Structural and molecular study of the supraspinatus muscle of modern humans (Homo sapiens) and common chimpanzees (Pan troglodytes).

OBJECTIVES: To analyze the muscle architecture and the expression pattern of the myosin heavy chain (MyHC) isoforms in the supraspinatus of Pan troglodytes and Homo sapiens in order to identify differences related to their different types of locomotion. MATERIALS AND METHODS: We have analyzed nine supraspinatus muscles of Pan troglodytes and ten of Homo sapiens. For each sample, we have recorded the muscle fascicle length (MFL), the pennation angle, and the physiological cross-sectional area (PCSA). In the same samples, by real-time quantitative polymerase chain reaction, we have assessed the percentages of expression of the MyHC-I, MyHC-IIa, and MyHC-IIx isoforms. RESULTS: The mean MFL of the supraspinatus was longer (p = 0.001) and the PCSA was lower (p < 0.001) in Homo sapiens than in Pan troglodytes. Although the percentage of expression of MyHC-IIa was lower in Homo sapiens than in Pan troglodytes (p = 0.035), the combination of MyHC-IIa and MyHC-IIx was expressed at a similar percentage in the two species. DISCUSSION: The longer MFL in the human supraspinatus is associated with a faster contractile velocity, which reflects the primary function of the upper limbs in Homo sapiens-the precise manipulation of objects-an adaptation to bipedal locomotion. In contrast, the larger PCSA in Pan troglodytes is related to the important role of the supraspinatus in stabilizing the glenohumeral joint during the support phase of knuckle-walking. These functional differences of the supraspinatus in the two species are not reflected in differences in the expression of the MyHC isoforms.

Inter- and Intraspecific Variations in the Pectoral Muscles of Common Chimpanzees (Pan troglodytes), Bonobos (Pan paniscus), and Humans (Homo sapiens).

We have analyzed anatomic variations in the pectoralis major and pectoralis minor muscles of common chimpanzees (Pan troglodytes) and bonobos (Pan paniscus) and compared them to anatomic variations in these muscles in humans (Homo sapiens). We have macroscopically dissected these muscles in six adult Pan troglodytes, five Pan paniscus of ages ranging from fetus to adult, and five adult Homo sapiens. Although Pan troglodytes are thought to lack a separate pectoralis abdominis muscle, we have identified this muscle in three of the Pan troglodytes; none of the Pan paniscus, however, had this muscle. We have also found deep supernumerary fascicles in the pectoralis major of two Pan troglodytes and all five Pan paniscus. In all six Pan troglodytes, the pectoralis minor was inserted at the supraspinatus tendon, while, in Pan paniscus and Homo sapiens, it was inserted at the coracoid process of the scapula. Some of the anatomic features and variations of these muscles in common chimpanzees and bonobos are similar to those found in humans, therefore enhancing our knowledge of primate comparative anatomy and evolution and also shedding light on several clinical issues.

Ultrastructure of Lingual Papillae in Common Chimpanzee (Pan troglodytes) Foetus, Newborn and Adult Specimens.

Among primates, the two recognized species of chimpanzees (common chimpanzee, Pan troglodytes; pygmy chimpanzee, Pan paniscus) are considered to be the most similar to humans. Importantly, in mammals, the food intake behaviour largely determines the tongue morphology, including the type, proportion and distribution of gustatory and non-gustatory tongue papillae. The lingual papillae form during its development and mature in post-natal life depending on the different feeding. In this study, we have used scanning electron microscopy to analyse the age-related changes in the lingual papillae of foetal, newborn and adult P. troglodytes. Four main types of lingual papillae, denominated filiform, fungiform, foliate and vallate, and one subtype of filiform papillae called conical papillae, were found. The main age-related changes observed in all kinds of papillae were a progressive keratinization and morphological complexity along the lifespan. During the foetal period, there was scarce keratinization, which progressively increases in young animals to adulthood. The number of filiform increased with ageing, and both filiform and fungiform papillae in adult tongues are divided into pseudopapillae. On the other hand, the vallate papillae vary from smooth simple surfaces in foetal tongues to irregular surfaces with grooves and pseudopapillae (microscopic papilla-shaped formations within the papilla itself) in adults. These results describe for the first time the age-related variations in the three-dimensional aspect of lingual papillae of the chimpanzee tongue and provide new data to characterize more precisely these structures in the human closest specie.

