RESUMO
OBJECTIVES: One of the main findings in asthma is chronic inflammation of the airway. Exhaled nitric oxide may be a useful marker of airway inflammation in asthmatic children. To evaluate the concentration of nitric oxide (NO) in exhaled air in healthy and asthmatic children and to relate this variable with different treatments. MATERIAL AND METHODS: A cross-sectional study was performed in a tertiary hospital. The concentration of exhaled NO (in parts per billion) was analyzed by chemoluminescence using the T technique of exhaling against expiratory resistance with positive mouthpiece pressure in two different study groups: group A (control group) consisted of 105 healthy children aged 6-14 years old without a history of respiratory disease; group B (asthmatic group) consisted of 79 children aged 6-14 years old diagnosed with asthma and undergoing anti-asthmatic treatment for at least the previous two months, depending on the severity of their disease. Quantitative variables were analyzed using Student's t-test and the relationship between qualitative variables was analyzed using the chi-squared test. RESULTS: Expired NO concentrations were significantly higher in the asthma group (15.02 ppb) than in the control group (5.40 ppb) (p < 0.01). No significant differences were found among the asthmatic children in asthma severity. Expired NO was higher in asthmatic children treated with nedocromil (16.62 ppb) than in those treated with inhaled corticosteroids (11.8 ppb) but this difference was not significant (p 0.08). Children who presented signs of acute asthma (22.87 ppb) and those with a positive bronchial dilatation test (20.65) showed higher expired NO values (p < 0.05) than those without asthmatic crises and/or a negative bronchial dilatation test. Likewise, children with atopic dermatitis showed higher expired NO concentrations (23.07 ppb) than those without atopic dermatitis (11.68 ppb) (p < 0.001). CONCLUSIONS: Children with asthma of various degrees of severity have higher levels of expired NO than do healthy children. Measurement of expired NO concentrations can be used to monitor bronchial inflammation and to evaluate the efficacy of anti-inflammatory treatments.
Assuntos
Asma/diagnóstico , Óxido Nítrico/análise , Adolescente , Criança , Doença Crônica , Estudos Transversais , Feminino , Nível de Saúde , Humanos , MasculinoRESUMO
A case history of a two years seven months old boy with hyperinsulinism and marked psychomotor retardation is presented. In this patient, biochemical analysis revealed persistent hypoglycemia. Basal insulin levels (RIA) were consistently superior to 10 mU/ml. and were higher than would be expected from the child's glycemias. Angiographic study of the celiac trunk revealed diffuse pancreatic enlargement. Therapy was initiated with oral diazoxide normalizing and stabilizing the patient's glycemia. The importance of an early diagnosis of this condition and the necessary biochemical analyses involved are discussed as well as the use of diazoxide in the medical treatment of hypoglycemia.
Assuntos
Hiperinsulinismo/etiologia , Pâncreas/patologia , Pré-Escolar , Diazóxido/uso terapêutico , Humanos , Hiperinsulinismo/tratamento farmacológico , Hiperplasia , Hipoglicemia/etiologia , Masculino , Transtornos Psicomotores/etiologiaRESUMO
Authors present a case of Maestre de San Juan-Kallman syndrome in a male 14 year old, with hypogonadotrophic hypogonadism and anosmia, and with other associated malformations (cryptorchism, epilepsy, hypoacusia and chromosomal anomalies). Standard values of FSH and LH are undetectable. After stimulation with LH-RH, a continued release of gonadotropins is produced. With the clomiphene stimulation there is a negative response.
