RESUMEN
Beginning in early 2006, an ocular disease of unknown etiology was routinely observed in American crocodiles (Crocodylus acutus) inhabiting the highly polluted Tarcoles River in west-central Costa Rica. We examined the nature and incidence of ocular disease in Tarcoles crocodiles and assessed the possible association between the disease and accumulation of chemical pollutants in diseased individuals. During 12-15 September and 12-13 December 2007, crocodiles were captured and examined for ocular disease and sampled to determine environmental contaminant accumulation. Three of 11 (27.3%) crocodiles captured (all males) exhibited unilateral ocular disease, primarily characterized by corneal opacity and scarring, anterior synechia, and phthisis bulbi. Multiple pollutants were detected in crocodile caudal scutes (organochlorine pesticides [OCPs] and metals), crocodile blood (OCPs), and sediments (OCPs and metals) from the Tarcoles, but no associations were found between contaminant accumulation and the incidence of eye disease. On the basis of the limited number of diseased animals examined and the potential exposure of crocodiles to pathogens and other pollutants not targeted in this study, we cannot rule out infection or chemical toxicosis as causes of the eye lesions. However, circumstantial evidence suggests that the observed ocular disease is likely the result of injury-induced trauma (and possibly secondary infection) inflicted during aggressive encounters (e.g., territorial combat) among large adult crocodiles living at relatively high densities.
Asunto(s)
Caimanes y Cocodrilos , Monitoreo del Ambiente/métodos , Oftalmopatías/veterinaria , Contaminación del Agua/efectos adversos , Animales , Costa Rica , Contaminación Ambiental/efectos adversos , Monitoreo Epidemiológico , Oftalmopatías/epidemiología , Oftalmopatías/etiología , Masculino , Metales Pesados/análisis , Plaguicidas/análisisRESUMEN
OBJECTIVE: To assess the long-term systemic and neurologic responses to enzyme replacement therapy (ERT) with macrophage-targeted glucocerebrosidase in patients with type 3 Gaucher's disease. STUDY DESIGN: Patients with type 3 Gaucher's disease (n = 21), aged 8 months to 35 years, were enrolled in a prospective study. Enzyme dose was adjusted to control systemic manifestations. Clinical and laboratory evaluations were performed at baseline and every 6 to 12 months thereafter. Patients were followed up for 2 to 8 years. RESULTS: Significant improvement in hemoglobin levels, platelet count, and acid phosphatase values occurred. Liver and spleen volume markedly decreased, and bone structure improved. Nineteen patients had asymptomatic interstitial lung disease unresponsive to ERT. Supranuclear gaze palsy remained stable in 19 patients, worsened in one patient, and improved in one. Cognitive function remained unchanged or improved over time in 13 patients but decreased in 8 patients, 3 of whom developed progressive myoclonic encephalopathy accompanied by cranial magnetic resonance imaging and electroencephalographic deterioration. CONCLUSIONS: At relatively high doses, ERT reverses almost all the systemic manifestations in patients with type 3 Gaucher's disease. Most treated patients do not deteriorate neurologically. Novel therapeutic strategies are required to reverse the pulmonary and neuronopathic aspects of the disease.
Asunto(s)
Enfermedad de Gaucher/tratamiento farmacológico , Glucosilceramidasa/uso terapéutico , Adolescente , Adulto , Determinación de la Edad por el Esqueleto , Niño , Preescolar , Electroencefalografía/métodos , Femenino , Enfermedad de Gaucher/sangre , Enfermedad de Gaucher/diagnóstico , Enfermedad de Gaucher/psicología , Glucosilceramidasa/administración & dosificación , Humanos , Pruebas de Inteligencia , Imagen por Resonancia Magnética , Masculino , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
We report a case of Mycobacterium marinum facial sporotrichoid infection in an otherwise healthy 2-year-old child, probably acquired through contact with pets in an aquarium. The M. marinum isolate was susceptible to clarithromycin, and the child was successfully treated with oral antibiotic therapy. This unusual case emphasizes the importance of a thorough history in the evaluation of a patient with chronic sporotrichoid skin lesions.
Asunto(s)
Dermatosis Facial/diagnóstico , Infecciones por Mycobacterium no Tuberculosas/diagnóstico , Administración Oral , Antibacterianos/administración & dosificación , Preescolar , Enfermedad Crónica , Claritromicina/administración & dosificación , Diagnóstico Diferencial , Quimioterapia Combinada/administración & dosificación , Dermatosis Facial/tratamiento farmacológico , Dermatosis Facial/etiología , Humanos , Masculino , Infecciones por Mycobacterium no Tuberculosas/tratamiento farmacológico , Infecciones por Mycobacterium no Tuberculosas/etiología , Rifampin/administración & dosificación , Esporotricosis/diagnósticoRESUMEN
Partial splenectomy was introduced to achieve the benefits of splenectomy and to avoid the risk of overwhelming infection in patients with symptomatic Gaucher disease. We observed regrowth of the splenic remnant, reemergence of preoperative symptoms, and new bone involvement among most of our patients who had undergone partial splenectomy. Enzyme replacement therapy has markedly limited indications for splenectomy, partial or total, for Gaucher disease.
Asunto(s)
Enfermedad de Gaucher/cirugía , Esplenectomía , Adolescente , Adulto , Enfermedades Óseas/etiología , Niño , Femenino , Estudios de Seguimiento , Enfermedad de Gaucher/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Osteonecrosis/etiología , Bazo/crecimiento & desarrollo , Resultado del TratamientoRESUMEN
Long-term studies of a child with Gaucher disease indicated that the response to treatment with macrophage-targeted glucocerebrosidase (glucosylceramidase) is dose dependent, and that the hematologic response precedes the skeletal response.
Asunto(s)
Enfermedad de Gaucher/tratamiento farmacológico , Glucosilceramidasa/administración & dosificación , Abdomen/patología , Huesos/patología , Niño , Relación Dosis-Respuesta a Droga , Estudios de Seguimiento , Enfermedad de Gaucher/diagnóstico , Enfermedad de Gaucher/enzimología , Glucosilceramidasa/sangre , Humanos , Macrófagos/efectos de los fármacos , Imagen por Resonancia Magnética , MasculinoRESUMEN
Among immigrants resident in greater London from Europe, Ireland, the USSR, the old Commonwealth countries of Australia, Canada, and New Zealand, North and South America, Egypt, Turkey, and Iran the incidence of admission to hospital for probable multiple sclerosis (MS) between 1960 and 1972 was high or moderately high. The incidence was the same order as that found in those born in the United Kingdom. Immigrants from India, Pakistan, and other Asian countries and from new Commonwealth Africa and America, which includes the West Indies, had a low incidence of hospital admission for MS. Immigrants from countries where the risk of MS is low whose parents were born in Europe had a reduced incidence of admission to hospital but not the very low incidence found in those parents were also born in these countries. Emigrating to England from low risk parts of the world did not seem to increase the risk of developing MS.