Influence of zoledronic acid and pamidronate on tooth eruption in children with osteogenesis imperfecta.

INTRODUCTION: Osteogenesis imperfecta (OI) is a congenital disease comprising a heterogeneous group of inherited connective tissue disorders. The main treatment in children is bisphosphonate therapy. Previous animal studies have shown that bisphosphonates delay tooth eruption. The aim of this study is to determine whether patients with OI treated with pamidronate and/or zoledronic acid have a delayed eruption age compared to a control group of healthy children. METHODS: An ambispective longitudinal cohort study evaluating the age of eruption of the first stage mixed dentition in a group of children with OI (n = 37) all treated with intravenous bisphosphonates compared with a group of healthy children (n = 89). Within the study group, the correlation (Pearson correlation test) between the type of medication administered (pamidronate and/or zoledronic acid) and the chronology of tooth eruption is established, as well as the relationship between the amount of cumulative dose received and tooth eruption. RESULTS: The age of eruption of the study group was significantly delayed compared to the age of eruption of the control group for molars and lateral incisors (p < 0.05). Patients who received higher cumulative doses had a delayed eruption age compared to those with lower cumulative doses (p < 0.05). There is a high positive correlation between age of delayed tooth eruption and Zoledronic acid administration. CONCLUSION: Patients with OI have a delayed eruption of the 1st stage mixed dentition compared to a control group of healthy children. This delayed eruption is directly related to the cumulative dose of bisphosphonates and the administration of zoledronic ac.

Chorea as the presenting feature of acute rheumatic fever in childhood; case reports from a low-prevalence European setting.

BACKGROUND: Despite a notable decrease in acute rheumatic fever (ARF) incidence in the past few decades, there are still cases in our setting. Sydenham chorea (SC) may be the initial manifestation for this condition in childhood in a significant proportion of children. We report two cases of choreoathetosis in children as the first manifestation of ARF. CASE PRESENTATION: A previously healthy 8-year-old boy presented with right hemichorea with a predominance in the brachial region, orofacial dyskinesias and speech difficulties for the past 2 weeks. The only medical history of interest was a common catarrhal illness 3 weeks before and nonspecific bilateral tenosynovitis in both feet since a year prior. A brain computerized tomography was normal and the echocardiogram showed mild mitral and aortic regurgitation, meeting ARF criteria. He demonstrated clinical improvement with treatment based on prednisone and carbamazepine. The second patient was a 10-year-old girl with choreic movements of the right half of the body and repetitive right eye closure of 1 week duration. She had symptoms of fever and rash the previous week and pharyngitis that resolved without antibiotic 2 months before. Blood tests revealed elevated C reactive protein (12 mg/dl) and erythrocyte sedimentation rate (96 mm/h). Brain magnetic resonance was normal and echocardiogram showed left ventricle dilation and mild mitral regurgitation, leading to the diagnosis of ARF. Due to neurological involvement, she received corticosteroids and intravenous immunoglobulin treatment, with worsening of neurological symptoms that required valproic acid with remission of the hemichorea. In addition skin lessions compatible with erythema marginatum appeared on the upper limbs. CONCLUSIONS: SC should be the main diagnostic consideration in cases of hemichorea with normal neuroimaging in children. The cases reported highlight the need to maintain a high index of suspicion even in settings where incidende of ARF is low and the need to perform cardiological investigations in all patients with suspected SC, due to the possibility of subclinical valve lesions. Good adherence to secondary prophylaxis is crucial to avoid chorea relapses and worsening valve disease.

Una nueva imagen en osteogénesis imperfecta: "nube de humo" / A new radiological image in osteogenesis imperfecta: "Smoke cloud"

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Evaluación de una técnica de diagnóstico molecular Xpert EV (Cepheid(R)) en la meningitis por enterovirus / Evaluation of an Xpert EV (Cepheid(R)) molecular diagnostic technique for enteroviral meningitis

