Javaneh Abbasian

Cord Blood Nucleated Cells Induce Delayed T Cell Alloreactivity

Cord blood (CB) mononuclear cells (MNCs) can be transplanted in HLA mismatched recipients with limited graft rejection or graft-versus-host disease (GVHD). Previous studies have shown that naive T cells and hyporesponsive dendritic cells are largely represented in CB. Data presented here demonstrate that CB MNCs are unable to stimulate allogeneic T cell proliferative or cytotoxic responses in standard in vitro assays. However, a suppressive effect of CB MNCs was ruled out because purified CD34(+) cells or CD14(+) monocytes stimulated T cell responses that were not inhibited by add-back of CB MNCs. The lack of antigen-presenting cell (APC) activity of CB MNCs in primary mixed lymphocyte culture (MLC) did not induce allogeneic T cell anergy. In fact, rechallenge of T cells with CB CD34(+) cells, or immature monocyte-derived dendritic cells (iMo-DCs) in secondary MLC induced potent T cell proliferative responses. A delayed APC activity of CB MNCs was observed after stimulation with irradiated allogeneic T cells for 6 days, likely because of the upregulation of CD86 and HLA-DR on CB cells. Cytotoxic lymphocytes (CTL) were generated after stimulation of blood T cells with CB MNCs for 4 weeks or CB-derived iMo-DCs for 1 week. Concomitant stimulation of T cells with CB iMo-DC obtained from 2 CB units resulted in the generation of CTLs specific for each CB, independently of the CB:CB cell ratio. These data suggest that the APC activity of CB cells possibly increases posttransplant, and may contribute to delayed graft rejection and/or acute and chronic GVHD.

Immunologic and genetic markers in patients with idiopathic ocular inflammation and a family history of inflammatory bowel disease

PURPOSE To evaluate the prevalence of immunologic and genetic markers in patients with idiopathic ocular inflammation and a family history of inflammatory bowel disease. DESIGN Matched case-control study. METHODS Patients with a diagnosis of idiopathic ocular inflammation and family history of inflammatory bowel disease who did not have inflammatory bowel disease themselves were identified and matched to control patients with idiopathic ocular inflammation. Serum was evaluated for immunologic markers using Prometheus IBD Serology 7. Genomic DNA was analyzed for single nucleotide polymorphisms (SNP) of the NOD2 gene associated with Crohn disease. RESULTS Fifteen patients with idiopathic ocular inflammation and family history of inflammatory bowel disease were matched to 15 control patients based on age, sex, and race. Eight of 15 patients (53%) with a family history of inflammatory bowel disease had elevated p-ANCA antibody levels compared to 3 of 15 controls (20%) (1-sided P = .04) with a matched analysis odds ratio of 6.0 (1-sided P = .06). Four of 15 patients (27%) with family history of inflammatory bowel disease tested positive for immunologic markers predicting ulcerative colitis, while no control patients tested positive (1-sided P = .06). Carrier rates of NOD2 SNPs did not differ significantly between the test and control groups. CONCLUSIONS One-quarter of patients with idiopathic ocular inflammation and a family history of inflammatory bowel disease had immunologic markers predicting bowel disease, and one-half had elevated p-ANCA levels. Prometheus IBD Serology 7 may be useful in the evaluation of selected patients with unexplained uveitis.

Macular optical coherence tomography in patients with unilateral optic nerve hypoplasia.

PURPOSE To characterize the extent and location of macular thinning in patients with unilateral optic nerve hypoplasia (ONH) as compared to the contralateral normal eye. METHODS The medical records of patients with unilateral ONH who underwent spectral domain optical coherence tomography (SD-OCT) of the macula were retrospectively reviewed. SD-OCT scans were manually segmented by 3 observers in 3 macular regions (superior, central, inferior). Boundaries identified included the inner limiting membrane, the junction between the inner nuclear layer and outer plexiform layer, and the neural retina-retinal pigment epithelium interface. Using custom MATLAB software, inner and outer retinal thickness profiles were quantified. A paired t test was used to compare the retinal thickness between the ONH eye and the contralateral normal eyes. RESULTS Inner retinal thickness of the ONH eye was decreased in all areas of the macula (superior, central, and inferior) compared to the contralateral normal eye (P < 0.05). Outer retinal thicknesses were also decreased in the central and inferior sections compared with the normal eye (P < 0.05). CONCLUSIONS Optic nerve hypoplasia is a congenital disease known to result in thinning of the nerve fiber and ganglion cell layer. Our small cohort demonstrated thinning of the inner retinal layers as well as the outer retinal layers in the ONH eye compared with the contralateral normal eye.

