Clinical trial NCT01935661: A Case Control Study to Evaluate the Cognitive and Brain Function of β-thalassemia Patients.
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.
Study type: OBSERVATIONAL
Overall status: UNKNOWN
Conditions: Thalassemia
Brain Function (Measured by ERP Level)
Keywords: Blood transfusion
Hemosiderosis
ERP
Lead sponsor: HaEmek Medical Center, Israel
Sponsor class: OTHER
First posted: 2013-09-05
Enrollment: 120
Sex: ALL
Minimum age (years): 6
Maximum age (years): 70
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: yes
Observational model: CASE_CONTROL
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: 60 Thalasssemia major and intermedia patients. 60 healthy controls.

Brief summary: 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.


Eligibility criteria: 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.

Primary outcomes:
measure: Brain function assessment evaluated by Event-related potentials (ERPs of the information stream in the brain of Thalassemia patients.
timeFrame: one year
Secondary outcomes:
measure: correlation between brain function evaluated by ERP measurements and Hemosiderosis parameters like ferritin levels, Iron transferrin and Saturation.
timeFrame: one year
Other outcomes:
measure: correlation between pre and post transfusion status related to the Hemoglobin concentration and brain function measured by ERP.
timeFrame: one year
Locations:
city: Afula
country: Israel
facility: Haemek Medical Center
Study officials:
affiliation: Pediatric Hematology Unit - Emek Medical Center
name: Carina Levin, MD
role: PRINCIPAL_INVESTIGATOR
References: