The risk of glaucoma associated with phacomatosis cesioflammea and phacomatosis cesioflammeo-marmorata

Background: The ocular features of phacomatosis pigmentovascularis (PPV) have rarely been reported, and glaucoma is the leading cause of blindness in patients with this condition. To protect vision in these patients, it is important to identify glaucoma as early as possible. Objectives: To systematically report the systemic and ocular manifestations of phacomatosis cesioflammea and phacomatosis cesioflammeo-marmorata, and to investigate a glaucoma risk scoring system. Materials & Methods: In this prospective study, patients with PPV from 2014 to 2021 were included. Clinical information was collected, and associations with glaucoma were evaluated. The suitability of the scoring system was assessed. A systematic literature review and analysis of reported cases of PPV was performed. Results: A total of 28 participants with PPV were included. Their ocular findings were similar, ranging from episcleral hyperpigmentation (78.5%), glaucoma (75%), choroid haemangioma (38%), and retinal vascular abnormalities (48%), to hyperpigmentation of the cornea, iris, lens and fundus. Glaucoma was associated with multiple factors, especially a thick choroid (odds ratio: 2.61; p = 0.008) and a diffuse mass-type of episcleral hyperpigmentation (odds ratio: 41.3; p = 0.027). The risk scoring system was characterized by high sensitivity (84%) and specificity (80%; AUC = 0.91) in predicting glaucoma. Conclusion: In addition to involving the systemic system, phacomatosis cesioflammea and phacomatosis cesioflammeo-marmorata also represent a specific spectrum of ophthalmic vascular malformations and hyperpigmentation. Early and periodic detailed ocular examination are recommended. The novel scoring system will help to tailor follow-up for visual protection.

The Effect of Iron Content on the Ammonia Selective Catalytic Reduction Reaction (NH3-SCR) Catalytic Performance of FeOx/SAPO-34.

Iron-based catalysts are regarded as promising candidates for the ammonia selective catalytic reduction reaction (NH3-SCR) which show good catalytic activity at medium and high temperatures, whereas SAPO-34 molecular sieves have a micro-pore structure and are ideal catalyst carriers. In this paper, four FeOx/SAPO-34 molecular sieve catalysts with different iron contents (Fe = 1%, 2%, 3%, 4%) were prepared using an impregnation method. The effect of iron content on the surface properties and catalytic activity was investigated by a series of characterization techniques including XRD, SEM, BET, XPS, H2-TPR and NH3-TPD. Iron species in the FeOx/SAPO-34 catalysts exist in the form of isolated iron ions or well-dispersed small crystals and iron oxide species clusters. With the addition of iron content, the integrity of CHA (chabazite) zeolite structure remained, but the crystallinity was affected. The FeOx/SAPO-34 catalyst with 3% Fe loading showed a relatively flat surface with no large-diameter particles and strong oxidation-reduction ability. Meanwhile, more acidic sites are exposed, which accelerated the process of catalytic reaction. Thus, the FeOx/SAPO-34 catalyst with 3% Fe showed the best NO conversion performance among the four catalysts prepared and maintained more than 90% NO conversion efficiency in a wide temperature range from 310 °C to 450 °C.

Efficacy and safety of high-intensity focused ultrasound cyclo-plasty in glaucoma.

