Congenital cytomegalovirus infection: the state of the art and future perspectives.

Congenital cytomegalovirus (cCMV) infection is the most common congenital infection, with an estimated incidence of approximately one in 200 infants in high-income settings. Approximately one in four children may experience life-long consequences, including sensorineural hearing loss and neurodisability. Knowledge regarding prevention, diagnosis, and treatment increased in the recent years, but some challenges remain. In this review, we tried to summarize the current knowledge on both the obstetrical and pediatric areas, while also highlighting controversial aspects and future perspectives. There is a need to enhance awareness among the general population and pregnant women through specific information programs. Further research is needed to better define the classification of individuals at birth and to have a deeper understanding of the long-term outcomes for so defined children. Finally, the availability of valaciclovir medication throughout pregnancy, where appropriate, has prompted the assessment of a universal serological antenatal screening. It is recommended to establish a dedicated unit for better evaluation and management of both mothers and children.

Factores de mortalidad en niños con tosferina maligna / Mortality risk factors in children with severe pertussis

INTRODUCCIÓN: La hiperleucocitosis y la hipertensión pulmonar son factores de riesgo de mortalidad en niños con tosferina maligna. Las opciones terapéuticas disponibles para estos casos graves no se encuentran bien establecidas. Población y métodos: Se diseñó un estudio ambispectivo que incluía a niños diagnosticados de tosferina ingresados en una Unidad de Cuidados Intensivos Pediátricos (UCIP) de un hospital de tercer nivel en España entre enero de 2007 y octubre de 2015. Se compararon variables clínicas y demográficas entre el grupo de niños que sobrevivieron (grupo de supervivientes [GS]) y los que finalmente fallecieron (grupo exitus [EG]). RESULTADOS: Se identificaron un total de 31 pacientes. La mortalidad global fue del 19% (6/31 pacientes). Cinco niños fueron diagnosticados de hipertensión pulmonar. Cinco de seis niños que finalmente fallecieron precisaron canulación en oxigenación por membrana extracorpórea (ECMO). Ocho pacientes recibieron terapia mediante exanguinotransfusión (ET). La mediana de leucocitos antes de la realización de ET fue mayor (81.300 cél./μL) en EG que en GS (57.400 cél./μL), p= 0,05. Los pacientes que fallecieron tuvieron un mayor recuento pico de leucocitos totales, linfocitos, neutrófilos y niveles de proteína C reactiva (PCR) que los niños que sobrevivieron. Las variables que se identificaron como factores de riesgo de mortalidad fueron: una frecuencia cardiaca mayor de 170 lpm (OR 18; IC del 95%: 1,7-192,0), la presencia de neumonía (OR 16,5; IC del 95%: 1,7-165) y la presencia de hipertensión pulmonar (OR 179,6 [6,4-5.027]). CONCLUSIÓN: El uso de variables sencillas como la frecuencia cardiaca, el recuento total de leucocitos o los valores de PCR pueden servir para identificar de forma precoz a pacientes con riesgo de hipertensión pulmonar y tosferina maligna, de forma que procedimientos invasivos como la ET puedan utilizarse de una forma más precoz

BACKGROUND: Hyperleukocytosis and pulmonary hypertension are risk factors for death in infants with severe pertussis. Treatment options in severe pertussis are not well-established. METHODS: We designed an ambispective study of children with pertussis admitted to the pediatric intensive care unit (PICU) of a tertiary level hospital in Spain from January 2007 to October 2015. Clinical and demographical variables were compared between the group of children who survived (survivors group or SG) and those children who died (exitus group or EG). RESULTS: Thirty-one children were identified. Overall mortality rate was 19% (6/31 patients). Five children had pulmonary hypertension. Five out of 6 infants who eventually died had been placed on ECMO. Eight infants needed exchange transfusion (ET). Median leukocyte count immediately before exchange transfusion was higher (81300 cél./μL) in EG than in SG (57400 cél./μL), p= 0.05. Children who died had higher peak values in white blood cell counts (WBC), lymphocyte count, neutrophil counts and PCR levels than children who survived. The following variables were associated with risk of death: a heart rate above 170 bpm (OR 18, CI 95%: 1.7-192,0), the presence of pneumonia (OR 16.5, CI 95%: 1.7-165) and pulmonary hypertension (OR 179,6 [6,4-5027]. CONCLUSION: Early identification of patients at risk for pulmonary hypertension and fatal pertussis using heart rate, WBC and PCR levels would be appropriate so that invasive procedures such as exchange transfusion could be carried out precociously

