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Deferasirox Might Be Effective for Microcytic Anemia and Neurological Symptoms Associated with Aceruloplasminemia: A Case Report and Review of the Literature.

Internal medicine (Tokyo, Japan)
January 1, 1970
Zenshi Miyake et al. (5 authors)
Case ReportsJournal ArticleReviewHuman Study
Study Details

Study Goal

The researchers aimed to evaluate the effects of iron chelation therapy (deferasirox) on anemia and cerebellar symptoms in a patient with aceruloplasminemia, a condition linked to low copper levels.

Results Summary

The study found that deferasirox improved the patient's anemia and cerebellar symptoms, including dysarthria and limb ataxia, suggesting potential benefits for similar cases within a year of treatment.

Population

A 64-year-old man with aceruloplasminemia, refractory microcytic anemia, diabetes mellitus, and low serum copper and ceruloplasmin levels.

Effective Dosage

Not specified

Duration

Less than one year

Interactions

None mentioned

Extracted Claims (5)
InterventionDirectionEndpointPopulationDosageImpactClaim #
Iron chelation therapy with deferasirox
decrease
anemia
64-year-old man with aceruloplasminemia
-
improved
#1
Iron chelation therapy with deferasirox
decrease
cerebellar symptoms
64-year-old man with aceruloplasminemia
-
improved
#2
Iron chelation therapy with deferasirox
decrease
dysarthria
64-year-old man with aceruloplasminemia
-
improved
#3
Iron chelation therapy with deferasirox
decrease
limb ataxia
64-year-old man with aceruloplasminemia
-
improved
#4
deferasirox
decrease
cerebellar symptoms with aceruloplasminemia
patients with aceruloplasminemia
in less than one year
might respond to
#5
Abstract

The patient was a 64-year-old man presented with difficulty in walking, articulation, and swallowing, as well as cognitive impairment. He had refractory microcytic anemia and diabetes mellitus. His serum levels of iron, copper, and ceruloplasmin were low. Magnetic resonance imaging suggested iron deposition in the basal ganglia, thalami, cerebellar dentate nuclei, and cerebral and cerebellar cortices. He was diagnosed with aceruloplasminemia after a ceruloplasmin gene analysis. Iron chelation therapy with deferasirox improved his anemia and cerebellar symptoms, which included dysarthria and limb ataxia. The present study and previous reports indicate that cerebellar symptoms with aceruloplasminemia might respond to deferasirox in less than one year.

Medical Subject Headings (MeSH)
AdultAnemia, Iron-DeficiencyCeruloplasminCopperDeferasiroxFemaleHumansIronIron Chelating AgentsIron Metabolism DisordersMaleMiddle AgedNeurodegenerative DiseasesTreatment OutcomeYoung Adult
Study Links
Quality Scores
SafetyNot Assessed
Efficacy70/10
Quality60/10
Citation Metrics
Total Citations8
Citations/Year1.6
Relative Citation Ratio0.62
NIH Percentile33.4%
Research Impact Scores
APT Score0.25
Weight Score1.38
Normalized Score0.60
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