Crisis asmática en los servicios de urgencias en Espa˜na, ¿cuál es nuestra práctica habitual? / Asthma crisis in emergency departments in Spain: what is our usual practice?

Introducción: A pesar de la existencia de múltiples guías y documentos de consenso sobre el asma, persiste todavía una gran variabilidad en su abordaje, situación poco analizada en los Servicios de Urgencias (SU). Objetivo: Describir el manejo habitual de las crisis asmáticas en los SU españoles, conocer el grado de variabilidad entre sus diferentes profesionales y analizar los factores que pueden influir en su manejo. Material y métodos: Se elaboró un cuestionario, basado en supuestos clínicos, que se distribuyó entre los socios de la Sociedad Española de Urgencias de Pediatría y asistentes a la reunión en 2011. Resultados: Se analizan 300 encuestas. En 190 casos, el lugar de trabajo habitual es un hospital de tercer nivel. Existe una alta coincidencia en la valoración de la gravedad de los casos. Más del 90% utiliza beta 2 agonistas inhalados en la crisis asmática leve, el 75,3% utiliza corticoides orales en crisis moderadas y el 79% utiliza bromuro de ipratropio en las crisis graves. El 98% considera importante hacer educación terapéutica en los SU. Solo 8 (2,7%) utilizan la medición del pico de flujo espiratorio máximo. Los encuestados que trabajan en un hospital de tercer nivel utilizan con más frecuencia escalas de gravedad (90,5% vs. 80%, p = 0,009) y administran corticoides parenterales con menos frecuencia (53,2% vs. 66,4%, p = 0,017). Conclusiones: Aunque un porcentaje elevado de pediatras que trabajan en los SU siguen las guías de práctica clínica, se han detectado aspectos en los que el abordaje del asma es discordante entre los diferentes profesionales(AU)

Background: Despite there being various guidelines and consensus statements on asthma, great variability still exists in its treatment approach; although this has been little discussed in Emergency Departments (ED). Objective: To describe the usual management of asthma exacerbations in Spanish ED, and to determine the degree of variability among different professionals, as well as analyse factors that may influence their management. Material and methods: A questionnaire was developed based on clinical cases, which was distributed among members of the Spanish Society of Paediatric Emergencies and attendees at the meeting in 2011. Results: A total of 300 surveys were analysed. In 190 cases the place of work was in a tertiary care hospital. There was high agreement in the assessment of the severity of the cases mentioned. Over 90% use inhaled beta 2 agonists in mild asthma exacerbations, 75.3% used oral corticosteroids in moderate exacerbations, and 79% use ipratropium bromide in severe exacerbations. Almost all (98%) considered Therapeutic Education important in the Emergency Department. Only 8 (2.7%) used peak expiratory flow measurements. Respondents working in a tertiary hospital used severity scales more frequently (90.5% vs 80%, P=0.009) and administered parenteral corticosteroids less frequently (53.2% vs 66.4%, P=0.017). Conclusions: Although a high percentage of paediatricians working in the ED follow Clinical Practice Guidelines, some different points of the approach among professionals have been identified(AU)

[Asthma crisis in emergency departments in Spain: what is our usual practice?]. / Crisis asmática en los servicios de urgencias en España, ¿cuál es nuestra práctica habitual?

BACKGROUND: Despite there being various guidelines and consensus statements on asthma, great variability still exists in its treatment approach; although this has been little discussed in Emergency Departments (ED). OBJECTIVE: To describe the usual management of asthma exacerbations in Spanish ED, and to determine the degree of variability among different professionals, as well as analyse factors that may influence their management. MATERIAL AND METHODS: A questionnaire was developed based on clinical cases, which was distributed among members of the Spanish Society of Paediatric Emergencies and attendees at the meeting in 2011. RESULTS: A total of 300 surveys were analysed. In 190 cases the place of work was in a tertiary care hospital. There was high agreement in the assessment of the severity of the cases mentioned. Over 90% use inhaled ß2 agonists in mild asthma exacerbations, 75.3% used oral corticosteroids in moderate exacerbations, and 79% use ipratropium bromide in severe exacerbations. Almost all (98%) considered Therapeutic Education important in the Emergency Department. Only 8 (2.7%) used peak expiratory flow measurements. Respondents working in a tertiary hospital used severity scales more frequently (90.5% vs 80%, P=.009) and administered parenteral corticosteroids less frequently (53.2% vs 66.4%, P=.017). CONCLUSIONS: Although a high percentage of paediatricians working in the ED follow Clinical Practice Guidelines, some different points of the approach among professionals have been identified.

