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Schwannomas, also known as neurilemomas, are peripheral nerve sheath neoplasms. They can be sporadic or associated with genetic syndromes including neurofibromatosis type 2 (NF2). Schwannomas may lead to symptoms by exerting pressure on nearby structures, such as nerve and muscle fibers. In this study, we present the case of a 22-year-old female with a history of NF2 who, upon examination, presented with a visibly enlarged salmon-colored mass involving the left inferior rectus that she had since the age of 12 years. Ocular examinations revealed a small left hypertropia and exotropia in all gazes. Magnetic resonance imaging confirmed bilateral involvement of the inferior rectus muscles. She had a partial excisional biopsy of the mass involving the left inferior rectus muscle that confirmed the presence of schwannoma. This case highlights the importance of comprehensive evaluation of sensory and motor functions as well as considering orbital schwannomas in cases of strabismus, especially within the context of neurofibromatosis.
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Various gratings with 700 nm feature spacings are patterned on the reverse side of organic solar cell active layers to increase the path length and constrain light to the cell through total internal reflection. The absorption enhancement is studied for 15, 40, and 120 nm active layers. We were able to confine 9% of the incident light over the wavelength range of 400-650 nm, with thinner gratings having a greater enhancement potential. The measurement setup utilizing an integrating sphere to fully characterize scattered or diffracted light is also fully described.
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This study describes the clinical characteristics of a cohort of Hispanic patients living in Puerto Rico who were found to have West Indian punctate keratopathy (WIPK). This case also introduces the term "PR (Puerto Rican) spots" to describe the dots observed in the eyes of individuals with WIPK who have a documented history of residing in Puerto Rico. The methods of the study consist of a retrospective chart review of patients presenting with WIPK. The patient data were entered retrospectively into a new database and analyzed. Eighteen patients who had WIPK were identified. The median age at presentation was 60.5 years (range: 49-72); 61.1% were female. At presentation, only one patient had both eyes affected. The median number of PR spots on examination was 1 (range: 1-4). All the patients had a history of ocular disease, most frequently glaucoma (55.5%), and had lived in Puerto Rico for more than 40 years. A total of 33.3% of the patients were retired or unemployed at the time of their presentation. While the origin of these dots remains unclear, ongoing efforts to document and characterize WIPK and PR spots will persist, with the aim of enhancing our understanding of this clinical entity.
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We report on a case of West Indian (Caribbean) punctate keratopathy (WIPK) in a pediatric patient living in Puerto Rico, USA. A 9-year-old Hispanic girl presented for a routine ophthalmic follow-up. The patient had a history of juvenile idiopathic arthritis and chronic bilateral anterior uveitis. At the presentation, her visual acuity was 20/30 in the right eye and 20/20 in the left eye. An examination of the right eye was remarkable for one round, white subepithelial corneal opacity of approximately 0.1 mm in height by 0.1 mm in width, located slightly lateral to the center of the cornea, which was consistent with WIPK. This case highlights the importance of recognizing WIPK in children who have a history of living on any one or more of the Caribbean islands.
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We report on the case of a Hispanic male with bilateral acute retinal necrosis (BARN), whose disease was managed with intravenous acyclovir, topical prednisolone, topical atropine, intravitreal ganciclovir, oral valacyclovir, and oral prednisone. The oral corticosteroid was added to his treatment regimen eight days after his initial presentation. The 55-year-old patient presented with a two-week history of bilateral blurred vision. His medical history was pertinent for remote varicella and herpes zoster (the latter limited to the forehead). His best-corrected visual acuity (BCVA) was counting fingers in both eyes (OU). His examination was remarkable in OU for grade 3+ cells, pharmacologically dilated pupils, and grade 2+ vitreous cells. The patient's fundus was remarkable in OU for optic-nerve swelling, vitreous condensation, ghost vessels, and retinitis patches. Given the clinical and ancillary testing results, an assessment of BARN was made. The patient received acyclovir treatment initially, and systemic steroids were introduced eight days later. He ultimately experienced significant clinical improvement.
