Hydroxy Urea, Omega 3, Nigella Sativa,Honey on Oxidative Stress and Iron Chelation in Pediatric Major Thalassemia
Condition: Iron Overload; Oxidative Stress; Thalassemia Major
MeSH terms: Thalassemia; beta-Thalassemia; Iron Overload
Study type: Interventional
Phase: Phase 2/Phase 3

Summary: 
      The aim of the present study is evaluating the strength of combination therapy of hydroxy
      urea, omega 3, nigella sativa and honey on antioxidant-oxidant status (OXIDATIVE STRESS) in
      response to reactive oxygen species production (LIPID PEROXIDATION) and their effect on iron
      intoxication (IRON CHELATION) in pediatric major thalassemia.
    

Detailed description: 
      Beta thalassemia is a blood disorder that reduces the production of hemoglobin. Hemoglobin is
      the iron-containing protein in red blood cells that carries oxygen to cells throughout the
      body. In people with beta thalassemia, low levels of hemoglobin lead to a lack of oxygen in
      many parts of the body. Affected individuals also have a shortage of red blood cells
      (anemia), which can cause pale skin, weakness, fatigue, and more serious complications.
      People with beta thalassemia are at an increased risk of developing abnormal blood clots.
      Beta thalassemia is classified into two types depending on the severity of symptoms:
      thalassemia major (also known as Cooley's anemia) and thalassemia intermedia. Of the two
      types, thalassemia major is more severe.

      Beta-thalassemia syndromes are a group of hereditary blood disorders. It is characterized by
      reduced beta globin chain synthesis, resulting in reduced Hb in red blood cells (RBC),
      decreased RBC production and anemia.

      Homozygotes for beta-thalassemia may develop either thalassemia major or thalassemia
      intermedia. Individuals with thalassemia major usually come to medical attention within the
      first 2 years and require regular blood transfusion to survive.

      Affected infants with thalassemia major fail to thrive and become progressively pale. Feeding
      problems, diarrhea, irritability, recurrent bouts of fever, and enlargement of the abdomen,
      caused by splenomegaly, may occur. If a regular transfusion program that maintains a minimum
      Hb concentration of 95-105 g/L is initiated, then growth and development are normal until the
      age of 10-11 years. After the age of 10-11 years, affected individuals are at risk of
      developing severe complications related to posttransfusional iron overload, depending on
      their compliance with chelation therapy.

      Complications of iron overload include growth retardation and failure of sexual maturation
      and also those complications observed in adults with hemachromatosis -associated hereditary
      hemochromatosis (HH): involvement of the heart (dilated myocardiopathy and pericarditis),
      liver (chronic hepatitis, fibrosis, and cirrhosis), and endocrine glands (resulting in
      diabetes mellitus and insufficiency of the parathyroid, thyroid, pituitary, and, less
      commonly, adrenal glands).

      The underlying basis of b-thalassemia pathology is the diminished b-globin synthesis leading
      to a-globin accumulation and premature apoptotic destruction of erythroblasts, causing
      oxidative stress-induced ineffective erythropoiesis.
    

Intervention name(s): Omega 3; Nigella Sativa Oil; Hydroxyurea; Honey; Deferoxamine; blood transfusion session
Intervention type(s): Drug; Drug; Drug; Drug; Drug; Procedure
Intervention description(s): Omega-3 supplementation (300-400mg EPA & 200-300mg DHA) per day for 8 consecutive months up to 10 months; Nigella sativa supplementation (1g black seed oil contain 1% thymoquinone) per day for 8 consecutive months up to 10 months; hydroxyurea medication (5 to 15mg/kg) per day for 8 consecutive months up to 10 months.; Natural honey(2.5 mg/kg dissolved in 250 ml water) per day for 8 consecutive months up to 10 months.; deferoxamine (SubQ infusion: 20 to 40 mg/kg/day over 8 to 12 hours, 6 to 7 nights per week, maximum daily dose: 40 mg/kg/day)for 8 consecutive months up to 10 months.; Regular blood transfusion session based on patient hematological profile starts from one session every 2 weeks.
Other intervention name(s): Natural honey formulation
Intervention MeSH terms: Hydroxyurea; Deferoxamine
Allocation: Randomized
Intervention model: Parallel Assignment
Intervention model description: Four experimental groups, one control group.
Each experimental group receives different experimental treatments plus traditional treatment in the hospital and the control group receives only traditional treatment.
Masking: Double (Participant, Care Provider)
Primary purpose: Treatment
Keywords: thymoquinone; omega3; hydroxyurea; pediatric Thalassemia Major; oxidative stress; iron chelation; iron overload; chelation activity of thymoquinone; antioxidant effect of omega3; anti-hemolysis effect of hydroxyurea