Assuntos
Anormalidades Múltiplas/sangue , Gonadotropinas Hipofisárias/deficiência , Hipogonadismo/sangue , Transtornos do Olfato/sangue , Adolescente , Aberrações Cromossômicas , Clomifeno , Criptorquidismo/sangue , Epilepsia/sangue , Hormônio Liberador de Gonadotropina , Humanos , Masculino , SíndromeAssuntos
Infecções Urinárias/complicações , Refluxo Vesicoureteral/tratamento farmacológico , Adolescente , Fatores Etários , Criança , Pré-Escolar , Protocolos Clínicos , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Refluxo Vesicoureteral/complicações , Refluxo Vesicoureteral/diagnósticoAssuntos
Antagonistas Adrenérgicos beta/uso terapêutico , Anti-Hipertensivos/uso terapêutico , Hipertensão/terapia , Antagonistas Adrenérgicos alfa/uso terapêutico , Débito Cardíaco/efeitos dos fármacos , Criança , Diuréticos/uso terapêutico , Espaço Extracelular , Humanos , Hipertensão/fisiopatologia , Sistema Renina-Angiotensina/efeitos dos fármacos , Resistência Vascular/efeitos dos fármacosAssuntos
Arteriopatias Oclusivas/complicações , Arteriopatias Oclusivas/etiologia , Displasia Fibromuscular/complicações , Doença de Moyamoya/etiologia , Obstrução da Artéria Renal/complicações , Trombose das Artérias Carótidas/diagnóstico por imagem , Trombose das Artérias Carótidas/etiologia , Criança , Humanos , Masculino , Doença de Moyamoya/diagnóstico por imagem , Radiografia , Obstrução da Artéria Renal/diagnóstico por imagemAssuntos
Síndrome de Secreção Inadequada de HAD/tratamento farmacológico , Lítio/uso terapêutico , Antibacterianos/uso terapêutico , Morte Encefálica , Encefalopatias/complicações , Pré-Escolar , Feminino , Humanos , Síndrome de Secreção Inadequada de HAD/sangue , Síndrome de Secreção Inadequada de HAD/urina , Isoniazida/uso terapêutico , Lítio/farmacologia , Concentração Osmolar , Tuberculose Miliar/complicaçõesAssuntos
Apneia/etiologia , Malformação de Arnold-Chiari/complicações , Apneia/diagnóstico , Malformação de Arnold-Chiari/diagnóstico , Ventrículos Cerebrais/diagnóstico por imagem , Ventrículos Cerebrais/patologia , Humanos , Recém-Nascido , Imageamento por Ressonância Magnética , Masculino , Polissonografia , Ultrassonografia , Paralisia das Pregas Vocais/diagnóstico , Paralisia das Pregas Vocais/etiologiaRESUMO
La infección estafilocócica es una causa poco frecuente de neumonía en niños inmunocompetentes. Es casi exclusivamente una enfermedad de lactantes. La neumonía por Staphylococcus tiende a presentarse como una enfermedad aguda y grave, en especial porque muchos antibióticos usados para tratar las neumonías adquiridas en la comunidad (NAC) no proporcionan una cobertura apropiada para este agente. Los hallazgos radiológicos incluyen bronconeumonía con infiltrados alveolares, que habitualmente son unilaterales. Estos infiltrados pueden coalescer y originar grandes áreas de consolidación y cavitación. La destrucción de las paredes bronquiales puede dar lugar a la formación de neumatoceles en más del 50% de los casos y a empiemas. Aunque la aparición de los neumatoceles puede ser dramática, una vez que la infección es controlada, los neumatoceles se resuelven completamente en unos pocos meses. A pesar de su baja frecuencia, estas neumonías precisan ser tratadas conociendo el antibiótico más adecuado, pues este germen puede tener cepas meticilin resistentes, que obligan al uso de fármacos específicos como la vancomicina o la teicoplanina(AU)
Staphylococcus infection is a rare cause of pneumonia in immunocompetent children. It is almost exclusively a disease of infants. Staphylococcus aureus pneumonia tend to present as an acute and severe illness, especially because many antibiotics used to treat community acquired pneumonia (CAP) do not providec overage for this agent. Radiologic findings include bronchopneumonia with alveolar infiltrates, which is more commonly unilateral. The infiltrates may coalesce and may evolve areas of consolidation and cavitation. Destruction of bronchial walls may lead to pneumatocele formation in about more half of the cases and empyema. Although the appearance of staphylococcal pneumatoceles may be dramatic, usually once the infection has been overcome, the pneumatoceles resolve completely over a few months. In spite of its low frequency, these pneumonias are necessary to be treated knowing the most suitable antibiotic, since this germ can have meticillin-resistant strains, which force to the use of specific drugs as the vancomyc in or the teicoplanin(AU)
