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https://www.readbyqxmd.com/read/29674168/spikes-might-precede-seizures-and-predict-epilepsy-in-children-with-sturge-weber-syndrome-a-pilot-study
#1
Claire Bar, Anna Kaminska, Rima Nabbout
PURPOSE: Sturge-Weber syndrome (SWS) is a neurocutaneous disorder characterized by a facial port-wine stain, a glaucoma, and a leptomeningeal angioma. Epilepsy occurs in more than 75% of affected children, and seizures occurring in the first year of life are associated with a poor neurological prognosis. The aim of this study was to identify possible predictive markers of epilepsy on electroencephalogram (EEG) performed prior to seizure onset in children with SWS. METHODS: This study included children with a diagnosis of SWS who had an EEG performed prior to seizure onset...
March 28, 2018: Epilepsy Research
https://www.readbyqxmd.com/read/29607552/pathophysiology-and-management-of-glaucoma-associated-with-phakomatoses
#2
REVIEW
Alisa T Thavikulwat, Deepak P Edward, Abdulrahman AlDarrab, Thasarat S Vajaranant
The phakomatoses, encephalotrigeminal angiomatosis (ETA; Sturge-Weber Syndrome), neurofibromatosis type 1 (NF1 or von Recklinghausen disease), Von Hippel-Lindau (VHL) disease, tuberous sclerosis (TSC), oculodermal melanocytosis (ODM), and phakomatosis pigmentovascularis are a group of neurocutaneous disorders that have characteristic systemic and ocular manifestations. Through many different mechanisms, they may cause glaucomatous damage of the optic nerve and subsequent vision loss varying from mild to severe...
April 1, 2018: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/29583063/double-pass-595-nm-pulsed-dye-laser-does-not-enhance-the-efficacy-of-port-wine-stains-compared-with-single-pass-a-randomized-comparison-with-histological-examination
#3
Wenxin Yu, Jiafang Zhu, Lizhen Wang, Yajing Qiu, Yijie Chen, Xi Yang, Lei Chang, Gang Ma, Xiaoxi Lin
OBJECTIVE: To compare the efficacy and safety of double-pass pulsed dye laser (DWL) and single-pass PDL (SWL) in treating virgin port wine stain (PWS). BACKGROUND: The increase in the extent of vascular damage attributed to the use of double-pass techniques for PWS remains inconclusive. A prospective, side-by-side comparison with a histological study for virgin PWS is still lacking. MATERIALS AND METHODS: Twenty-one patients (11 flat PWS, 10 hypertrophic PWS) with untreated PWS underwent 3 treatments at 2-month intervals...
March 27, 2018: Photomedicine and Laser Surgery
https://www.readbyqxmd.com/read/29546617/intravenous-injection-of-artificial-red-cells-and-subsequent-dye-laser-irradiation-causes-deep-vessel-impairment-in-an-animal-model-of-port-wine-stain
#4
Naoaki Rikihisa, Mai Tominaga, Shoji Watanabe, Nobuyuki Mitsukawa, Yoshiaki Saito, Hiromi Sakai
Our previous study proposed using artificial blood cells (hemoglobin vesicles, Hb-Vs) as photosensitizers in dye laser treatment for port-wine stains (PWSs). Dye laser photons are absorbed by red blood cells (RBCs) and hemoglobin (Hb) mixture, which potentially produce more heat and photocoagulation and effectively destroy endothelial cells. Hb-Vs combination therapy will improve clinical outcomes of dye laser treatment for PWSs because very small vessels do not contain sufficient RBCs and they are poor absorbers/heaters of lasers...
