ABSTRACT
PURPOSE: The Sturge-Weber syndrome (SWS) is a rare congenital disorder that occurs sporadically and features lesions of both skin and nervous system. We report a case of 7 year-old girl with Sturge-Weber syndrome and discuss its clinicopathological features, differential diagnosis and also emphasize the importance of its diagnosis in the clinical oral practice. CASE DESCRIPTION: The classic pathognomonic manifestations include angioma of the leptomeninges extending to cerebral cortex with ipsilateral angiomatous lesions, unilateral facial nevus affecting a division of trigeminal nerve, hemiparesis, intracranial calcification, mental retardation and refractory epilepsy. The most apparent indication of SWS is a facial birthmark or "Port Wine Stain" present at birth and typically involving at least one upper eyelid and the forehead. Management of a patient with Sturge-Weber Syndrome may be challenging due to risk of hemorrhage. CONCLUSION: The dentists should focus on comprehensive therapy, starting with behavior management and stress on preventive measures.
OBJETIVO: A síndrome de Sturge-Weber (SWS) é uma rara doença congênita que ocorre esporadicamente e apresenta lesões de pele e do sistema nervoso. Este artigo é um relato de caso de uma menina de 7 anos com SWS para discutir suas características clinicopatológicas, diagnóstico diferencial e enfatizar a importância do seu diagnóstico na prática clínica odontológica. DESCRIÇÃO DO CASO: As manifestações clássicas patognomônicas incluem angioma das leptomeninges que se estendem para o córtex cerebral com lesões angiomatosas ipsilaterais, nevo facial unilateral, que afetam uma divisão do nervo trigêmeo, hemiparesia, calcificação intracraniana, retardo mental e epilepsia refratária. A indicação mais evidente de SWS é uma marca de nascença facial ou "mancha vinho do porto" presente no nascimento e tipicamente envolvendo pelo menos uma pálpebra superior e a testa. O controle clínico de um paciente com SWS pode ser desafiador devido ao risco de hemorragia. CONCLUSÃO: Os dentistas devem adotar uma abordagem terapêutica abrangente, começando com o controle de comportamento e de estresse com medidas preventivas.
Subject(s)
Humans , Female , Child , Angiomatosis/diagnosis , Mouth Mucosa/physiology , Sturge-Weber Syndrome/diagnosisABSTRACT
La definición explícita de prioridades en intervenciones de salud representa una oportunidad para México de equilibrar la presión y la complejidad de una transición epidemiológica avanzada, con políticas basadas en evidencias generadas por la inquietud de cómo optimizar el uso de los recursos escasos para mejorar la salud de la población. La experiencia mexicana en la definición de prioridades describe cómo los enfoques analíticos estandarizados en la toma de decisiones, principalmente los de análisis de la carga de la enfermedad y de costo-efectividad, se combinan con otros criterios -tales como dar respuesta a las expectativas legítimas no médicas de los pacientes y asegurar un financiamiento justo para los hogares-, para diseñar e implementar un grupo de tres paquetes diferenciados de intervenciones de salud. Éste es un proceso clave dentro de un conjunto más amplio de elementos de reforma dirigidos a extender el aseguramiento en salud, especialmente a los pobres. Las implicaciones más relevantes en el ámbito de políticas públicas incluyen lecciones sobre el uso de las herramientas analíticas disponibles y probadas para definir prioridades nacionales de salud; la utilidad de resultados que definan prioridades para guiar el desarrollo de capacidades a largo plazo; la importancia de favorecer un enfoque para institucionalizar el análisis ex-ante de costo-efectividad; y la necesidad del fortalecimiento de la capacidad técnica local como un elemento esencial para equilibrar los argumentos sobre maximización de la salud con criterios no relacionados con la salud en el marco de un ejercicio sistemático y transparente.
Explicit priority setting presents Mexico with the opportunity to match the pressure and complexity of an advancing epidemiological transition with evidence-based policies driven by a fundamental concern for how to make the best use of scarce resources to improve population health. The Mexican priority-setting experience describes how standardised analytical approaches to decision making, mainly burden of disease and cost-effectiveness analyses, combine with other criteria -eg, being responsive to the legitimate non-health expectations of patients and ensuring fair financing across households- to design and implement a set of three differentiated health intervention packages. This process is a key element of a wider set of reform components aimed at extending health insurance, especially to the poor. The most relevant policy implications include lessons on the use of available and proven analytical tools to set national health priorities, the usefulness of priority-setting results to guide long-term capacity development, the importance of favouring an institutionalised approach to cost-effectiveness analysis, and the need for local technical capacity strengthening as an essential step to balance health-maximising arguments and other non-health criteria in a transparent and systematic process.
Subject(s)
Adult , Female , Humans , Infant , Male , Middle Aged , Health Priorities , Public Health , Age Factors , Cause of Death , Cost of Illness , Cost-Benefit Analysis , Forecasting , Health Care Reform/economics , Mexico , Morbidity/trends , Mortality/trends , Poverty , Risk Factors , Sex FactorsABSTRACT
A FPLC purified 38kDa protease (Bm mf S-7) isolated from B. malayi microfilarial soluble antigen was identified. It showed pronounced reactivity with sera collected from 'putatively immune' asymptomatic and amicrofilaraemic individuals residing in an endemic area for bancroftian filariasis. Further the immune protective activity of Bm mf S-7 antigen was evaluated in susceptible hosts, jirds (Meriones unguiculatus) against B. malayi filarial infection. The antigen showed 89% cytotoxicity against mf and 87-89% against infective (L3) larvae in in vitro antibody dependent cellular cytotoxicity Assay (ADCC) and in situ micropore chamber methods. Bm mf S-7 immunized jirds after challenge infection showed 81.5% reduction in the adult worm burden. The present study has shown that, the 38kDa microfilarial proteases (Bm mf S-7) could stimulate a strong protective immune response against microfilariae and infective larvae in jird model to block the transmission of filariasis. Analysis of IgG subclasses against Bm mf S-7 revealed a significant increase in IgG2 and IgG3 antibodies in endemic normals. Lymphocyte proliferation to Bm mf S-7 was significantly high in endemic normal group as compared to that in clinical and microfilarial carriers. Significantly enhanced levels of IFN-gamma in the culture supernatant of PBMC of endemic normals followed by stimulation with Bm mf S-7 suggest that the cellular response in this group is skewed towards Th 1 type.