Sangrado por el pezón en un lactante / Bleeding nipple in a infant

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[An association between moya-moya disease and morning glory anomaly]. / Asociación entre enfermedad de moya moya y anomalía de morning glory.

INTRODUCTION: The morning glory disc anomaly is a not very frequent congenital alteration of the optic papilla associated with a retinal vascular dysgenesia. Several cases of association have been described between this disc anomaly and the presence of Moyamoya vessels. We report the case of a boy with this association. CASE REPORT: A 5 year-old boy was evaluated for several episodes of disconnection, some of them preceded by intense headaches and associated to incoherent language, and in other occasions with hypotonia and unmotivated laugh of variable duration. The physical and neurological exploration was normal, except for the optic disc papilla of the right eye that was very big and dug, compatible with Morning glory disc anomaly. No alterations were found at cranial CT and EEG. In the magnetic resonance imaging were visualized multiple small round low intensity areas in the basal ganglia, because of the high number of collateral vessels. The angioRM showed stenosis of the supraclinoid portions of both internal carotids as well as the proximal segments of the anterior cerebral artery and the left middle cerebral artery with abnormal network of collateral vessels at the base of the brain like a puff of smoke . This is a typical imaging for Moyamoya disease. CONCLUSIONS: In all patient with Morning glory disc anomaly, mainly if they presents neurological clinical features, the realization of angioRM studies will be evaluated, to discard the existence of Moyamoya disease.

Asociación entre la enfermedad de moya-moya y la anomalía de morning glory / An association between moya-moya disease and morning glory anomaly

Introducción. La anomalía de morning glory (AMG) es una alteración congénita de la papila óptica poco frecuente, que se asocia a una disgenesia vascular retiniana. Se han descrito varios pacientes que presentan esta entidad y alteraciones cerebrovasculares compatibles con la enfermedad de moya-moya (EMM). Presentamos el caso de un niño con esta asociación. Caso clínico. Niño de 5 años, que consultó por haber presentado varios episodios de desconexión con el medio, en algunos casos precedidos de cefalea intensa y asociados a lenguaje incoherente, y, en otras ocasiones, a hipotonía y risa inmotivada, de duración variable. La exploración física y la neurológica fueron normales, salvo la papila del ojo derecho, que era muy grande y excavada, compatible con AMG. La tomografía computarizada craneal y el EEG fueron normales. En la resonancia magnética (RM) se evidenciaron múltiples imágenes puntiformes de vacío de señal en los núcleos de la base sugerentes de aumento de la vascularización y la angio-RM mostró estenosis en la porción distal de ambas carótidas internas, así como en los segmentos proximales de la arteria cerebral anterior y la arteria cerebral media izquierda, con un aumento de la circulación colateral en la zona de los núcleos de la base, compatible con la típica imagen en `volutas de humo', que confirmó el diagnóstico de EMM. Conclusión. En todo paciente diagnosticado de AMG, sobre todo si presenta sintomatología neurológica, se deberá valorar la realización de estudios de angio-RM, para descartar la existencia de EMM (AU)

Introduction. The morning glory disc anomaly is a not very frequent congenital alteration of the optic papilla associated with a retinal vascular dysgenesia. Several cases of association have been described between this disc anomaly and the presence of Moyamoya vessels. We report the case of a boy with this association. Case report. A 5 year-old boy was evaluated for several episodes of disconnection, some of them preceded by intense headaches and associated to incoherent language, and in other occasions with hypotonia and unmotivated laugh of variable duration. The physical and neurological exploration was normal, except for the optic disc papilla of the right eye that was very big and dug, compatible with Morning glory disc anomaly. No alterations were found at cranial CT and EEG. In the magnetic resonance imagine were visualized multiple small round low intensity areas in the basal ganglia, because of the high number of collateral vessels. The angioRM showed stenosis of the supraclinoid portions of both internal carotids as well as the proximal segments of the anterior cerebral artery and the left middle cerebral artery with abnormal network of collateral vessels at the base of the brain like a ‘puff of smoke’. This is a typical imagine for Moyamoya disease. Conclusions. In all patient with Morning glory disc anomaly, mainly if they presents neurological clinical features, the realization of angioRM studies will be evaluated, to discard the existence of Moyamoya disease (AU)

