Hemolysis in Patients With Hereditary Spherocytosis (HS)

The recruitment status of this study is unknown because the information has not been verified recently.
Verified September 2010 by Wolfson Medical Center.
Recruitment status was  Not yet recruiting
Sponsor:
Information provided by:
Wolfson Medical Center
ClinicalTrials.gov Identifier:
NCT01201174
First received: September 12, 2010
Last updated: September 13, 2010
Last verified: September 2010
  Purpose

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


Condition Intervention
Hereditary
Hemolysis
Other: fermented papaya preparation (FPP)

Study Type: Interventional
Study Design: Allocation: Non-Randomized
Intervention Model: Parallel Assignment
Masking: Open Label
Primary Purpose: Diagnostic
Official Title: Oxidative Stress Contributes to Hemolysis in Patients With Hereditary Spherocytosis (HS) and Can be Ameliorated by Fermented Papaya Preparation (FPP)

Resource links provided by NLM:


Further study details as provided by Wolfson Medical Center:

Primary Outcome Measures:
  • ROS, reduced glutathione (GSH) and lipid peroxides will be measured in RBC [ Time Frame: year ] [ Designated as safety issue: Yes ]
    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 .


Secondary Outcome Measures:
  • Hemolysis [ Time Frame: year ] [ Designated as safety issue: Yes ]
    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)


Estimated Enrollment: 15
Study Start Date: September 2010
Estimated Study Completion Date: September 2011
Estimated Primary Completion Date: September 2011 (Final data collection date for primary outcome measure)
Arms Assigned Interventions
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.

Detailed 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].

  Eligibility

Ages Eligible for Study:   5 Years and older
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   Yes
Criteria

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
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT01201174

Contacts
Contact: Ghoti Hossam, doctor 035028110 drghoti123@yahoo.com

Locations
Israel
Wolfsson Medical Center Not yet recruiting
Holon,, Israel,, Israel
Contact: GHOTI HOSSAM, doctor    970-35028110    drghoti123@yahoo.com   
Sponsors and Collaborators
Wolfson Medical Center
Investigators
Principal Investigator: GHOTI HOSSAM, doctor HEMATOLOGY DEPARTMENT ON WOLFSSON MEDICAL CENTER
  More Information

No publications provided

Responsible Party: Dr. Ghoti Hossam, Organization: hematology department on Wolfsson Medical Center
ClinicalTrials.gov Identifier: NCT01201174     History of Changes
Other Study ID Numbers: SH-01CTIL
Study First Received: September 12, 2010
Last Updated: September 13, 2010
Health Authority: United States: Institutional Review Board

Keywords provided by Wolfson Medical Center:
Hemolysis in Patients with Hereditary Spherocytosis
with documented family history of HS.
spherocyte morphology,
splenomegaly and non-immune mediated hemolysis.

Additional relevant MeSH terms:
Hemolysis
Spherocytosis, Hereditary
Pathologic Processes
Anemia, Hemolytic, Congenital
Anemia, Hemolytic
Anemia
Hematologic Diseases
Genetic Diseases, Inborn

ClinicalTrials.gov processed this record on September 18, 2014