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NCT Number: NCT01048905

Glutamine Therapy for Hemolysis-Associated Pulmonary Hypertension

The primary hypothesis of this study is that glutamine supplementation will improve the erythrocyte glutamine/glutamate ratio, a biomarker of oxidative stress, hemolysis and pulmonary hypertension (PH) in sickle cell disease (SCD) and thalassemia (Thal) patients with PH. PH is defined as a tricuspid regurgitant jet velocity (TRV) on Doppler echocardiography > 2.5 m/s. We also predict that glutamine therapy will increase arginine bioavailability and subsequently alter sickle red cell endothelial interaction that can be identified using endo-PAT technology through nitric oxide (NO) generation, leading to changes in biological markers, and clinical outcome. Specifically our second hypothesis is that oral glutamine will decrease biomarkers of hemolysis and adhesion molecules, and improve the imbalanced arginine-to-ornithine ratio that occurs in hemolytic anemias, leading to improved arginine bioavailability and clinical endpoints of endothelial dysfunction and PH in patients with SCD and Thal.

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Key information

Conditions

Pulmonary Hypertension Anemia Anemia, Hemolytic Anemia, Hemolytic, Congenital Anemia, Sickle Cell Cardiovascular Diseases Congenital, Hereditary, and Neonatal Diseases and Abnormalities Genetic Diseases, Inborn Hematologic Diseases Hemic and Lymphatic Diseases Hemoglobinopathies Hypertension Hypertension, Pulmonary Lung Diseases Respiratory Tract Diseases Sickle Cell Disease Thalassemia Vascular Diseases

Age range

4 year and older

Sex eligibility

All sexes

Study type

Interventional

Phase

Phase 2

Primary location

Children's Hospital & Research Center Oakland

Oakland, California, 94608, United States

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • Established diagnosis of SCD (Hb SS, SC or SBeta- thalassemia) or Thal
  • PH documented by echocardiography, defined as at TRV greater than 2.5 m/s
  • Age greater than or equal to 4 years

Exclusion criteria

  • Inability to take or tolerate oral medication
  • Acute crisis or hospitalization within 1 month of enrollment
  • Hepatic dysfunction (SGPT greater than 3X normal)
  • Renal dysfunction (Creatinine greater than 2X normal)
  • Allergy to glutamine
  • Pregnancy or breastfeeding
  • Patients on sildenafil (Viagra), calcium channel blockers, or amino acid/protein supplements (other therapies acceptable if stable more than 3 months)

Treatment and study plan

L-glutamine

Drug

Oral L-glutamine 10 grams TID or (0.1g/kg TID) for children < 15 years of age.

Primary outcomes

  1. Erythrocyte Glutamine/Glutamate Ratio at 8 Weeks

    Time frame: 8 weeks

    Erythrocyte Glutamine/Glutamate Ratio: a novel biomarker of oxidative stress

Secondary outcomes

  1. Plasma Glutamine

    Time frame: 8 weeks

  2. Tricuspid Regurgitant Jet Velocity on Doppler Echocardiography

    Time frame: 8 week

    Tricuspid Regurgitant Jet Velocity was measured using Doppler Echocardiography in meters per second.

  3. 6 Minute Walk Distance

    Time frame: 8 weeks

    The six-minute walk test (6MWT) measures the distance in meters an individual is able to walk over a total of six minutes on a hard, flat surface.

  4. Liver Function Tests

    Time frame: 8 weeks

    Alanine aminotransferase (ALT) Aspartate aminotransferase (AST)

  5. Renal Function Tests

    Time frame: 8 weeks

    Creatinine Blood urea nitrogen (BUN)

Sponsors and collaborators

Lead sponsor

UCSF Benioff Children's Hospital Oakland

Other

Collaborators

  • Food and Drug Administration (FDA)

Registry information

Official study title

Phase 2 Trial for Glutamine Therapy for Hemolysis-Associated Pulmonary Hypertension

Important dates

Study start
2009
Primary completion
2014
Study completion
2014
First posted
Jan 14, 2010
Registry last updated
Jun 10, 2021

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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