BACKGROUND: High-intensity focused ultrasound cyclo-plasty (UCP) is a recently developed glaucoma surgery. This study collected and analysed the clinical data of patients who underwent UCP to observe the efficacy and safety of this surgery in Chinese glaucoma patients. METHODS: This was a retrospective study. The clinical data of all the patients who underwent UCP at Affiliated Foshan Hospital, Southern Medical University, were collected and analysed to evaluate the efficacy and safety of UCP. The main outcome measure was intraocular pressure, and the secondary outcome measures were best corrected visual acuity (logMAR) and complications. RESULTS: Fifty-eight patients (61 eyes) were recruited for this study. IOP was dramatically decreased during the 12 months after UCP (p<0.05). The median IOP reduction during the 18 months post-procedure was more than 30%. The greatest reduction was at 1 month post-UCP (60.86%). The qualified success rate was more than 60% during the 18-month follow-up (Fig. 1). Poor follow up was found after 6-month post-UCP. The highest success rate was obtained at 7 days post-UCP (94.55%). No statistically significant decrease in BCVA in the vison group was observed at the follow-up visits, except for 1 day post-UCP. There was a statistically significant reduction in the use of IOP lowering medications during the 6 months post-UCP. No severe complications occurred. CONCLUSION: UCP is a safe and effective procedure for primary and refractive glaucoma at least during the 6 months post-UCP procedure. Studies with longer follow-up time and better follow up are needed to further confirm the long-term efficacy and safety of UCP in Chinese glaucoma patients.

The Antiangiogenesis Effect of Pirfenidone in Wound Healing In Vitro.

Abstracts Purpose: Pirfenidone is mostly used in antifibrotic and anti-inflammatory therapies. We have previously demonstrated that pirfenidone had antifibrotic and anti-inflammatory effects on the wound healing process after glaucoma filtration surgery in vitro and in vivo. Since the wound healing and reactive scarring process simultaneously involves inflammation, fibrosis, and angiogenesis, and angiogenesis plays a more important role in chronic or prolonged wound healing, we tried to explore the antiangiogenesis effect in pirfenidone and its potential multitarget function in regulating excessive scarring. The aim of the present study was to investigate the antiangiogenesis effect of pirfenidone. METHODS: The proliferation of human umbilical vein endothelial cells (HUVECs) and human Tenon's fibroblasts (HTFs) were detected by WST-1 assay. The cell viability of HUVECs was measured by Trypan Blue together with lactate dehydrogenase, Annexin 5 experiment, and Ki-67 immunofluorescence assay. The functions of HUVECs and HTFs were demonstrated using cell migration assay, transwell invasion assay, and tube formation assay. The expression levels of vascular endothelial growth factor-A (VEGF-A), VEGF receptor-2 (VEGFR-2), neuropilin-1(NRP-1), and their downstream signaling proteins p-PI3K, PI3K, p-AKT, AKT, p-mTOR, and mechanistic target of rapamycin (mTOR) were indicated by western blot assay. The secretion of VEGF-A was detected by enzyme-linked immunosorbent assay. RESULTS: Pirfenidone inhibited proliferation, migration, invasion, and tube formation of HUVECs in vitro, and had an equivalent antiangiogenesis effect when compared with Ranibizumab in HUVECs and HTFs. Pirfenidone downregulated VEGF-A/VEGFR-2, VEGF-A/NRP-1, and its downstream signaling pathway protein expression. CONCLUSIONS: Pirfenidone has an antiangiogenesis effect in the wound healing process and may become an ideal multitarget antiscarring agent after glaucoma filtration surgery.

Pirfenidone Induces G1 Arrest in Human Tenon's Fibroblasts In Vitro Involving AKT and MAPK Signaling Pathways.