[Utility of urine levels of interleukins in the diagnosis of vesicoureteral reflux: a case-control study in children]. / Utilidad de los niveles urinarios de interleuquinas en el diagnóstico del reflujo vésico-ureteral: estudio de casos y controles en niños.

UNLABELLED: Invasive imaging methods that require catheterization are used for the diagnosis of vesicoureteral reflux. Our aim is to assess the usefulness of interleukin urinary levels for the diagnosis of reflux in children without urinary tract infection. METHODS: Case-control study in children who underwent a voiding cystourethrogram: forty cases diagnosed of reflux and 80 controls. Concentrations of IL-1beta, IL-6 and IL-8 related to creatinine levels (pg/micromol) were determined in urine samples in all. RESULTS: Sixty-two patients were males and fifty-eight females, with a mean age of 2.4 years. Indications for cystography were previous urinary tract infection in 78 cases (65%), prenatal diagnosis in 24 cases (20%) and postnatal diagnosis of uropathy or family history in 18 cases (15.1%). No significant differences were observed between cases and controls in IL-1beta/creatinine and IL-6/creatinine levels. However, IL-8/creatinine levels were almost significant higher in case group (median 3.5 pg/micromol; SD 9.2) than in control group (median 1.54 pg/micromol; SD 3) (P=0.001). The odds ratio was 5.57 (CI95%: 1.51 a 20.60) (X(MH)=2.80; p=0.005). CONCLUSIONS: Urinary levels of IL-8/creatinine are elevated in children with vesicoureteral reflux, even in absence of urinary tract infection. It could be used as a non-invasive marker for detection of subclinical cases of disease.

Utilidad de los niveles urinarios de interleuquinas en el diagnóstico del refl ujo vésico-ureteral: estudio de casos y controles en niños / Utility of urine levels of interleukins in the diagnosis of vesicoureteral reflux: a case-control study in children

En el diagnóstico del reflujo vésico-ureteral se emplean métodos de imagen invasivos que requieren sondajes. Nuestro objetivo es evaluarla utilidad de los niveles urinarios de interleuquinas en el diagnóstico del reflujo en niños sin infección urinaria en curso. Métodos. Estudio de casos y controles en niños que acuden a realizar una cistouretrografía miccional (CUMS): 40 casos con reflujo y80 controles. Se determinó en todos la concentración en orina de IL-1,IL-6 e IL-8 en relación con la creatinina. Resultados. 62 niños y 58 niñas, con 2,4 años de edad media. La CUMS se indicó por infección urinaria previa en 78 (65%), hallazgos prenatales en 24 (20%) y diagnóstico postnatal de uropatía o historia familiar en 18 (15,1%). No se han observado diferencias significativas en los niveles urinarios de IL-1/creatinina e IL-6/creatinina entre ambos grupos; sin embargo, los niveles de IL-8/creatinina fueron significativamente más altos en los casos (media 3,5 pg/μmol; SD 9,2) que en los controles (media 1,54 pg/μmol; SD 3) (p=0,001). La odds ratio fue de5,57 (IC 95%: 1,51 a 20,60) (XMH=2,80; p=0,005).Conclusiones. Los niveles urinarios de IL-8/creatinina están elevados en niños con reflujo, incluso en ausencia de infección. Estos podrían utilizarse como biomarcador no invasivo para la detección de los casos subclínicos de enfermedad (AU)

Invasive imaging methods that require catheterization are used for the diagnosis of vesicoureteral refl ux. Our aim is to assess the usefulness of interleukin urinary levels for the diagnosis of reflux in children without urinary tract infection. Methods. Case-control study in children who underwent a voiding cystourethrogram: forty cases diagnosed of refl ux and 80 controls. Concentrations of IL-1, IL-6 and IL-8 related to creatinine levels (pg/μmol) were determined in urine samples in all. Results. Sixty-two patients were males and fi fty-eight females, with a mean age of 2.4 years. Indications for cystography were previousurinary tract infection in 78 cases (65%), prenatal diagnosis in 24 cases(20%) and postnatal diagnosis of uropathy or family history in 18 cases(15.1%). No significant differences were observed between cases and controls in IL-1/creatinine and IL-6/creatinine levels. However, IL-8/creatinine levels were almost significant higher in case group (median3.5 pg/μmol; SD 9.2) than in control group (median 1.54 pg/μmol; SD 3)(P=0.001). The odds ratio was 5.57 (CI 95%: 1.51 a 20.60) (XMH=2.80;p=0.005).Conclusions. Urinary levels of IL-8/creatinine are elevated in children with vesicoureteral reflux, even in absence of urinary tractinfection. It could be used as a non-invasive marker for detection of subclinical cases of disease (AU)

[Isolated conjunctival involvement in Langerhans cell histiocytosis]. / Histiocitosis de células de Langerhans con afectación conjuntival aislada.