March 15, 2018: Lasers in Medical Science
https://www.readbyqxmd.com/read/29542044/multiple-laser-pulses-in-conjunction-with-an-optical-clearing-agent-to-improve-the-curative-effect-of-cutaneous-vascular-lesions
#5
Jun Ma, Bin Chen, Dong Li, Yue Zhang, Zhaoxia Ying
Port-wine stain (PWS) birthmark is a congenital microvascular malformation of the skin. A 1064-nm Nd:YAG laser can achieve a deeper treatment, but the weak absorption by blood limits its clinical application. Multiple laser pulses (MLPs) are a potential solution to enhance the curative effect of a Nd:YAG laser. To reduce the pulse number (pn ) required for the thermal destruction of the blood vessel, the effect of glucose in conjunction with MLP was investigated. In vivo experiments were performed on a dorsal skin chamber model...
March 14, 2018: Lasers in Medical Science
https://www.readbyqxmd.com/read/29541691/acute-primary-angle-closure-in-sturge-weber-syndrome
#6
Wei-Wen Su
Purpose: Sturge-Weber syndrome (SWS) is a neurocutaneous disorder characterized by facial cutaneous venous dilatation (port-wine stain), leptomeningeal angioma, and ocular abnormalities. Here we report a case of SWS who experienced acute primary angle-closure in the same side of the nevus flammeus. Observations: A 64-years-old female patient with SWS port wine stain on the left side of her face was referred to our ER for acute primary angle-closure (PAC). The IOP was 64 mmHg in the left eye (12 mmHg in OD) and the visual acuity was 20/100...
June 2018: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29533491/a-dose-finding-study-for-hemoporfin-in-photodynamic-therapy-for-port-wine-stain-a-multicenter-randomized-double-blind-phase-iib-trial
#7
Qiuju Wu, Ping Tu, Guoyu Zhou, Huilan Yang, Zhanchao Zhou, Yi Zhao, Qingshan Zheng, Ying Gu, Jining Tao, Xuejun Zhu
BACKGROUND/PURPOSE: Previous studies have shown that hemoporfin-mediated photodynamic therapy (PDT) was a treatment for port-wine stain (PWS). Our current study aimed to identify optimal hemoporfin dose. METHODS: A prospective, multicenter, double-blind, randomized clinical trial was conducted. Patients were assigned into low or high dose hemoporfin (2.5 mg/kg and 5 mg/kg intravenously, respectively), or control (placebo) group, at a rate of 2: 2: 1. Treatment efficacy was evaluated at week 8...
March 13, 2018: Photodermatology, Photoimmunology & Photomedicine
https://www.readbyqxmd.com/read/29518883/-research-progress-of-sturge-weber-syndrome-induced-glaucoma
#8
Y Wu, W Y Guo
Sturge-Weber Syndrome is a sporadic rare disease. Its characteristic feature of Port-wine Stain in patient's face has been an important indicator for screening secondary glaucoma, which further provides patients with possibility of early diagnosis and treatment of Sturge-Weber Syndrome induced glaucoma. This paper focuses on the pathogenesis, risk factors and treatment of Sturge-Weber Syndrome secondary glaucoma. (Chin J Ophthalmol, 2018, 54: 229-233) .
March 11, 2018: [Zhonghua Yan Ke za Zhi] Chinese Journal of Ophthalmology
https://www.readbyqxmd.com/read/29476210/a-child-with-apert-syndrome-and-sturge-weber-syndrome-could-fibronectin-or-the-ras-mapk-signaling-pathway-be-the-connection
#9
Ai Peng Tan, Wui Khean Chong
BACKGROUND: Apert syndrome is one of the most common craniosynostosis syndrome caused by mutation in genes encoding fibroblast growth factor receptor 2 (FGFR2). Craniosynostosis, midfacial hypoplasia, and syndactyly/symphalangism are features of this syndrome. Sturge-Weber syndrome (SWS) on the other hand is a congenital neurocutaneous disorder characterized by facial port-wine stains (PWSs) and leptomeningeal vascular capillary malformations. In 2013, the causative mutation underlying SWS (p...