Congenital cavitary optic disc anomaly and Axenfeld’s anomaly in Wolf-Hirschhorn syndrome: A case report and review of the literature

ABSTRACT Background: Wolf-Hirschhorn syndrome is a rare genetic syndrome caused by a heterozygous deletion on chromosome 4p16.3 and is characterized by a “Greek warrior helmet” facies, hypotonia, developmental delay, seizures, structural central nervous system defects, intrauterine growth restriction, sketelal anomalies, cardiac defects, abnormal tooth development, and hearing loss. A variety of ocular manifestations may occur in up to 40% of patients. Materials/methods: We report the genetic testing results, systemic findings, and complete ophthalmologic examination findings in a patient with Wolf-Hirschhorn syndrome, including external photography, RetCam3 (Clarity Medical Systems, Pleasonton, CA) goniography, and fundus photography. In addition, we review the literature on ocular manifestations of Wolf-Hirschhorn syndrome. Results: Microarray analysis revealed an unbalanced translocation between 4p16.3–15.3 and Xp22.33-p22.2. Systemic findings included “Greek warrior helmet” facies, hypotonia, cleft palate, neonatal tooth eruption, talipes equinovarus, bilateral clinodactyly, clitoromegaly, partial agenesis of the corpus callosum, bilateral renal hypoplasia, and two atrial septal defects. Ocular findings included normal intraocular pressures and corneal diameters, large-angle exotropia, downward slanting of the palpebral fissures, absent eyelid creases, upper and lower eyelid retraction with shortage of the anterior eyelid lamellae, euryblepharon, lagophthalmos with poor Bell’s reflex and exposure keratopathy, hypertelorism, Axenfeld’s anomaly, megalopapillae, and cavitary optic disc anomaly. Conclusions: We describe the ocular phenotype of a patient with Wolf-Hirschhorn syndrome, including the rare descriptions and photographs of Axenfeld’s anomaly, megalopapilla, and cavitary optic disc anomaly in this condition.

Bilateral Optic Nerve Head Changes in an Asymptomatic Preadolescent Girl

A preadolescent girl presented to the pediatric ophthalmology clinic for evaluation and treatment of possible papilledema, discovered by an outside eye care professional during routine eye examination. Aside from mild hyperopic astigmatism, there was no significant ocular, medical, or family history. The patient denied any change in vision, scotomas, transient visual obscurations, dimming of light, photophobia, or pain. She denied headache, pulsatile tinnitus, weight gain, joint pain, lethargy, decreased appetite, fever, or chills. The patient was not taking any prescription or over-the-counter medicines; however, she had recently used a short-term course of oral antibiotics for a tooth infection. On examination, Snellen visual acuity with correction was 20/20 OD and 20/25 OS. Confrontation visual fields and ocular motility were normal. External examination was unremarkable, and slitlamp examination revealed normal eyelids, conjunctiva, and sclera. There were 1+ anterior chamber white blood cells and trace anterior vitreous cells bilaterally. Posterior segment examination revealed 360° nerve hyperemia with elevation of nasal disc borders bilaterally (Figure 1), without vitreous haze, snowballs, or snowbanks. There was no vascular sheathing, choroidal granulomas, or chorioretinal scarring in the periphery. Right eye A Left eye B

The use of irradiated corneal patch grafts in pediatric Ahmed drainage implant surgery.

PURPOSE To describe the use of irradiated cornea for scleral reinforcement in Ahmed glaucoma valve drainage implant (AGV) devices in children. METHODS The medical records of patients <18 years of age who underwent AGV surgery with irradiated cornea as scleral reinforcement were reviewed retrospectively. The primary outcome measure was erosion of the drainage tube through the corneal patch graft. Secondary outcome measures included other major complications: persistent inflammation, wound dehiscence, transmission of infectious disease, endophthalmitis, and tube/plate self-explantation. RESULTS A total of 25 procedures (20 patients) met inclusion criteria. Average patient age was 70 months (range, 2 months to 17 years). Mean follow-up was 24.8 months (range, 6 months to 6.2 years). One tube experienced conjunctival exposure through two separate corneal grafts (2/25 cases [8%]), sequentially in the same eye. The first event occurred at month 3.5 after primary implantation of the tube shunt; the second erosion occurred following revision of the existing implant at month 1.5 postoperatively. There were 2 cases of auto-explantation, 2 cases of wound dehiscence, and 1 case of persistent inflammation. There were no cases of endophthalmitis or other infections. CONCLUSIONS To our knowledge, this is the first report describing the use of corneal patch grafts in children. Irradiated cornea improves cosmesis and enhances visualization of the tube. The risk of tube exposure was found to be low and comparable to other materials used as a patch graft.