Giant coronary aneurysm in a 6-week-old infant, an unusual finding?

Kawasaki disease (KD) is a self-limited vasculitis with significant morbidity and even mortality if not treated early. The diagnosis and timely treatment in children younger than 3 months is challenging as most of them have an incomplete or atypical presentation. Coronary artery abnormalities are frequent in this type of patients. We present a 6-week-old female infant with KD who developed a giant coronary aneurysm. An early diagnosis and promptly treatment, as well as the echocardiographic and multimodality follow-up allowed us to improve our clinical approach and management.

Nuubo ™ improving the diagnosis of pediatric arrhythmias.

We report an 11-year-old male with intermittent palpitations episodes, and no other pathological findings were found. With the standard Holter electrocardiogram, there is only the option of 24 to 72 hours monitoring of the patient. A Holter Nuubo ™ Vest was placed for 33 days and a supraventricular tachycardia was found.

Resultados de cirugía endoscópica nasal en el tratamiento de sinusitis fúngica invasiva en niños con cáncer e inmunosupresión / Results of endoscopic nasal surgery in the treatment of invasive fungal sinusitis in children with cancer and immunosuppression

Antecedentes y objetivo: El objetivo del estudio ha sido describir los resultados del tratamiento de sinusitis fúngica invasiva con cirugía endoscópica nasal en una población oncológica pediátrica con inmunosupresión e informar sobre la seguridad, la eficacia y las complicaciones del procedimiento. Métodos: Se realizó un estudio retrospectivo de la totalidad de los pacientes con diagnóstico de sinusitis fúngica invasiva operados en la Unidad Nacional de Oncología Pediátrica entre los años 2012 y 2016. Los datos tomados de su historial médico incluyeron: características epidemiológicas, diagnóstico oncológico, datos hematológicos, síntomas, estudios tomográficos, intervenciones quirúrgicas, resultados de enfermedad y cultivos, medicamentos recibidos, complicaciones, evolución y supervivencia. Los datos fueron analizados utilizando estadística descriptiva, las variables continuas con medidas de tendencia central y las variables categóricas de forma porcentual. Resultados: Se identificó a 18 pacientes, 7 de sexo masculino y 11 de sexo femenino. El promedio de edad fue de 12 años, 13 tuvieron diagnóstico de leucemia linfoide aguda y 5 de leucemia mieloide aguda; 17 pacientes presentaron neutropenia severa en el momento del diagnóstico. El agente etiológico más frecuentemente identificado fue Aspergillus en 13 pacientes. En 16 pacientes (89%) se controló la enfermedad con cirugía endoscópica nasal. Diez pacientes fallecieron por causas no relacionadas a lo largo del estudio. Discusión y conclusiones: La sinusitis fúngica invasiva es una enfermedad cuya incidencia va en aumento entre pacientes con inmunosupresión y debe de considerarse una urgencia médica debido a su alta mortalidad. El diagnóstico se basa en un alto índice de sospecha en pacientes con factores predisponentes (leucemia, neutropenia, fiebre persistente, sonda nasogástrica) y la evaluación endoscópica nasal. El tratamiento médico antifúngico y cirugía endoscópica nasal agresiva está indicado independientemente del estado del paciente para disminuir la carga fúngica y la alta mortalidad asociada. El tratamiento debe de ser suministrado por un equipo multidisciplinario que incluye pediatría, hemato-oncología, infectología y otorrinolaringología

Background and objective: to describe the results of the treatment of invasive fungal sinusitis with nasal endoscopic surgery in an immunocompromised paediatric oncological population. Methods: retrospective study of all patients diagnosed with invasive fungal sinusitis operated in the National Paediatric Oncology Unit between 2012 and 2016. Data taken from their medical history included: epidemiological characteristics, oncological diagnosis, haematological data, symptoms, tomographic studies, surgical interventions, results of pathology and cultures, medications received, complications, evolution and survival. Results: 18 patients were identified, 7 male and 11 female. The average age was 12 years, 13 had a diagnosis of acute lymphocytic leukemia and 5 of acute myeloid leukemia. Seventeen patients presented severe neutropenia at the time of diagnosis. The most frequently identified aetiological agent was Aspergillus in 13 patients. In 16 patients (89%) the disease was controlled with nasal endoscopic surgery. Ten patients died due to unrelated causes throughout the study. Discussion and conclusions: Invasive fungal sinusitis should be considered a medical emergency due to its high mortality. The diagnosis is based on a high index of suspicion in patients with predisposing factors (leukaemia, neutropenia, persistent fever, nasogastric tube) and endoscopic nasal evaluation. Antifungal medical treatment and aggressive nasal endoscopic surgery is indicated regardless of the patient's condition to reduce the fungal burden and associated high mortality. The treatment must be provided by a multidisciplinary team that includes paediatrics, haemato-oncology, infectology and otorhinolaryngology

Results of endoscopic nasal surgery in the treatment of invasive fungal sinusitis in children with cancer and immunosuppression. / Resultados de cirugía endoscópica nasal en el tratamiento de sinusitis fúngica invasiva en niños con cáncer e inmunosupresión.

