A Case Control Study to Evaluate the Cognitive and Brain Function of β-thalassemia Patients.

This study is enrolling participants by invitation only.
Information provided by (Responsible Party):
HaEmek Medical Center, Israel
ClinicalTrials.gov Identifier:
First received: August 7, 2013
Last updated: September 16, 2015
Last verified: September 2015

β-thalassemia syndromes are a group of hereditary disorders characterized by a genetic deficiency in the synthesis of beta-globin chains.

In recent studies done in β-thalassemia major patients abnormal iron deposition was evident using MRI in brain structures, cortex, putamen, and caudate nucleus . In most of the cases the neurological involvement is subclinical. Cognitive functioning was evaluated in beta thalassemia major, compared with healthy controls, using a neuropsychological battery including tests of abstract reasoning, attention, executive functions, language, constructional/visuospatial skills, and memory. Patients with beta thalassemia major, in particular those showing signs of hemosiderosis, had significantly impaired function in all neuropsychological tests. There was no relationship between cognitive performances and signs of deferoxamine toxicity, deferoxamine dosage, and levels of hemoglobin and ferritin. Event-related potentials (ERPs) are one of the most informative and dynamic methods of monitoring the information stream in the living brain. ERPs are linked in time with a physical or mental event, and are typically extracted from the scalp-recorded electroencephalogram (EEG) by means of signal averaging.

ERPs have been used in the assessment of cognitive function in several disorders, including anemia and iron deficiency anemia. However, literature regarding cognitive function and ERP activity in thalassemia patients is extremely limited, especially in adults.

The purpose of this study is to evaluate the cognitive and brain function in a group of 60 thalassemia patients and compare the results to healthy controls.

Brain Function (Measured by ERP Level)

Study Type: Observational
Study Design: Observational Model: Case Control
Time Perspective: Prospective
Official Title: A Case Control Study to Evaluate the Cognitive and Brain Function of β-thalassemia Patients in EMEK MEDICAL CENTER Compare to Healthy Controls.

Resource links provided by NLM:

Further study details as provided by HaEmek Medical Center, Israel:

Primary Outcome Measures:
  • Brain function assessment evaluated by Event-related potentials (ERPs of the information stream in the brain of Thalassemia patients. [ Time Frame: one year ] [ Designated as safety issue: No ]

Secondary Outcome Measures:
  • correlation between brain function evaluated by ERP measurements and Hemosiderosis parameters like ferritin levels, Iron transferrin and Saturation. [ Time Frame: one year ] [ Designated as safety issue: No ]

Other Outcome Measures:
  • correlation between pre and post transfusion status related to the Hemoglobin concentration and brain function measured by ERP. [ Time Frame: one year ] [ Designated as safety issue: No ]

Estimated Enrollment: 120
Study Start Date: September 2013
Estimated Study Completion Date: December 2016
Estimated Primary Completion Date: December 2016 (Final data collection date for primary outcome measure)

Ages Eligible for Study:   6 Years to 70 Years
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   Yes
Sampling Method:   Non-Probability Sample
Study Population

60 Thalasssemia major and intermedia patients. 60 healthy controls.


Inclusion Criteria:

  • Thalassemia major and intermedia patients older than 6 years treated at Emek Medical Center Afula Israel.
  • healthy controls matched for age and ethnicity.

Exclusion Criteria:

  • patients suffering from acute diseases.
  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: NCT01935661

Haemek Medical Center
Afula, Israel
Sponsors and Collaborators
HaEmek Medical Center, Israel
Principal Investigator: Carina Levin, MD Pediatric Hematology Unit - Emek Medical Center
  More Information

No publications provided

Responsible Party: HaEmek Medical Center, Israel
ClinicalTrials.gov Identifier: NCT01935661     History of Changes
Other Study ID Numbers: 111-12-EMC
Study First Received: August 7, 2013
Last Updated: September 16, 2015
Health Authority: Israel: Ministry of Health

Keywords provided by HaEmek Medical Center, Israel:
Blood transfusion

Additional relevant MeSH terms:
Anemia, Hemolytic
Anemia, Hemolytic, Congenital
Genetic Diseases, Inborn
Hematologic Diseases

ClinicalTrials.gov processed this record on October 13, 2015