Ocular manifestations in egyptian children and young adults with sickle cell disease.

In sickle cell disease (SCD), ocular lesions result from stasis and occlusion of small eye vessels by sickled erythrocytes. Vaso-occlusive disease of the retina can be responsible for nonproliferative (NPR) and proliferative retinopathy (PR). Patients are often asymptomatic until serious complications arise as, vitreous hemorrhage and retinal detachment. This work aimed to study the frequency and pattern of ocular manifestations in Egyptian children and young adults with SCD. In this cross-sectional study, 40 steady state patients (80 eyes) aged 2-28 years (30 children and 10 young adults) with established diagnosis of SCD (26 with homozygous SS and 14 with S/ß thalassemia underwent complete ophthalmic examination with dilated fundoscopy. Fluorescein angiography was performed for patients ≥12 years old. The overall frequency of retinal lesions was 47.5 % (46.2 and 50 % of SS and S/ß patients respectively). PR and NPR were evident in 32.5 and 27.5 % of all enrolled patients respectively (five patients having both). Peripheral retinal occlusion was a frequent ocular finding in both groups; the youngest patient showing PR was 15 years old. Older age, longer disease duration and splenectomy were significantly more prevalent among patients with PR. Despite lack of visual symptoms, children and young adults are at risk of PR. Frequency of retinal lesions was comparable in SS and S/ß patients. Periodic ophthalmologic examination starting at the age of 12 years is recommended for timely-identification of retinal lesions thus minimizing the risk of sight threatening retinopathy.

Assessment of anti-neutrophil cytoplasmic antibodies and selected serum pro-inflammatory cytokines in systemic lupus erythematosus: prevalence and clinical significance.

This study was designed to elucidate the prevalence and clinical implications of various cytokines such as TNF-alpha, IL-6 and IL-2 in presence or absence of anti-neutrophil cytoplasmic auto- antibodies (ANCAs) in children with SLE. The present study was conducted on 29 children with SLE and 20 healthy children of matched age and sex as controls. Patients were selected from the Rheumatology Clinic, Cairo University Pediatric Hospital. Serum levels of ANCAs, ANA, anti-ds-DNA were assayed by an indirect immuno-fluorescence technique (IIF). Serum levels of cytokines (TNF-alpha, IL-6 and IL-2) were assayed by ELISA technique. The anti-neutrophil cytoplasmic auto- antibodies were present in 55% of children with SLE. Furthermore, serum levels of TNF-alpha, IL-6 were increased and IL-2 level was decreased in SLE children positive for ANCAs. In addition, SLE complications were increased in those children. These results demonstrate that ANCAs may be detectable in SLE sera and their presence could be associated with particular clinical manifestations.