BACKGROUND AND OBJECTIVE: to describe the results of the treatment of invasive fungal sinusitis with nasal endoscopic surgery in an immunocompromised paediatric oncological population. METHODS: retrospective study of all patients diagnosed with invasive fungal sinusitis operated in the National Paediatric Oncology Unit between 2012 and 2016. Data taken from their medical history included: epidemiological characteristics, oncological diagnosis, haematological data, symptoms, tomographic studies, surgical interventions, results of pathology and cultures, medications received, complications, evolution and survival. RESULTS: 18 patients were identified, 7 male and 11 female. The average age was 12 years, 13 had a diagnosis of acute lymphocytic leukemia and 5 of acute myeloid leukemia. Seventeen patients presented severe neutropenia at the time of diagnosis. The most frequently identified aetiological agent was Aspergillus in 13 patients. In 16 patients (89%) the disease was controlled with nasal endoscopic surgery. Ten patients died due to unrelated causes throughout the study. DISCUSSION AND CONCLUSIONS: Invasive fungal sinusitis should be considered a medical emergency due to its high mortality. The diagnosis is based on a high index of suspicion in patients with predisposing factors (leukaemia, neutropenia, persistent fever, nasogastric tube) and endoscopic nasal evaluation. Antifungal medical treatment and aggressive nasal endoscopic surgery is indicated regardless of the patient's condition to reduce the fungal burden and associated high mortality. The treatment must be provided by a multidisciplinary team that includes paediatrics, haemato-oncology, infectology and otorhinolaryngology.

Left ventricular volumetry in healthy children and adolescents: comparison of two different real-time three-dimensional matrix transducers with cardiovascular magnetic resonance.

AIMS: To assess the accuracy of different hardware and software settings for left ventricular (LV) volume quantification in children using real-time three-dimensional echocardiography (RT3DE). METHODS AND RESULTS: The impact of different matrix transducers (IE 33, X3-1 and VIVID 7, V3) and quantification software settings [TOMTEC; contour-finding activity (tCFA) values ranging from 30 to 70 U] on the accuracy of LV indices was tested in 24 healthy children/adolescents (median = 12.6 years) and 25 paediatric patients with Tetralogy-of-Fallot (TOF) (median = 7.3 years) with abnormally shaped ventricles. RT3DE was compared with cardiovascular magnetic resonance (CMR) volumetry as reference. Best agreement (Bland-Altman analysis) was achieved using a tCFA value of 30 U. Applying the V3 device, end-diastolic volume (EDV) and end-systolic volume (ESV) were underestimated by 14.8 +/- 10.6% (mean +/- SD) and 11.2 +/- 16.3%, respectively (r = 9.42, P < 0.001 and r = 0.937, P = 0.003); with the X3-1 system 24.2 +/- 11.0 and 14.6 +/- 15.2%, respectively (r = 0.951, P < 0.001 and r = 0.912, P = 0.001). Negligible differences <1% (P = n.s.) between both transducers were detected applying a tCFA value of 70 U but with significant underestimation (EDV: approximately 35%, P < 0.001; ESV: approximately 26%, P < 0.001) compared with CMR. EDV and ESV of TOF patients were underestimated by 3.2 +/- 15.4 and 8.1 +/- 22.6%, respectively. Intra- and interobserver variability was <4%. CONCLUSION: In contrast to recommendations of the manufacturer, data sets from both RT3DE transducers showed acceptable agreement to CMR for volumetric parameters only for low tCFA. Fine-tuning of software settings is mandatory to improve accuracy.

Is torsion a suitable echocardiographic parameter to detect acute changes in left ventricular afterload in children?