Assuntos
Humanos , Masculino , Feminino , Lactente , Pneumonia Estafilocócica/complicações , Pneumonia Estafilocócica/epidemiologia , Pneumonia Estafilocócica/terapia , Vancomicina/uso terapêutico , Teicoplanina/uso terapêutico , Pneumotórax/complicações , Diagnóstico Diferencial , Broncopneumonia/complicações , Broncopneumonia/epidemiologia , Staphylococcus aureus/isolamento & purificação , Staphylococcus aureus/patogenicidade , Radiografia Torácica , Pneumotórax , Tomografia Computadorizada de EmissãoRESUMO
El pulmón constituye el lugar más común de asentamiento de los microorganismos causales de sepsis del recién nacido. Este origen corresponde tanto a la vida prenatal y al parto como a la vida posnatal. Es por ello por lo que deben observarse estrechamente los signos sugerentes de dificultad respiratoria en el neonato, con el fin de llevar a cabo un diagnóstico y un tratamiento precoces. Estos cuadros se asocian a una gran morbilidad, pues se estima en unas 800.000 las muertes neonatales al año debidas a infecciones respiratorias en países en vías de desarrollo. En nuestro medio se estima que la incidencia es menor del 1%, pero sería cercana al 10% en niños con comorbilidad si se englobasen tanto los recién nacidos con peso adecuado para la edad gestacional como los de bajo peso. Además, en la práctica clínica se observa que casi en la totalidad de los cuadros sépticos existe afectación neumónica más o menos significativa tanto clínica como radiológica. En este artículo revisamos los diversos aspectos y retos que plantean las neumonías neonatales (en especial los gérmenes más habituales), y su enfoque terapéutico(AU)
The lung is the most common site of entry of the microorganisms causing sepsis in the newborn infant. This invasion can occur during the prenatal period and labor, as well as during the postnatal period. Thus, we must closely monitor signs suggestive of respiratory distress in the newborn infant in order to establish an early diagnosis and initiate treatment. These conditions are associated with a high rate of morbidity, and it is estimated that, in developing countries, approximately 800,000 neonatal deaths a year are due to respiratory infections. In our population, the incidence is calculated to be less than 1%, but it would be nearly10% in children with co-morbidity if the population includes those with a birth weight adequate for their gestational age and full term and premature infants. In addition, in clinical practice, it is observed that in nearly every case of sepsis, there is more or less significant evidence of pulmonary involvement in both the clinical and radiological examinations. In this article, we review the different aspects and challenges associated with neonatal pneumonia, focusing especially on the most common microorganisms and the therapeutic approach (AU)
Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Pneumonia/complicações , Pneumonia/diagnóstico , Prognóstico , Pneumonia/epidemiologia , Morbidade/tendências , Infecções Respiratórias/complicações , Infecções Respiratórias/epidemiologia , Síndrome do Desconforto Respiratório do Recém-Nascido/complicações , Ampicilina/uso terapêutico , Gentamicinas/uso terapêutico , Pneumonia/prevenção & controle , Pneumonia/fisiopatologia , Infecções Respiratórias/prevenção & controle , Comorbidade/tendências , Infecções Pneumocócicas/complicações , Streptococcus agalactiae/isolamento & purificação , Chlamydia trachomatis/isolamento & purificação , Chlamydia trachomatis/patogenicidade , Pneumocystis/isolamento & purificação , Pneumonia por Pneumocystis/complicaçõesRESUMO
