Hemolysis in Patients With Hereditary Spherocytosis (HS)

Hemolysis Clinical Trial

Official title:

Oxidative Stress Contributes to Hemolysis in Patients With Hereditary Spherocytosis (HS) and Can be Ameliorated by Fermented Papaya Preparation (FPP)

Clinical Trial Details — Status: Not yet recruiting

Administrative data

• NCT number: NCT01201174
• Other study ID #: SH-01CTIL
• Status: Not yet recruiting
• Phase: N/A
• First received: September 12, 2010
• Last updated: September 13, 2010
• Start date: September 2010
• Est. completion date: September 2011

Study information

• Verified date: September 2010
• Source: Wolfson Medical Center
• Contact: Ghoti Hossam, doctor
• Phone: 035028110
• Email: drghoti123[@]yahoo.com
• Is FDA regulated: No
• Health authority: United States: Institutional Review Board
• Study type: Interventional

Clinical Trial Summary

In the present study the investigators are going to explore the oxidative status of HS-RBC and its contribution to hemolysis

Description:

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 [3].

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 [4]. Secondary protein deficiencies resulting from oxidative stress are often observed and may be involved in the clinical manifestations of the disease [5].

Recruitment information / eligibility

• Status: Not yet recruiting
• Enrollment: 15
• Est. completion date: September 2011
• Est. primary completion date: September 2011
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: Both
• Age group: 5 Years and older

Eligibility

Inclusion Criteria:

• patients > 5 years

• with documented family history of HS

• 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.

Exclusion Criteria:

• non

Study Design

Allocation: Non-Randomized, Intervention Model: Parallel Assignment, Masking: Open Label, Primary Purpose: Diagnostic

Related Conditions & MeSH terms

• Hemolysis
• Hereditary

Intervention

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.

Locations

Country	Name	City	State
Israel	Wolfsson Medical Center	Holon,	Israel,

Sponsors (1)

Lead Sponsor	Collaborator
Wolfson Medical Center

Country where clinical trial is conducted

Israel, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	ROS, reduced glutathione (GSH) and lipid peroxides will be measured in RBC	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 [8] and with mercury orange for GSH [9]. After being washed twice, the cells will be resuspended in PBS and analyzed by flow cytometry .	year	Yes
Secondary	Hemolysis	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)	year	Yes