BACKGROUND: Congenital heart defects such as coarctation or valvular aortic stenosis are followed by changes in left ventricular myocardial deformation mechanics induced by pressure overload. METHODS: Echocardiography was performed in 37 patients (aged 0-27 years, 15 female) with coarctation (27) or valvular aortic stenosis (10) before and after interventional catheterization and compared with 37 body surface area/age-matched healthy children. Deformation was calculated by 2-dimensional strain speckle tracking. RESULTS: Stress gradients under provocation with orciprenaline in coarctation decreased from 51.8 +/- 20.0 mm Hg to 6.0 +/- 12.0 mm Hg (P < .0001), and resting gradients in aortic stenoses decreased from 57.5 +/- 18.8 mm Hg to 25.5 +/- 14.0 mm Hg (P < .0001) after intervention. Patients had an increased maximal torsion (tor(max): 16.7 +/- 6.7 deg vs 11.0 +/- 4.7 deg (controls; P < .0001), which decreased significantly after therapy (11.8 +/- 4.9 deg, P < .0001). CONCLUSION: Compensatory elevation of left ventricular tor(max) in children with moderate left ventricular pressure load changes rapidly after successful interventional treatment. tor(max) may be a valuable tool to assess cardiac unloading or indicate the need for interventional treatment.

Diagnóstico tardío, videotoracoscópico de hernias diafragmáticas / Delayed videothoracoscopy diagnosis of diaphragmatic hernia

Objetivo. Determinar la utilidad de la toracoscopía en pacientes con trauma penetrante toracoabdominal.Población. 44 pacientes. Diseño. Estudio prospectivo observacional.Métodos. Durante el período de junio 1996 a mayo de 1997, se sometieron a cirugía electiva, bajo anestesia general, en la posición de decúbito lateral con el hemitórax afectado hacia arriba para efectuar toracoscopía. Resultados. El toráx izquierdo fue el más afectado con 68 de los casos; el 62 estaban clínica y radiológicamente asintomáticos. Las heridas por arma blanca fueron la causa más frecuente (77); el toráx lateral y posterior fueron las áreas de mayor índice de penetración con 100 y 94 respectivamente. El trauma diafragmático grado II fue la presentación más frecuente (40) y el 70 de las lesiones requirieron sutura con técnica endoscópica.Conclusión. El trauma penetrante toracoabdominal debe ser explorado quirúrgicamente aún en pacientes asintomáticos, debido a la alta incidencia de lesiones diafragmáticas, que si bien pueden causar lesiones autolimitantes intraabdominales, constituyen un riesgo de desarrollar hernias diafragmáticas y postraumáticas

Toracoscopía de rutina en trauma penetrante toracoabdominal: reporte preliminar / Routine thoracoscopy in penetrating thoraco-abdominal trauma: preliminary report

Se presenta el reporte de 27 casos de pacientes con heridas penetrantes en la región toracoabdominal, hemodinámicamente estables y sin indicación de cirugía abdominal o torácica de urgencia, a quienes se les realizó video toracoscopía diagnóstica y/o terapéutica de manera electiva. La penetración hacia la cavidad torácica sucedió en 81/100 de los pacientes (22/27),a pesar de que el 74/100 de ellos (20/27) no presentaban evidencia clínica ni radiológica de penetración. Se encontró lesión del diafragma en 74/100, hemotórax en 56/100, lesión pulmonar en 11/100 y lesión del pericardio en 4/100 de los casos.Las lesiones que penetraron la cavidad torácica en la región lateral y posterior del tórax, ocasionaron lesión diafragmática en 100/100 de los casos. Dichas lesiones fueron reparadas por vía toracoscópica.Tuvimos un 7/100 de complicaciones un 11/100 de los casos fueron verificacos por video laparoscopía por problemas técnicos en la toracoscopía. Nuestro estudio concluye que la toracoscopía es un método útil en el diagnóstico y tratamiento de las lesiones diafragmáticas ocasionadas por heridas penetrantes de la región toracoabdominal

Envenenamiento mortal por hongos en Guatemala / Mortal poisoning by mushrooms in Guatemala

Una mezcla de posibles hongos comestibles, provocó un envenenamiento en dos familias de San Yuyo, en el Departamento de Jalapa, al SO de Guatemala. Se intoxicaron 19 personas, de las cuales murieron 8 con síntomas de ataque hepático. Los hongos que se identificaron según las investigaciones entre la población, son Amanita caesarea (Scop. ex. Fr.) Grev., A. gemmata (Fr.) Gill. y A. magnivelaris Peck, el primero es comestible de excelente calidad, el segundo provoca únicamente molestias gastrointestinales y el tercero produce la muerte; dicha especie pertence al grupo de A. bisporigera Atk., caracterizada por su contenido en alcaloides altamente venenosos. Este es el primer registro de A. magnivelaris en Guatemala