El parto domiciliario a debate / Home births under debate

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Cribado de citomegalovirus en prematuros menores de 1.500 g. Comité Científico del Registro Estatal de Infección Congénita por Citomegalovirus / Cytomegalovirus screening in less than 1500 g premature newborns. National Congenital Cytomegalovirus Disease Registry Scientific committee

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Presencia de los familiares durante la realización de procedimientos invasivos / Presence of family members while performing invasive procedures. A prospective study

Introducción: En nuestro medio tradicionalmente no se ha permitido a los familiares de los pacientes permanecer junto al niño cuando se realizaban procedimientos invasivos. Objetivo: Evaluar el grado de satisfacción de los familiares, el personal sanitario y del propio paciente con la presencia de los familiares durante la realización de los procedimientos dolorosos en un servicio de urgencias pediátricas. Material y métodos: Se realizó un estudio observacional prospectivo. Se diseñó un protocolo de actuación y se instruyó al personal sanitario. Se diseñó una encuesta con datos demográficos, datos del procedimiento y grado de satisfacción tanto del paciente y su familiar como del profesional sanitario. Resultados: Se obtuvieron datos de 75 procedimientos. En 5 de ellos los familiares rechazaron la opción de estar presentes. Los más frecuentes fueron punciones lumbares (44%), sutura/cura de heridas (22,7%) y venopunciones (17,3%). El 100% de los niños quisieron que sus familiares estuvieran presentes. El 90% de los familiares y el 57% de los profesionales opinaron que la presencia de los familiares había facilitado el procedimiento. El 90% de los familiares y el 76% de los profesionales opinaron que había sido beneficioso para el niño. El 95% de los familiares y el 71% de los profesionales opinaron que se debería dar la opción a los familiares de estar presentes. El 73% de los profesionales quedaron satisfechos. En una escala del 1 al 10 la satisfacción global de los familiares fue de un 9,5. Conclusiones: En nuestra experiencia la presencia familiar es una práctica posible que facilita la realización de los procedimientos dolorosos y resulta beneficiosa para el niño. Encontramos una alta satisfacción familiar y al mismo tiempo una amplia aceptación por parte del personal sanitario(AU)

Introduction: Family members of child patients have traditionally not been allowed to be present during invasive procedures. Objectives: To evaluate the level of satisfaction of family members, healthcare professionals, and the patients themselves, when family members are present during invasive procedures carried out in the pediatric emergency department. Materials and methods: A prospective observational study was carried out, which included a questionnaire containing demographic information, the details of the procedure, and the level of satisfaction of the patient, their family members, and the healthcare professionals present. Results: Data was obtained from 75 procedures. In 5 of these, family members chose not to be present during the procedure. The most frequent procedures were lumbar punctures (44%), laceration repairs (22,7%) and venopunctures (17,3%). All (100%) the children who were asked wanted their family members to be present. 90% of family members and 57% of healthcare professionals were of the opinion that the presence of family members facilitated the procedure. Furthermore, 90% of family members and 76% of healthcare professionals thought that family presence was beneficial to the patient. 95% of family members and 71% of healthcare professionals thought that the option to be present during invasive procedures should be given to family members. 73% of healthcare professionals were satisfied with the presence of family members. On a scale of one to ten, overall satisfaction of family members was 9.5.Conclusions: In our experience, family presence during invasive procedures is possible, and we have found this to be beneficial to the child. We also found that both family members and healthcare professionals were accepting and also satisfied with this new practice policy(AU)