PURPOSE: To investigate the underlying mechanism by which pirfenidone blocks the transition from the G1 to S phase in primary human Tenon's fibroblasts. METHODS: Primary human Tenon's fibroblasts were characterized by immunocytofluorescence staining with vimentin, fibroblast surface protein, and cytokeratin. After treating Tenon's fibroblasts with pirfenidone under proliferation conditions (10% fetal bovine serum), cell proliferation was measured using a WST-1 assay. Progression through the cell cycle was analyzed by flow cytometry. The expression of CDK2, CDK6, cyclinD1, cyclinD3, and cyclinE and the phosphorylation of AKT, ERK1/2/MAPK, JNK/MAPK, and p38 MAPK were estimated using western blot analysis. RESULTS: Under proliferative conditions, pirfenidone inhibited Tenon's fibroblasts proliferation and arrested the cell cycle at the G1 phase; decreased the phosphorylation of AKT, GSK3ß, ERK1/2/MAPK, and JNK/MAPK; increased the phosphorylation of p38 MAPK; and inhibited CDK2, CDK6, cyclin D1, cyclin D3, and cyclin E in a dose-dependent manner. Inhibitors of AKT (LY294002), ERK1/2 (U0126), and JNK (SP600125) arrested the G1/S transition, similar to the effect of pirfenidone. The p38 inhibitor (SB202190) decreased the G1-blocking effect of pirfenidone. The expression of CDK2, CDK6, cyclin D1, and cyclin D3 were inhibited by LY294002, U0126, and SP600125. SB202190 attenuated the pirfenidone-induced reduction of CDK2, CDK6, cyclin D1, cyclin D3, and cyclin E. CONCLUSIONS: Pirfenidone inhibited HTFs proliferation and induced G1 arrest by downregulating CDKs and cyclins involving the AKT/GSK3ß and MAPK signaling pathways.

Phakomatosis Pigmentovascularis Associated With Sturge-Weber Syndrome, Ota Nevus, and Congenital Glaucoma.

Phakomatosis pigmentovascularis (PPV) is a rare congenital malformation syndrome that is characterized by a combination of capillary abnormalities and dermal melanocytosis.We describe 3 cases of PPV combined with bilateral Sturge-Weber syndrome (SWS), Ota nevus, and congenital glaucoma.Case 1 was a 2-year-old boy. Facial port-wine stains distributed along the 3 branches of his trigeminal nerves, which suggested the existence of SWS. Gray-blue patches were spread over the frontal and temporal areas of bilateral face, waist, buttocks, and thigh. Bilateral triangular alopecia was found on the temporal scalp. The diagnosis of Ota nevus was made by the bilateral scleral malanocystosis. Increased intraocular pressure, enlarged cornea, and pathologic optic disc cupping supported the diagnoses of infantile bilateral glaucoma. Case 2 was a 4-year-old boy. Port-wine stains were found on the face along the 3 branches of the trigeminal nerve and distributed along the trunk, arms, and legs. Mongolian spots spread over his frontal and temporal areas of the bilateral face, waist, buttocks, thigh, abdomen, and back. Infantile glaucoma was found in both eyes. Ota nevus were found in the both eyes. Optic coherent tomography (OCT) scans revealed increased thickness of choroid. Case 3 was a 5-year-old boy. Besides Ota nevus and infantile glaucoma in both eyes, color Doppler ultrasonography showed choroidal hemagioma. OCT scan showed increased choroidal thickness. The bilateral triangular alopecia on the child's temporal scalp was similar to that of Case 1. Cases 1 and 2 presented with port-wine stain patches that were consistent with the characteristic manifestation of PPV type IIb. However, the CMTC of Case 3 met the diagnostic criteria for PPV type Vb.Case 1 was treated with trabeculotomies in both eyes. For Cases 2 and 3, surgical interventions were not considered due to the high risks of antiglaucomatous operation complications. We prescribed them antiglaucoma indications.The simultaneously coexistence of PPV with SWS, Ota nevus, and congenital glaucoma is rare. In the clinic, additional detailed examinations and tests of PPV patients to exclude other ocular abnormalities or extraocular involvements are necessary.

Immunostimulatory effect of a composition isolated from white peony root oral liquid in the treatment of radiation-induced esophagitis.

