Ocular Findings in Hemoglobin SC Disease in Jamaica

Condon, P. I.; Serjeant, G. R.

doi:10.1016/0002-9394(72)91213-5

Cited by 98 publications

(53 citation statements)

References 14 publications

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“…(5)(6)(7)(8)(9)(10) With regard to the fundoscopic lesions, vascular tortuosity and "black sunbursts" were the most frequent changes identified, corroborating previous Brazilian studies. (7)(8)(9)(10) The overall frequency of vascular tortuosity was similar in both patient groups (HBSS and HBSC ), as previously described.…”

Section: Discussionsupporting

confidence: 86%

Ocular lesions in sickle cell disease patients from Bahia, Brazil

Cury

Boa‐Sorte

Lyra³

et al. 2010

Rev. bras.oftalmol.

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Objective: The present study aims to describe ocular alterations in sickle cell disease patients in Bahia, a Northeast state, with the highest prevalence of the disease in Brazil. Methods: We carried out a cross-sectional study in a group of 146 (292 eyes) sickle cell disease patients (90 HBSS and 56 HBSC). Ophthalmologic examination including indirect binocular ophthalmoscopy was performed. Examination was completed by fluorescein angiography to detect retinal lesions. Results: The most frequent ocular lesions identified were "vascular tortuosity" and "black sunburst". Proliferative retinopathy was found in 22 (12.2%) eyes of HBSS patients and 25 (22.3%) eyes of HBSC patients (OR=2.06; CI95%: 1.5-4.06, p=0.022); Its frequency was higher among HBSS patients aged 20-39 years, while in HBSC patients, it peaked after 40 years (35.7% and 42.8%) and dropped sharply afterwards. Conclusion: Proliferative retinopathy was described as early as 10 years of age in both patients groups. Proliferative sickle retinopathy can result in blindness and the knowledge of the most prevalent ocular alterations and age risk will be important to establish a protocol of ophthalmologic follow-up, in order to prevent a severe visual loss and increase patient's life quality.

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Section: Discussionsupporting

confidence: 86%

Ocular lesions in sickle cell disease patients from Bahia, Brazil

Cury

Boa‐Sorte

Lyra³

et al. 2010

Rev. bras.oftalmol.

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“…Este, muito frequente, mostrou-se presente em 95% dos olhos examinados e a tortuosidade vascular retiniana (Figura 2) em 36,6%. Os "black sunburst" (Figura 3), que correspondem à hiperplasia e hipertrofia do epitélio pigmentado da retina, secundários às hemorragias, foram encontrados em 24,4% dos exames, sendo a terceira alteração mais frequente, ratificando estudos anteriores (8)(9)(10)(11)(12)(13)(14)(15) . A presença de estrias angióides em 5,5% dos casos merece ênfase em função de sua evolução natural que somada à retinopatia falciforme, aumenta o risco de seus portadores ao longo do tempo de desenvolver perda visual grave (16) .…”

Section: Resultsunclassified

“…A variável genótipo mostrou maior número de homozigotos SS, seguidos dos SC e S-Tal respectivamente, conforme todos os estudos relacionados (8)(9)(10)(11)(12)(13)(14)(15) . Sobre os estudos que concluíram que o eletrorretinograma seria útil no monitoramento da evolução da retinopatia falciforme (19,20) , o presente estudo obteve dados opostos.…”

Section: Resultsunclassified

Alterações oculares e eletrorretinográficas na doença falciforme

David¹,

Júnior²,

Rodrigues³

2011

Arq. Bras. Oftalmol.

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INTRODUÇÃODentre as anemias hemolíticas crônicas, a doença falciforme é a mais frequente e incapacitante em nosso meio. Aproximadamente 0,3% a 3% dos nascidos vivos no Estado do Rio de Janeiro, apresentam anemia falciforme e traço falcêmico, respectivamente (1) ; cerca de 0,3% das crianças negras nos Estados Unidos são homozigotos SS (2) . A anemia falciforme é caracterizada pela produção de hemoglobinas anormais que deformam e tornam rígidas as hemácias, causando o aumento da viscosidade sanguínea e oclusão da microcirculação em graus variáveis (3) . As limitações físicas por todo o organismo aumentam em frequência e intensidade com o passar dos anos. Sabe-se que suas alterações oculares têm grande incidência e podem levar à amaurose irreversível. Estas possibilitam excepcional visualização e documentação, viabilizando sua análise precisa e bem documentada. Deve-se ressaltar que os quadros oculares são de início assintomático, o que torna imperioso o exame oftalmoló-gico completo e sob midríase dos portadores de doença falciforme. RESUMO

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“…Haemoglobinopathies in which ocular involvement has been reported include homozygous sickle cell anaemia (HbSS) (Goodman et al, 1957;Condon and Serjeant, 1976), sickle cell trait (HbAS) (Gerde, 1974;Radius and Finkelstein, 1976), sickle cell haemoglobin C disease (HbSC) (Goldberg, 1971;Condon and Serjeant, 1972a;Ryan, 1974), sickle cell P-thalassaemia (HbS P-thal) Condon and Serjeant, 1972b), and haemoglobin C trait (HbAC) (Moschandreau et al, 1974).…”

mentioning

confidence: 99%

“…Angioid streaks have been noted in association with 4 of the above disorders, namely, HbSS (Geeraets and Guerry, 1960;Condon and Serjeant, 1976), HbAS (Gerde, 1974), HbSC (Condon and Serjeant, 1972a;Nagpal et al, 1976), and HbS ,-thalassaemia Nagpal et al, 1976). Their incidence in association with haemoglobinopathies as a whole has been estimated at 0-94% (Paton, 1972).…”

mentioning

confidence: 99%

Ocular findings in a case of haemoglobin H disease.

Daneshmend¹

1979

British Journal of Ophthalmology

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Ocular Findings in Hemoglobin SC Disease in Jamaica

Cited by 98 publications

References 14 publications

Ocular lesions in sickle cell disease patients from Bahia, Brazil

Ocular lesions in sickle cell disease patients from Bahia, Brazil

Alterações oculares e eletrorretinográficas na doença falciforme

Ocular findings in a case of haemoglobin H disease.

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