Carga viral indetectable tras la adición de raltegravir al tratamiento de una adolescente con virus de la inmunodeficiencia humana embarazada de 36 semanas y en fracaso virológico / Undetectable viral load after the addition of raltegravir in a 36 week pregnant adolescent with high-level HIV viraemia

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[Presence of family members while performing invasive procedures. A prospective study]. / Presencia de los familiares durante la realización de procedimientos invasivos.

INTRODUCTION: Family members of child patients have traditionally not been allowed to be present during invasive procedures. OBJECTIVES: To evaluate the level of satisfaction of family members, healthcare professionals, and the patients themselves, when family members are present during invasive procedures carried out in the pediatric emergency department. MATERIALS AND METHODS: A prospective observational study was carried out, which included a questionnaire containing demographic information, the details of the procedure, and the level of satisfaction of the patient, their family members, and the healthcare professionals present. RESULTS: Data was obtained from 75 procedures. In 5 of these, family members chose not to be present during the procedure. The most frequent procedures were lumbar punctures (44%), laceration repairs (22,7%) and venopunctures (17,3%). All (100%) the children who were asked wanted their family members to be present. 90% of family members and 57% of healthcare professionals were of the opinion that the presence of family members facilitated the procedure. Furthermore, 90% of family members and 76% of healthcare professionals thought that family presence was beneficial to the patient. 95% of family members and 71% of healthcare professionals thought that the option to be present during invasive procedures should be given to family members. 73% of healthcare professionals were satisfied with the presence of family members. On a scale of one to ten, overall satisfaction of family members was 9.5. CONCLUSIONS: In our experience, family presence during invasive procedures is possible, and we have found this to be beneficial to the child. We also found that both family members and healthcare professionals were accepting and also satisfied with this new practice policy.

Documento de Consenso de la Sociedad de Infectología Pediatrica y la Sociedad de Reumatología Pediatrica sobre el diagnóstico diferencial y el abordaje terapéutico de la fiebre recurrente / Consensus Document on the differential diagnosis and therapeutic approach to recurrent fever by the Paediatric Infectology Society and the Paediatric Rheumatology Society

La fiebre recurrente es un problema relativamente frecuente en la infancia. En la mayoría de las ocasiones es sencillo establecer su etiología generalmente asociada a episodios infecciosos banales. No obstante, en un pequeño porcentaje de casos estos episodios se deben a procesos de causa no infecciosa a menudo de complejo diagnóstico. En este documento se analiza el diagnóstico diferencial de la fiebre recurrente o periódica frente a otros procesos, con especial atención a las enfermedades autoinflamatorias (EA). Las EA son alteraciones de la inmunidad innata recientemente incluidas dentro de las inmunodeficiencias, sin embargo no se caracterizan por presentar infecciones lo que las diferencia de las inmunodeficiencias clásicas. Un importante número de las EA tienen una base genética conocida. La sintomatología que ocasionan se deriva de una inflamación sistémica que puede dar clínica y procesos muy variados. Uno de los grupos mejor conocidos es el formado por los síndromes hereditarios de fiebre periódica. Este grupo se caracteriza por presentar fiebre recurrente, asociada a diversos síntomas, con una relativa periodicidad y con intervalos libres o casi libres de síntomas. Para algunas de las entidades más frecuentes se dispone de criterios diagnósticos que son aquí recogidos, así como las características que deben hacernos iniciar el estudio genético. El tratamiento debe ser individualizado dada la complejidad de estos cuadros si bien se pueden dar algunas recomendaciones generales (AU)

