Study of Ventilatory Mechanics in Patients With Sickle Cell Anemia

NCT02565849 | Completed DMC

• 1 other identifier: EAF-8421
• Study Type: observational
• Target: 80
• Locations: 0 countries
• Sites: N/A

Brief Summary

The Sickle Cell Anemia (SCA) is a recessive genetic condition, monogenic, resulting in defects in the red cell structure. In the investigators' country, this disease affects about 3,000 children each year and is considered one of the most prevalent disorders among the group of existing hereditary diseases. The lungs are frequently affected in this disease by Acute Chest Syndrome (STA). Besides being the leading cause of death and the second leading cause of hospitalization in SCA, the STA is correlated with cognitive impairment frame these patients, resulting secondary Stroke vaso-occlusion of capillaries that supply the brain tissue. Traditional tests of pulmonary function allow assess whether the person has any commitment in the respiratory system, whether obstructive, restrictive or mixed. To run these tests it is necessary that the patient understands and performs a forced expiratory maneuver to obtain reliable results. In the particular case of SCA, performing these tests it is very difficult due to the presence of cognitive impairment of varying degrees. This results in underdiagnosis of early changes in the lung parenchyma during the therapeutic window, committing the proper monitoring and treatment offered to these patients.

Conditions

Sickle Cell Anemia

Outcome Measures

Primary Outcomes (18)

• spirometrics parameters of pulmonary function tests assessed by forced expiratory volume in one second (FEV1)

  1 day

• spirometrics parameters of pulmonary function tests assessed by forced vital capacity (FVC)

  1 day

• spirometrics parameters of pulmonary function tests assessed by ratio forced expiratory volume in one second and forced vital capacity (FEV1 / FVC)

  1 day

• spirometrics parameters of pulmonary function tests assessed by FEF max (Flow Forced Expiratory maximum)

  1 day

• plethysmographics parameters of pulmonary function tests assessed by Total Lung Capacity (TLC)

  1 day

• plethysmographics parameters of pulmonary function tests assessed by functional residual capacity (FRC)

  1 day

• plethysmographics parameters of pulmonary function tests assessed by residual volume (RV)

  1 day

• plethysmographics parameters of pulmonary function tests assessed by Ratio Residual Volume and Total Lung Capacity (RV / TLC)

  1 day

• plethysmographics parameters of pulmonary function tests assessed by Airway resistance (Rva)

  1 day

• diffusion parameters of pulmonary function tests assessed by Diffusing Capacity Carbon monoxide (DLCO)

  1 day

• diffusion parameters of pulmonary function tests assessed by VA (Volume Alveolar)

  1 day

• diffusion parameters of pulmonary function tests assessed by diffusion coefficient (DLCO / VA)

  1 day

• forced oscillation technique assessed by zero resistance at the intercept (R0) in cmH2O/l/s

  1 day

• forced oscillation technique assessed by resistance at 4 Hz (Rrs4Hz) in cmH2O/l/s

  1 day

• forced oscillation technique assessed by average resistance (Rm) in cmH2O/l/s

  1 day

• reactive parameters of the forced oscillation technique assessed by mean reactance (Xm) in cmH2O/l/s is a parameter related to the inhomogeneity of the respiratory system

  1 day

• reactive parameters of the forced oscillation technique of the respiratory system compliance was measured by dynamic compliance (Crs, dyn) in cmH2O

  1 day

• reactive parameters of the forced oscillation technique of total mechanical loading of the respiratory system was assessed using the absolute value of the respiratory impedance (Z4Hz) in cmH2O/l/s

  1 day

Study Arms (3)

Control group

Recruited volunteers aged above 18 years with no history of smoking or hospitalization in the last three months without cardiac dysfunction, orthopedic or lung.

Sickle Cell Anemia normal spirometry

Recruited patients aged above 18 years with no history of smoking or hospitalization in the last three months, ability to independent ambulation and spirometry tests and plethysmography with normal medical report.

Sickle Cell Anemia spirometry abnormal

Recruited patients aged above 18 years with no history of smoking or hospitalization in the last three months, ability to independent ambulation and spirometry tests and plethysmography with altered medical report.

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

Patients with sickle cell anemia, type of hemoglobin SS, above 18 years of age, accompanied by the Hematology Clinic at the University Pedro Ernesto hospital and no contraindications for study participation

You may qualify if:

• Patients older than 18 years
• Typing of Hemoglobin SS

You may not qualify if:

• History absence of respiratory infections and hospitalized in the last three months
• Asthma , smoking, rheumatological and oncological diseases, acute pain and inability to independent ambulation

Sponsors & Collaborators

• Rio de Janeiro State University: lead

MeSH Terms

Conditions

Anemia, Sickle Cell

Condition Hierarchy (Ancestors)

Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Anemia; Hematologic Diseases; Hemic and Lymphatic Diseases; Hemoglobinopathies; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Cirlene Marinho

  University Hospital Pedro Ernesto

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: CROSS SECTIONAL
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Study of Ventilatory Mechanics in Patients With Sickle Cell Anemia

Study Record Dates

• First Submitted: August 22, 2015
• First Posted: October 1, 2015
• Study Start: February 1, 2013
• Primary Completion: July 1, 2015
• Study Completion: August 1, 2015
• Last Updated: October 1, 2015

Record last verified: 2015-09