PEDIATRICS Vol. 48 No. 5 November 1971, pp. 740-744
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INTELLIGENCE AND BEHAVIORAL PATTERNS IN PATIENTS WITH COOLEY'S ANEMIA (HOMOZYGOUS BETA-THALASSEMIA); A STUDY BASED ON 138 CONSECUTIVE CASES

John Logothetis M.D.1, Mary Haritos-Fatouros Ph.D.1, Mathios Constantoulakis M.D.1, Joanna Economidou M.D.1, Olga Augoustaki M.D.1, and Ruth B. Loewenson Ph.D.1

1 Department of Neurology, University of Minnesota Medical School, Minneapolis; the Research Hematology Section of Hellenic Red Cross, Athens, Greece; and the Center of Social Psychiatry, Thessaloniki, Greece

A study of 138 consecutive cases of Cooley's anemia (homozygons beta-thalassemia) was conducted in Greece for evaluation of their mental status. Intelligence Quotients were computed with the Wechsler Intelligence Scales (WISC and WAIS) and Porteus-maze test in 125 patients above the age of 5. The means and distributions of IQ scores revealed no significant deviation from the expected norms. Correlation with various clinical and laboratory parameters of the illness suggested relatively lower IQ scores in patients presenting more severe stigmata of the illness and in patients subjected to less vigorous transfusion programs. Abnormalities in character and behavior were noted in 96 of 138 cases. About half of the patients presented abnormal emotional responses most commonly in the direction of a depressed mood and floating anxiety.

Submitted on January 15, 1971
Accepted on May 3, 1971




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