Infecciones virales de vías respiratorias inferiores en lactantes hospitalizados: etiología, características clínicas y factores de riesgo / Viral infection of the lower respiratory tract in hospitalized infants: etiology, clinical features and risk factors

Objetivos: Analizar las características clínicas y virológicas de las infecciones del tracto respiratorio inferior (ITRI) de los lactantes hospitalizados en nuestro medio y encontrar parámetros clínicos, analíticos o radiológicos que se relacionen, en el momento del ingreso, con una evolución más grave. Pacientes y métodos: Se realizó un estudio prospectivo de los niños menores de 24 meses ingresados por ITRI durante seis temporadas epidemiológicas consecutivas. Resultados: Se estudiaron 617 lactantes de los cuales el 64% presentaba bronquiolitis; el 24,6%, bronquitis espástica; el 4,4%, laringitis, y el 6,8%, neumonía. La edad media fue de 269 ± 188 días, la razón varón/mujer de 1,6 y la estancia media 6,6 ± 3,5 días. Se aisló al menos un agente viral en el 55,6% de los episodios, que en el 83,6% correspondió al virus respiratorio sincitial (VRS). Otros virus aislados con menor frecuencia fueron: para influenza (7 %), adenovirus (4,3%) e influenza (4%). Se detectaron coinfecciones en el 6,2% ciento de los casos VRS( ). Los niños VRS( ) tuvieron menor edad (p 38,5 °C y concentraciones de proteína C reactiva > 30 mg/l (p 6 (p < 0,003), SaO2 87 por ciento (p < 0,01) y aislamiento de VRS (p < 0,001). En el análisis multivariado sólo la SaO2 87% y la presencia de VRS se asociaron con hospitalización de más de 5 días. Conclusión: Las ITRI más frecuentes del lactante hospitalizado en nuestro medio son las bronquiolitis VRS( ), con características clínicas similares a las descritas en otros países. La hipoxia al ingreso y el aislamiento de VRS en aspirado nasofaríngeo son los factores de riesgo más importantes de hospitalización prolongada (AU)

[Viral infection of the lower respiratory tract in hospitalized infants: etiology, clinical features and risk factors]. / Infecciones virales de vías respiratorias inferiores en lactantes hospitalizados: etiología, características clínicas y factores de riesgo.

OBJECTIVES: The aim of this study was to assess the clinical and virological characteristics of lower respiratory tract (LRT) infection in hospitalized infants in Spain and to identify clinical, radiological or laboratory parameters that could, on admission, be associated with a more severe clinical course. PATIENTS AND METHODS: A prospective study of infants less than 24 months old hospitalized for LRT infection during six consecutive seasons was performed. RESULTS: A total of 617 infants were included in the study. Diagnosis was bronchiolitis in 64 %, wheezy bronchitis in 24.6 %, laryngitis in 4.4 % and pneumonia in 6.8 %. The mean age was 269 188 days, the male/female ratio was 1:6 and the mean hospital stay was 6.6 3.5 days. At least one viral agent was identified in 55.6 % of the episodes, of which 83.6 % were due to respiratory syncytial virus (RSV). Other less frequently identified viruses were parainfluenza in 7 %, adenovirus in 4.3 % and influenza in 4 %. Coinfection was identified in 6.2 % of RSV-positive infants. These infants were younger (p < 0.005), had higher respiratory distress assessment instrument (RDAI) scores and longer hospital stay than infants in the other etiologic groups. Chest radiographs were performed in 94.3 % of the infants and 39.5 % showed infiltrate or atelectasis. This radiological alteration was significantly associated with a fever of more than 38.5 C and reactive C protein concentrations of more than 30 mg/L (p < 0.001 and p < 0.002), but not with higher RDAI score, SaO2 equal to or less than 87 %, or longer hospital stay. In the crude analysis, hospitalization for more than 5 days was associated with lower age (p < 0.01), a mean RDAI score of more than 6 (p < 0.003), SaO2 equal to or less than 87 % (p < 0.01) and RSV infection (p < 0.001). However, in the multivariate analysis only SaO2 equal to or less than 87 % and RSV infection were significantly associated with prolonged hospitalization. CONCLUSION: The most common lower respiratory tract infections in hospitalized infants in Spain are the various types of RSV-positive bronchiolitis, which have a clinical pattern similar to that described in other countries. Hypoxia on admission and RSV infection are the most important risk factors for prolonged hospitalization.

