resistance and physiologic desaturations. In healthy subjects these features are almost completely compensated but, in LAM patients, may lead to airway obstruction and/or pathologic oxygen desaturation that could be managed. So far, none have investigated the changes in sleep architecture and related blood gas exchange in patients with LAM relating these changes with disease severity.

This pilot study investigate if the physiologic modifications of breathing during sleep lead to sleep disorders in LAM patients, measured through abnormalities in polysomnography (PSG) and ih sleep disorders in LAM are associated with respiratory functional abnormalities and disease severity.

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Study Type : Interventional  (Clinical Trial)
Estimated Enrollment : 15 participants
Allocation: N/A
Intervention Model: Single Group Assignment
Intervention Model Description: cohort study, pilot
Masking: None (Open Label)
Primary Purpose: Screening
Official Title: Sleep Patterns in Patients Affected by Lymphangioleiomiomatosis
Actual Study Start Date : June 30, 2020
Estimated Primary Completion Date : October 8, 2020
Estimated Study Completion Date : November 1, 2020

Resource links provided by the National Library of Medicine


Arm Intervention/treatment
Experimental: PSG in LAM patients
Patients affected by LAM underwent whole-night PSG
Diagnostic Test: LAM patients underwent PSG
Patients with confirmed diagnosis of LAM underwent a whole-night polysomnography. The presence of either acute or chronic respiratory failure or the use of long-term oxygen therapy are considered exclusion criteria




Primary Outcome Measures :
  1. prevalence of sleep disorders in LAM [ Time Frame: 1 year ]
    Aim of the project is to assess the presence of sleep disorders (oxygen destaruration during sleep and/or sleep apnea or hypopnea) in patients affected by LAM.

  2. correlation between sleep-related disorders and LAM severity measured by spirometry [ Time Frame: 1 year ]
    Another aim of the project is to stratify the presence of sleep-related disorders (oxygen desaturation during sleep, sleep apnea or hypopnea) according to the severity of LAM measured through functional impairment. By spirometry performed at the time of PSG the study population will be divided in: subjects with obstructive impairment; subjects with restrictive abnormality and subjects with no alterations.

  3. correlation between sleep-related disorders and LAM severity measured by annual decline of forced-expiratory volume at the first second (FEV1) [ Time Frame: 1 year ]
    Another aim of the project is to stratify the presence of sleep-related disorders (oxygen desaturation during sleep, sleep apnea or hypopnea) according to the severity of LAM measured by annual decline of forced-expiratory volume at the first second (FEV1), expressed in ml/year. Annual decline of FEV1 will be measured subtracting the FEV1 value (ml) at the time of PSG test to the FEV1 value (ml) at the time of diagnosis and then, dividing it by disease duration in years.

  4. correlation between sleep-related disorders and LAM severity measured by arterial blood gas exchange [ Time Frame: 1 year ]
    Another aim of the project is to stratify the presence of sleep-related disorders (oxygen desaturation during sleep, sleep apnea or hypopnea) according to the severity of LAM measured through arterial blood gas exchanges values: partial pressure of oxygen (PaO2 - mmHg), partial pressure of carbon dioxide (PaCO2 - mmHg), oxygen saturation (SatO2, %).

  5. correlation between sleep-related disorders and clincal parameters [ Time Frame: 1 year ]

    Moreover, another aim is to assess the distribution of sleep disorders (oxygen desaturation during sleep, sleep apnea or hypopnea) according to age, sex, body mass index (BMI), comorbidities in patients affected by LAM.

    BMI will be calculated using height (mt) and weight (Kg) at the time of PSG and expressed in Kg/m^2.




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Ages Eligible for Study:   18 Years and older   (Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Criteria

Inclusion Criteria:

  • confirmed diagnosis of LAM according to current guidelines
  • sign of informed consent to participate

Exclusion Criteria:

  • the presence of either acute or chronic respiratory failure
  • the use of long-term oxygen therapy

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT04577937


Contacts
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Contact: Stefano Nava, MD-FERS 00390512143253 stefano.nava@aosp.bo.it
Contact: Irene Prediletto, MD-PhD 00390512143253 irene.prediletto@aosp.bo.it

Locations
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Italy
Azienda Ospedaliero-Universitaria di Bologna Recruiting
Bologna, Italy, 40138
Contact: Stefano Nava, MD    00390512143253    stefano.nava@aosp.bo.it   
Contact    00390512143253      
Sponsors and Collaborators
dr. Stefano Nava
Investigators
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Principal Investigator: Stefano Nava, MD-FERS AOSP - Azienda Ospedaliero Universitaria Di Bologna
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Responsible Party: dr. Stefano Nava, Professor Stefano Nava, MD-FERS, Azienda Ospedaliera Universitaria di Bologna Policlinico S. Orsola Malpighi
ClinicalTrials.gov Identifier: NCT04577937    
Other Study ID Numbers: 270/2016/O/Oss/AOUB
First Posted: October 8, 2020    Key Record Dates
Last Update Posted: October 8, 2020
Last Verified: October 2020
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: No

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
Additional relevant MeSH terms:
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Lymphangioleiomyomatosis
Sleep Apnea Syndromes
Sleep Wake Disorders
Syndrome
Disease
Pathologic Processes
Apnea
Respiration Disorders
Respiratory Tract Diseases
Sleep Disorders, Intrinsic
Dyssomnias
Nervous System Diseases
Neurologic Manifestations
Mental Disorders
Lymphangiomyoma
Lymphatic Vessel Tumors
Neoplasms by Histologic Type
Neoplasms
Perivascular Epithelioid Cell Neoplasms
Neoplasms, Connective and Soft Tissue
Lymphoproliferative Disorders
Lymphatic Diseases
Immunoproliferative Disorders
Immune System Diseases