RESUMEN
BACKGROUND: To analyze the clinical course and outcomes of autoimmune vs. non-autoimmune surgically induced scleral necrosis (SISN). METHODS: Multicentric, retrospective, comparative cohort study. Eighty-two eyes of 70 patients with SISN were classified according to pathogenic mechanism into autoimmune vs. non-autoimmune. Main outcome measures included necrosis onset, type of surgery, associated systemic disease, visual acuity, and treatment were analysed in patients followed for ≥ 6 months. RESULTS: Forty-six (65.7%) patients were women, and the median age was 66 (range: 24-90) years. Most patients (82.9%) had unilateral disease. The median time between surgery and SISN onset was 58 (1-480) months. Thirty-one (37.8%) eyes were classified as autoimmune, and 51 (62.2%) as non-autoimmune SISN. Autoimmune SISN was associated with a shorter time between the surgical procedure and SISN onset than non-autoimmune cases (median of 26 vs. 60 months, p = 0.024). Also, autoimmune SISN was associated with cataract extraction (93.5% vs. 25.5%, p < 0.001), severe scleral inflammation (58.1% vs. 17.6%, p < 0.001), and higher incidence of ocular complications (67.7% vs. 33.3%, p = 0.002) than non-autoimmune cases. Remission was achieved with medical management alone in 44 (86.3%) eyes from the non-autoimmune and in 27 (87.1%) from the autoimmune group (p = 0.916). Surgical management was required in 11 (13.4%) eyes, including two requiring enucleations due to scleral perforation and phthisis bulbi. CONCLUSIONS: Eyes with autoimmune SISN had a higher rate of cataract surgery, severe scleral inflammation, and ocular complications. Early SISN diagnosis and appropriate management, based on clinical features and pathogenic mechanisms, are critical to avoid sight-threatening complications.
RESUMEN
The onset of scleral necrosis after ocular surgery may have catastrophic ocular and systemic consequences. The two most frequent surgeries causing surgically-induced scleral necrosis (SISN) are pterygium excision and cataract extraction. Several pathogenic mechanisms are involved in surgically induced scleral necrosis. All of them are poorly understood. Ocular trauma increasing lytic action of collagenases with subsequent collagen degradation, vascular disruption leading to local ischemia, and immune complex deposition activating the complement system represents some of the events that lead to scleral necrosis. The complex cascade of events involving different pathogenic mechanisms and the patient's abnormal immune response frequently leads to delayed wound healing that predisposes the development of scleral necrosis. The management of SISN ranges from short-term systemic anti-inflammatory drugs to aggressive immunosuppressive therapy and surgical repair. Therefore, before performing any ocular surgery involving the sclera, a thorough ophthalmic and systemic evaluation must be done to identify high-risk patients that may develop SISN.
Asunto(s)
Pterigion , Escleritis , Humanos , Necrosis/complicaciones , Necrosis/patología , Esclerótica/cirugía , Escleritis/tratamiento farmacológico , Escleritis/etiología , Escleritis/patología , Trasplante Autólogo/efectos adversosRESUMEN
The 2015 Paediatric European Network for Treatment of AIDS (PENTA) guidelines provide practical recommendations on the management of HIV-1 infection in children in Europe and are an update to those published in 2009. Aims of treatment have progressed significantly over the last decade, moving far beyond limitation of short-term morbidity and mortality to optimizing health status for adult life and minimizing the impact of chronic HIV infection on immune system development and health in general. Additionally, there is a greater need for increased awareness and minimization of long-term drug toxicity. The main updates to the previous guidelines include: an increase in the number of indications for antiretroviral therapy (ART) at all ages (higher CD4 thresholds for consideration of ART initiation and additional clinical indications), revised guidance on first- and second-line ART recommendations, including more recently available drug classes, expanded guidance on management of coinfections (including tuberculosis, hepatitis B and hepatitis C) and additional emphasis on the needs of adolescents as they approach transition to adult services. There is a new section on the current ART 'pipeline' of drug development, a comprehensive summary table of currently recommended ART with dosing recommendations. Differences between PENTA and current US and World Health Organization guidelines are highlighted and explained
Asunto(s)
Humanos , Niño , Infecciones por VIH/diagnóstico , Infecciones por VIH/tratamiento farmacológico , Síndrome de Inmunodeficiencia Adquirida/diagnóstico , Síndrome de Inmunodeficiencia Adquirida/tratamiento farmacológico , Terapia Antirretroviral Altamente ActivaRESUMEN
Birdshot retinochoroidopathy (BSRC) is a distinct type of posterior uveitis originally described in the 1940s. Its characteristics include minimal anterior segment inflammation and diffuse posterior choroidopathy with vitritis and retinal vasculitis. The precise etiology of this disease is yet to be elucidated. However, various treatment modalities have been employed with the ultimate goal of durable remission of this vision threatening intraocular disease. The purpose of this review is not only to emphasize the importance of recognizing BSRC, but also to discuss the new discoveries, immune mediators, current and new therapies, and techniques applied to monitor and accomplish disease remission.
