Secondary Pulmonary Hypertension in Adults With Sickle Cell Anemia

This study is currently recruiting participants.
Verified August 2013 by National Institutes of Health Clinical Center (CC)
Sponsor:
Collaborator:
Information provided by:
National Institutes of Health Clinical Center (CC)
ClinicalTrials.gov Identifier:
NCT00011648
First received: February 24, 2001
Last updated: March 14, 2014
Last verified: August 2013

February 24, 2001
March 14, 2014
February 2001
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Complete list of historical versions of study NCT00011648 on ClinicalTrials.gov Archive Site
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Secondary Pulmonary Hypertension in Adults With Sickle Cell Anemia
Determining the Prevalence and Prognosis of Secondary Pulmonary Hypertension in Adult Patients With Sickle Cell Anemia

The purpose of this study is to determine how often people with sickle cell anemia develop pulmonary hypertension a serious disease in which blood pressure in the artery to the lungs is elevated.

Men and women 18 years of age and older with sickle cell anemia may be eligible for this study. Participants will undergo an evaluation at Howard University s Comprehensive Sickle Cell Center in Washington, D.C. or at the National Institutes of Health in Bethesda, Maryland. It will include the following:

  • medical history
  • physical examination
  • blood collection (no more than 50 ml., or about 1/3 cup) to confirm the diagnosis of sickle cell anemia, sickle cell trait or beta-thalassemia (Some blood will be stored for future research testing on sickle cell anemia.)
  • echocardiogram (ultrasound test of the heart) to check the pumping action of the heart and the rate at which blood travels through the tricuspid valve.

Following this evaluation, a study nurse will contact participants twice a month for 2 months and then once every 3 months for the next 3 years for a telephone interview. The interview will include questions about general health and recent health-related events, such as hospitalizations or emergency room visits.

Sickle cell anemia is an autosomal recessive disorder and the most common genetic disease affecting African-Americans. Approximately 0.15% of African-Americans are homozygous for sickle cell disease, and 8% have sickle cell trait. Acute pain crisis, acute chest syndrome (ACS), and secondary pulmonary hypertension are common complications of sickle cell anemia. Mortality rates of sickle cell patients with pulmonary hypertension are significantly increased as compared to patients without pulmonary hypertension. Recent studies report up to 40% mortality at 22 months after detection of elevated pulmonary artery pressures in sickle cell patients. Furthermore, pulmonary hypertension is thought to occur in up to 30% of clinic patients with sickle cell anemia.

This study is designed to determine the prevalence and prognosis of secondary pulmonary hypertension in adult patients with sickle cell anemia, and to determine whether genetic polymorphisms in candidate genes contribute to its development or response to treatment.

Observational
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  • Pulmonary Hypertension
  • Sickle Cell Anemia
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*   Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
 
Recruiting
1500
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  • INCLUSION CRITERIA:

All volunteer subjects must be at least 18 years of age and must be able to provide informed, written consent for participation in this study. Decisional impaired subjects will not be included in this study because it does not offer the prospect of direct benefit.

Sickle Cell Patients:

Male and females over 18 years of age.

Diagnosis of sickle cell disease (electrophoretic documentation of SS, SC, or S-beta thallassemia genotype is required).

EXCLUSION CRITERIA:

Sickle Cell Patients:

Hb A-only phenotype and sickle cell trait.

Decisionally impaired subjects.

INCLUSION CRITERIA:

Control Subjects:

Male and females African American subjects over 18 years of age.

Exclusion of sickle cell disease (electrophoretic documentation of hemoglobin A is required).

EXCLUSION CRITERIA:

Control Subjects:

Diagnosis of sickle cell disease (electrophoretic documentation of SS, or SC, or SB thallassemia genotype is required.

Decisionally impaired subjects.

Both
18 Years and older
Yes
Contact: Darlene T Allen (301) 435-1495 darlenea@mail.nih.gov
Contact: James G Taylor, M.D. (301) 435-7895 jamesta@mail.nih.gov
United States
 
NCT00011648
010088, 01-H-0088
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National Heart, Lung, and Blood Institute (NHLBI)
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Principal Investigator: James G Taylor, M.D. National Heart, Lung, and Blood Institute (NHLBI)
National Institutes of Health Clinical Center (CC)
August 2013

ICMJE     Data element required by the International Committee of Medical Journal Editors and the World Health Organization ICTRP