Hydroxy Urea, Omega 3, Nigella Sativa,Honey on Oxidative Stress and Iron Chelation in Pediatric Major Thalassemia
Słowa kluczowe
Abstrakcyjny
Opis
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.
Daktyle
| Ostatnia weryfikacja: | 06/30/2020 |
| Pierwsze przesłane: | 02/27/2020 |
| Szacowana liczba przesłanych rejestracji: | 02/27/2020 |
| Wysłany pierwszy: | 03/02/2020 |
| Ostatnia aktualizacja przesłana: | 07/09/2020 |
| Ostatnia opublikowana aktualizacja: | 07/13/2020 |
| Rzeczywista data rozpoczęcia badania: | 10/31/2019 |
| Szacowana data zakończenia podstawowej działalności: | 09/30/2020 |
| Szacowana data zakończenia badania: | 10/31/2020 |
Stan lub choroba
Interwencja / leczenie
Drug: Omega-3 experimental group
Drug: Nigella sativa experimental group
Drug: Hydroxyurea experimental group
Drug: Natural honey experimental group
Drug: Deferoxamine
Procedure: blood transfusion session
Faza
Grupy ramion
| Ramię | Interwencja / leczenie |
|---|---|
| Experimental: Omega-3 experimental group 50 patients from each participating hospital that will receive Omega-3 supplementation (300-400mg EPA & 200-300mg DHA) per day for 8 consecutive months up to 10 months.
in addition to experimental treatment this group will receive the traditional treatment of deferoxamine/deferasirox plus regular blood transfusion with dose de-escalation methods till efficacy of experimental treatment proved. | Drug: Omega-3 experimental group Omega-3 supplementation (300-400mg EPA & 200-300mg DHA) per day for 8 consecutive months up to 10 months |
| Experimental: Nigella sativa experimental group 50 patients from each participating hospital that will receive Nigella sativa supplementation (1g black seed oil contain 1% thymoquinone) per day for 8 consecutive months up to 10 months.
in addition to experimental treatment this group will receive the traditional treatment of deferoxamine/deferasirox plus regular blood transfusion with dose de-escalation methods till efficacy of experimental treatment proved. | Drug: Nigella sativa experimental group Nigella sativa supplementation (1g black seed oil contain 1% thymoquinone) per day for 8 consecutive months up to 10 months |
| Experimental: Hydroxyurea experimental group 50 patients from each participating hospital that will receive hydroxyurea medication (5 to 15mg/kg) per day for 8 consecutive months up to 10 months.
in addition to the experimental treatment, this group will receive the traditional treatment of deferoxamine/deferasirox plus regular blood transfusion with dose de-escalation methods until the efficacy of experimental treatment proved. | Drug: Hydroxyurea experimental group hydroxyurea medication (5 to 15mg/kg) per day for 8 consecutive months up to 10 months. |
| Experimental: Natural honey experimental group 50 patients from each participating hospital that will receive natural honey(2.5 mg/kg dissolved in 250 ml water) per day for 8 consecutive months up to 10 months.
in addition to the experimental treatment, this group will receive the traditional treatment of deferoxamine/deferasirox plus regular blood transfusion with dose de-escalation methods until the efficacy of experimental treatment proved. | Drug: Natural honey experimental group Natural honey(2.5 mg/kg dissolved in 250 ml water) per day for 8 consecutive months up to 10 months. |
| Active Comparator: Ordinary hospital treatment group 50 patients from each participating hospital that will receive the ordinary treatment of iron chelator agent of deferoxamine or deferasirox (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.
in addition to iron chelator agent, this group receive regular blood transfusion session. |
Kryteria kwalifikacji
| Wiek kwalifikujący się do nauki | 7 Years Do 7 Years |
| Płeć kwalifikująca się do nauki | All |
| Przyjmuje zdrowych wolontariuszy | tak |
| Kryteria | Inclusion Criteria: 1. Any case with full manifestation of β-THALASSEMIA major disease 2. #Aged from 7-15 years old 3. # accompanied with ineffective erythropoiesis 4. # with low hemoglobin level 5. # with iron overload Exclusion Criteria: 1. The presence of any other chronic illness. 2. Patient age>15 years old or < 7 years old. 3. The presence of concomitant myocardial infarction, stroke, acute chest syndrome. 4. The patient suffers from any other type of anemia. |
Wynik
Podstawowe miary wyników
1. F 2 -isoprostanes pg/mL [3 months]
2. Total cholesterol Mg/dl [10 months]
3. HDL cholesterol Mg/dl [10 months]
4. LDL cholesterol Mg/dl [10 months]
5. Triglycerides Mg/dl [10 months]
6. Serum total iron mcg/dL [10 months]
7. % transferrin saturation [10 months]
8. C-reactive protein mg/L [10 months]
9. Serum Ferritin ng/ml [10 months]
10. Total Iron Binding Capacity (TIBC) mcg/dL [10 months]
11. hemoglobin (Hbg) g/dL [10 months]
12. mean corpuscular hemoglobin (MCH) pg/ml [10 months]
13. leukocytes count μl [10 months]
14. % Chelation activity Fe+++ - thymoquinone complex [3 months]
15. % Chelation activity Fe++ - thymoquinone complex [3 months]
16. Lactic acid dehydrogenase U/L [10 months]
17. Reticulocyte count % [10 months]
18. Hb-F level g/dL [10 months]
19. Reticulocyte absolute count [10 months]
20. White blood cells count [10 months]