Introducción: Las técnicas de biología molecular han demostrado ser útiles en la detección del enterovirus en niños con meningitis aséptica. El objetivo de nuestro estudio fue analizar cambios en la práctica clínica tras la introducción de una técnica de RT-PCR a tiempo real, ensayo Xpert EV (Cepheid(R)), para la detección de enterovirus en muestras de líquido cefalorraquídeo de niños con sospecha de meningitis vírica. Métodos: Estudio retrospectivo de los niños mayores de 1año diagnosticados de meningitis por enterovirus en un hospital de tercer nivel desde noviembre de 2006 a febrero de 2013. Se comparó el periodo previo a la introducción del ensayo Xpert EV (Cepheid(R)) (grupo1: noviembre 2006-agosto de 2010) con el periodo posterior (grupo2: septiembre 2010-febrero 2013). Se compararon las características clínicas, los tiempos de estancia media y los costes por hospitalización. Resultados: Se incluyeron 41 pacientes con una mediana de edad de 64 meses (rango intercuartílico, 28-96). En el grupo 2 se incluyeron 26 pacientes (63,4%). No hubo diferencias epidemiológicas, de gravedad, ni de laboratorio estadísticamente significativas entre los pacientes valorados en ambos grupos. Se observó una disminución significativa en la duración de estancia media hospitalaria en el grupo 2 (48 h vs 40,5 h, p = 0,039) y una disminución significativa en el gasto por paciente hospitalizado (779,77 Euros vs. 656,05 Euros, p<0,05). Conclusiones: La incorporación de la técnica Xpert EV (Cepheid(R)) permitió disminuir la estancia y el gasto asociado a hospitalización en niños con meningitis por enterovirus (AU)

Introduction: Polymerase chain reaction (PCR) assays have shown to be useful and quick for the diagnosis of enterovirus in aseptic meningitis. The aim of our study was to analyse the changes in clinical practice after the introduction of a real-time polymerase chain reaction (RT-PCR) technique using the Xpert EV (Cepheid(R)) assay for the qualitative detection of enterovirus RNA in cerebrospinal fluid specimens from children with suspected viral meningitis. Methods: A retrospective study was performed in children older than 1 year, diagnosed with enterovirus meningitis in a third level hospital from November 2006 to February 2013. The first period, before the availability of Xpert EV (Cepheid(R)) (Group 1, November 2006-August 2010) was compared with the later period (Group 2, September 2010-February 2013). Clinical characteristics, the mean length of stay, and the cost per inpatient cases, were compared between the 2 periods. Results: Forty-one patients (60.9% male) were included, with a median age of 64 months (interquartile range 28-96). Twenty-six patients (63.4%) were included in Group 2. There were non-statistically significant differences in the epidemiological, disease severity, and laboratory characteristics between both periods of study. A significant difference was observed in the mean length of stay, with it being shorter in Group2 (48 hours vs 40.5 hours, P = .039), and a significant lower inpatient cost per case (Euros 779.77 vs Euros 656.05, P < .05). Conclusion: Xpert EV (Cepheid(R)) assay was useful for decreasing the length of hospital stay and the costs associated with hospitalisation in children with enterovirus meningitis (AU)

[Evaluation of an Xpert EV (Cepheid®) molecular diagnostic technique for enteroviral meningitis]. / Evaluación de una técnica de diagnóstico molecular Xpert EV (Cepheid®) en la meningitis por enterovirus.

INTRODUCTION: Polymerase chain reaction (PCR) assays have shown to be useful and quick for the diagnosis of enterovirus in aseptic meningitis. The aim of our study was to analyse the changes in clinical practice after the introduction of a real-time polymerase chain reaction (RT-PCR) technique using the Xpert EV (Cepheid®) assay for the qualitative detection of enterovirus RNA in cerebrospinal fluid specimens from children with suspected viral meningitis. METHODS: A retrospective study was performed in children older than 1year, diagnosed with enterovirus meningitis in a third level hospital from November 2006 to February 2013. The first period, before the availability of Xpert EV (Cepheid®) (Group1, November 2006-August 2010) was compared with the later period (Group2, September 2010-February 2013). Clinical characteristics, the mean length of stay, and the cost per inpatient cases, were compared between the 2periods. RESULTS: Forty-one patients (60.9% male) were included, with a median age of 64 months (interquartile range 28-96). Twenty-six patients (63.4%) were included in Group2. There were non-statistically significant differences in the epidemiological, disease severity, and laboratory characteristics between both periods of study. A significant difference was observed in the mean length of stay, with it being shorter in Group2 (48hours vs 40.5hours, P=.039), and a significant lower inpatient cost per case (€779.77 vs €656.05, P<.05). CONCLUSION: Xpert EV (Cepheid®) assay was useful for decreasing the length of hospital stay and the costs associated with hospitalisation in children with enterovirus meningitis.