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This study describes a cohort of patients presenting with histocompatibility leukocyte antigen (HLA)-A29-associated retinal vasculitis without choroidal lesions that may share clinical features with birdshot retinochoroiditis. The methods include a retrospective chart review of patients presenting with HLA-A29-associated retinal vasculitis without choroidal lesions. The data on the patients were entered retrospectively into a new database and analyzed. Four patients who had HLA-A29-associated retinal vasculitis without choroidal lesions were identified. The median age at presentation was 40 years (range: 14-71); 75% were female. At presentation, all four patients had a visual acuity of 20/50 or better in both eyes. All the eyes had mild vitritis, three eyes (37.5%) had cystoid macular edema, and two eyes (25%) had optic disc edema. All the patients required treatment with systemic steroids and immunosuppressive therapy. HLA-A29-associated retinal vasculitis without choroidal lesions appears to share many clinical features with birdshot chorioretinitis, including the need for systemic immunosuppressive therapy. Whether this entity represents an early form of birdshot retinochoroiditis or a more localized variant of the disease is a topic for additional studies.
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The article addresses some forms of sexual violence suffered by Latin American women during migratory transit through Mexico. In-depth, semi-structured, qualitative-phenomenological interviews were conducted with 10 women in different stages of major transmigration states. The findings demonstrate the ubiquity of sexual violence during the migration. Five themes were identified: (a) The migration dimension of "being a woman"; (b) Recognition of contextual factors associated with the migratory process: "Intermediaries, road, shelters and sisterhood"; (c) The costs of migration: Abuse, discrimination, and persecution: "Criminal groups, physical violence, and "La Bestia"; (d) Triggers and supports: "Escape and future expectations"; and (e) God as a support.
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Estupro , Delitos Sexuais , Feminino , Humanos , América Latina , Masculino , México , Pesquisa QualitativaRESUMO
This article contains data of the horizontal hydraulic conductivity of five different fine-grained soils of the former Lake Texcoco. The data were back-calculated from excess pore pressure dissipation measurements collected at 119 locations using piezocone tests (CPTu). The test campaign was part of the geotechnical survey performed for the construction of the New Mexico City International Airport (NAICM). Descriptive statistical parameters of each soil unit are presented and lognormal probability distributions are fitted to describe the natural variability of the horizontal hydraulic conductivity of this site.
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BACKGROUND: Kids B-LONG was a multicentre, open-label, phase 3 study assessing the safety, efficacy, and pharmacokinetics of recombinant factor IX Fc fusion protein (rFIXFc) in previously treated paediatric patients younger than 12 years with severe haemophilia B. METHODS: The study enrolled 30 previously treated boys younger than 12 years with haemophilia B (≤2 IU/dL [≤2%] endogenous coagulation factor IX [FIX] activity). All patients were initially given rFIXFc prophylaxis (50-60 IU/kg) once per week with adjustments to dose (≤100 IU/kg per infusion) or dosing frequency (up to two times per week) as needed. The primary outcome measure was development of inhibitors (neutralising antibodies). Secondary outcomes were pharmacokinetics, annual bleeding rate (ABR), spontaneous joint ABR, the number of infusions and dose required to resolve a bleed, time from last infusion of rFIXFc to a bleeding episode, assessment of response to treatment, and total annualised rFIXFc consumption for prevention and treatment of bleeding episodes. All patients underwent sequential pharmacokinetic evaluations of their prestudy FIX and rFIXFc. The completed trial is registered with ClinicalTrials.gov, number NCT01440946. FINDINGS: No patients developed inhibitors to rFIXFc; in the 30 enrolled patients the most common adverse events were nasopharyngitis (n=7; 23%) and fall (n=6; 20%); four patients (13%) had serious adverse events. Overall, rFIXFc exhibited a prolonged half-life of 68·6 h (95% CI 61·8-76·0), reduced clearance, and similar recovery compared with prestudy FIX. The median ABR was 2·0 (0·0-3·1) overall and 0·0 (0·0-0·0) for spontaneous joint bleeds; ten (33%) of 30 patients reported no bleeding, and 19 (63%) reported no joint bleeding on-study. The median average prophylactic dose of rFIXFc was 58·6 IU/kg (IQR 52·3-64·8) per week. Throughout the study, 29 (97%) of 30 patients remained on once per week infusions. INTERPRETATION: Weekly infusions of rFIXFc were well tolerated and resulted in low bleeding rates in children with severe haemophilia B. FUNDING: Biogen, Sobi.