Asunto(s)
Coriorretinitis , Enfermedades de la Coroides , Enfermedades de la Retina , Anticuerpos Monoclonales Humanizados/uso terapéutico , Coriorretinitis/diagnóstico , Coriorretinitis/tratamiento farmacológico , Coriorretinitis/inmunología , Enfermedades de la Coroides/diagnóstico , Enfermedades de la Coroides/tratamiento farmacológico , Enfermedades de la Coroides/inmunología , Diagnóstico Diferencial , Quimioterapia Combinada , Electrorretinografía , Angiografía con Fluoresceína , Antígenos HLA-A/inmunología , Humanos , Inmunosupresores/uso terapéutico , Inducción de Remisión , Enfermedades de la Retina/diagnóstico , Enfermedades de la Retina/tratamiento farmacológico , Enfermedades de la Retina/inmunologíaRESUMEN
Birdshot retinochoroidopathy (BSRC) is a distinct type of posterior uveitis originally described in the 1940s. Its characteristics include minimal anterior segment inflammation and diffuse posterior choroidopathy with vitritis and retinal vasculitis. The precise etiology of this disease is yet to be elucidated. However, various treatment modalities have been employed with the ultimate goal of durable remission of this vision threatening intraocular disease. The purpose of this review is not only to emphasize the importance of recognizing BSRC, but also to discuss the new discoveries, immune mediators, current and new therapies, and techniques applied to monitor and accomplish disease remission.
Retinocoroidopatia do tipo "birdshot" é um tipo de uveíte posterior originalmente descrita na década de 1940. Achados característicos incluem inflamação mínima do segmento anterior, retinocoroidopatia difusa associada à vitreíte e vasculite retiniana. A etiologia da doença ainda não foi completamente definida, entretanto várias modalidades de tratamento têm sido utilizadas com o objetivo de atingir a remissão. O objetivo desta revisão é enfatizar não só a importância do reconhecimento da doença como também discutir novas descobertas relacionadas a mediadores imunes, formas de tratamentos e como monitorar a doença.
Asunto(s)
Humanos , Enfermedades de la Retina , Enfermedades de la Coroides , Coriorretinitis , Anticuerpos Monoclonales Humanizados/uso terapéutico , Enfermedades de la Retina/diagnóstico , Enfermedades de la Retina/inmunología , Enfermedades de la Retina/tratamiento farmacológico , Inducción de Remisión , Angiografía con Fluoresceína , Antígenos HLA-A/inmunología , Enfermedades de la Coroides/diagnóstico , Enfermedades de la Coroides/inmunología , Enfermedades de la Coroides/tratamiento farmacológico , Coriorretinitis/diagnóstico , Coriorretinitis/inmunología , Coriorretinitis/tratamiento farmacológico , Diagnóstico Diferencial , Quimioterapia Combinada , Electrorretinografía , Inmunosupresores/uso terapéuticoRESUMEN
A 58-year-old woman presented with rash over the left side of the face and intense acute uveitis. Following careful review of the symptoms and dilated fundus examination unilateral optic neuritis was discovered. The rash was typical of varicella zoster dermatitis. Patients presenting with herpes zoster ophthalmicus should always undergo dilated fundus examination, as there is a potential risk of unexpected posterior segment inflammation. Early diagnosis and prompt treatment can avoid visual sequelae.
Paciente de 58 anos de idade apresentando erupção cutânea no lado esquerdo da face e intensa uveíte unilateral. Após cuidadosa revisão dos sintomas e exame de fundo do olho foi detectada neurite óptica. O rash era típico de dermatite por varicella zoster. Pacientes apresentando quadro de herpes zoster oftálmico devem ser submetidos ao exame de fundo do olho devido ao risco de inesperada inflamação do segmento posterior. Diagnóstico precoce e tratamento imediato podem evitar danos visuais.
