Hemolysis in Patients With Hereditary Spherocytosis (HS)
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|ClinicalTrials.gov Identifier: NCT01201174|
Recruitment Status : Unknown
Verified September 2010 by Wolfson Medical Center.
Recruitment status was: Not yet recruiting
First Posted : September 14, 2010
Last Update Posted : September 14, 2010
|Condition or disease||Intervention/treatment||Phase|
|Hereditary Hemolysis||Other: fermented papaya preparation (FPP)||Not Applicable|
The oxidative status of cells, which is determined by the balance between pro-oxidants, such as the reactive oxygen species (ROS), and antioxidants, is a major regulator of cellular functions. Impaired balance between pro- and antioxidants causes oxidative stress which may result in oxidation of proteins, lipids and DNA with the final outcome of premature cell aging and apoptosis [1,2]. Oxidatively stressed red blood cell (RBC) have been observed in various congenital and acquired hemolytic anemias, including thalassemia, sickle cell anemia, congenital dyserythropoietic anemia, G6PD deficiency and paroxysmal nocturnal hemoglobinuria (PNH) as well as in myelodysplastic syndrome (MDS). Although the primary etiology is different in these anemias, oxidative stress mediates several of their pathologies, mainly hemolysis .
Hereditary Spherocytosis (HS) is a genetic disorder of the RBC skeleton with primary deficiency in spectrin, ankyrin-1, band 3 or protein 4.2 associated with chronic hemolytic anemia . Secondary protein deficiencies resulting from oxidative stress are often observed and may be involved in the clinical manifestations of the disease .
|Study Type :||Interventional (Clinical Trial)|
|Estimated Enrollment :||15 participants|
|Intervention Model:||Parallel Assignment|
|Masking:||None (Open Label)|
|Official Title:||Oxidative Stress Contributes to Hemolysis in Patients With Hereditary Spherocytosis (HS) and Can be Ameliorated by Fermented Papaya Preparation (FPP)|
|Study Start Date :||September 2010|
|Estimated Primary Completion Date :||September 2011|
|Estimated Study Completion Date :||September 2011|
No Intervention: Patients with Hereditary Spherocytosis
All patients should have clinical and laboratory findings, consistent with mild to severe HS, diagnosed on the basis of spherocyte morphology, elevated MCHC (33-38 g/dl), with a mean value of (35.47 g/dl), increased osmotic fragility , splenomegaly and non-immune mediated hemolysis.
Other: fermented papaya preparation (FPP)
Hemolysis will be assayed by suspending 3 ml of packed RBC in PBS or in the autologous plasma and overnight incubation in the presence of various concentrations of antioxidants such as fermented papaya preparation (FPP) at 37oC in humidified atmosphere of 5% CO2 in air (10). Following 5 min centrifugation at 800 rpm, the supernatants will collected for Hb determination by measuring the absorbance at 540 nm.
- ROS, reduced glutathione (GSH) and lipid peroxides will be measured in RBC [ Time Frame: year ]ROS, reduced glutathione (GSH) and lipid peroxides will be measured in RBC following incubation with with 100 μM 2'-7'-dichlorofluorescin diacetate, 40 μM [1-(4-chloromercuryphenyl-azo-2-naphthol)] and fluor-DHPE, respectively for ROS  and with mercury orange for GSH . After being washed twice, the cells will be resuspended in PBS and analyzed by flow cytometry .
- Hemolysis [ Time Frame: year ]Hemolysis will be assayed by suspending 3 ml of packed RBC in PBS or in the autologous plasma and overnight incubation in the presence of various concentrations of antioxidants such as fermented papaya preparation (FPP)
To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT01201174
|Contact: Ghoti Hossam, email@example.com|
|Wolfsson Medical Center|
|Holon,, Israel,, Israel|
|Contact: GHOTI HOSSAM, doctor 970-35028110 firstname.lastname@example.org|
|Principal Investigator:||GHOTI HOSSAM, doctor||HEMATOLOGY DEPARTMENT ON WOLFSSON MEDICAL CENTER|