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Fator IX/uso terapêutico , Hemofilia B/tratamento farmacológico , Fragmentos Fc das Imunoglobulinas/uso terapêutico , Proteínas Recombinantes de Fusão/uso terapêutico , Criança , Pré-Escolar , Hemartrose , Hemorragia , Humanos , Masculino , Resultado do TratamentoRESUMO
The safety, efficacy, and prolonged half-life of recombinant factor IX Fc fusion protein (rFIXFc) were demonstrated in the Phase 3 B-LONG (adults/adolescents ≥12 years) and Kids B-LONG (children <12 years) studies of subjects with haemophilia B (≤2 IU/dl). Here, we report interim, long-term safety and efficacy data from B-YOND, the rFIXFc extension study. Eligible subjects who completed B-LONG or Kids B-LONG could enrol in B-YOND. There were four treatment groups: weekly prophylaxis (20-100 IU/kg every 7 days), individualised prophylaxis (100 IU/kg every 8-16 days), modified prophylaxis (further dosing personalisation to optimise prophylaxis), and episodic (on-demand) treatment. Subjects could change treatment groups at any point. Primary endpoint was inhibitor development. One hundred sixteen subjects enrolled in B-YOND. From the start of the parent studies to the B-YOND interim data cut, median duration of rFIXFc treatment was 39.5 months and 21.9 months among adults/adolescents and children, respectively; 68/93 (73.1 %) adults/adolescents and 9/23 (39.1 %) children had ≥100 cumulative rFIXFc exposure days. No inhibitors were observed. Median annualised bleeding rates (ABRs) were low in all prophylaxis regimens: weekly (≥12 years: 2.3; <6 years: 0.0; 6 to <12 years: 2.7), individualised (≥12 years: 2.3; 6 to <12 years: 2.4), and modified (≥12 years: 2.4). One or two infusions were sufficient to control 97 % (adults/adolescents) and 95 % (children) of bleeding episodes. Interim data from B-YOND are consistent with data from B-LONG and Kids B-LONG, and confirm the long-term safety of rFIXFc, absence of inhibitors, and maintenance of low ABRs with prophylactic dosing every 1 to 2 weeks.
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Coagulantes/administração & dosagem , Fator IX/administração & dosagem , Hemofilia B/tratamento farmacológico , Hemorragia/prevenção & controle , Hemostasia/efeitos dos fármacos , Fragmentos Fc das Imunoglobulinas/administração & dosagem , Proteínas Recombinantes de Fusão/administração & dosagem , Adolescente , Adulto , Anticorpos Neutralizantes/sangue , Criança , Coagulantes/efeitos adversos , Esquema de Medicação , Fator IX/efeitos adversos , Fator IX/imunologia , Hemofilia B/sangue , Hemofilia B/diagnóstico , Hemorragia/sangue , Humanos , Fragmentos Fc das Imunoglobulinas/efeitos adversos , Fragmentos Fc das Imunoglobulinas/imunologia , Masculino , Pessoa de Meia-Idade , Proteínas Recombinantes de Fusão/efeitos adversos , Proteínas Recombinantes de Fusão/imunologia , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
INTRODUCTION: The south area of Puerto Rico has a high density of a college student population. This population is exposed to a series of hypertension risk factors, for instance: they are exposed to the stress associated with their studies, they are the target of publicitary promotion of fast foods, alcoholic drinks, smoking, and they share with the rest of the Puertorican population predisposition to diabetes mellitus. We collected data about the risk factors for High Blood Pressure (HBP) to which this specific population was exposed. METHODS: This is an observational-descriptive, cross-sectional study with a data collection period from February to March 2004. SUBJECTS: students of a health science institution of the south area of Puerto Rico (N = 310). Sample availability (155). The Prediagnostic History of Francisco Fragachán (2002), High Blood Pressure Unit, questionaire Universitary Hospital, Central University of Venezuela was utilized. We also performed the questionnaire of Vulnerability Stress Test of Beech and Scheffield (adapted for a Hispanic community). The blood pressure was measured with an electronic calibrated Omrom 4M equipment. Measured anthropometric variables: Weight and height with a calibrated scale; Hip and waist circumference under the anatomical references of Siegell et al. RESULTS: Age Average: 25.4 +/- 3.19. Ninety three percent (93%) of the population had at least one risk factor for HBP. Of our population, 90% consider themselves vulnerable to stress at least in one of the items proposed. 45% of our subjects were overweight and 17% were obese. 94.8% of the study population had an adequate W/H ratio. Mean values for BP: 112/71; Pulse Presure: 41; Mean Blood Presure: 85,4; Heart Rate: 79,5. We found seven subjects with hypertension. CONCLUSION: In our study population the presence of risk factors (modifiable and non-modifiable) in an early stage of life (25.4 +/- 3.19 years) increases the possibility to develop hypertension.