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Varicela/complicaciones , Neuritis Óptica/diagnóstico , Neuritis Óptica/etiología , Herpes Zóster Oftálmico/complicaciones , Herpes Zóster Oftálmico/diagnóstico , Herpesvirus Humano 3/inmunología , Nervio Óptico/patología , Nervio Óptico/diagnóstico por imagen , Sulfonamidas/uso terapéutico , Timolol/uso terapéutico , Activación Viral , Prednisona/uso terapéutico , Angiografía con Fluoresceína , Neuritis Óptica/tratamiento farmacológico , Neuritis Óptica/virología , Uveítis Anterior/diagnóstico , Uveítis Anterior/virología , Hipertensión Ocular/etiología , Hipertensión Ocular/tratamiento farmacológico , Herpes Zóster Oftálmico/tratamiento farmacológico , Herpes Zóster Oftálmico/virología , Corticoesteroides/uso terapéutico , Tomografía de Coherencia Óptica , Microscopía con Lámpara de Hendidura , Valaciclovir/uso terapéutico , Fondo de Ojo , Presión Intraocular/fisiología , Midriáticos/uso terapéuticoRESUMEN
This exploratory, multicenter, open-label study evaluated the efficacy and safety of FTY720, as a part of an immunosuppressive regimen, in combination with everolimus and steroids in de novo renal transplant recipients at increased risk of delayed graft function (DGF). Patients received FTY720 (5 mg) and everolimus (4 mg) 2-12 h pre-transplantation, followed by 2.5 mg/d FTY720 and concentration-controlled everolimus (4-8 ng/mL) post-transplant for 12 months. Induction therapy was prohibited. After enrollment of 56 of the planned 200 patients between 2000 and 2002, the recruitment was terminated. The primary endpoint, rate of graft loss, or death at three months was 15.4% and the biopsy-confirmed acute rejection was 42.3%. Death or graft loss at 12 months in the DGF and non-DGF arms was 36.0% and 25.9%, respectively. The mean estimated creatinine clearance at three months was 63 and 55 mL/min in the non-DGF and DGF groups, respectively, while at 12 months it was 56 mL/min in both the groups. Although there was no comparator arm, the results from this exploratory study (compared with data from other phases II and III trials) indicated no apparent benefits of FTY720-based regimens for prevention of acute rejection and preservation of renal function in renal transplant recipients at high risk of DGF.
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Funcionamiento Retardado del Injerto/prevención & control , Rechazo de Injerto/prevención & control , Inmunosupresores/uso terapéutico , Trasplante de Riñón , Glicoles de Propileno/uso terapéutico , Sirolimus/análogos & derivados , Esfingosina/análogos & derivados , Adulto , Funcionamiento Retardado del Injerto/etiología , Quimioterapia Combinada , Everolimus , Femenino , Clorhidrato de Fingolimod , Rechazo de Injerto/etiología , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Prospectivos , Sirolimus/uso terapéutico , Esfingosina/uso terapéutico , Tasa de Supervivencia , Resultado del TratamientoAsunto(s)
Antibacterianos/uso terapéutico , Biopsia , Queratitis/diagnóstico , Queratitis/microbiología , ADN Bacteriano/análisis , Glaucoma/diagnóstico , Glaucoma/microbiología , Leprostáticos/uso terapéutico , Lepra Lepromatosa/diagnóstico , Lepra Lepromatosa/microbiología , Lepra Lepromatosa/tratamiento farmacológico , Humor Acuoso/microbiología , Infecciones Bacterianas del Ojo/diagnóstico , Infecciones Bacterianas del Ojo/microbiología , Infecciones Bacterianas del Ojo/tratamiento farmacológico , Iris/microbiología , Iris/patología , Mycobacterium leprae/genética , Mycobacterium leprae/aislamiento & purificación , Piel/microbiología , Quimioterapia Combinada , Reacción en Cadena de la Polimerasa , Uveítis Anterior/diagnóstico , Uveítis Anterior/microbiología , Uveítis Anterior/tratamiento farmacológicoRESUMEN
La conjuntiva desempeña un papel muy importante en los procesos inflamatorios de las estructuras oculares externas, incluyendo a la misma conjuntiva, la córnea y la esclera. La quetoconjuntivitis cicatrizante crónica representa un grupo de enfermedades con manifestaciones clínicas similares, pero con un diagnóstico diferencial muy extenso y en el cual la confirmación diagnóstica por medio de los hallazgos inmunohistopatológicos de la conjuntiva es de crucial importancia para instituir un tratamiento adecuado. Lo mismo es cierto para la queratitis ulcerativa periférica y la excleritis necrotizante, en las cuales la historia clínica detalla, en conjunto con la biopsia conjuntival, representan las dos armas diagnósticas fundamentales. Además, esta última es el punto crítico en la toma de decisiones respecto a si instituir o no quimioterapia inmunosupresiva en estos casos. De esta manera, el estudio histopatológico e inmunopatológico de la conjuntiva representa una arma fundamental en el diagnóstico, en el tratamiento, así como en el entendimiento de la patogénesis de las enfermedades inflamatorias oculares externas de origen inmunológico.