[Benign transient hyperphosphatasemia: the contribution of 20 new cases]. / Hiperfosfatasemia transitoria benigna: aportación de 20 nuevos casos.

We report 20 children diagnosed with transient benign hyperphosphatasemia (THI) during a period of 2 years. The mean age was 29.3 months, ranging from 3 months to 10.2 years, with a male predominance (1.5:1). There were several associated disorders, with the most frequent being acute gastroenteritis, failure to thrive and asthma, the mean alkaline phosphatase (AP) value was 4,137 +/- 2,624 U/L (mean +/- SD). All AP values were above the 97th percentile for each age, with a mean of 3.8 times the level in the 97th percentile for every one-year period (between 0 and 14 years), 6.2 times the 50th percentile mean level and 14.8 times higher than the mean normal upper limit value for adults. The range was from 1,199 to 9,950 U/L. Their serum AP levels returned to normal in a mean period of 3.16 months (1 to 6 months) i 18 cases. The remaining 2 cases are pending on a new checkup. There was no seasonal predominance and the frequency was uniform throughout the year In 2 cases the onset age was more than 5 years and in another 2 patients follow-up serum AP levels did not return to reference values within 4 months. THI is a benign and self-limited entity. We think that the age and duration limits proposed by Kraut may be too rigorous; hence, it would be advisable to review these criteria.

[A score for the differential diagnosis of bacterial and viral meningitis]. / Score para el diagnóstico diferencial entre meningitis bacteriana y viral.

The purpose of this study was to verify the statistical validity of the score proposed by Thomé et al. for the differential diagnosis between bacterial and viral meningitis and to study the utility of two new parameters (CRP and the patient's age). A retrospective review of 136 consecutive cases of meningitis was made. The cases were classified into three groups according to the culture results and the use or not of intravenous antibiotics. There were 20 cases of bacterial meningitis, 60 non-bacterial and 56 cases of meningitis of uncertain etiology. Considering only the patients in the first two groups, the capacity for differential diagnosis between bacterial and non-bacterial meningitis of the 8 parameters in the original score, the CRP and the patient's age was analyzed with a numeric value of 0, 1, or 2 assigned to each parameter. Finally, the results of applying the score that includes the two new parameters with the original score are compared. We found that all parameters showed statistical significance for the differential diagnosis between bacterial and viral meningitis. The resulting score can be used in order to decide the need for intravenous antibiotic therapy, with only a few cases being uncertain. The score with the two new parameters correctly classified 78 of the 80 cases, while leaving only two case uncertain as compared to the four that remained unclassified with the original score. We conclude that the score is a useful instrument in the differential diagnosis between bacterial and viral meningitis. Furthermore, the introduction of CRP and the patient's age improves the diagnostic value of the test.

[Familial erythrophagocytic lymphohistiocytosis with neonatal presentation]. / Linfohistiocitosis eritrofagocítica familiar de presentación neonatal.

A neonatal case of familial erythrophagocytic lymphohistiocytosis is presented. Evolution was rapidly fatal under a course in which fever, hepatosplenomegaly, pancytopenia, severe coagulation alteration and jaundice were especially significant. Pathology findings after nine days of life, were all conclusive. Diagnostic difficulties of this uncommon entity in its' congenital form only once previously reported, are emphasized.

[Lumbosacral agenesis. Presentation of one case. Literature review and embriological considerations (author's transl)]. / Agenesia lumbosacra. Presentación de un caso. Revisión de la literatura y consideraciones embriológicas.

A case of a complete lumbosacral agenesis is presented, whose etiological factors are unknown. Clinically, motor deficit corresponded to the agenesis level, but the sensibility and the autonomous nervous system functions, which were conserved, partially, in lower levels than the normal ones in the agenesis. The case report is completed with electromiography, mielography, rectal biopsy, urography, cistography, opaque enema and inborn infection study. There are three facts: a partially conserved sensibility in the lower extremities, normal bladder function and above all, the presence of nervous parasympatics normal fibers in rectum wall, in contrast with the serious motor alteration just in the same medullar levels. Explanation of these facts suggest origin of an embryological source. It is assumed that the noxa must have acted between the third and the fourth weeks of pregnancy at a caudal cordomesoblast level, supposing that it did not affect neural crest.