- Treatment of facial telangiectasia with narrow-band intense pulsed light in Chinese patients. [Journal Article]
- JCJ Cosmet Laser Ther 2018 Feb 20; :1-5
- CONCLUSIONS: Narrow-band intense pulsed light DPL is effective and safe in treating facial telangiectasia.
- Vascular deficiency of Smad4 causes arteriovenous malformations: a mouse model of Hereditary Hemorrhagic Telangiectasia. [Journal Article]
- AAngiogenesis 2018 Feb 19
- Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder that leads to abnormal connections between arteries and veins termed arteriovenous malformations (AVM). Mutation...
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder that leads to abnormal connections between arteries and veins termed arteriovenous malformations (AVM). Mutations in TGFβ pathway members ALK1, ENG and SMAD4 lead to HHT. However, a Smad4 mouse model of HHT does not currently exist. We aimed to create and characterize a Smad4 endothelial cell (EC)-specific, inducible knockout mouse (Smad4f/f;Cdh5-CreERT2) that could be used to study AVM development in HHT. We found that postnatal ablation of Smad4 caused various vascular defects, including the formation of distinct AVMs in the neonate retina. Our analyses demonstrated that increased EC proliferation and size, altered mural cell coverage and distorted artery-vein gene expression are associated with Smad4 deficiency in the vasculature. Furthermore, we show that depletion of Smad4 leads to decreased Vegfr2 expression, and concurrent loss of endothelial Smad4 and Vegfr2 in vivo leads to AVM enlargement. Our work provides a new model in which to study HHT-associated phenotypes and links the TGFβ and VEGF signaling pathways in AVM pathogenesis.
- Benign and malignant hepatocellular lesions in patients with vascular liver diseases. [Journal Article]
- ARAbdom Radiol (NY) 2018 Feb 19
- A variety of vascular liver disorders can induce hepatocellular tumors. They may be related to portal venous deprivation, venous outflow obstruction, or arterial diseases. Their common feature is an ...
A variety of vascular liver disorders can induce hepatocellular tumors. They may be related to portal venous deprivation, venous outflow obstruction, or arterial diseases. Their common feature is an imbalance between hepatic arterial and portal venous blood flow leading to an increased hepatic arterial inflow. Consequently, hepatocellular tumors may arise, most commonly focal nodular hyperplasia-like lesions but hepatocellular adenomas and hepatocellular carcinoma may be seen as well. This article will review the most common vascular liver diseases associated with hepatocellular nodules (Budd-Chiari syndrome, congenital portosystemic shunt, hereditary hemorrhagic telangiectasia, and portal cavernoma). For each condition, imaging findings will be described as well as the differential diagnosis and the diagnostic clues.
- Antiviral activity of pyrrole-imidazole polyamides against SV40 and BK polyomaviruses. [Journal Article]
- ARAntiviral Res 2018 Feb 16
- The ability of antiviral polyamides (AVP) to act upon polyomaviruses (PyV) was evaluated. Initial studies found that a single treatment of AVP protected SV40-infected BSC-1 cells from cytopathic effe...
The ability of antiviral polyamides (AVP) to act upon polyomaviruses (PyV) was evaluated. Initial studies found that a single treatment of AVP protected SV40-infected BSC-1 cells from cytopathic effect (CPE) for as long as 11 days p.i.. AVP substantially suppressed SV40 genome copy numbers over the duration of the experiment. Immunofluorescence analysis of ataxia-telangiectasia mutated (ATM) activation and large T antigen (LTag) expression clearly demonstrated that AVP treatment at day 1 p.i. delayed the onset of productive SV40 replication by approximately 3 days, and substantially limited the infection relative to vehicle-treated controls. AVP dose-response experiments recorded IC50s in the low nM range that were similar to IC50s previously reported for HPV16. The ability of AVPs to act on BKPyV was next examined. Again, IC50s in the low nM range were obtained with the exception of an AVP (PA1) that gave an IC50 of 437 nM against the BKPyV Dunlop strain. The Mre11 inhibitor Mirin substantially reduced the AVP IC50 against SV40 demonstrating that Mre11 protects PyV genomes from AVP action as previously shown for HPV. Together these experiments show that AVPs are potent antiviral agents for PyV that act via a mechanism with similarities to that found for HPV. The results demonstrate that AVPs are useful tools for controlling and studying PyV biology. The potential use of these agents against BKPyV and other PyV pathogens also has clinical implications.
- Comparison of Selected Parameters of Redox Homeostasis in Patients with Ataxia-Telangiectasia and Nijmegen Breakage Syndrome. [Journal Article]
- OMOxid Med Cell Longev 2017; 2017:6745840
- This study compared the antioxidant status and major lipophilic antioxidants in patients with ataxia-telangiectasia (AT) and Nijmegen breakage syndrome (NBS). Total antioxidant status (TAS), total ox...