Functional and comparative study of lingual papillae in four species of bear (Ursidae) by scanning electron microscopy.

The eight current species of bear (Ursidae) are widely distributed throughout Europe, Asia, and America. They are mainly encountered in the northern hemisphere, except for the spectacled bear and the sun bear, which are also found in the south of the equator. Adaptations of the masticatory apparatus (teeth, tongue, and musculature) to diet are one of the factors that imply the greatest structural changes in the cranium. This diet may be carnivorous, herbivorous, melliferous, or insectivorous, with one type of food predominating according to the time of year. The way in which food is eaten determines the morphology of the lingual surface; generally speaking, all bears put their mouth to the food, which, initially, they lick or they let the food stick to their tongue, as occurs when insects are eaten. As in all mammals, a distinction can be made between mechanical and gustatory papillae and the development and distribution of which depend on the species and their eating habits. In this study of the complete tongues of four species of adult bears, we describe the morphology of the lingual surfaces, the different types of papillae, their characteristics, and topographic distribution. It was seen that there were five main types of lingual papillae: filiform, conical, fungiform, foliate, and vallate. Morphology of the microgrooves and pores was similar to that observed in other mammals. In general, there were no great differences among the four species of bears studied, perhaps due to the similarity in the kind of food they consume in captivity.

Adrenalectomía y cirugía mayor ambulatoria. ¿Es posible? / No disponible

Objetivo: El propósito de nuestro estudio fue determinar la factibilidad y seguridad de un programa de adrenalectomía laparoscópica (AL) en régimen ambulatorio (CMA) en el Hospital General de Ciudad Real (HGCR).Material y método: Realizamos una revisión retrospectiva de las adrenalectomías realizadas en el HGCR desde enero de 2006a junio de 2008. La morbilidad y las características de las mas asadrenales extirpadas fueron evaluadas, al igual que la estancia hospitalaria y las complicaciones postoperatorias para ver si podrían haber sido incluidos en un programa de CMA según estrictoscriterios de selección. Resultados: Se realizaron 16 adrenalectomías durante esteperiodo; 12 por vía laparoscópica. Aplicando los criterios de Gill para cirugía ambulatoria, el grupo de candidatos consistiría en 6 pacientes que presentaban las siguientes características: la edad media fue de 51,67 años (44-65), 3 eran funcionantes y 3 incidentalomas. El ratio izquierdas/derechas fue 2:1. El tamaño tumoral medio fue de 3,75 cm (1-5 cm). En todos los casos se practicó AL transperitoneal lateral con tiempo operatorio medio de 83 minutos(30-150 minutos). No se registraron complicaciones postoperatorias. La estancia media fue de 2,83 días (2-3 días), con requerimientos de analgesia oral habitual durante 1-3 días. Conclusión: Nuestros resultados muestran que aplicando estrictos criterios de selección, es seguro y factible iniciar un programa ambulatorio de adrenalectomías por cirujanos expertos en técnicas laparoscópicas avanzadas, con alta satisfacción y nula morbilidad. Aún se necesitan estudios controlados que propicien criterios adecuados para unos resultados óptimos (AU)

Objective: The purpose of our study was to determine the feasibility and safety of a program for laparoscopic adrenalectomy(LA), on an ambulatory basis, at the General Hospital of Ciudad Real (HGCR).Material and method: A retrospective study was undertaken from January 2006 to June 2008. Co-morbidity and characteristics of the adrenal masses to be excised were assessed, as well as hospital length of stay and postoperative complications, to see whether they could have been included in an outpatient program according to strict selection criteria. Results: During the study period 16 adrenalectomies were performed at our institution; 12 of them by laparoscopy. Applying Gill’s criteria for outpatient surgery, the group of candidates consisted of 6 patients who met the following characteristics: mean age was 51.67 years (44-65), 3 tumours were functioning and the other 3 incidental findings. The ratio left/right was 2:1.The mean tumour size was 3.75 cm (1-5 cm). All cases underwent a lateral transperitoneal LA approach, the mean operative time was 83 minutes (30-150). No postoperative complications occurred.The average length of stay was 2.83 days (2-3), with regularoral analgesia requirements for 1-3 days.Conclusion: Our results show that, by applying strict selectioncriteria, it would be safe and feasible to under go an ambulatory program of adrenalectomy by experienced surgeons in advanced laparoscopic techniques, achieving high patients’ satisfaction and no morbidity. Controlled studies are still required to provide appropriate criteria for optimal results (AU)