CASE REPORT: A 43-year-old woman referred for a hyperemic node in the inferior conjunctival fornix. No improvement was observed after 10 days of antibiotic and anti-inflammatory topical treatment, so biopsy-extirpation was performed. Histopathological and immunohistochemical findings suggested the diagnosis of Langerhans cell histiocytosis (LCH). No findings of extraocular manifestations were shown after an intensive clinical and analytical investigation. DISCUSSION: LCH is a disease with different organ manifestations whose diagnosis depends on histological findings. We present a patient with isolated conjunctival affectation, which to our knowledge is the third case published in the literature.

Histiocitosis de células de Langerhans con afectación conjuntival aislada / Isolated conjunctival involvement in langerhans cell histiocytosis

Caso clínico: Mujer de 43 años que consulta por unnódulo hiperémico en fondo de saco conjuntivalinferior. Tras diez días de tratamiento antibiótico yantiinflamatorio tópico no se observa mejoría, porlo que se realiza una biopsia-extirpación. El resultadoanatomopatológico e inmunohistoquímico esinformado como histiocitosis de células de Langerhans(HCL), procediéndose a realizar un estudiosistémico con diferentes pruebas complementarias,sin hallar afectación a otro nivel.Discusión: La histiocitosis de células de Langerhanses una enfermedad de afectación muy variada,cuyo diagnóstico se establece mediante hallazgoshistológicos. Presentamos un caso de implicaciónexclusivamente conjuntival, habiéndose publicadosólo tres casos previamente en la literatura médica consultada(AU)

Case report: A 43-year-old woman referred for ahyperemic node in the inferior conjunctival fornix.No improvement was observed after 10 days ofantibiotic and anti-inflammatory topical treatment,so biopsy-extirpation was performed. Histopathologicaland immunohistochemical findings suggestedthe diagnosis of Langerhans cell histiocytosis(LCH). No findings of extraocular manifestationswere shown after an intensive clinical and analyticalinvestigation.Discussion: LCH is a disease with different organmanifestations whose diagnosis depends on histologicalfindings. We present a patient with isolatedconjunctival affectation, which to our knowledge isthe third case published in the literature(AU)

[Hemophagocytic syndrome secondary to visceral leishmaniasis]. / Síndrome hemofagocítico secundario a leishmaniasis visceral.

Haemophagocytic syndrome is a disease diagnosed according to clinical and analytical criteria, related to many infectious diseases. It is exceptionally described in patients infected with Leishmania. Visceral leishmaniasis is an uncommon disease in our country except in some areas where it is endemic. Its diagnosis is sometimes difficult and the use of other methods currently available is needed. Haemophagocytic syndrome treatment is based on established chemotherapy protocols, but when it is secondary to Visceral Leishmaniasis, it may be an exception, since the abnormalities can be resolved by treatment of the infection itself. This treatment has improved recently as Liposomal Amphotericin B has replaced classic antimonials, being more beneficial due to less adverse effects and a shorter treatment time.

Síndrome hemofagocítico secundario a leishmaniasis visceral / Hemophagocytic syndrome secondary to visceral leishmaniasis

El síndrome hemofagocítico es una enfermedad diagnosticada basándose en criterios clínicos y analíticos, relacionada con numerosas entidades infecciosas. De forma excepcional se ha descrito en pacientes infectados con el parásito Leishmania. La leishmaniasis visceral es una patología infrecuente en nuestro país, salvo en zonas concretas donde es endémica. Su diagnóstico en ocasiones es difícil y hay que recurrir a varios de los métodos actualmente disponibles. El tratamiento del síndrome hemofagocítico se fundamenta en pautas quimioterápicas protocolizadas, aunque puede representar una excepción cuando es secundario a la leishmaniasis visceral, ya que el tratamiento antiinfeccioso suele resolver las alteraciones por sí mismo. Dicha terapia ha evolucionado en los últimos tiempos al sustituir la anfotericina B liposomal a las pautas clásicas con antimoniales y logrando beneficios por sus menores efectos secundarios y por acortar el tiempo de tratamiento (AU)

