Asunto(s)
Artritis/genética , Artritis/patología , Exantema/etiología , Sinovitis/genética , Sinovitis/patología , Uveítis/genética , Uveítis/patología , Enfermedad Aguda , Adalimumab/administración & dosificación , Adalimumab/uso terapéutico , Antiinflamatorios/administración & dosificación , Antiinflamatorios/uso terapéutico , Artritis/diagnóstico , Artritis/tratamiento farmacológico , Diagnóstico Diferencial , Exantema/diagnóstico , Femenino , Humanos , Lactante , Proteína Adaptadora de Señalización NOD2/genética , Sarcoidosis , Sinovitis/tratamiento farmacológico , Resultado del Tratamiento , Uveítis/diagnóstico , Uveítis/tratamiento farmacológicoRESUMEN
INTRODUCTION: Prompt detection of childhood uveitis is key to minimising negative impact. From an internationally unique inception cohort, we report pathways to disease detection.UNICORNS is a national childhood non-infectious uveitis study with longitudinal collection of a standardised clinical dataset and patient reported outcomes. Descriptive analysis of baseline characteristics are reported.Amongst 150 recruited children (51% female, 31% non-white ethnicity) age at detection ranged from 2-18yrs (median 10). In 69%, uveitis was diagnosed following onset of symptoms: time from first symptoms to uveitis detection ranged from 0-739days (median 7days), with longer time to detection for those presenting initially to their general practitioner. Non symptomatic children were detected through JIA/other disease surveillance (16%), routine optometry review (5%) or child visual health screening (1%). Commonest underlying diagnoses at uveitis detection were JIA (17%), TINU (9%, higher than pre-pandemic reported UK disease frequency) and sarcoid (1%). 60% had no known systemic disease at uveitis detection. At disease detection, in at least one eye: 34% had structural complications (associated with greater time to detection - 17 days versus 4 days for uncomplicated presentation).The larger relative proportions of children with non-JIA uveitis reported here increase the importance of improving awareness of childhood uveitis amongst the wider clinical communities. There is scope for improvement of pathways to detection. Forthcoming analysis on the full cohort (251 recruited to date across 33 hospitals and 4 nations) will provide nationally representative data on management and the determinants of visual and broader developmental/well-being outcomes.
Asunto(s)
Artritis Juvenil , Uveítis , Niño , Humanos , Femenino , Preescolar , Adolescente , Masculino , Estudios de Cohortes , Artritis Juvenil/complicaciones , Uveítis/diagnóstico , Reino Unido/epidemiologíaRESUMEN
Reactive astrocytes adjacent to a forebrain stab injury were selectively ablated in adult mice expressing HSV-TK from the Gfap promoter by treatment with ganciclovir. Injured tissue that was depleted of GFAP-positive astrocytes exhibited (1) a prolonged 25-fold increase in infiltration of CD45-positive leukocytes, including ultrastructurally identified monocytes, macrophages, neutrophils, and lymphocytes, (2) failure of blood-brain barrier (BBB) repair, (3) substantial neuronal degeneration that could be attenuated by chronic glutamate receptor blockade, and (4) a pronounced increase in local neurite outgrowth. These findings show that genetic targeting can be used to ablate scar-forming astrocytes and demonstrate roles for astrocytes in regulating leukocyte trafficking, repairing the BBB, protecting neurons, and restricting nerve fiber growth after injury in the adult central nervous system.
Asunto(s)
Astrocitos/patología , Lesiones Encefálicas/patología , Movimiento Celular , Leucocitos/patología , Degeneración Nerviosa/patología , Neuritas/patología , Heridas Punzantes/patología , Animales , Astrocitos/metabolismo , Barrera Hematoencefálica , Recuento de Células , Femenino , Ganciclovir/farmacología , Regulación de la Expresión Génica , Proteína Ácida Fibrilar de la Glía/biosíntesis , Proteína Ácida Fibrilar de la Glía/genética , Hipocampo/patología , Histocitoquímica , Leucocitos/metabolismo , Ratones , Ratones Transgénicos , Neuritas/metabolismo , Neuronas/metabolismo , Neuronas/patología , Simplexvirus/enzimología , Simplexvirus/genética , Timidina Quinasa/biosíntesis , Timidina Quinasa/genéticaRESUMEN
We report the case history of a 41-year-old patient who, following bronchial infection, presented with progressive ophthalmoplegia in both eyes, dilated pupils unresponsive to light or convergence and ataxia. The suspected diagnosis of Miller-Fisher syndrome was confirmed by the detection of anti-ganglioside GQ1b antibodies.
Asunto(s)
Síndrome de Miller Fisher/diagnóstico , Midriasis/diagnóstico , Oftalmoplejía/diagnóstico , Adulto , Diagnóstico Diferencial , Humanos , MasculinoRESUMEN
PURPOSE: To establish how good we are as clinicians at advising glaucoma patients with bilateral visual field defects of their legal responsibility to inform the Driver and Vehicle Licensing Agency (DVLA). By using a sticker placed in the patients' notes to highlight driving status and visual fields, we sought to improve our success in providing and documenting this advice. METHODS: We interviewed and examined the notes of two groups of 100 consecutive glaucoma patients before and after the introduction of a 'driver sticker' placed into patients' notes at the time of visual field testing. We examined the documentation of driving status, and the provision and documentation of advice regarding the DVLA. RESULTS: In the first audit, we found only 9% of patients had driving status documented. Only 20% of drivers with bilateral field defects were advised to inform the DVLA with 11.4% documentation of this advice. After the introduction of the sticker, we succeeded in improving the documentation of driving status to 99%. We advised and documented the advice to inform the DVLA in 97% of drivers with bilateral field defects. CONCLUSIONS: We found that as a unit we were poor at documenting driving status and advising glaucoma patients with bilateral field defects to inform the DVLA. By the simple measure of introducing a sticker into patients' notes, we were able to highlight this critical group and improve our provision and documentation of appropriate advice regarding informing the DVLA.