The aim of this study was to explore the immune repairing effect of a composition isolated from white peony root oral liquid (cWPROL), a traditional Chinese herbal composition, in the treatment of experimental radiation-induced esophagitis in rats. A total of 128 Wistar rats were randomly divided into eight groups, irradiated with 43 Gy 60Co γ-rays to induce esophagitis and treated by different methods. Flow cytometry, hematological analysis and immune nephelometry were used to detect the absolute numbers and percentages of CD3+, CD4+ and CD8+ T lymphocytes, numbers and classification of leukocytes, and the levels of IgG and complement C3 in the peripheral blood of the rats at each experimental time point. Following irradiation, the total number of leukocytes, absolute numbers and percentages of CD3+, CD4+ and CD8+ T lymphocytes, and levels of IgG and complement C3 in the peripheral blood of the rats were decreased. Furthermore, the total numbers of leukocytes, absolute numbers and percentages of CD3+, CD4+ and CD8+ T lymphocytes, and levels of IgG and complement C3 in the peripheral blood were higher in the administered with cWPROL by intra-esophageal perfusion compared with those in the untreated irradiated groups, but lower in the groups treated with a mixture of lidocaine hydrochloride, dexamethasone sodium phosphate and gentamicin sulfate. This study suggested that cWPROL is able to repair the impaired cellular and humoral immunity of rats with radiation-induced esophagitis.

Improved retinal ganglion cell survival through retinal microglia suppression by a chinese herb extract, triptolide, in the DBA/2J mouse model of glaucoma.

To investigate the changes in retinal microglia and retinal ganglion cell (RGC) survival after long-term administration of a Chinese herb extract, triptolide, in a DBA/2J mice. DBA/2J mice (n = 96) were administered triptolide (n = 48) 25 µg/kg or vehicle (n = 48) and were judged at 7, 9, 11 months of age. Long-term triptolide treatment tended to attenuate the anterior segment pathology in experimental group, though intraocular pressure was not significantly different between the two groups. In the experimental group, RGC survival was improved (7, 9, 11 months: p = 0.035, 0.004, 0.014), and microglia activation was suppressed based on a more ramified appearance (9, 11 months: p = 0.024, 0.013) and a lower total microglial cell count (7, 9, 11 months: p = 0.028, 0.025, 0.014). Double-immunofluorescence staining revealed TNF? localized to microglia, TNFR1 localized to the RGCs and nerve fiber layer. These findings indicate that long-term triptolide administration suppressed microglia activation and improved RGC survival in DBA/2J mice.

Baicalein reduces inflammatory process in a rodent model of diabetic retinopathy.

PURPOSE: This study was designed to elucidate the role of inflammatory process in diabetic retinopathy and to investigate the effect of baicalein treatment on diabetic rat. METHODS: Retinal microglial cells were identified with CD11b antibody, and retinal Müller cells were identified with glial fibrillary acidic protein (GFAP). The gene expression of interleukin (IL)-18, tumor necrosis factor (TNF)-alpha, and IL-1beta was examined by quantitative real-time PCR. The expression of GFAP and vascular endothelial growth factor (VEGF) was examined by quantitative real-time PCR, immunohistochemistry, and Western blot analysis. Vascular permeability was measured in vivo by bovine serum albumin conjugated with FITC. Baicalein was given by oral administration (150 mg/kg/d) with an animal feeding needle beginning 5 days after streptozotocin (STZ) injection. RESULTS: By 24 weeks after onset of diabetes, microglial cells were activated and proliferated, and Müller cells upregulated their GFAP and VEGF expression. Pro-inflammatory factors, including IL-18, TNF-alpha, and IL-1beta, were significantly upregulated. Obvious vascular leakage and abnormality were demonstrated, and ganglion cell loss was significant. Baicalein treatment ameliorated diabetes-induced microglial activation and pro-inflammatory expression, reduced the GFAP and VEGF expression from Müller cells, and significantly reduced vascular abnormality and ganglion cell loss within the retina. CONCLUSIONS: Inflammatory process, characterized by microglial activation and Müller cells dysfunction, was implicated in STZ-induced diabetic retinopathy. Baicalein treatment ameliorated inflammatory process, and therefore inhibited vascular abnormality and neuron loss in diabetic retinas.