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Neurophysiologic study of β-thalassemia patients

By A Li, ACW Lee and V Wong


Neurophysiologic investigations were performed in 34 Chinese patients with β-thalassemia major maintained on long-term desferrioxamine treatment to look for subclinical toxicity in the auditory, visual, peripheral, or central neural pathways. In the auditory pathway study, four patients (12%) had mild sensorineural hearing impairment. Two patients (6%) had increased P 100 latencies in the visual evoked potential study, and nine patients (26%) had abnormal electroretinogram results. All had normal electrooculograms. Ophthalmoscopic examination was abnormal in three patients (9%), and three (9%) had a visual field defect. In the peripheral or central nervous pathways, seven patients (21%) had sensory neuropathy, of which three cases were probably related to diabetes mellitus. All had normal motor conduction velocities. Four patients (12%) had increased cortical latencies of median or posterior tibial somatosensory evoked potential. Abnormalities in multiple neural pathways were seen in four patients (12%). There was a significant association between subclinical toxicity to the peripheral or central nervous systems and serum ferritin level (P ≤ .03) and the presence of diabetes mellitus (P < .002). There was no significant relationship between the age, dosage, or duration of desferrioxamine used and the increased risk of neurotoxicity to the auditory, visual, peripheral, or central nervous systems. There was also no association between the risk of neurotoxicity and the serum zinc, copper, or fructosamine levels.link_to_subscribed_fulltex

Topics: Electroretinography, Evoked Potentials/drug effects, Evoked Potentials, Auditory, Brain Stem, Female, Ferritins/analysis/blood/metabolism, Fructosamine, Hexosamines/analysis/blood/metabolism, Humans, Male, Neural Pathways/drug effects, Zinc/analysis/blood/metabolism, beta-Thalassemia/blood/*drug therapy, Adolescent, Adult, Brain/drug effects, Copper/analysis/blood/metabolism, Deferoxamine/pharmacology/*therapeutic use, Diabetes Mellitus/blood/metabolism, Electrooculography
Publisher: 'United States Sports Academy'
Year: 1993
DOI identifier: 10.1177/088307389300800407
OAI identifier:
Provided by: HKU Scholars Hub
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