February 23, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29449213/inflammatory-morphea-presenting-as-a-hemifacial-acquired-port-wine-stain
#10
Andrea Bassi, Paola Piccini, Cesare Filippeschi, Teresa Oranges, Teresa Giani, Iria Neri, Rolando Cimaz, Maurizio de Martino
No abstract text is available yet for this article.
February 15, 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29406329/no-braf-v600e-mutation-identified-in-28-periocular-pyogenic-granuloma
#11
Guosheng Li, Emily Adams, James R Eshleman, Charles G Eberhart
PURPOSE: BRAF V600E mutations were recently identified in some pyogenic granulomas of skin, particularly lesions arising in patients with port wine stains. In this study, the authors analyzed BRAF gene status in 28 periocular pyogenic granulomas to determine its role in their pathogenesis. METHODS: Retrospective review of ophthalmic pathology archives from 2007 to 2016 was used to identify pyogenic granulomas of the conjunctiva, eyelid, and orbit. Three eyelid nevi were also analyzed as positive controls...
February 5, 2018: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/29399306/topical-halometasone-reduces-acute-adverse-effects-induced-by-pulsed-dye-laser-for-treatment-of-port-wine-stain-birthmarks
#12
Lin Gao, Linhan Qian, Li Wang, Kai Li, Rong Yin, Yanting Wang, Hanmei Kang, Wenting Song, Gang Wang
Introduction: Pulsed dye laser (PDL) for treatment of port wine stain (PWS) usually causes some acute adverse effects, including pain, erythema, scabbing and swelling. This study aimed to determine whether topical halometasone can be used to reduce these acute adverse effects for post-PDL care of patients. Methods: A total of 40 PWS subjects were enrolled in this study and randomly assigned into two regimens: PDL alone and PDL + halometasone. All subjects were given a single treatment of PDL with wavelength of 595 nm, fluence of 8...
2018: Journal of Lasers in Medical Sciences
https://www.readbyqxmd.com/read/29390578/analysis-of-quality-of-life-and-influencing-factors-in-197-chinese-patients-with-port-wine-stains
#13
Juan Wang, Yu-You Zhu, Zhong-Ying Wang, Xiu-Hua Yao, Lan-Fang Zhang, Hong Lv, Si-Ping Zhang, Bai Hu
Port-wine stains (PWS) are congenital capillary malformations, usually occurring on the face, neck, and other exposed parts of the skin, that have serious psychological and social impact on the patient. Most researchers focus on the treatment of PWS, but the quality of life (QoL) of PWS patients is seldom researched. The objective of this study is to evaluate the QoL of patients with PWS on exposed parts and explore the factors influencing the QoL of PWS patients. The QoL of 197 cases with PWS on exposed parts were prospectively studied using the Dermatology Life Quality Index questionnaire (DLQI), and the factors influencing the patients' QoL were analyzed by single-factor analysis and multiple-factor logistic regression analysis...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29384394/prospective-analysis-of-the-port-wine-stain-patient-population-in-the-netherlands-in-light-of-novel-treatment-modalities
#14
M Ingmar van Raath, Caroline A Bambach, Lea M Dijksman, Albert Wolkerstorfer, Michal Heger
BACKGROUND: Pulsed dye laser is the gold standard for port-wine stain (PWS) treatment. However, pulsed dye lasers achieve suboptimal clinical results in a majority of patients. Patient demand for novel therapies and willingness to participate in clinical studies is currently unknown, yet, imperative for steering R&D activity. The objective of this study was to evaluate these two factors in relation to PWS patient demographics. METHODS: A questionnaire was used to assess patient and PWS characteristics, treatment history, efficacy, and satisfaction, stress level, willingness to travel and pay for an effective treatment, participation in clinical studies, and amenability to intravenous drug administration...