Neumonías neonatales / Neonatal pneumonia

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)

Neumonías virales / Viral pneumonia

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)

Neumonía estafilocócica / Staphylococcus aureus pneumonia

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)

Morphological study of the lingual papillae of the giant panda (Ailuropoda melanoleuca) by scanning electron microscopy.

Due to the scarcity of giant pandas, there are few descriptions of their morphology and even fewer of their microscopic anatomy and the ultrastructure of their organs. In this study of the complete tongue of an adult male giant panda, we describe the morphology of its lingual surface, the different types of papillae, their characteristics and topographic distribution. It was seen that there are four main types of lingual papillae: filiform, conical, fungiform and vallate. There was no sign of foliate papillae, tuberculum intermolare or sublingua. Papilla distribution was not limited to the dorsum of the tongue, but was also seen on the anterior and ventral surfaces of the tongue. In the anterior third of the midline there is a smooth area with no papillae at all. Morphology of the microgrooves and pores is similar to that observed in other mammals. The papillae share characteristics encountered in Carnivora and herbivorous species of mammals. A narrow bamboo-based diet and specialized manner of eating have together resulted in modification of the tongue of a carnivoran, giving it some characteristics typical of an herbivore.

Enfoque terapéutico de las neumonías en la infancia / General management of pneumonia in childhood

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)

Hipercolesterolemia en la infancia y la adolescencia / Hypercholesterolemia during childhood and adolescence

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)

Neumonías adquiridas en la comunidad / Community-acquired pneumonia

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

Prevalencia de la sensibilización a neumoalérgenos en nuestro medio / Prevalence of sensitization to aeroallergens in our general population

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

Síndrome de activación de macrófagos y artritis juvenil de inicio sistémico / Macrophage activation syndrome and juvenile rheumatoid arthritis

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

[Mycobacterium marinum infection. Case report and review of the literature]. / Infección por Mycobacterium marinum. Presentación de un nuevo caso y revisión de la literatura.

Atypical mycobacterial infections are increasingly important in immunosuppressed patients as well as in healthy hosts. The atypical mycobacterium that most commonly affects the skin is Mycobacterium marinum. The infection should be suspected upon the presence of ulcers, nodules or chronic plaques and a history of contact with fresh or salt water. Optimal therapy is yet to be established. We report a case of Mycobacterium marinum infection in a patient receiving immunosuppressive therapy that responded favourably to treatment with doxicycline. We review the different antibiotic regimens prescribed in the past years for the treatment of Mycobacterium marinum infection.

Infección por Mycobacterium marinum. Presentación de un nuevo caso y revisión de la literatura / Mycobacterium marinuminfection. Case report and review of the literature

Las infecciones por micobacterias atípicas están adquiriendo cada vez mayor importancia en los pacientes inmunodeprimidos, así como en huéspedes sanos. El Mycobacterium marinum es la micobacteria atípica que afecta la piel con mayor frecuencia. Debe sospecharse ante la existencia de úlceras, nódulos o placas crónicas y el antecedente de un contacto con medios acuáticos. El tratamiento óptimo no está aún bien establecido. Presentamos un nuevo caso de infección por Mycobacterium marinum en un paciente que seguía tratamiento con fármacos inmunosupresores, que respondió favorablemente al tratamiento con doxiciclina y revisamos los distintos regímenes antibióticos utilizados para el tratamiento de la infección por Mycobacterium marinum en los últimos años

Atypical mycobacterial infections are increasingly important in immunosuppressed patients as well as in healthy hosts. The atypical mycobacterium that most commonly affects the skin is Mycobacterium marinum. The infection should be suspected upon the presence of ulcers, nodules or chronic plaques and a history of contact with fresh or salt water. Optimal therapy is yet to be established. We report a case of Mycobacterium marinum infection in a patient receiving immunosuppressive therapy that responded favourably to treatment with doxicycline. We review the different antibiotic regimens prescribed in the past years for the treatment of Mycobacterium marinum infection

Tuberculosis (II): diagnóstico y tratamiento / Tuberculosis: diagnoses and treatment

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

Estado actual de la tuberculosis (I) / Current status of tuberculosis

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

Neurofibromatosis of atypical presentation.

