Posterior subcapsular opacity in two patients with thalassaemia major following deferiprone consumption

Mehdizadeh, Mehrzad; Nowroozzadeh, Mohammad Hosein

doi:10.1111/j.1444-0938.2009.00385.x

Cited by 11 publications

(12 citation statements)

References 9 publications

(11 reference statements)

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“…Deferiprone is an oral equivalent that has been introduced to the market more recently. A recent report of two cases of posterior subcapsular cataract after using deferiprone 9 raised the issue of a possible association between deferiprone consumption and development of posterior subcapsular cataract. The authors proposed that a deferiprone‐induced shift of iron (as it occurs in other tissues) might be responsible for posterior subcapsular iron deposition and opacity.…”

Section: Discussionmentioning

confidence: 99%

“…The aim of the present study was to investigate the biometric and refractive characteristics of patients with thalassaemia major. Because a previous report 9 raised the issue of possible association between deferiprone consumption and development of posterior subcapsular cataract, we have studied the presumed association as a secondary goal.…”

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confidence: 99%

“…1 Ophthalmologic changes might occur as a result of the disease itself or as sideeffects of iron chelators and include ocular surface disorders, cataract, angioid streaks, retinal venous tortuosity, retinal toxicity, retinal pigment epithelial degeneration and mottling, optic neuropathy and decreased visual acuity. [2][3][4][5][6][7][8][9][10][11][12] Functional changes such as abnormalities in the electro-retinogram, the electrooculogram, the visual-evoked potentials and contrast sensitivity function have been demonstrated in these patients. 13 Thalassaemia major patients have characteristic skeletal changes, including typical craniofacial changes and deformities of the long bones that result from expansion of the bone marrow.…”

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Ocular refractive and biometric characteristics in patients with thalassaemia major

Nowroozzadeh

Kalantari

Namvar

et al. 2011

Clinical and Experimental Optometry

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Background: Thalassaemia major is associated with characteristic findings in craniofacial bony structures and thereby may render abnormal bony orbit and subsequently distinctive ocular biometry. The purpose of the present study was to evaluate the ocular refractive and biometric characteristics in patients with thalassaemia major. Methods: This case-controlled study comprised 94 eyes of 47 patients with thalassaemia major and 88 eyes of 44 age-and sex-matched healthy control subjects. All participants had a complete ocular examination including slitlamp biomicroscopy, fundoscopy, ocular biometry, keratometry, refraction and analysis using Fourier transformation. Results: There were no significant differences in spherical equivalent (p = 0.66) and total astigmatism (p = 0.83) between groups. Mean uncorrected vision and visual acuities (logMar) were similar (p = 0.32 and p = 0.71, respectively). Compared with controls, thalassaemic patients had a shorter axial length (23.01 Ϯ 0.12 [SEM] mm versus 23.46 Ϯ 0.12 mm, p = 0.035), thicker crystalline lens (4.01 Ϯ 0.11 mm versus 3.87 Ϯ 0.1 mm, p = 0.046) and steeper average keratometry (44.02 Ϯ 0.24 D versus 43.44 Ϯ 0.24, p = 0.03). Fifty-seven per cent of thalassaemic patients had against-the-rule astigmatism (total), while 64.6 per cent of controls showed a with-the-rule pattern (p < 0.05). These patterns were also found for corneal astigmatism. The magnitudes of total, corneal and lenticular astigmatism were similar among groups. Regression analysis showed significant correlation between corneal ( J0 and J45) and total ( J0 and J45) astigmatism. The correlations were less prominent between lenticular and total J0 and J45. The mean intraocular pressure was 14.68 Ϯ 0.27 mmHg and 13.3 Ϯ 0.26 mmHg in the thalassaemia and control groups, respectively (p < 0.001). Six eyes (12.8 per cent) of four patients in the thalassaemia group had posterior subcapsular cataract, while the condition had not been observed in controls (p = 0.049). Conclusions: Shorter axial length, thicker lens, steeper corneal curvature and more against-the-rule pattern were characteristic findings in patients with thalassaemia major. Key words: astigmatism, biometry, refraction, thalassaemia major Haemoglobin is essential for normal oxygen delivery to tissues. Betathalassaemia is one of the most common haemoglobinopathies (with an incidence of up to 14 per cent in some populations) and has an autosomal recessive inheritance pattern. In this disorder, abnormalities in b-globin biosynthesis diminish production of haemoglobin tetramers, resulting in hypochromia and microcytosis. Management of thalassaemia major consists of regular long-life blood Functional changes such as abnormalities in the electro-retinogram, the electrooculogram, the visual-evoked potentials and contrast sensitivity function have been demonstrated in these patients. 13Thalassaemia major patients have characteristic skeletal changes, including typical craniofacial changes and deformities of the long bones that result from expansion of t...

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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confidence: 99%

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Ocular refractive and biometric characteristics in patients with thalassaemia major

Nowroozzadeh

Kalantari

Namvar

et al. 2011

Clinical and Experimental Optometry

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“…Although ocular side effects are more commonly reported in patients of TM on DFO therapy, they are known to occur in patients with DFP therapy as well. 10,11 Similar to the postulated mechanism for neurological toxicity, the ocular toxicity of DFP may be related to its small size and water-solubility that allows it to move freely across cell membranes including the blood-ocular barrier. 12 Thus, DFP may achieve high levels in the aqueous humor where it may decrease the activity of the enzyme glutathione peroxidase leading to lenticular opacities.…”

Section: Discussionmentioning

confidence: 97%

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

Parakh

Sharma

Prakash

et al. 2015

Journal of Pediatric Hematology/Oncology

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The oral iron chelator deferiprone is associated with various side effects including agranulocytosis, arthropathy, and deranged liver function tests. Rarely, neurological and visual side effects have been reported with high doses. The authors describe rare neurological manifestations of cerebellar ataxia, hypertonia, and bilateral cataract in an 11-year-old boy with thalassemia major on recommended therapeutic doses of deferiprone. The neurological abnormalities resolved with stoppage of deferiprone. Central nervous system toxicity and lenticular opacities may be attributed to the low molecular weight of deferiprone and its ability to cross the blood-brain and blood-ocular barrier, respectively. Clinicians should be alert to the possibility of neurological abnormalities that may occur during deferiprone therapy.

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“…It has a low molecular weight (139.15 g/mol) and is lipophilic: characteristics ideal for corneal penetration . Apart from a case study showing two incidences of posterior subcapsular deposits associated with systemic deferiprone for thalassemia, there have not been any other ocular complications . This was accepted as a manageable risk compared with penetrating keratoplasty.…”

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Novel topical therapy for corneal blood staining

Chan

White

Meades

et al. 2017

Clinical Exper Ophthalmology

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Posterior subcapsular opacity in two patients with thalassaemia major following deferiprone consumption

Cited by 11 publications

References 9 publications

Ocular refractive and biometric characteristics in patients with thalassaemia major

Ocular refractive and biometric characteristics in patients with thalassaemia major

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

Novel topical therapy for corneal blood staining

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