+ Site Statistics
References:
54,258,434
Abstracts:
29,560,870
PMIDs:
28,072,757
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Clinical and laboratory factors associated with the severity of proliferative sickle cell retinopathy in patients with sickle cell hemoglobin C (SC) and homozygous sickle cell (SS) disease



Clinical and laboratory factors associated with the severity of proliferative sickle cell retinopathy in patients with sickle cell hemoglobin C (SC) and homozygous sickle cell (SS) disease



Medicine 90(6): 372-378



Proliferative sickle cell retinopathy (PSCR) is the most frequent vision-threatening complication of sickle cell disease (SCD). We investigated the relationship between the severity of sickle cell retinopathy in heterozygous (SC) or homozygous (SS) adult SCD patients and the clinical and laboratory data obtained during visits to a national SCD referral center. This retrospective longitudinal analysis included 942 SCD patients (313 patients with SC and 629 with SS disease) with ophthalmologic evaluations who were followed over a 19-year period by a multidisciplinary team in a referral center. PSCR was graded using the Goldberg classification. We identified patient and SCD characteristics associated with sickle cell retinopathy severity using multinomial logistic-regression models. Multivariate analysis associated severe PSCR forms (stages III-V) with older age (p=0.032), pulmonary involvement (documented pulmonary hypertension with pulmonary arterial pressure≥40 mm Hg, restrictive syndrome>20%, or previous history of pulmonary embolism diagnosed by vascular imaging) (p=0.029), deafness or tinnitus (p=0.026), and no history of osteomyelitis (p=0.013) for SC patients; and with older age (p<0.001), male sex (p=0.003), and acute pyelonephritis (p=0.04) for SS patients. The model of severe PSCR versus no PSCR showed good calibration and discrimination for SC and SS patients. Awareness of the clinical and laboratory factors significantly associated with severe PSCR in patients with SC or SS SCD may contribute to improved preventive strategies.

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 052106748

Download citation: RISBibTeXText

PMID: 22033449

DOI: 10.1097/md.0b013e3182364cba


Related references

Severe proliferative retinopathy is associated with blood hyperviscosity in sickle cell hemoglobin-C disease but not in sickle cell anemia. Clinical Hemorheology and Microcirculation 55(2): 205-212, 2014

Haematological factors associated with proliferative retinopathy in homozygous sickle cell disease. British Journal of Ophthalmology 65(1): 29-35, 1981

Normal vitamin e status in homozygous sickle cell anemia and hemoglobin sickle cell disease. Clinical Research 34(1): 137A, 1986

Sickle-resistant sickle cells in patients with homozygous sickle cell disease. Blood 98(11 Part 2): 18b, November 16, 2001

Fetal hemoglobin and F-cell responses to long-term hydroxyurea treatment in young sickle cell patients. The French Study Group on Sickle Cell Disease. Blood 91(12): 4472-4479, 1998

Effective sickle hemoglobin reduction by automated red cell exchange using Spectra Optia in three Emirati patients with sickle cell disease before allogeneic hematopoietic stem cell transplantation. Blood Research 53(4): 325-329, 2018

Red blood cell aggregation, aggregate strength and oxygen transport potential of blood are abnormal in both homozygous sickle cell anemia and sickle-hemoglobin C disease. Haematologica 94(8): 1060-1065, 2009

Fetal hemoglobin and clinical severity of homozygous sickle cell disease in early childhood. Journal of Pediatrics 98(1): 37-41, 1981

Proliferative sickle cell retinopathy associated with sickle cell trait and gestational diabetes: case report. Arquivos Brasileiros de Oftalmologia 72(3): 400-402, 2009

Sickle cell disease in Madhya Pradesh, Central India: A comparison of clinical profile of sickle cell homozygote vs. sickle-beta thalassaemia individuals. Hematology 21(9): 558-563, 2017

Studies in sickle-cell anemia. V. Sickle-cell hemoglobin C disease; report of two cases in siblings with clinical and genetic observations and a brief review of the literature. A.M.A. American Journal of Diseases of Children 90(1): 35-42, 1955

Outcome of pregnancy in sickle cell anemia and sickle cell-hemoglobin C disease. An analysis of 181 pregnancies in 98 patients, and a review of the literature. American Journal of Obstetrics and Gynecology 138(3): 239-245, 1980

The spectrum of sickle hemoglobin-related nephropathy: from sickle cell disease to sickle trait. Expert Review of Hematology 10(12): 1087-1094, 2017

Blood rheology and proliferative retinopathy in homozygous sickle cell disease. British Journal of Ophthalmology 70(7): 522-525, 1986

Red cell exchange transfusion halts progressive proliferative sickle cell retinopathy in a teenaged patient with hemoglobin SC disease. Pediatric Blood and Cancer 62(4): 721-723, 2015