Arginine Treatment of Acute Chest Syndrome (Pneumonia) in Sickle Cell Disease Patients
Status: | Completed |
---|---|
Conditions: | Pneumonia, Anemia |
Therapuetic Areas: | Hematology, Pulmonary / Respiratory Diseases |
Healthy: | No |
Age Range: | 5 - Any |
Updated: | 3/16/2015 |
Start Date: | August 2001 |
End Date: | July 2004 |
Arginine Therapy for Acute Chest Syndrome in Sickle Cell Disease
This is a study to determine if oral arginine will increase nitric oxide in sickle cell
disease (SCD) patients with acute chest syndrome (ACS). It will also assess the effects of
arginine in the body and how the body uses nitric oxide in ACS.
disease (SCD) patients with acute chest syndrome (ACS). It will also assess the effects of
arginine in the body and how the body uses nitric oxide in ACS.
Pneumonia in patients with SCD can be particularly severe and has come to be called acute
chest syndrome. ACS is a common cause of morbidity in SCD patients and is the most common
cause of death in SCD. Multiple factors are involved in the severity of acute pulmonary
injury in SCD. Nitric oxide has a multitude of related functions, many of which could impact
favorably on ACS in SCD. Nitric oxide is an important inflammatory mediator which is
produced by the conversion of L-arginine to citrulline by nitric oxide synthase. Nitric
oxide and its precursor, arginine, are known to be low in SCD patients with ACS, suggesting
that therapies, such as arginine, aimed at increasing nitric oxide production will improve
the clinical course of ACS. Patients will receive 1 of 3 doses of arginine hydrochloride
orally 3 times a day for 3 days while hospitalized. The efficacy of arginine will be
measured by the increase in nitric oxide production and the physiological effects will be
assessed.
chest syndrome. ACS is a common cause of morbidity in SCD patients and is the most common
cause of death in SCD. Multiple factors are involved in the severity of acute pulmonary
injury in SCD. Nitric oxide has a multitude of related functions, many of which could impact
favorably on ACS in SCD. Nitric oxide is an important inflammatory mediator which is
produced by the conversion of L-arginine to citrulline by nitric oxide synthase. Nitric
oxide and its precursor, arginine, are known to be low in SCD patients with ACS, suggesting
that therapies, such as arginine, aimed at increasing nitric oxide production will improve
the clinical course of ACS. Patients will receive 1 of 3 doses of arginine hydrochloride
orally 3 times a day for 3 days while hospitalized. The efficacy of arginine will be
measured by the increase in nitric oxide production and the physiological effects will be
assessed.
Inclusion criteria:
- Diagnosis of sickle cell disease (Hb SS, SC, or Sbeta thalassemia)
Acute chest syndrome with the following:
- New pulmonary infiltrate on chest radiography involving a full segment of the lung
and 1 of the following:
- Fever
- Cough, tachypnea, retractions, rales, or wheezing
- Chest pain
Exclusion criteria:
- Inability to take or tolerate oral medications
- Hepatic dysfunction (SGPT greater than 2 times normal)
- Renal dysfunction (creatinine greater than 2 times normal)
- Mental status or neurological changes
- Allergy to arginine
- History of priapism
- Pregnancy
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