Recurrent fever is a relatively common problem during childhood. Diagnosis is often easy and related to mild viral infections. However a small proportion of these cases originate from an underlying non-infectious process that is generally difficult to diagnose. In this paper we describe the differential diagnosis of recurrent or periodic fever versus other processes, with especial attention to autoinflammatory disorders (AD). AD are alterations of innate immunity, and they have been recently classified as an immunodeficiency. Anyhow, since infections are not present, these processes are different to the classic primary immunodeficiency. An important part of AD is of known genetic aetiology. The symptoms originate from an underlying inflammatory process and can have different clinical expressions. One of the most relevant groups is the hereditary syndromes of periodic fever. This group of diseases associates recurrent fever and several clinical symptoms with a relative periodicity, separated by intervals free or almost free of symptoms. We include the diagnostic criteria for some processes as well as the characteristics that should, eventually, lead to a genetic study. Although treatment should be individualised, we also include some general recommendations (AU)

Lactante con exantema maculopapuloso / Infant with maculopapular exanthema

La sarna es una infestación frecuente de la piel, caracterizada por la aparición de un exantema pruriginoso. El diagnóstico es fundamentalmente clínico, a partir de la historia clínica y la distribución de las lesiones. Ante un niño con un exantema pruriginoso es esencial sospechar la posibilidad de una sarna a la hora de establecer un correcto diagnóstico diferencial. Presentamos un caso con imágenes de una presentación atípica de esta ectoparasitosis (AU)

Scabies is a frequently infestation of the skin characterized by the appearance of a pruritic exanthema. The diagnosis is generally made from the history and the distribution of lesions. When we are in the presence of a child with a pruritic exanthema it is vital to suspect the possibility of scabies when establishing a correct differential diagnosis. We would like to present a case with images of an infant with atypical presentation of this ectoparasitoses (AU)

[Consensus Document on the differential diagnosis and therapeutic approach to recurrent fever by the Paediatric Infectology Society and the Paediatric Rheumatology Society]. / Documento de Consenso de la Sociedad de Infectología Pediatrica y la Sociedad de Reumatología Pediatrica sobre el diagnóstico diferencial y el abordaje terapéutico de la fiebre recurrente.

Recurrent fever is a relatively common problem during childhood. Diagnosis is often easy and related to mild viral infections. However a small proportion of these cases originate from an underlying non-infectious process that is generally difficult to diagnose. In this paper we describe the differential diagnosis of recurrent or periodic fever versus other processes, with especial attention to autoinflammatory disorders (AD). AD are alterations of innate immunity, and they have been recently classified as an immunodeficiency. Anyhow, since infections are not present, these processes are different to the classic primary immunodeficiency. An important part of AD is of known genetic aetiology. The symptoms originate from an underlying inflammatory process and can have different clinical expressions. One of the most relevant groups is the hereditary syndromes of periodic fever. This group of diseases associates recurrent fever and several clinical symptoms with a relative periodicity, separated by intervals free or almost free of symptoms. We include the diagnostic criteria for some processes as well as the characteristics that should, eventually, lead to a genetic study. Although treatment should be individualised, we also include some general recommendations.

Meningitis pediátrica. ¿Cuándo podemos estar tranquilos? / Pediatric meningitis. When can we be calm?