Asunto(s)
Adulto , Persona de Mediana Edad , Humanos , Masculino , Femenino , Esclerótica/fisiopatología , Biopsia , Conjuntiva/fisiopatología , Enfermedades de la Conjuntiva/diagnóstico , Córnea/fisiopatología , Oftalmopatías/diagnóstico , Queratoconjuntivitis/fisiopatología , Úlcera de la Córnea/diagnósticoRESUMEN
A stratified random sample of 464 persons aged 40 to 79 years, drawn from enumeration registers in the Bridgetown area of Barbados, participated in this survey. The prevalence of hypertension (defined as systolic blood pressure of at least 160 mm Hg, diastolic blood pressure of at least 95 mm Hg, or use of antihypertensive medication) was 47% and 43% for women and men, respectively. Diabetes was present in 17% of all subjects (18% of women and 15% of men). Of the 209 hypertensive subjects, 82% were aware of their blood pressure status. The proportion of previously diagnosed hypertensive subjects on medication was 72% for men and 68% for women. Fifty-three percent of men and 42% of women were overweight (body mass indices [weight in kilograms divided by height in meters squared] between 25 and 30). However, 30% of women and 10% of men were obese (body mass indices over 30), supporting the growing recognition of the marked gender disparity in obesity among persons of African origin in the Caribbean. Body mass index was positively associated with hypertension (OR = 1.33; 95% CI: 1.1-1.6). Obese persons experienced a 2.6 times greater risk of hypertension compared to those with body mass indices below 25. Similar statistically significant associations were observed between diabetes and body mass index: OR comparing body mass index over 30 with body mass index under 25 was 2.5 (95% CI: 1.3-5.1) for all subjects, 1.0 (0.3-4.1) for men only, and 5.2 (1.9-14) for women only. Preventing obesity in this population could reduce the incidence of hypertension and diabetes by approximately 30% and 33% among men and women, respectively.
Asunto(s)
Diabetes Mellitus/epidemiología , Hipertensión/epidemiología , Obesidad , Adulto , Anciano , Barbados/epidemiología , Presión Sanguínea , Índice de Masa Corporal , Complicaciones de la Diabetes , Diabetes Mellitus/prevención & control , Femenino , Conductas Relacionadas con la Salud , Conocimientos, Actitudes y Práctica en Salud , Humanos , Hipertensión/complicaciones , Hipertensión/prevención & control , Modelos Logísticos , Masculino , Persona de Mediana Edad , Oportunidad Relativa , Prevalencia , Factores de Riesgo , Encuestas y CuestionariosRESUMEN
We hypothesized that prepubertal girls with gonadotropin deficiency would produce less follicle-stimulating hormone (FSH) in response to synthetic gonadotropin-releasing hormone (GnRH) than would gonadotropin-sufficient children. To test this hypothesis, we performed 103 GnRH tests serially in 21 children who had idiopathic hypopituitarism with growth hormone deficiency. We tried to predict whether puberty would occur in the 17 girls with bone ages of 8 years or less. Of these 17 girls, 4 failed to have spontaneous secondary sexual characteristics by age 16 1/2 years, and 12 had spontaneous complete pubertal development. One girl had incomplete pubertal maturation with partial gonadotropin deficiency; her results were combined with those of the girls who had no spontaneous pubertal development. With increasing bone age, the girls with complete pubertal development had a decrease in the increment of FSH released in response to GnRH, although basal gonadotropin concentrations did not change. For GnRH tests performed at bone ages of 8 years or less, basal luteinizing hormone (LH) values did not differ between girls with complete puberty and those with absent or incomplete puberty. However, basal FSH and the incremental response of LH and FSH to GnRH were greater in those with complete puberty. Only two girls with prepubertal bone ages at the time of testing, who subsequently had complete puberty, had incremental FSH responses to GnRH that were less than 5 IU/L. Individual incremental LH responses to GnRH did not discriminate well between groups. None of the girls with adrenocorticotropic hormone deficiency, either originally or subsequently, had spontaneous puberty, but 4 of 12 girls with thyrotropin deficiency, either originally or subsequently, had complete puberty. We conclude that a significant increase in GnRH-stimulated FSH suggests that spontaneous pubertal development will occur in girls with idiopathic hypopituitarism. However, a low FSH response to GnRH may not be diagnostic of gonadotropin deficiency.