This study compared the antioxidant status and major lipophilic antioxidants in patients with ataxia-telangiectasia (AT) and Nijmegen breakage syndrome (NBS). Total antioxidant status (TAS), total oxidant status (TOS), oxidative stress index (OSI), and concentrations of coenzyme Q10 (CoQ10) and vitamins A and E were estimated in the plasma of 22 patients with AT, 12 children with NBS, and the healthy controls. In AT patients, TAS (median 261.7 μmol/L) was statistically lower but TOS (496.8 μmol/L) was significantly elevated in comparison with the healthy group (312.7 μmol/L and 311.2 μmol/L, resp.). Tocopherol (0.8 μg/mL) and CoQ10 (0.1 μg/mL) were reduced in AT patients versus control (1.4 μg/mL and 0.3 μg/mL, resp.). NBS patients also displayed statistically lower TAS levels (290.3 μmol/L), while TOS (404.8 μmol/L) was comparable to the controls. We found that in NBS patients retinol concentration (0.1 μg/mL) was highly elevated and CoQ10 (0.1 μg/mL) was significantly lower in comparison with those in the healthy group. Our study confirms disturbances in redox homeostasis in AT and NBS patients and indicates a need for diagnosing oxidative stress in those cases as a potential disease biomarker. Decreased CoQ10 concentration found in NBS and AT indicates a need for possible supplementation.
- Intense pulsed light is effective in treating postburn hyperpigmentation and telangiectasia in Chinese patients. [Journal Article]
- JCJ Cosmet Laser Ther 2018 Feb 16; :1-6
- Intense pulsed light (IPL) has been used to treat postinflammatory hyperpigmentation and telangiectasia in Fitzpatrick type I -II skin. However, its therapeutic effects after superficial second-degre...
Intense pulsed light (IPL) has been used to treat postinflammatory hyperpigmentation and telangiectasia in Fitzpatrick type I -II skin. However, its therapeutic effects after superficial second-degree burns in Asian populations with Fitzpatrick type III-IV skin are uncertain. Thirty-five Han Chinese patients with facial or hand hyperpigmentation and telangiectasia due to second-degree fire burns received treatment with IPL. Each patient underwent 2-6 treatments over 3-5 weeks. The laser wavelength was 560-615 nm. Skin pigmentation was evaluated by two plastic surgeons as well as by the patients themselves (self-evaluation) before treatment at the end of the treatment cycle and 1 year after the first treatment. Blood flow in telangiectasia skin was measured by laser Doppler flow. The results showed that IPL significantly lessened hyperpigmentation so that close to normal skin color was achieved after the treatment cycles, and pigmentation did not reoccur 1 year after the first treatment. Approximately 82.9% of the patients were satisfied with their treatment outcomes. There were no post-treatment complications. Doppler showed a significant decreased blood flow in telangiectasia after treatment. In conclusion, IPL is an effective and safe modality for Chinese patients with hyperpigmentation and telangiectasia after fire burns.
- Pazopanib effective for bevacizumab-unresponsive epistaxis in hereditary hemorrhagic telangiectasia. [Case Reports]
- LLaryngoscope 2018 Feb 16
- Hereditary hemorrhagic telangiectasia (HHT) most commonly manifests with nasal mucosal telangiectasias, and vascular endothelial growth factor (VEGF) plays a significant role in this angiodysplasia. ...
Hereditary hemorrhagic telangiectasia (HHT) most commonly manifests with nasal mucosal telangiectasias, and vascular endothelial growth factor (VEGF) plays a significant role in this angiodysplasia. We describe a patient with HHT with epistaxis recalcitrant to several endonasal procedures and six cycles of intravenous bevacizumab, for which he was dependent on iron infusions and packed red blood cells transfusions. He then started pazopanib at 100 mg with dramatic improvements in epistaxis and normalization of hemoglobin and iron levels, without replenishment needs for 12 months. This is the first report on the efficacy of pazopanib with high selectivity for abrogating VEGF receptor-2 signaling in HHT, and needs to be explored further. Laryngoscope, 2018.
- ALK1 (Activin-Receptor Like Kinase 1) Loss Results in Vascular Hyperplasia in Mice and Humans Through PI3K (Phosphatidylinositol 3-Kinase) Activation. [Journal Article]
- ATArterioscler Thromb Vasc Biol 2018 Feb 15
- CONCLUSIONS: Overall, our results indicate that the BMP9/ALK1 hub critically mediates vascular quiescence by limiting PI3K signaling and suggest that PI3K inhibitors could be used as novel therapeutic agents to treat hereditary hemorrhagic telangiectasia.
- Heart involvement in systemic sclerosis. [Journal Article]
- TMTunis Med 2017; 95(3):215-220
- Primary and secondary heart involvement in systemic sclerosis are important mortality predictors. Aim of this study was to investigate by standard echocardiography associated to pulsed-tissue Doppler...
Primary and secondary heart involvement in systemic sclerosis are important mortality predictors. Aim of this study was to investigate by standard echocardiography associated to pulsed-tissue Doppler imaging, six-minute walk test (6MWT) and BNP level cardiac manifestation in 30 patients with ScS and to establish a strategy to detect and to evaluate this complication.
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- Lenalidomide as a novel therapy for gastrointestinal angiodysplasia in von Willebrand disease. [Journal Article]
- HHaemophilia 2018 Feb 15
- CONCLUSIONS: This case series demonstrates significantly reduced number of endoscopies and increased bleed-free duration with lenalidomide treatment in selected patients with VWD and recurrent GIB from AD. Prospective multicenter trials are needed to further define the role of lenalidomide in the management of GIB from angiodysplasia and VWD.