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Hipertensão/epidemiologia , Adulto , Estudos Transversais , Feminino , Humanos , Masculino , Porto Rico/epidemiologia , Fatores de Risco , Estudantes , Adulto JovemRESUMO
Sodium appetite reflects the importance of sodium homeostasis. The sodium ion is one of the most important risk factors in the development of hypertension. Humans, for various reasons, seem to have a specific preference for salt which is consumed in excess of need and this has been characterized as an important contributor to hypertension. Salt intake is related to the salt taste sensitivity threshold and the salt good taste level. Gustatory sensibility responds to various physiological mechanisms and salt taste is directly modified by cultural and socio-economical factors. We measured the salt taste sensitivity threshold and salt good taste level of a young student population. Air popped popcorn sprayed with different Molar concentrations of salt where given to students to taste and a questionnaire to evaluate diet salt intake preferences. Both salt taste sensitivity threshold and salt good taste level graph patterns are different from each other. Salt taste sensitivity threshold has a bell shape distribution with different molar salt concentrations. The major tendency of the salt umbral sensitivity of our population was the 0.5 M concentration. Salt good taste level has an exponential shape distribution with different molar salt concentrations. The tendency for the good taste level of our population was 3 M. Smoking does not seem to modify the salt taste sensitivity thresholds or the salt good taste level graphs. Also, salt shaker use does not seem to modify salt taste sensitivity thresholds or salt good taste level graphs in our population. Salt taste sensitivity threshold is probably associated to morpho-physiological factors. Salt good taste level is mainly associated with the cultural environment. The majority of subjects have a tendency to prefer foods with higher concentrations of salt increasing the possibility of exposure to the salt intake risk factor.
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Cloreto de Sódio na Dieta , Limiar Gustativo , Paladar , Adulto , Estudos Transversais , Feminino , Humanos , Masculino , Porto Rico , Estudantes , Adulto JovemRESUMO
Zinc homeostasis is achieved after intake variation by changes in the expression levels of zinc transporters. The aim of this study was to evaluate dietary intake (by 24-h recall), absorption, plasma zinc (by absorption spectrophotometry) and the expression levels (by quantitative PCR), of the transporters ZIP1 (zinc importer) and ZnT1 (zinc exporter) in peripheral white blood cells from 24 adolescent girls before and after drinking zinc-fortified milk for 27 day. Zinc intake increased (p < 0.001) from 10.5 ± 3.9 mg/day to 17.6 ± 4.4 mg/day, and its estimated absorption from 3.1 ± 1.2 to 5.3 ± 1.3 mg/day. Mean plasma zinc concentration remained unchanged (p > 0.05) near 150 µg/dL, but increased by 31 µg/dL (p < 0.05) for 6/24 adolescents (group A) and decreased by 25 µg/dL (p < 0.05) for other 6/24 adolescents (group B). Expression of ZIP1 in blood leukocytes was reduced 1.4-fold (p < 0.006) in group A, while for the expression of ZnT1 there was no difference after intervention (p = 0.39). An increase of dietary zinc after 27-days consumption of fortified-milk did not increase (p > 0.05) the plasma level of adolescent girls but for 6/24 participants from group A in spite of the formerly appropriation, which cellular zinc uptake decreased as assessed by reduction of the expression of ZIP1.