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Hormona Folículo Estimulante/sangre , Hormona Liberadora de Gonadotropina , Hormona del Crecimiento/deficiencia , Hipopituitarismo/sangre , Hormona Luteinizante/sangre , Niño , Femenino , Hormona Folículo Estimulante/deficiencia , Hormonas , Humanos , Hipopituitarismo/complicaciones , Hormona Luteinizante/deficiencia , Pubertad/fisiologíaRESUMEN
For 9 years we have observed a girl who has ossification in the dermis with a strikingly limited distribution. Recently a second girl with similar dermal ossification restricted to a single extremity was identified. The ectopic bone is histologically identical to normal membranous bone. These two patients have no obvious underlying cause for soft tissue bone formation, and no disorder of calcium or phosphate metabolism. Ossification first involved the dermal and subcutaneous connective tissue, and with time advanced locally in the affected areas to bridge joints and limit mobility. The ossification has now extended to involve muscle fascia but has not involved the muscle itself. This disease appears to represent a heretofore unrecognized disorder of mesenchymal differentiation.
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Osificación Heterotópica/patología , Enfermedades de la Piel/patología , Huesos/diagnóstico por imagen , Huesos/metabolismo , Niño , Preescolar , Femenino , Humanos , Minerales/metabolismo , Osificación Heterotópica/diagnóstico por imagen , Osificación Heterotópica/metabolismo , Radiografía , Piel/patología , Enfermedades de la Piel/diagnóstico por imagen , Enfermedades de la Piel/metabolismoRESUMEN
Between 1979 and 1983, 129 children (95 girls) with precocious puberty were referred to the National Institutes of Health and received treatment for at least 6 months with the long-acting LHRH analogue D-Trp6-Pro9-NEt-LHRH. The majority (107 of 129) of the children had central precocious puberty mediated by activation of the hypothalamic-pituitary-gonadal axis in association with hypothalamic hamartomas (24 of 107) or other central nervous system lesions (21 of 107), or idiopathic precocious puberty (62 of 107). Hypothalamic hamartomas or other central nervous system lesions were a frequent cause of central precocious puberty in girls (27 of 87), but idiopathic precocious puberty was still the most frequent diagnosis (63%). Idiopathic precocious puberty was uncommon in boys (6%). The patients with peripheral precocious puberty included six girls with McCune-Albright syndrome and six boys with familial male precocious puberty. These children had peripheral sex steroid secretion in the absence of hypothalamic-pituitary-gonadal axis maturation. The children with combined peripheral and central precocious puberty included nine children with congenital adrenal hyperplasia and one girl with a virilizing adrenal tumor. In the patients with central precocious puberty or combined peripheral and central precocious puberty, LHRHa therapy caused suppression of gonadotropin and sex steroid levels (P less than 0.001), stabilization or regression of secondary sexual characteristics, and decreases in growth rate and in the rate of bone age maturation (P less than 0.005). Patients with peripheral precocious puberty, however, had no significant change in gonadotropin or sex steroid levels, growth rate, or the rate of bone age maturation, and no improvement in secondary sexual characteristics. Thus, LHRHa is an effective treatment of central precocious puberty and combined peripheral and central precocious puberty, but is ineffective in the therapy of peripheral precocious puberty.