Neurological Complications and Cataract in a Child With Thalassemia Major Treated With Deferiprone

Parakh, Nupur; Sharma, Rajni; Prakash, Om; Mahto, Deonath; Dhingra, Bhavna; Sharma, Suvasini; Chandra, Jagdish

doi:10.1097/mph.0000000000000391

Cited by 8 publications

(7 citation statements)

References 14 publications

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“…Chemical chelators (deferiprone, deferoxamine, or deferasirox) have already been evaluated in neurogenerative diseases after systemic administration ( 22 ). However, long-term iron chelation therapy, routinely used for instance in beta-thalassemia and chronic iron overload diseases, negatively affects patient’s quality of life and induced ocular toxicity, including cataracts, maculopathy, and optic neuritis ( 23 , 24 ). Thus, the use of these chemical chelators in patients with RD without systemic iron overload could be deleterious for the eye.…”

Section: Discussionmentioning

confidence: 99%

Iron is neurotoxic in retinal detachment and transferrin confers neuroprotection

et al. 2019

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Section: Discussionmentioning

confidence: 99%

Iron is neurotoxic in retinal detachment and transferrin confers neuroprotection

et al. 2019

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“…Other identified events were neurological symptoms including cerebellar signs with dizziness, axial hypotonia and impaired motor coordination seen in two case reports and one clinical trial .…”

Section: Discussionmentioning

confidence: 99%

Safety profiles of iron chelators in young patients with haemoglobinopathies

Botzenhardt

Chan

et al. 2017

European J of Haematology

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Background: This review describes the safety of deferoxamine (DFO), deferiprone (DFP), deferasirox (DFX) and combined therapy in young patients less than 25 yr of age with haemoglobinopathies. Methods: Searches in electronic literature databases were performed. Studies reporting adverse events associated with iron chelation therapy were included. Study and reporting quality was assessed using AHRQ Risk of Bias Assessment Tool and McMaster Quality Assessment Scale of Harms. Prospective clinical studies were pooled in a random-effects meta-analysis of proportions. Results: Safety data of 2040 patients from 34 studies were included. Ninety-two case reports of 246 patients were identified. DFX (937 patients) and DFP (667 patients) possess the largest published safety evidence. Fewer studies on combination regimens are available. Increased transaminases were seen in all regimens (3.9-31.3%) and gastrointestinal disorders with DFP and DFX (3.7-18.4% and 5.8-18.8%, respectively). Therapy discontinuations due to adverse events were low (0-4.1%). Reporting quality was selective and poor in most of the studies. Conclusion: Iron chelation therapy is generally safe in young patients, and published data correspond to summary of product characteristics. Each iron chelation regimen has its specific safety risks. DFO seems not to be associated with serious adverse effects in recommended doses. In DFP and DFX, rare, but serious, adverse reactions can occur. Data on combined therapy are scarce, but it seems equally safe compared to monotherapy.

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“…[13][14][15] While iron chelating drugs are essential for overall survival and complication-free survival, they carry considerable side effects. 13,16,17 Among the less studied ones are ophthalmic complications: various case reports [18][19][20][21][22][23][24][25][26][27][28] described a relationship between initiation of chelation therapy and onset of ocular symptoms. However, no systematic reviews published to date indicated whether these events are frequently related to specific risk factors or preventable.…”

Section: Introductionmentioning

confidence: 99%

“…Furthermore, it has not yet been established whether ophthalmological complications may be related to the dosage of chelating agents or their intake time. The majority of articles refer to deferoxamine, 16 , 20–41 while data about deferiprone 18 , 19 , 32 and deferasirox 42 , 43 are poor and do not allow to draw any conclusions.…”

Section: Introductionmentioning

confidence: 99%

Long-Term Effects of Iron Chelating Agents on Ocular Function in Patients with Thalassemia Major

Nuzzi¹,

Geronazzo²,

Tridico³

et al. 2021

OPTH

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Background:The aim of this study is to evaluate eye structures and function in patients receiving iron chelating therapy and to assess whether a correlation exists between the onset of ocular alterations and the intake of iron chelating drugs. Methods: A prospective cohort study was performed. Eighty-eight patients, composed of children and adults with thalassemia major (TM) who are taking or had taken iron chelating drugs (deferoxamine, deferiprone or deferasirox), have been initially enrolled in the study. The final sample featured 80 patients, including 18 children and 62 adults. These subjects received an eye examination to evaluate intraocular pressure (IOP), best corrected visual acuity (BCVA), the presence of refractive defects, cornea, anterior chamber, lens, fundus oculi, visual field and mean retinal nerve fiber layer (RNFL) thickness. Logistic regression model analysis was performed in order to assess any correlation. In addition, a literature search regarding the relation between iron chelating drugs and ocular adverse events was carried out to compare the results obtained with the evidence in the literature. Results: Logistic regression did not report a significant correlation between the intake of iron chelating drugs and the onset of anterior ocular segment alterations, lens opacities, retinal diseases, optical neuropathies, astigmatism, visual field and RNFL thickness defects. Logistic regression returned a statistically significant correlation between myopia and iron chelation therapy (p-value 0.04; OR 1.05) and also between presbyopia and total duration of therapy with deferoxamine (p-value 0.03; OR 1.21). Although intraocular pressure levels remained within the normal range, a significant correlation with the length of deferoxamine therapy has been found (p-value 0.002; association coefficient −0.12). A negative correlation between deferiprone and presbyopia has also been observed. Conclusion: Iron chelation therapy is not associated with severe visual function alterations. Limitation of deferoxamine treatment can help prevent ocular complications. Deferiprone and/or deferasirox may be preferable, especially in patients over age 40 years.

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Neurological Complications and Cataract in a Child With Thalassemia Major Treated With Deferiprone

Cited by 8 publications

References 14 publications

Iron is neurotoxic in retinal detachment and transferrin confers neuroprotection

Iron is neurotoxic in retinal detachment and transferrin confers neuroprotection

Safety profiles of iron chelators in young patients with haemoglobinopathies

Long-Term Effects of Iron Chelating Agents on Ocular Function in Patients with Thalassemia Major

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