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Incidence and natural history of proliferative sickle cell retinopathy: observations from a cohort study.
Ophthalmology. 2005 Nov; 112(11):1869-75.O

Abstract

OBJECTIVE

To describe the incidence, prevalence, and natural history of proliferative sickle cell retinopathy (PSR).

DESIGN

Prospective longitudinal study over 20 years.

PARTICIPANTS

Newborn screening of 100000 consecutive deliveries from 1973 to 1981 identified 315 children with homozygous sickle cell (SS) disease and 201 with SS-hemoglobin C (SC) disease. By the age of 5 years, 307 SS patients and 166 SC patients were alive and living in Jamaica and were recruited for this ophthalmic study.

METHODS

Description of retinal vascular changes on annual angiography and angioscopy.

MAIN OUTCOME MEASURES

Incidence and prevalence of PSR and its behavior on follow-up. Progression of PSR was investigated using the number of eyes affected (none, one, both) and the interval until PSR onset.

RESULTS

At last review in January 2000, PSR had developed in 59 patients (14 SS, 45 SC), unilaterally in 36 patients and bilaterally in 23. Incidence increased with age in both genotypes, with crude annual incidence rates of 0.5 cases (95% confidence interval [CI], 0.3-0.8) per 100 SS subjects and 2.5 cases (95% CI, 1.9-3.3) per 100 SC subjects. Prevalence was greater in SC disease, and by the ages of 24 to 26 years, PSR had occurred in 43% subjects with SC disease and in 14% subjects with SS disease. Patients with unilateral PSR had a 16% (11% SS, 17% SC) probability of regressing to no PSR and a 14% (16% SS, 13% SC) probability of progressing to bilateral PSR. Those with bilateral PSR had an 8% (8% SS, 8% SC) probability of regressing to unilateral PSR and a 1% (0 SS, 2% SC) probability of regressing to a PSR-free state. Irretrievable visual loss occurred in only 1 of 82 PSR-affected eyes, and 1 required detachment surgery and recovered normal visual acuity.

CONCLUSIONS

Longitudinal observations over 20 years in a cohort of patients followed from birth confirms a greater incidence and severity of PSR in SC disease, and shows that spontaneous regression occurred in 32% of PSR-affected eyes. Permanent visual loss was uncommon in subjects observed up to the age of 26 years.

Authors+Show Affiliations

MRC Laboratories, Kingston, Jamaica.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

16171867

Citation

Downes, Susan M., et al. "Incidence and Natural History of Proliferative Sickle Cell Retinopathy: Observations From a Cohort Study." Ophthalmology, vol. 112, no. 11, 2005, pp. 1869-75.
Downes SM, Hambleton IR, Chuang EL, et al. Incidence and natural history of proliferative sickle cell retinopathy: observations from a cohort study. Ophthalmology. 2005;112(11):1869-75.
Downes, S. M., Hambleton, I. R., Chuang, E. L., Lois, N., Serjeant, G. R., & Bird, A. C. (2005). Incidence and natural history of proliferative sickle cell retinopathy: observations from a cohort study. Ophthalmology, 112(11), 1869-75.
Downes SM, et al. Incidence and Natural History of Proliferative Sickle Cell Retinopathy: Observations From a Cohort Study. Ophthalmology. 2005;112(11):1869-75. PubMed PMID: 16171867.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Incidence and natural history of proliferative sickle cell retinopathy: observations from a cohort study. AU - Downes,Susan M, AU - Hambleton,Ian R, AU - Chuang,Elaine L, AU - Lois,Noemi, AU - Serjeant,Graham R, AU - Bird,Alan C, Y1 - 2005/09/19/ PY - 2005/01/14/received PY - 2005/05/09/accepted PY - 2005/9/21/pubmed PY - 2005/12/13/medline PY - 2005/9/21/entrez SP - 1869 EP - 75 JF - Ophthalmology JO - Ophthalmology VL - 112 IS - 11 N2 - OBJECTIVE: To describe the incidence, prevalence, and natural history of proliferative sickle cell retinopathy (PSR). DESIGN: Prospective longitudinal study over 20 years. PARTICIPANTS: Newborn screening of 100000 consecutive deliveries from 1973 to 1981 identified 315 children with homozygous sickle cell (SS) disease and 201 with SS-hemoglobin C (SC) disease. By the age of 5 years, 307 SS patients and 166 SC patients were alive and living in Jamaica and were recruited for this ophthalmic study. METHODS: Description of retinal vascular changes on annual angiography and angioscopy. MAIN OUTCOME MEASURES: Incidence and prevalence of PSR and its behavior on follow-up. Progression of PSR was investigated using the number of eyes affected (none, one, both) and the interval until PSR onset. RESULTS: At last review in January 2000, PSR had developed in 59 patients (14 SS, 45 SC), unilaterally in 36 patients and bilaterally in 23. Incidence increased with age in both genotypes, with crude annual incidence rates of 0.5 cases (95% confidence interval [CI], 0.3-0.8) per 100 SS subjects and 2.5 cases (95% CI, 1.9-3.3) per 100 SC subjects. Prevalence was greater in SC disease, and by the ages of 24 to 26 years, PSR had occurred in 43% subjects with SC disease and in 14% subjects with SS disease. Patients with unilateral PSR had a 16% (11% SS, 17% SC) probability of regressing to no PSR and a 14% (16% SS, 13% SC) probability of progressing to bilateral PSR. Those with bilateral PSR had an 8% (8% SS, 8% SC) probability of regressing to unilateral PSR and a 1% (0 SS, 2% SC) probability of regressing to a PSR-free state. Irretrievable visual loss occurred in only 1 of 82 PSR-affected eyes, and 1 required detachment surgery and recovered normal visual acuity. CONCLUSIONS: Longitudinal observations over 20 years in a cohort of patients followed from birth confirms a greater incidence and severity of PSR in SC disease, and shows that spontaneous regression occurred in 32% of PSR-affected eyes. Permanent visual loss was uncommon in subjects observed up to the age of 26 years. SN - 1549-4713 UR - https://www.unboundmedicine.com/medline/citation/16171867/Incidence_and_natural_history_of_proliferative_sickle_cell_retinopathy:_observations_from_a_cohort_study_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0161-6420(05)00889-4 DB - PRIME DP - Unbound Medicine ER -