La neumonía viral es una de las enfermedades más comunes que afecta a lactantes y niños. La OMS comunica que la infección respiratoria aguda continúa siendo una causa importante de morbimortalidad infantil; se estima que aproximadamente 2 millones de niños menores de 5 años de edad mueren por esta causa en los países del Tercer Mundo y el 40% de estas muertes son debidas a infecciones virales. El problema que plantean las neumonías virales es triple: por un lado, el descubrimiento de nuevos virus patógenos, algunos de los cuales causan graves cuadros neumónicos; por otro, las dificultades para conocer la etiología, problema de difícil solución, y un problema no menor, su coexistencia con otros patógenos, con el probable aumento de la morbimortalidad asociada. Una cuestión todavía no resuelta y que preocupa en todo el mundo es la infección por el virus de la gripe aviar, tipo H1N5, cuyo hipotético paso a humanos por infección humana (no animal-humano) no descarta la posibilidad de una pandemia. Parece razonable pensar que el enfoque futuro para un aumento del control de estas infecciones virales vendrá por bioingienería genética, lo que mejorará su identificación y el desarrollo de vacunas para los virus más agresivos causantes de neumonías virales graves(AU)
Viral pneumonia is one of the most common diseases affecting infants and children throughout the world. The WHO reports that acute respiratory infections continue to be a leading cause of morbidity and mortality in young children; they kill approximately two million children under the age of 5 years in developing countries, and about 40% of these deaths are due to viral infections. The challenge posed by the viral pneumonias is triple: on the one hand, the discovery of new pathogenic viruses, some of which cause serious pulmonary problems; on the other hand, the difficulties involved in determining the etiology, a question that is difficult to resolve; and another that is by no means minor, their coexistence with other pathogens, probably accompanied by an increase in the associated morbidity and mortality. Another problem that remains to be solved, and has become a worldwide concern, is infection by the H5N1 avian flu virus, the hypothetical transfer of which from an infected human to other humans (not animal-to-human) does not rule out the possibility of a pandemic. It seems to be reasonable to think that the future approach to controlling an increase in these viral infections will come from genetic engineering to improve their identification and the development of vaccines for the most aggressive causative viruses of serious viral pneumonias(AU)
Assuntos
Humanos , Masculino , Feminino , Criança , Lactente , Pneumonia Viral/epidemiologia , Mortalidade Infantil/tendências , Infecções por Paramyxoviridae/diagnóstico , Síndrome do Desconforto Respiratório do Recém-Nascido/complicações , Síndrome do Desconforto Respiratório do Recém-Nascido/diagnóstico , Bronquiolite Obliterante/diagnóstico , Bronquiolite Obliterante , Prognóstico , Indicadores de Morbimortalidade , Pneumonia Viral/diagnóstico , Pneumonia Viral/fisiopatologia , Infecções Respiratórias/diagnóstico , Infecções Respiratórias/epidemiologia , Pneumonia Viral/etiologia , Derrame Pleural/complicações , Hipoventilação/complicações , Atelectasia Pulmonar/complicações , Radiografia Torácica/métodosRESUMO
Los niños con neumonía y afectación media o moderada pueden ser cuidados en su domicilio con seguridad y reexaminados a las 48 horas del inicio del tratamiento. En los niños pequeños, una afectación más intensa como tener una SaO2 <92%, cianosis, polipnea >70 rpm, esfuerzo respiratorio, apneas intermitentes, quejido, incapacidad para ingerir alimentos y limitación para observación o supervisión familiar es indicación para su ingreso hospitalario. En los niños más mayores, estas indicaciones serían una SaO2 <92%, cianosis, polipnea >50 rpm, dificultad para ingerir líquidos o alimentos, quejido, signos de deshidratación o dificultad familiar para su observación o supervisión. Como los virus son la causa única de muchos casos de neumonía en la infancia no parece apropiado tratar a cada niño con antibióticos. Las decisiones terapéuticas en un niño concreto pueden ser difíciles, pues la mayoría de las pruebas no son útiles para diferenciar de modo adecuado las infecciones virales de las bacterianas. La resistencia antibacteriana se ha incrementado constantemente en los últimos años y está relacionada con su uso abusivo en situaciones en que la afectación viral es muy probable o la enfermedad es leve y limitada a vías superiores. Por esta razón, es preferible restringir los antibióticos tanto como sea posible y, si se usan, utilizar agentes de espectro estrecho siempre que sea posible, pues la menor presión antibiótica limita la emergencia de resistencia antibiótica. Finalmente, se comenta el enfoque terapéutico de las neumonías, de acuerdo con la edad, la repercusión sobre el estado general, el patrón radiológico, los datos de laboratorio y la epidemiología de estas infecciones en nuestro medio (AU)