Introducción: Desde que en 1980 se publicó la Escala de Boyer, se han propuesto muchas herramientas clínicas para distinguir entre meningitis viral (MV) y meningitis bacteriana(MB). En los últimos años se ha validado el Bacterial Meningitis Score (BMS), instrumento que identifica a los niños con muy bajo riesgo de padecer MB. Nuestro objetivo es determinarla eficacia de la Escala de Boyer modificada con la proteína C reactiva (PCR), y validar en nuestra población pediátrica el BMS. Métodos: Se realizó un análisis retrospectivo de los niños de3 meses a 15 años de edad hospitalizados con el diagnóstico de meningitis entre 1994 y 2007. Se aplicó la Escala de Boyer, añadiéndose una puntuación correspondiente a la PCR. La sensibilidad y la especificidad se calcularon en los puntos de corte determinantes para la toma de decisiones (puntuaciones 3 y 5). En el caso del BMS, se valoró su sensibilidad y especificidad a partir del cumplimiento de los criterios de inclusión propuestos por sus autores. Resultados: En total se incluyeron 145 casos, de los cuales46 fueron MB y 99 MV. Se determinó la PCR en 73 de ellos. La modificación de la Escala de Boyer con la PCR aumentó la sensibilidad en el punto de corte 5 de un 83 a un 100%, permaneciendo la sensibilidad en el 100% en ambos casos. En el caso del BMS, el cumplimiento de al menos uno de los cinco criterios de riesgo identificó los casos de MB con una sensibilidad del 100% y una especificidad del 61,22%. Conclusión: Una puntuación de más de 5 en la Escala de Boyer modificada con la PCR identifica de forma precisa los casos de meningitis de etiología bacteriana en nuestro medio. El BMS es muy eficaz para determinar los casos de muy bajo riesgo de MB. La combinación de ambas herramientas resulta muy útil en el manejo de los pacientes con sospecha de meningitis (AU)

Introduction: Since Boyer's Score was published in 1980; many clinical tools have been proposed to distinguish between viral meningitis (VM) and bacterial meningitis (BM). Recently, it has been validated the Bacterial Meningitis Score (BMS), an instrument that identifies children at very low risk of BM. Our objective is to determine the accuracy of Boyer's Score modified with C-reactive protein (CRP) and to validate the BMS in our pediatric population. Methods: A retrospective study analysis of children (aged from 3 months to 15 years) hospitalized with a meningitis diagnosis in our pediatric center between 1994 and 2007. We calculated Boyer's Score and added a punctuation corresponding to CRP. The sensitivity and specificity were estimated in the scores cut off point that determine the taking of decisions (punctuations 3 and 5). We applied the BMS to all children using the inclusion criteria proposed by the authors, and calculated the sensitivity and specificity. Results: Among the 145 cases included, 46 had BM and 99 had VM. CRP was determined in 73 of them. The modification of Boyer's Score with CRP increased the sensibility of punctuation 5 from 83% to 100%; the specificity of this punctuation remained in 100% even if CRP was included or not. In the case of BMS, the fact of presenting at least one of the 5 criteria identified the cases of BM with a sensibility of 100% and a specificity of 61.22%. Conclusion: A punctuation of more than 5 in Boyer's Score modified with CRP identifies with high efficacy the cases of bacterial etiology. The BMS is very accurate for the identification of very low risk cases of BM. The combination of both scales is very useful in the management of patients with suspicion of meningitis ( AU)

Malaria en un niño procedente de adopción internacional / Malaria in an international adoptee

La malaria importada en la edad pediátrica es una enfermedad emergente en nuestro medio gracias al aumento de los viajes internacionales a países endémicos y a la llegada de niños inmigrantes o adoptados desde estas zonas. Describimos el caso de una niña adoptada procedente de Etiopía con parasitación por P. falciparum, que se encontraba asintomática a su llegada y sólo presentaba esplenomegalia como único hallazgo clínico. El diagnóstico y tratamiento precoz de esta enfermedad resulta fundamental para disminuir la morbimortalidad asociada, por lo que siempre debe descartarse en aquellos pacientes procedentes de área endémica(AU)

Imported childhood malaria has become an emerging disease in Spain. The two main reasons are international travel to endemic countries and the increase in immigrant and adopted children coming from those geographical regions. We describe the case of an adopted Ethiopian girl who was infected with Plasmodium falciparum, although with the exception of splenomegaly, she was asymptomatic at the time of her arrival. Early diagnosis and treatment of malaria is essential to decrease the associated morbidity and mortality. Therefore, steps should be taken to rule out this disease in patients coming from endemic areas(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)