Pulmonary Hypertension in Patients With Sickle Cell Disease in Nigeria

NCT00367523 | Completed

• 2 other identifiers: 99990618906-H-N189
• Study Type: observational
• Target: 308
• Locations: 1 country
• Sites: 1

Brief Summary

This study will explore how people with sickle cell disease (SCD) develop a complication called pulmonary hypertension (PHTN), a serious disease in which blood pressure in the lungs is higher than normal. PHTN is also caused by HIV, hepatitis C and schistosomiasis. Patients who have both SCD and one of these other infections may develop more severe PHTN. The number of Nigerians with SCD who also have PHTN is not known, nor is the cause of PHTN in this population. This study will examine genetic material in people with and without SCD to determine whether certain genes will allow doctors to predict which patients with SCD are likely to develop PHTN. Nigerian males and females 10 years of age and older with or without SCD may be eligible for this study. Patients must have SS, SC, or SB thalassemia or other genotype; control subjects must have hemoglobin A or AS genotype. Participants undergo a complete medical history and physical examination, blood tests, electrocardiogram (EKG), ultrasound tests of the heart and abdomen, and a 6-minute walk (distance test) to determine exercise capacity. Blood tests include screening for HIV, hepatitis B and C, schistosomiasis, hookworm and malaria. Patients who test positive for HIV, hepatitis B or C, schistosomiasis, hookworm or malaria are referred for treatment at Ahmadu Bello University Teaching Hospital in Zaria, Nigeria, and those who test negative for hepatitis B are referred for vaccination. Genetic tests focus on genes involved in SCD, PHTN, inflammation, blood vessel function and red blood cell function.

Conditions

Secondary Pulmonary Arterial Hypertension; Sickle Cell Disease; Pulmonary Hypertension

Keywords

Echocardiogram; Nigeria; Screening; Sickle Cell Disease; Genetics

Eligibility Criteria

• Age: 5 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

• All volunteer subjects 5 years of age and above and able to provide informed, written consent for participation in this study. Children will be included in this study provided that a legally authorized representative provides fully informed consent. Assent of children will also be required.
• Nigerian male and females over 5 years of age
• Diagnosis of sickle cell disease (electrophoretic documentation of SS, SC, or SBeta thalassemia or other genotype is required)
• Nigerian males and females over 5 years of age.
• Electrophoretic documentation of hemoglobin A or AS genotype.

You may not qualify if:

• Hb A-only phenotype and sickle cell trait
• Decisionally impaired subjects.
• Persons not able to understand the investigational nature of the study or give informed consent.
• Diagnosis of sickle cell disease (electrophoretic documentation of SS, SC, SBeta thallassemia or other sickling genotype)
• Decisionally impaired subjects.
• Persons not able to understand the investigational nature of the study or give informed consent.

Sponsors & Collaborators

• National Heart, Lung, and Blood Institute (NHLBI): lead

Study Sites (1)

Ahmadu Bello University Teaching Hospital

Kaduna, Nigeria

Location

Related Publications (3)

• Aliyu ZY, Tumblin AR, Kato GJ. Current therapy of sickle cell disease. Haematologica. 2006 Jan;91(1):7-10. No abstract available.

  PMID: 16434364 BACKGROUND

• Rother RP, Bell L, Hillmen P, Gladwin MT. The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease. JAMA. 2005 Apr 6;293(13):1653-62. doi: 10.1001/jama.293.13.1653.

  PMID: 15811985 BACKGROUND

• Morris CR, Kato GJ, Poljakovic M, Wang X, Blackwelder WC, Sachdev V, Hazen SL, Vichinsky EP, Morris SM Jr, Gladwin MT. Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease. JAMA. 2005 Jul 6;294(1):81-90. doi: 10.1001/jama.294.1.81.

  PMID: 15998894 BACKGROUND

MeSH Terms

Conditions

Anemia, Sickle Cell; Hypertension, Pulmonary

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; Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases

Study Officials

• Swee Lay Thein, M.D.

  National Heart, Lung, and Blood Institute (NHLBI)

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Sponsor Type: NIH
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: August 22, 2006
• First Posted: August 23, 2006
• Study Start: June 15, 2006
• Study Completion: September 28, 2017
• Last Updated: November 29, 2019

Record last verified: 2017-09-28

Locations

NG (1)