Neurofibromatosis (NF) is considered to be a heterogeneous neuroectodermal disease clinically defined by the presence of neurofibromas, multiple café-au-lait spots, intertriginous freckles and Lisch nodules. Mosaicism explains atypical presentations of the disease. Early mutations, before tissue differentiation, give rise to generalized disease. We report an atypical presentation of neurofibromatosis with an unusual distribution of neurofibromas, a peculiar, clinically and pathologically, neurofibroma on the trunk and the association with an ovarian serous cystoadenofibroma.

Óxido nítrico exhalado en niños asmáticos tratados con corticoides inhalados / Exhaled nitric oxide in asthmatic children treated with inhaled corticosteroids

Objetivos: Estudiar los valores de óxido nítrico exhalado (ONE), la evolución clínica y la función pulmonar en una población de niños con asma episódica frecuente de nuevo diagnóstico, antes y después de recibir tratamiento con corticoides inhalados durante 8 semanas. Material y métodos: Estudio prospectivo de ámbito hospitalario en el que se analizaron distintos parámetros en un grupo de 21 niños asmáticos: 1. valores de ONE, mediante la técnica en T de exhalación lenta contra resistencia; 2. clínicos, evaluados mediante encuesta: episodios de sibilancias y síntomas nocturnos a la semana, y número de semanas que transcurrían entre las exacerbaciones asmáticas; 3. función pulmonar mediante espirometría. Resultados: Tras el tratamiento se constató: 1. las cifras de ONE disminuyeron de 22,47 antes del tratamiento a 7,95 ppb tras éste (p <0,0001); 2. mejoría clínica de los pacientes: disminución de los episodios de sibilancias de 2,68 días a la semana antes del tratamiento a 0,25 tras él (p <0,012); síntomas nocturnos de 2,45 días a la semana a 0,13 días (p <0,039); de una exacerbación asmática cada 3,64 semanas a una cada 7,94 (p <0,001); y 3. la función pulmonar mejoró en todos los parámetros evaluados FVC, FEV1, y FEF25-75%. Conclusiones: Los corticoides inhalados disminuyen los valores elevados de ONE en pacientes asmáticos. La medición de la concentración de ONE puede ser un medio complementario para monitorizar la inflamación en el tracto respiratorio inferior, y así poder valorar la eficacia de tratamientos antinflamatorios como los corticoides inhalados

Objectives. To study the exhaled nitric oxide (NO) levels, the clinical course and pulmonary function in a population of children with newly diagnosed asthma, before and after 8 weeks of treatment with inhaled corticosteroids. Material and methods. A prospective, hospital-based study was carried out to analyze different parameters in a group of 21 asthmatic children: the exhaled NO level, analyzed by the T-piece technique, which involves exhaling against expiratory resistance; clinical symptoms were evaluated by means of a survey to establish the number of episodes of wheezing and nocturnal symptoms a week and number of weeks between asthma exacerbations, and pulmonary function by spirometry. Results: Exhaled NO concentrations decreased from 22.47 ppb before treatment to 7.95 ppb afterwards (p <0.0001). Clinical symptoms improved after treatment. The frequency of episodes of wheezing decreased from 2.68 days a week before treatment to 0.25 after treatment (p <0.012), and nocturnal symptoms decreased from 2.45 days a week before treatment to 0.13 days (p <0.039). The children went from presenting an asthma exacerbation every 3.64 weeks before treatment to one every 7.94 weeks (p <0.001). Pulmonary function parameters (FVC, FEV1, MMEF25-75) improved after treatment. Conclusions: Inhaled corticosteroids decrease exhaled nitric oxide in asthmatic children. The measurement of expired NO concentrations can be used to monitor bronchial inflammation and, therefore, to evaluate the efficacy of antiinflammatory treatments such us inhaled corticosteroids