Infants and children with moderate pneumonia can be safely cared for at home and should be re-examined within 48 hours of the beginning of treatment. An SaO2 of 92% or less, cyanosis, respiratory rate greater than70 breaths per minute, difficulty in breathing, intermittent apnea, grunting, inability to eat, and a family incapable of providing appropriate observation or supervision are indicators for hospital admission among infants. In the case of older children, theses indicators are an SaO2 of 92% or less, cyanosis, respiratory rate greater than 50 breaths per minute, difficulty in the intake of fluids or solids, grunting, signs of dehydration, or a family incapable of providing either appropriate observation or supervision. Since viruses are the sole cause of many cases of pneumonia in childhood, it is not appropriate to treat every child with antibiotics. However, therapeutic decisions can be difficult, because most tests do not adequately differentiate viral from bacterial infection in a given child. The problem of bacterial resistance to antibiotics has increased steadily in recent years, and it is certainly related to their overuse in situations in which viral involvement is quite probable or the illness is trivial and self-limiting, such as an upper respiratory infection. Hence, it is preferable to restrict antibiotics as much as possible and to use narrow-spectrum agents whenever appropriate, because less antibiotic pressure limits the emergence of bacterial resistance. Finally, we comment on the general management of pneumonias depending on the age, the impact on the general condition of the patient and the radiological pattern, laboratory data and the epidemiology of these infections in our general population (AU)
Assuntos
Humanos , Masculino , Feminino , Criança , Pneumonia/diagnóstico , Pneumonia/epidemiologia , Pneumonia/terapia , Resistência a Medicamentos/fisiologia , Farmacorresistência Bacteriana/fisiologia , Choque Séptico/complicações , Cianose/complicações , Apneia/complicações , Staphylococcus aureus/isolamento & purificação , Staphylococcus aureus/patogenicidade , Choque/complicaçõesRESUMO
La enfermedad cardiovascular continúa siendo la primera causa de morbimortalidad en los países industrializados en la edad adulta, pero de los datos disponibles podemos afirmar que las alteraciones iniciales, poco evidentes pero con gran significado futuro, se inician en la mayoría de las personas durante la infancia. El objeto de este trabajo es una puesta al día de esta epidemia, contemplando inicialmente el metabolismo complejo del colesterol, la evaluación del riesgo futuro y las medidas que deben adoptarse, tanto desde un punto de vista preventivo como de enfoque de esta situación, así como de los tratamientos farmacológicos de que disponemos, sus indicaciones y sus riesgos, que no deben ser minusvalorados. Las medidas preventivas que se pueden aconsejar en la población pediátrica sin factores de riesgo no son fáciles de tomar y, hasta que no se disponga de un marcador biológico, el problema continuará sin resolverse. Distinto es el problema cuando se conocen los factores de riesgo por la historia familiar, en cuyo caso, además de medidas dietéticas y ejercicio regular, puede ser aconsejable el uso de estatinas, pero sin olvidar sus riesgos potenciales y la experiencia escasa que existe todavía al respecto(AU)
Cardiovascular disease continues to be the leading cause of morbidity and mortality among adults in the industrialized countries, but the available information indicates that the initial changes, which are barely evident but are of great significance for the future, begin to take place in the majority of individuals during childhood. The objective of this work is to provide an update on this epidemic, first taking into account the complexity of cholesterol metabolism, the evaluation of future risk and the measures to be taken, both from a preventive point of view, and as an approach to this situation and to the pharmacological treatments available to us, as well as the indications and risks, which should not be underestimated. The preventive measures that can be recommended in the pediatric population with no risk factors are not easy to take, and until we discover a biological marker, the problem will remain unsolved. The problem changes when the family history reveals the existence of risk factors. In this case, in addition to dietary measures and regular exercise, the use of statins or other drugs might be advisable, without overlooking the potential risks and the still limited experience available(AU)
Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Hipercolesterolemia/epidemiologia , Apolipoproteínas/análise , /análise , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Fatores de Risco , Biomarcadores/análiseRESUMO
Los alérgenos son sustancias, habitualmente de naturaleza proteica, capaces de inducir en individuos predispuestos genéticamente (atópicos) anticuerpos IgE específicos, y que resultan inocuas para el resto de la población1-3. Según la vía de entrada y el órgano de contacto, la respuesta inmunitaria o de hipersensibilidad alérgica dará lugar a manifestaciones clínicas oculares (conjuntivitis), digestivas (alergias alimentarias) o respiratorias (asma y/o rinitis). Los alérgenos más frecuentemente relacionados con el asma son los que están presentes en el aire (aeroalérgenos o neumoalérgenos) y, al ser inhalados, ejercen sus efectos sobre la mucosa respiratoria, siendo los principales factores desencadenantes del asma y contribuyendo a la persistencia de síntomas en niños con asma establecida4. Los principales neumoalérgenos sensibilizantes en nuestro medio, con una población bastante significativa, son los pólenes (gramíneas y olivo), los árboles ornamentales (arizónicas y plátano de sombra), el epitelio de animales, los ácaros del polvo y los hongos
Allergens are substances, usually protein in nature, that are capable of inducing specific IgE antibodies in genetically predisposed individuals (atopic), but are innocuous in the remainder of the population. Depending on the route of entry and the organ of involved, the immune response or allergic hypersensitivity will give rise to clinical manifestations at the ocular level (conjunctivitis), in the gastrointestinal tract (food allergies) or in the respiratory tract (asthma and/or rhinitis). The allergens most frequently related to asthma are those present in air (Aeroallergens), which, when inhaled, exert their effects on the respiratory mucosa. They are the leading causes of asthma and contribute to the persistence of symptoms in children with established asthma. The main sensitizing aeroallergens among our general population, which is quite significant in number, are the pollens (grasses and olive), ornamental trees (cypress and shade plantain), animal dander, house dust mites and fungi
Assuntos
Masculino , Feminino , Criança , Humanos , Alérgenos/análise , Alérgenos/imunologia , Alérgenos/uso terapêutico , Asma/imunologia , Asma/prevenção & controle , Hipersensibilidade Imediata/complicações , Hipersensibilidade Imediata/diagnóstico , Imunoglobulina E/análise , Imunoglobulina E/imunologia , Imunoglobulina E/uso terapêutico , Imunização/métodos , Infestações por Ácaros/imunologia , Ácaros/imunologiaRESUMO
Las neumonías adquiridas en la comunidad (NAC) y otras infecciones neumónicas son uno de los problemas de salud más importantes que afectan a los niños de todo el mundo. Hay 4-5 millones de muertes anuales registradas en niños menores de 5 años, de forma especial en los países en vías de desarrollo, donde figuran entre las más frecuentes causas de morbimortalidad. No es ése el caso de nuestro país, donde sin embargo constituyen una de las causas de mayor morbilidad, por lo que el problema que representan no es menor. Quisiera agradecer a la Dirección de Acta Pediátrica Española su amabilidad por permitirnos exponer nuestra experiencia y una puesta al día de esta problemática infantil, cambiante en estos últimos años por la inmigración y el cambio epidemiológico, y quizás ecológico, que se está originado en nuestro medio por esta nueva situación
Community-acquired pneumonia is one of the most important health problems affecting children all over the world. Four to five million deaths are reported annually in children younger than 5 years of age, especially in developing countries, where it ranks among the most common causes of both mortality and morbidity. This is not the case in Spain, but it does represent one of the major causes of morbidity and, thus, can not be considered a minor problem. I wish to express my appreciation to the Editorial Board of Acta Pediátrica Española for enabling us to relate our experience and provide an update on this disease in childhood, which, in recent years, is being affected by immigration and the epidemiological and possibly ecological changes that this new situation may be originating