Haemophagocytic syndrome is a disease diagnosed according to clinical and analytical criteria, related to many infectious diseases. It is exceptionally described in patients infected with Leishmania. Visceral leishmaniasis is an uncommon disease in our country except in some areas where it is endemic. Its diagnosis is sometimes difficult and the use of other methods currently available is needed. Haemophagocytic syndrome treatment is based on established chemotherapy protocols, but when it is secondary to Visceral Leishmaniasis, it may be an exception, since the abnormalities can be resolved by treatment of the infection itself. This treatment has improved recently as Liposomal Amphotericin B has replaced classic antimonials, being more beneficial due to less adverse effects and a shorter treatment time (AU)

[Pena-Shokeir syndrome type I, associated to Klippel-Feil syndrome type II in the same family]. / Secuencia de Pena-Shokeir tipo I, asociada a síndrome de Klippel-Feil tipo II en la misma familia.

INTRODUCTION: In 1974 Pena and Shokeir described an early lethal disorder (OMIM 208150) that was characterised by neurogenic arthrogryposis, facial abnormalities and pulmonary hypoplasia. It has recently been suggested that it is secondary to the reduction of movements in the uterus due to an intrinsic pathology regardless of the cause (FADS, foetal akinesia deformation sequence). Klippel-Feil (K-F) syndrome (OMIM 118100) is defined by the congenital fusion of one or two cervical vertebrae, and clinically manifests as a shortened neck, with limited head movements, and may also be associated to other malformations. CASE REPORTS: We report the case of a family diagnosed with K-F syndrome type II. It was observed in the father and one daughter; another child presented Pena-Shokeir type I and died during the neonatal period. Both siblings presented anomalies in the central nervous system. CONCLUSIONS: The incidence of FADS syndrome is 1/10,000 deliveries and that of K-F syndrome is between 1/35,000 and 1/42,000 births. We reviewed the literature on FADS syndrome and no familiar association with K-F syndrome was found among its causes. Our aim is to report that an association between the two conditions is possible, which is very important for establishing suitable genetic counselling.

Secuencia de Pena-Shokeir tipo I, asociada a síndrome de Klippel-Feil tipo II en la misma familia / Pena-shokeir syndrome type i, associated to klippel-feil syndrome type ii in the same family

Introducción. Pena y Shokeir, en 1974, describen un trastorno letal temprano (OMIM 208150) caracterizado por artogriposis neurogénica, anormalidades faciales e hipoplasia pulmonar. Actualmente se ha sugerido que es secundario a la disminución de movimientos en el útero por patología intrínseca sin importar la causa (síndrome FADS, fetal akinesia deformation sequence). El síndrome de Klippel-Feil (K-F) –OMIM 118100– está definido por la fusión congénita de una o dos vértebras cervicales y clínicamente presenta cuello corto, limitación de los movimientos de la cabeza, y puede asociar además otras malformaciones. Casos clínicos. Presentamos una familia diagnosticada de síndrome de K-F tipo II. Se evidenció en el padre y una hija, y otro hijo presentó secuencia de Pena-Shokeir tipo I y falleció en período neonatal. Los dos hermanos presentaron anomalías del sistema nervioso central. Conclusiones. El síndrome FADS presenta una incidencia de 1/10.000 nacidos, y el síndrome de K-F, 1/35.000-42.000 nacimientos. Revisamos la bibliografía del síndrome FADS y entre su etiología no hemos encontrado la asociación familiar con el síndrome de K-F. Nuestro objetivo es comunicar que la asociación entre ambas entidades es posible, lo cual resulta de gran importancia para establecer un consejo genético adecuado

Introduction. In 1974 Pena and Shokeir described an early lethal disorder (OMIM 208150) that was characterised by neurogenic arthrogryposis, facial abnormalities and pulmonary hypoplasia. It has recently been suggested that it is secondary to the reduction of movements in the uterus due to an intrinsic pathology regardless of the cause (FADS, foetal akinesia deformation sequence). Klippel-Feil (K-F) syndrome (OMIM 118100) is defined by the congenital fusion of one or two cervical vertebrae, and clinically manifests as a shortened neck, with limited head movements, and may also be associated to other malformations. Case reports. We report the case of a family diagnosed with K-F syndrome type II. It was observed in the father and one daughter; another child presented Pena-Shokeir type I and died during the neonatal period. Both siblingspresented anomalies in the central nervous system. Conclusions. The incidence of FADS syndrome is 1/10,000 deliveries and that of K-F syndrome is between 1/35,000 and 1/42,000 births. We reviewed the literature on FADS syndrome and no familiar association with K-F syndrome was found among its causes. Our aim is to report that an association between the two conditions is possible, which is very important for establishing suitable genetic counselling