Bilateral giant retinal pigment epithelial tears in hypertensive choroidopathy.

PURPOSE: To report a case of hypertensive choroidopathy with bilateral bullous serous retinal detachments (SRDs), retinal pigment epithelial detachments (PEDs), and giant retinal pigment epithelial (RPE) tears. OBSERVATIONS: A 68-year-old man with a history of hypertension and diabetes mellitus presented with bilateral visual loss of about 10 day's duration. He discontinued his oral medications for 2 months without the advice of a physician. At his first visit, the best-corrected visual acuities (BCVAs) were 0.02 in the right eye and 0.3 in the left eye (decimal notation), and the respective intraocular pressures were 15 and 13 mmHg. Bullous SRDs, large PEDs, and giant RPE tears were present bilaterally. Blot retinal hemorrhages and hard exudates were seen in the left eye. The systemic blood pressure was 231/77 mmHg, and bilateral lower leg edema was observed. Biochemical blood tests showed deteriorated renal function, hypoalbuminemia, and hyperglycemia. Ultra-wide-field fundus fluorescein angiography showed leakage at the areas of the SRDs and hyperfluorescent areas corresponding to the RPE tears bilaterally. Indocyanine green angiography showed hypofluorescent areas corresponding to the PEDs. Systemic computed tomography and magnetic resonance imaging were performed, and no malignancy was found. Based on these findings, hypertensive choroidopathy was diagnosed. A week after antihypertensive treatment, the SRDs and PEDs resolved bilaterally, and the BCVAs improved to 0.4 and 0.5 in the right and left eyes, respectively. The RPE tears remained in both eyes, although the SRDs and PEDs did not recur. CONCLUSIONS AND IMPORTANCE: Hypertensive choroidopathy must be considered in the differential diagnosis of SRDs and/or PEDs.

Identification of Domains for Efficient Notch Signaling Activity in Immobilized Notch Ligand Proteins.

Notch is a critical signaling pathway that controls cell fate and tissue homeostasis, but the functional characterization of Notch ligand domains that activate Notch receptors remains incomplete. Here, we established a method for immobilizing Notch ligand proteins onto beads to measure time-dependent Notch activity after the addition of Notch ligand-coated beads. A comparison between activities by the Notch ligand found on the cell surface to that of the ligand immobilized on beads showed that immobilized Notch ligand protein produces comparable signal activity during the first 10 h. Follow-up truncation studies showed that the N-terminal epidermal growth factor (EGF) repeat three region of delta like canonical Notch ligand 4 (DLL4) or jagged 1 (JAG1) is the minimum region for activating Notch signaling, and the DLL4 EGF repeat three domain may have a role in activation through a mechanism other than by increasing binding affinity. In addition, we found that reconstruction of the DLL4 delta and OSM-11 (DOS) motif (N257P) resulted in an increase in both binding affinity and signaling activity, which suggests that the role of the DOS motif is conserved among Notch ligands. Furthermore, active DLL4 protein on beads promoted T cell differentiation or inhibited B cell differentiation in vitro, whereas JAG1 proteins on beads did not have any effect. Taken together, our findings provide unambiguous evidence for the role of different Notch ligands and their domains in Notch signal activation, and may be potential tools for controlling Notch signaling activation. J. Cell. Biochem. 118: 785-796, 2017. © 2016 Wiley Periodicals, Inc.