Assuntos
Masculino , Feminino , Lactente , Pré-Escolar , Criança , Humanos , Pneumonia/epidemiologia , Infecções Comunitárias Adquiridas/epidemiologia , Proteína C-Reativa/análise , Vírus Sincicial Respiratório Humano/isolamento & purificação , Vírus Sincicial Respiratório Humano/patogenicidade , Emigração e Imigração/tendências , Chlamydophila pneumoniae/patogenicidade , Chlamydia trachomatis/patogenicidade , Bordetella pertussis/patogenicidade , Diagnóstico DiferencialRESUMO
El síndrome de activación de macrófagos es una activación anómala del sistema histiocitario e indica una respuesta inadecuada del sistema inmunitario. Puede ser primario o reactivo; en los casos secundarios se asocia sobre todo con enfermedades de origen autoinmunitario, especialmente la artritis idiopática juvenil de inicio sistémico (AIJS). Su etiología es aún desconocida, pero se han implicado diferentes agentes infecciosos. Se define como un cuadro clínico agudo y grave de insuficiencia hepática, coagulopatía de consumo y encefalopatía, asociado con la presencia inconstante en la médula ósea de macrófagos activados con signos de hemofagocitosis. El diagnóstico y el tratamiento precoz mejoran el pronóstico de esta entidad potencialmente mortal. Presentamos el caso de una niña de 18 meses que había sido diagnosticada de AIJS 9 meses antes y que, tras una infección por varicela, desarrolló un cuadro de fiebre alta, con un empeoramiento progresivo de su estado general hasta desarrollar un fracaso multisistémico, y en la que los datos de la autopsia revelaron signos de hemofagocitosis
Macrophage activation syndrome is an abnormal activation of the histiocytic system, which reflects an inappropriate response of the immune system. It can be primary or reactive; in secondary cases, it is mainly associated with autoimmune disorders, most frequently with systemic onset juvenile rheumatoid arthritis (SOJRA). The etiology is still unknown, but several infectious agents have been implicated. It is defined as an acute clinical state of severe hepatic failure, disseminated intravascular coagulation and encephalopathy, associated with the variable presence in bone marrow of activated macrophages with signs of hemophagocytosis. Early diagnosis and treatment improve prognosis of this potentially lethal disorder. We report the case of an 18-month-old girl who had been diagnosed as having SOJRA 9 months earlier and developed a high fever associated with varicella infection, followed by progressive systemic deterioration leading to multiorgan failure. The pathological study revealed signs of hemophagocytosis
Assuntos
Feminino , Lactente , Humanos , Ativação de Macrófagos , Artrite Juvenil/fisiopatologia , Insuficiência Hepática/etiologia , Coagulação Intravascular Disseminada/etiologia , Histiocitose de Células não Langerhans/diagnóstico , Lesão Encefálica Crônica/etiologiaRESUMO
La tuberculosis es un problema de salud pública mundial. Más del 95% de los casos ocurren en países en vías de desarrollo; el continente africano es donde aparecen más casos, superior en número a cualquier otra región del mundo. En España, como en los países desarrollados, la mayoría de los casos se produce en grupos de riesgo (inmigrantes, niños con sida). En nuestro medio, parece que la incidencia de tuberculosis ha aumentado en los últimos años, motivo que nos ha inducido a efectuar una revisión clínica de la tuberculosis en la edad pediátrica
Tuberculosis is a worldwide public health problem. Over 95% of the cases occur in countries of the developing world, particularly Africa, which has the highest incidence of all the regions of the globe. In Spain, as in other developed nations, the majority of the cases are produced in risk groups (immigrants, children with acquired immunodeficiency syndrome). The incidence of tuberculosis among our general population appears to be increasing in recent years. This trend has led us to carry out a clinical review of tuberculosis in the pediatric age group