January 31, 2018: Journal of Cosmetic and Laser Therapy: Official Publication of the European Society for Laser Dermatology
https://www.readbyqxmd.com/read/29381541/a-port-wine-stain-treated-with-pulsed-dye-laser-a-complication-from-contaminated-makeup
#15
Kathleen M Nemer, Eva A Hurst
No abstract text is available yet for this article.
January 29, 2018: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
https://www.readbyqxmd.com/read/29356033/gold-nanospheres-enhanced-photothermal-therapy-in-a-rat-model
#16
Linzhuang Xing, Bin Chen, Dong Li, Wenjuan Wu, Zhaoxia Ying
BACKGROUND AND OBJECTIVE: Efficient photothermal conversion of gold nanoparticles with strong light absorption suggests their wide use as selective photothermal agents in biomedical fields. The aim of this study is to investigate the use of gold nanospheres (GNPs) as exogenous visible light absorbers to improve laser treatment of port-wine stains. MATERIALS AND METHODS: Thiol-terminated methoxypolyethylene glycol modified GNPs (PEG-GNPs) with peak extinction matching the visible light wavelength of the laser being used were synthesized...
January 22, 2018: Lasers in Surgery and Medicine
https://www.readbyqxmd.com/read/29313918/b1-and-b2-a-role-for-ephrin-signalling-in-port-wine-stain
#17
M Fear
No abstract text is available yet for this article.
December 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/29247433/emla-cream-does-not-influence-efficacy-and-pain-reduction-during-pulsed-dye-laser-treatment-of-port-wine-stain-a-prospective-side-by-side-comparison
#18
Wenxin Yu, Tianyou Wang, Jiafang Zhu, Yajing Qiu, Hui Chen, Yunbo Jin, Xi Yang, Xiaojie Hu, Lei Chang, Yijie Chen, Gang Ma, Xiaoxi Lin
EMLA cream was developed to reduce pain during pulsed-dye laser (PDL) treatment; however, no standard assessment for the therapeutic outcomes of PDL with EMLA creams thus far available. This comparative, prospective clinical trial evaluates laser efficacy and pain reduction during PDL treatment with EMLA cream for local topical anesthesia. Nineteen patients with untreated port-wine stain (PWS) were treated using PDL and examined in this study. Treatment specifications included Vbeam® PDL (Candela Corp.), 595-nm wavelength, 9 J/cm2 radiant exposure, 0...
April 2018: Lasers in Medical Science
https://www.readbyqxmd.com/read/29237924/glaucoma-in-patients-with-eyes-close-to-areas-affected-by-port-wine-stain-has-lateral-and-gender-predilection
#19
Yue Wu, Ru-Jing Yu, Di Chen, Li Xu, Mao Li, Li Zhu, Chun-Yu Guo, Wen-Yi Guo
BACKGROUND: The location of facial port-wine stain (PWS) may be helpful for predicting some associated anomalies; high glaucoma incidence is found in patients with eyes close to PWS-affected areas (V1, ophthalmic branch area of the trigeminal nerve). This study aimed to investigate the characteristics of glaucoma in V1-affected PWS. METHODS: A total of 569 patients with V1 area-affected PWS were reviewed in the study. The large series was based on the referral system between the Department of Plastic and Reconstructive Surgery and the Department of Ophthalmology...
December 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/29217063/capillary-malformations-portwine-stains-of-the-head-and-neck-natural-history-investigations-laser-and-surgical-management
#20
REVIEW
Jeong Woo Lee, Ho Yun Chung
Capillary malformations (CMs), also known as port-wine stains, are the most common type of congenital vascular malformations. Facial CM often occurs with a quasidermatomal distribution according to the sensory trigeminal nerve distribution. With time, these lesions darken progressively, and soft tissue hypertrophy, bony hypertrophy, and/or nodule formation can develop. Multiple treatments for CM have been reported. However, the mainstay and gold standard therapy for facial or aesthetically sensitive CM is still the pulsed dye laser treatment...
February 2018: Otolaryngologic Clinics of North America
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